October 2024 Critical Care Case of the Month: Respiratory Failure in a Patient with Ulcerative Colitis

Lewis J. Wesselius MD

Pulmonary Department

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

The patient is a 57-year-old woman with a history of ulcerative colitis (UC) complicated by toxic megacolon with subsequent colectomy. She presented to the emergency department with cough, shortness of breath and hypoxemia (87% on RA).

PMH, SH

• UC with history of toxic megacolon (4 years prior) with a total colectomy.
• History of a prior episode of respiratory failure a year earlier thought possibly medication-induced (ustekinumab, Stelara®) which she was taking for her UC. She was treated with steroids with a good response.
• Pyoderma gangrenosum of both ankles (attributed to UC).
• Anemia of chronic disease.
• She is a lifelong non-smoker.
• No exposures to toxic dusts, birds, down, humidifiers, mold or other antigens associated with hypersensitivity pneumonitis.

Physical Exam

• Afebrile, Oxygen saturation 94% on 2 lpm supplemental oxygen.
• Chest: crackles noted at left base.
• CV regular rhythm, no murmur.
• Ext: scarring and erythema on both ankles consistent with resolving pyoderma gangrenosum.

Current Medications

• Clonazepam 1.0 mg daily at bedtime
• Gabapentin 300 mg TID
• Pantoprazole 40 mg BID
• Prednisone 5 mg daily

Laboratory

• Hgb 9.7, WBC 16.9
• Swabs for Influenza A/B and Covid were negative
• Cocci serology negative

A chest radiograph was performed (Figure 1).

Figure 1. Portable chest X-ray performed in the emergency department. (To view Figure 1 in a separate, enlarged window click here).

Which of the following is/are true regarding the chest X-ray?

1. There is a left lower lobe consolidation.
2. The portable chest X-ray may be normal.
3. A chest CT scan is required to definitely view any consolidation.
4. There is a right upper lobe consolidation.
5. All of the above.

Cite as: Wesselius LJ. October 2024 Critical Care Case of the Month: Respiratory Failure in a Patient with Ulcerative Colitis. Southwest J Pulm Crit Care Sleep. 2024;29(4):30-33. doi: https://doi.org/10.13175/swjpcc2046-24 PDF

May 2023 Critical Care Case of the Month: Not a Humerus Case

Carli S. Ogle¹ DO

Billie Bixby² MD

Janet Campion² MD

Departments of Family and Community Medicine¹ and Internal Medicine²

Banner University Medical Center-South Campus

Tucson, AZ USA

History of Present Illness:

A 57-year-old woman with history of bone disease presented with a 3-day history of cough with thick yellow phlegm and progressive shortness of breath. No fever, chest pain or abdominal pain was noted. In the emergency department, she had SpO2 of 55% on room air, and then 90% on 15L NRB.

Past Medical History/Social History/Family History

• Bone disease since birth
• Asthma
• Severe scoliosis
• Gastrointestinal reflux disease
• Cholecystectomy
• Spinal growth rods
• Lives in adult care home, supportive family
• No smoking or alcohol use
• No illicit drug use
• There is no family history of any bone disease

Home Medications:

• Albuterol MDI PRN
• Alendronate 10mg daily
• Budesonide nebulizer BID
• Calcium carbonate BID
• MVI daily
• Lisinopril 10mg daily
• Loratadine 10mg daily
• Metformin 500mg BID
• Metoprolol 12.5mg BID
• Montelukast 10mg daily
• Naprosyn PRN
• Omeprazole 20mg daily
• Simvastatin 10mg daily
• Tizanidine PRN
• Vitamin D 2000 IU daily

Allergies:

• Cefazolin, PCN, Sulfa - all cause anaphylaxis

Physical Examination :

• Vital signs: BP 135/95, HR 108, RR 36, Temp 37.0 C Noted to desaturate to SpO2 in 70-80s off of Bipap even when on Vapotherm HFNC
• General: Alert, slightly anxious woman, tachypneic, able to answer questions
• Skin: No rashes, warm and dry
• HEENT: No scleral icterus, dry oral mucosa, normal conjunctiva
• Neck: No elevated JVP or LAD, short length
• Pulmonary: Diminished breath sounds at bases, no wheezes or crackles
• Cardiovascular: Tachycardic, regular rhythm without murmur
• Abdomen: Soft nontender, nondistended, active bowel sounds
• Extremities: Congenital short upper and lower limb deformities
• Neurologic: Oriented, fully able to make health care decisions with family at bedside

Laboratory Evaluation:

• Na 142, K 4.3, CL 100, CO2 29, BUN 15, Cr 0.38, Glu 222
• WBC 21.9, Hgb 13.6, Hct 42.9, Plt 313 with 83% N, 8% L, 1% E
• Normal LFTs
• Lactic acid 2.2
• Venous Blood Gases (peripheral) on Bipap 10/5, FiO2 90%: pH 7.36, pCO2 58, pO2 55
• COVID-19 positive

Radiologic Evaluation:

A thoracic CT scan was performed (Figure 1).

Figure 1. Representative images from thoracic CT scan in lung windows (A,C) and soft tissue windows (B,D).

The CT images show all the following except: (Click on the correct answer to be directed to the second of seven pages)

1. Severe scoliosis
2. Diffuse ground glass opacities
3. Right lower lobe consolidation
4. Pneumothorax
5. Atelectasis in bilateral lower lobes

Cite as: Ogle CS, Bixby B, Campion J. May 2023 Critical Care Case of the Month: Not a Humerus Case. Southwest J Pulm Crit Care Sleep. 2023;26(5):76-79. doi: https://doi.org/10.13175/swjpccs018-23 PDF

MSSA Pericarditis in a Patient with Systemic Lupus Erythematosus Flare

Antonious Anis MD

Marian Varda DO

Ahmed Dudar MD

Evan  D. Schmitz MD

Saint Mary Medical Center

Long Beach, CA 90813

 

Abstract

Bacterial pericarditis is a rare yet fatal form of pericarditis. With the introduction of antibiotics, incidence of bacterial pericarditis has declined to 1 in 18,000 hospitalized patients. In this report, we present a rare case of MSSA pericarditis in a patient that presented with systemic lupus erythematosus flare, which required treatment with antibiotics and source control with pericardial window and drain placement.

Abbreviations

• ANA: Anti-nuclear Antibody
• Anti-dsDNA: Anti double stranded DNA 
• IV: intravenous
• MSSA: Methicillin-sensitive staphylococcus aureus
• SLE: systemic lupus erythematosus 
• TTE: Transthoracic Echocardiogram

Case Presentation

History of Present Illness

31-year-old female with history of SLE, hypertension and type 1 diabetes mellitus presented with several days of pleuritic chest pain.

Physical Examination

Vitals were notable for blood pressure 204/130. She had normal S1/S2 without murmurs and had trace bilateral lower extremity edema.

Laboratory and radiology

Admission labs were notable for creatinine of 1.8, low C3 and C4 levels, elevated anti-smith, anti-ds DNA and ANA titers. ESR was elevated at 62. Troponin was normal on 3 separate samples 6 hours apart. CT Angiography of the chest showed moderate pericardial effusion (Figure 1).

Figure 1. CT Angiography of the chest on admission with moderate pericardial effusion (arrows).

Transthoracic echocardiography (TTE) showed a moderate effusion, but no tamponade physiology.

Hospital Course

Given the ongoing lupus flare, pleuritic chest pain, elevated ESR, normal troponin and pericardial effusion, the patient’s chest pain was thought to be caused by acute pericarditis secondary to SLE flare. The patient was treated with anti-hypertensives, though her creatinine worsened, which prompted a kidney biopsy, that showed signs of lupus nephritis. The patient was treated with methylprednisolone pulse 0.5 mg/kg for three days, then prednisone taper. Her home hydroxychloroquine regimen was resumed. The patient became febrile on hospital day 15 and blood cultures were obtained. These later revealed MSSA bacteremia, which is thought to be secondary to thrombophlebitis from an infected peripheral IV line in her left antecubital fossa. On hospital day 16, the patient complained of worsening chest pain and had an elevated troponin of 2, but no signs of ischemia on EKG. Repeat echo was performed, which showed increase in size of the pericardial effusion and right ventricular collapse during diastole, concerning for impending tamponade (Figure 2).

Figure 2. Video of the transthoracic echocardiography showing a pericardial effusion (top arrow) with RV collapse during diastole (bottom arrow), concerning for impending cardiac tamponade.

The patient remained hemodynamically stable. Pericardial window was performed. 500 cc of purulent fluid was drained, and a pericardial drain was placed. Intra-operative fluid culture grew MSSA. The drain was left in place for 13 days. The patient was treated with a 4-week course of oxacillin. Blood cultures obtained on hospital day 28 were negative. A repeat echo was normal. The patient was discharged without further complications.

Discussion

Bacterial pericarditis is a rare, but fatal infection, with 100% mortality in untreated patients (1). After the introduction of antibiotics, the incidence of bacterial pericarditis declined to 1 in 18,000 hospitalized patients, from 1 in 254 (2). The most implicated organisms are Staphylococcus, Streptococcus, Hemophilus and M. tuberculosis (3).  Historically, pneumonia was the most common underlying infection leading to purulent pericarditis, especially in the pre-antibiotic era (2). Since the widespread use of antibiotics, purulent pericarditis has been linked to bacteremia, thoracic surgery, immunosuppression, and malignancy (3).

Acute pericarditis is a common complication in SLE with incidence of 11-54% (4), though few cases of bacterial pericarditis were reported in SLE patients. The organisms in these cases were staphylococcus aureus, Neisseria gonorrhea and mycobacterium tuberculosis (5). Despite these reports, acute pericarditis secondary to immune complex mediated inflammatory process remains a much more common cause of pericarditis than bacterial pericarditis in SLE (6). There’s minimal data to determine whether the incidence of bacterial pericarditis in patients with SLE is increased compared to the general population; however, there is a hypothetically increased risk for purulent pericarditis in SLE given the requirement for immunosuppression. Disease activity is yet another risk factor for bacterial infections in SLE, which is thought to be a sequalae of treatment with high doses of steroids (7). In this case, the patient had an SLE flare on presentation with SLEDAI-2K score of 13. Both immunosuppression and bacteremia may have precipitated this patient’s infection with bacterial pericarditis.   

Diagnosis of bacterial pericarditis requires high index of suspicion, as other etiologies of pericarditis are far more common. In this case, we initially attributed the patient’s pericarditis to her SLE flare. The patient’s fever on hospital day 15 prompted the infectious work up. MSSA pericarditis was diagnosed later after the pericardial fluid culture grew MSSA. Delay in the diagnosis can be detrimental as patients may progress rapidly to cardiac tamponade. 

Treatment requires surgical drainage for source control along with antibiotics (8). In our case, the patient required pericardial window and placement of a drain for 13 days. In bacterial pericarditis, the purulent fluid tends to re-accumulate; therefore, subxiphoid pericardiostomy and complete drainage is recommended (8). In some cases, intrapericardial thrombolysis therapy may be required if adhesions develop (8). With appropriate source control and antibiotics therapy, survival rate is up to 85% (8). 

Conclusion

Bacterial pericarditis is a rare infection in the antibiotic era, though some patients remain at risk for acquiring it. Despite the high mortality rate, patients can have good outcomes if bacterial pericarditis is recognized early and treated.

References

1. Kaye A, Peters GA, Joseph JW, Wong ML. Purulent bacterial pericarditis from Staphylococcus aureus. Clin Case Rep. 2019 May 28;7(7):1331-1334. [CrossRef] [PubMed]
2. Parikh SV, Memon N, Echols M, Shah J, McGuire DK, Keeley EC. Purulent pericarditis: report of 2 cases and review of the literature. Medicine (Baltimore). 2009 Jan;88(1):52-65. [CrossRef] [PubMed}
3. Kondapi D, Markabawi D, Chu A, Gambhir HS. Staphylococcal Pericarditis Causing Pericardial Tamponade and Concurrent Empyema. Case Rep Infect Dis. 2019 Jul 18;2019:3701576. [CrossRef] [PubMed]
4. Dein E, Douglas H, Petri M, Law G, Timlin H. Pericarditis in Lupus. Cureus. 2019 Mar 1;11(3):e4166. [CrossRef] [PubMed]
5. Coe MD, Hamer DH, Levy CS, Milner MR, Nam MH, Barth WF. Gonococcal pericarditis with tamponade in a patient with systemic lupus erythematosus. Arthritis Rheum. 1990 Sep;33(9):1438-41. [CrossRef] [PubMed]
6. Buppajamrntham T, Palavutitotai N, Katchamart W. Clinical manifestation, diagnosis, management, and treatment outcome of pericarditis in patients with systemic lupus erythematosus. J Med Assoc Thai. 2014 Dec;97(12):1234-40. [PubMed]
7. Nived O, Sturfelt G, Wollheim F. Systemic lupus erythematosus and infection: a controlled and prospective study including an epidemiological group. Q J Med. 1985 Jun;55(218):271-87. [PubMed]
8. Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC)Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015 Nov 7;36(42):2921-2964. [CrossRef] [PubMed]

Cite as: Anis A, Varda M, Dudar A, Schmitz ED. MSSA Pericarditis in a Patient with Systemic Lupus Erythematosus Flare. Southwest J Pulm Crit Care Sleep. 2022;24(2):32-35. doi: https://doi.org/10.13175/swjpccs057-21 PDF

Rapidly Fatal COVID-19-associated Acute Necrotizing Encephalopathy in a Previously Healthy 26-year-old Man

Robert A. Raschke MD and Cristian Jivcu MD

HonorHealth Scottsdale Osborn Medical Center

Scottsdale, AZ USA

Case Presentation

A 26-year-old man presented to our Emergency Department at 0200 on the day of admission with chief complaints of subjective fever, leg myalgias, and progressive dyspnea of one week duration. An oropharyngeal swab PCR had revealed SARS-CoV-2 RNA three days previously. He had not received a SARS CoV-2 vaccination, but had made an appointment to receive it just a few days prior to the onset of his symptoms.

The patient had no significant past medical history, was taking no medications except for ibuprofen and acetaminophen over the past week, and did not take recreational drugs. He specifically denied headache and had no prior history of seizure.

On admission, his HR was 150 bpm (sinus), RR 22, BP 105/46 mmHg, temp 40.2° C. and SpO₂ 92% on room air. He was ill-appearing, but alert and oriented, his neck was supple and lung auscultation revealed bilateral rhonchi, but physical examination was otherwise unremarkable.

A CBC showed WBC 17.3 10³/uL, hemoglobin 13.9 g/dl, and platelet count 168 K/uL. A complete metabolic profile was normal except for the following: Na 135 mmol/L, creatinine 1.7 mg/dL, AST 95 and ALT 134 IU/L. D-dimer was 1.08 ug/ml (normal range 0.00-0.50 ug/ml), and ferritin 783 ng/ml. A urine drug screen was negative. Chest radiography showed subtle bilateral pulmonary infiltrates. CT angiography of the chest was negative for pulmonary embolism but showed bilateral patchy infiltrates consistent with COVID19 pneumonia. One liter NS bolus and dexamethasone 10mg were given intravenously, acetaminophen administered orally, and the patient was admitted to telemetry.

Shortly thereafter, the patient experienced a brief generalized seizure associated with urinary incontinence. He was stuporous post-ictally, exhibiting only arm flexion to painful stimuli. A stroke alert was called and radiographic studies emergently obtained. CT of the brain was normal and CT angiography of the head and neck showed no large vessel occlusion or flow-limiting stenosis, and a CT perfusion study (Figure 1) showed patchy Tmax prolongation in the right cerebellum and bilateral parietal occipital lobes “which may reflect artifact or relative ischemia” with no matching core infarct.

Figure 1. CT perfusion study showing mild bilateral posterior distribution ischemia (Tmax > 6 secs) without matching core infarct (CBF<30%), interpreted by a neuroradiologist as possible artifact.

The patient was transferred to the ICU at 10:00, and experienced a 40-second generalized tonic-clonic seizure shortly thereafter. Lorazepam 2mg was administered intravenously. The HR was 104, RR 21, BP 105/61, temp 36.5 C. and SpO₂ 96% on 2L /min nasal canula oxygen. On neurological examination, the Glasgow Coma Scale was 3, right pupil was 3mm, left pupil 2mm - both reactive, the gaze was disconjugate and directed downward, there was no blink to visual threat, and glabellar ridge pressure did not elicit grimace, but minimal arm flexion. The gag reflex was positive. Peripheral reflexes were 2+ with down-going toes bilaterally. Levetiracetam 1000mg bolus was administered intravenously. Glucose was 147 mg/dL. An EEG obtained at 12:00 showed diffuse bilateral slowing without seizure activity. A presumptive diagnosis of post-ictal encephalopathy was made. The patient seemed to be protecting his airway and nasal canula oxygen was continued.

The patient’s condition was not noted to significantly change over the next 12 hours. There were no episodes of hypoxia, hypotension or hypoglycemia. Around 0100 on the second day of hospitalization, the patient exhibited extensor-posturing and appeared to be choking on his oral secretions. HR rose to 135, BP 155/99, RR 12 and temp 37.8 C. His SpO₂ fell into the mid 80% range. He no longer had a gag or cough reflex and he was emergently intubated without complication. MRI (Figure 2) and MRV of the brain were emergently obtained. 

Figure 2. A: T2-weighted image demonstrating bilateral thalamic and L occipital white matter hypoattenuation. B: DWI and GRE images showing bilateral thalamic infarctions with hemorrhage. C: Representative DWI images of cerebrum and cerebellum and pons showing widespread diffusion restriction.

The MRI showed extensive diffusion restriction involving bilateral thalami, cerebellar hemispheres, pons, and cerebral hemispheres with scattered hemorrhage most obvious/confluent in the bilateral thalami.

Normal flow voids were present in intracranial arteries and venous structures. Partial effacement of the lateral and third ventricles was noted, with early uncal herniation. The MRV showed no evidence of dural venous sinus thrombosis.

At 05:00 of the second hospital day, it was noted that the patient’s pupils were dilated and unreactive and his respiratory rate was 16 – equal to the respiratory rate set on the ventilator. BP fell to 85/45 and norepinephrine infusion was started to maintain MAP >65 mmHg. STAT CT brain (Figure 3) showed hemorrhagic infarcts of the bilateral thalami with surrounding edema, interval development of low attenuation of the bilateral cerebrum and cerebellum, and mass effect with total effacement of fourth ventricle, basal cisterns and cerebral sulci consistent with severe cerebral edema.

Figure 3. STAT CT brain from 05:30 on the second hospital day showing bilateral thalamic infarctions and diffuse cerebral edema with effacement of the sulci and loss of grey/white differentiation.

Two neurologists confirmed the clinical diagnosis of brain death, including an apnea test. A venous ammonia level ordered that morning was not drawn. An autopsy was requested by the physicians, but not able to be obtained.

Discussion

Acute necrotizing encephalopathy (ANE) is a rarely-reported clinical-radiographic syndrome lacking pathopneumonic laboratory test or histological findings (1-3). It is characterized by an acute febrile viral prodrome, most commonly due to influenza or HHV-6, followed by rapidly progressive altered mental status and seizures. The most specific finding of ANE is necrosis of the bilateral thalami, appearing on MRI as hypoattenuated lesions on T2 and FLAIR images with diffusion restriction on DWI, and often with hemorrhage demonstrated on GRE images (as shown in figure 2 above). Symmetric multifocal lesions are typically seen throughout various other locations in the brain including the cerebral periventricular white matter, cerebellum, brainstem and spinal cord. Mizuguchi (who first described ANE in 1995) proposed elevation of serum aminotransferase without hyperammonemia, and cerebrospinal albuminocytologic dissociation (elevated CSF protein without leukocytosis) as laboratory criteria supporting the diagnosis of ANE (1,2). These were only partially evaluated in our patient. The mortality of ANE is 30% and significant neurological sequelae are common in survivors (2).

The clinical, radiographic and laboratory findings in our case are all characteristic of ANE, but our work-up was abbreviated by the patient’s fulminant presentation. The differential diagnosis includes hyper-acute forms of acute disseminated encephalomyelitis (ADEM) or acute hemorrhagic leukoencephalitis that may also occur after a viral prodrome and may be associated with diffuse white matter lesions (4,5), although bilateral thalamic necrosis is not characteristic of either of these entities. Examination of cerebral spinal fluid (CSF) for pleocytosis, oligoclonal bands, and testing for the myelin oligodendrocyte glycoprotein IgG autoantibody and the aquaporin-4 IgG serum autoantibody would have been indicated to further evaluate for the initial presentation of a relapsing CNS demyelinating disease (5,6). CSF examination would also have been helpful in ruling out viral encephalitis affecting the thalami, such as that caused by West Nile Virus (WNV) (7). An acute metabolic encephalopathy with diffuse brain edema, such as that caused by severe hyperammonemia associated with late-onset ornithine transcarbamylase deficiency (8) was not ruled out. Arterial or venous thromboembolism associated with COVID-19 were effectively ruled out by CT angiogram, CT perfusion and MRI and MRV findings.    

We found five previous case reports of ANE as a complication of COVID-19, ranging 33-59 years of age (9-13). The onset of altered mental status occurred 3, 4, 7,10 and 21 days after onset of COVID-19 symptoms and rapidly progressed to coma. Two had generalized seizures, one myoclonus and another “rhythmic movements” of an upper extremity. All had bilateral hypoattenuation of the thalami on CT and MRI with variable involvement of temporal lobes, subinsular regions, cerebellum, brainstem and supratentorial grey and white matter. Two patients had EEGs that showed generalized slow waves. All underwent examination of CSF with negative PCR tests for various common encephalopathy viruses including herpes simplex virus 1&2 and WNV - four reported CSF protein and cell counts, three of which demonstrated albuminocytologic dissociation. Three patients received IVIG. Two patients died on days 5 and 8 after onset of neurological symptoms. Two recovered after prolonged ICU care and the outcome of the third patient was not reported. ANE may be less rare than these few case reports suggest. A retrospective study carried out at 11 hospitals in Europe describes radiographic findings of 64 COVID-19 patients with neurological symptoms (14). The most common finding was ischemic stroke, but 8 patients had MRI findings consistent with encephalitis and two had findings characteristic of ANE.

The pathogenesis of ANE is unknown. Ten cases of fatal ANE with brain biopsy are reported (1,15-19). These showed diffuse cerebral edema, and hemorrhagic necrosis invariably involving the thalami. An exudative small vessel vasculopathy with endothelial necrosis was found in 7/10 patients (This could perhaps explain the early CT perfusion findings interpreted as artifactual in our patient). Demyelination or inflammatory infiltration of the brain or leptomeninges was absent. There has been conjecture that these pathological findings might be due to disruption of the blood brain barrier caused by hypercytokinemia but there is scant supportive evidence (20). 

There is no proven treatment for ANE. Corticosteroids, IVIg and plasma exchange have been previously used (3,9-11,21). Clinical trials are unlikely given the rarity of the disorder.

It was unfortunate that this young man had not availed himself of SARS CoV-2 vaccination. We did not make a pre-mortem diagnosis of ANE between his first abnormal CT brain at 0100 and his death at 06:00. We would have performed an LP, measured serum ammonia and given a trial of corticosteroids and IVIg if we had had more time.

References

1. Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, Kamoshita S. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry. 1995 May;58(5):555-61. [CrossRef] [PubMed]
2. Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997 Mar;19(2):81-92. [CrossRef] [PubMed]
3. Wu X, Wu W, Pan W, Wu L, Liu K, Zhang HL. Acute necrotizing encephalopathy: an underrecognized clinicoradiologic disorder. Mediators Inflamm. 2015;2015:792578. [CrossRef] [PubMed]
4. Marchioni E, Ravaglia S, Montomoli C, et al. Postinfectious neurologic syndromes: a prospective cohort study. Neurology. 2013 Mar 5;80(10):882-9. [CrossRef] [PubMed]
5. Manzano GS, McEntire CRS, Martinez-Lage M, Mateen FJ, Hutto SK. Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Leukoencephalitis Following COVID-19: Systematic Review and Meta-synthesis. Neurol Neuroimmunol Neuroinflamm. 2021 Aug 27;8(6):e1080. [CrossRef] [PubMed]
6. López-Chiriboga AS, Majed M, et al. Association of MOG-IgG Serostatus With Relapse After Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for MOG-IgG-Associated Disorders. JAMA Neurol. 2018 Nov 1;75(11):1355-1363. [CrossRef] [PubMed]
7. Guth JC, Futterer SA, Hijaz TA, Liotta EM, Rosenberg NF, Naidech AM, Maas MB. Pearls & oy-sters: bilateral thalamic involvement in West Nile virus encephalitis. Neurology. 2014 Jul 8;83(2):e16-7. [CrossRef] [PubMed]
8. Cavicchi C, Donati M, Parini R, et al. Sudden unexpected fatal encephalopathy in adults with OTC gene mutations-Clues for early diagnosis and timely treatment. Orphanet J Rare Dis. 2014 Jul 16;9:105. [CrossRef] [PubMed]
9. Poyiadji N, Shahin G, Noujaim D, Stone M, et al.  COVID19-associated acute necrotizing encephalopathy: CT and MRI features.  Radiology. 2020;296:E119-E120. [CrossRef]
10. Virhammar J, Kumlien E, Fällmar D,et al. Acute necrotizing encephalopathy with SARS-CoV-2 RNA confirmed in cerebrospinal fluid. Neurology. 2020 Sep 8;95(10):445-449. [CrossRef] [PubMed]
11. Delamarre L, Galion C, Goudeau G, et al. COVID-19-associated acute necrotising encephalopathy successfully treated with steroids and polyvalent immunoglobulin with unusual IgG targeting the cerebral fibre network. J Neurol Neurosurg Psychiatry. 2020 Sep;91(9):1004-1006. [CrossRef] [PubMed]
12. Dixon L, Varley J, Gontsarova A, Mallon D, Tona F, Muir D, Luqmani A, Jenkins IH, Nicholas R, Jones B, Everitt A. COVID-19-related acute necrotizing encephalopathy with brain stem involvement in a patient with aplastic anemia. Neurol Neuroimmunol Neuroinflamm. 2020 May 26;7(5):e789. [CrossRef] [PubMed]
13. Elkady A, Rabinstein AA. Acute necrotizing encephalopathy and myocarditis in a young patient with COVID-19. Neurol Neuroimmunol Neuroinflamm Sep 2020, 7 (5) e801. [CrossRef]
14. Kremer S, Lersy F, Anheim M, et al. Neurologic and neuroimaging findings in patients with COVID-19: A retrospective multicenter study. Neurology. 2020 Sep 29;95(13):e1868-e1882. [CrossRef] [PubMed]
15. Kirton A, Busche K, Ross C, Wirrell E. Acute necrotizing encephalopathy in caucasian children: two cases and review of the literature. J Child Neurol. 2005 Jun;20(6):527-32. [CrossRef] [PubMed]
16. Mastroyianni SD, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M. Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006 Oct;21(10):872-9. [CrossRef] [PubMed]
17. Nakano I, Otsuki N, Hasegawa A. Acute Stage Neuropathology of a Case of Infantile Acute Encephalopathy with Thalamic Involvement: Widespread Symmetrical Fresh Necrosis of the Brain. Neuropathology 1993;13: 315-25. [CrossRef]
18. Yagishita A, Nakano I, Ushioda T, Otsuki N, Hasegawa A. Acute encephalopathy with bilateral thalamotegmental involvement in infants and children: imaging and pathology findings. AJNR Am J Neuroradiol. 1995 Mar;16(3):439-47. [PubMed]
19. San Millan B, Teijeira S, Penin C, Garcia JL, Navarro C. Acute necrotizing encephalopathy of childhood: report of a Spanish case. Pediatr Neurol. 2007 Dec;37(6):438-41. [CrossRef] [PubMed]
20. Wang GF, Li W, Li K. Acute encephalopathy and encephalitis caused by influenza virus infection. Curr Opin Neurol. 2010 Jun;23(3):305-11. [CrossRef] [PubMed]
21. Okumura A, Mizuguchi M, Kidokoro H, et al. Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin. Brain Dev. 2009 Mar;31(3):221-7. [CrossRef] [PubMed]

Cite as: Raschke RA, Jivcu C. Rapidly Fatal COVID-19-associated Acute Necrotizing Encephalopathy in a Previously Healthy 26-year-old Man. Southwest J Pulm Crit Care. 2021;23(5):138-43. doi: https://doi.org/10.13175/swjpcc039-21 PDF

Utility of Endobronchial Valves in a Patient with Bronchopleural Fistula in the Setting of COVID-19 Infection: A Case Report and Brief Review

Nazanin Sheikhan, MD¹, Elizabeth J. Benge, MD¹, Amanpreet Kaur, MD¹, Jerome K Hruska, DO², Yi McWhorter DO³, Arnold Chung MD⁴

¹Department of Internal Medicine, HCA Healthcare; MountainView Hospital, Las Vegas, NV, USA

²Department of Pulmonology, HCA Healthcare; MountainView Hospital, Las Vegas, NV, USA

³Department of Anesthesiology Critical Care Medicine, HCA Healthcare; MountainView Hospital, Las Vegas, NV, USA

⁴MountainView Cardiovascular and Thoracic Surgery Associates, HCA Healthcare; MountainView Hospital, Las Vegas, NV, USA

Abstract

Patients with COVID-19 pneumonia frequently develop acute respiratory distress syndrome (ARDS), and in severe cases, require invasive mechanical ventilation. One complication that can develop in patients with ARDS who are mechanically ventilated is a bronchopleural fistula (BPF). Although rare, the frequency of BPF in patients with COVID-19 pneumonia is increasingly recognized. Here, we present a 48-year old man with BPF associated with COVID-19 pneumonia. Treatment with a commercial endobronchial valve (EBV) system resulted in reduced air leak allowing for tracheostomy placement. Our case adds to a growing body of evidence suggesting that the presence of COVID-19 pneumonia does not hinder the utility of EBV’s in the treatment of BPF’s.

Abbreviation List

• ARDS = acute respiratory distress syndrome
• BIPAP = Bilevel Positive Airway Pressure
• BPF = Bronchopleural Fistula
• COVID-19 = Coronavirus Disease-2019
• CT = Computed Tomography
• CTA = Computed Tomography Angiography
• EBV = Endobronchial Valve
• HFNC = High Flow Nasal Cannula
• ICU = Intensive Care Unit
• RML = Right Middle Lobe
• RUL = Right Upper Lobe
• SARS-CoV-2 = Severe Acute Respiratory Syndrome Coronavirus-2
• VATS = Video-Assisted Thoracoscopic Surgery

Introduction

The COVID-19 pandemic has resulted in over one hundred million infections worldwide, in addition to millions of deaths (1). A less common sequelae of COVID-19 is bronchopleural fistula (2). A bronchopleural fistula is an abnormal sinus tract that forms between the lobar, main stem, or segmental bronchus, and the pleural space (3). BPF is typically treated by surgical repair, via a video-assisted thoracoscopic surgical approach (VATS) (3). Bronchoscopic approach with placement of airway stents, coils or transcatheter occlusion devices can be considered for those who are not suitable for surgical intervention (3).  A newer therapeutic modality for bronchopleural fistulae are endobronchial valves, which have been used successfully to treat COVID-19 patients diagnosed concurrently with bronchopleural fistulae (4). 

Here, we present a case of a critically ill patient developing a bronchopleural fistula with a concurrent COVID-19 infection, whose respiratory status was stabilized with an endobronchial valve.  To our knowledge, this is one of four case reports of a bronchopleural fistula arising in the setting of COVID-19.

Brief Review of Endobronchial Valves in COVID-19

Several other studies report success using endobronchial valves to treat bronchopleural fistulae in patients with COVID-19 pneumonia. One case series documents two cases of COVID-19 pneumonia complicated by bacterial super-infections, in which both patients experienced pneumothorax and persistent air leaks after mechanical invasive ventilation.  Both patients were successfully treated via EBV positioning. These researchers speculate that the severe inflammation associated with COVID-19 related ARDS induces inflammatory-related tissue frailty, pre-disposing lung tissue to damage via barotrauma, and the subsequent development of BPF (5).  

Another case documents the treatment of a 49-year-old male with COVID-19 pneumonia who was treated with steroids and tocilizumab. He also had a 3-week history of persistent air leak, which was successfully treated with an EBV. This team emphasizes that the thick, copious sections evident in patients afflicted by COVID-19 pose a risk for EBV occlusion. They highlight the importance of medically optimizing the patient and draining the air leak to mitigate the potential of this procedural complication developing (4).

In conjunction with the treatment course presented in our case, these case reports provide compelling evidence indicating that endobronchial valves can be successfully used to treat persistent air leaks in patients with COVID-19 pneumonia.

Case Presentation

Our patient is a 48-year-old male with a medical history significant for essential hypertension and Type 1 diabetes mellitus who presented to the emergency department complaining of acute onset generalized weakness, shortness of breath, and a near-syncopal event that had occurred the day prior. Vital signs on admission showed an oxygen saturation of 86% on ambient air, respiratory rate of 18 breaths per min, heart rate of 111 beats per min with a temperature of 37.6°C. He was tested for SARS-CoV-2 on admission and was found to be positive.

Initial computed tomography (CT) chest showed diffuse bilateral ground-glass opacities compatible with COVID-19 pneumonia. On admission, his inflammatory markers were elevated, with C-reactive protein 4.48 mg/dL, ferritin 1230 ng/ml, lactate dehydrogenase 281 IU/L, and D-dimer 0.76 mg/L. He received 1 dose of tocilizumab, convalescent plasma, as well as 5-day course of Remdesivir. His oxygen requirement increased as well as his work of breathing requiring High Flow Nasal Cannula (HFNC) and subsequently Bilevel Positive Airway Pressure (BiPAP); patient was transferred to the medical intensive care unit (ICU) 17 days after admission requiring intubation. Computed tomography angiography (CTA) chest could not be obtained to rule out pulmonary embolism as patient was too unstable. Patient was started on Heparin drip empirically which had to be discontinued due to gastrointestinal bleeding. He had worsening oxygenation, ventilator asynchrony, with P:F ratio of 47, requiring high-dose sedation and neuromuscular blockade, as well as prone positioning. Repeat CT chest on day 21 demonstrated bilateral pneumothoraces and pneumomediastinum as well as interval worsening of diffuse ground glass infiltrates (Figure 1), requiring bilateral chest tube placement.

Figure 1. Computed tomography chest showing pneumomediastinum, bilateral pneumothoraces, and diffuse ground glass attenuation of the lungs bilaterally.

On the 34th day of admission, he developed a right-sided tension pneumothorax likely secondary to ongoing severe ARDS, requiring replacement of dislodged right chest tube. Patient subsequently had worsening of right pneumothorax requiring an additional second chest tube placement. Patient developed persistent air leak concerning for right bronchopleural fistula. On hospital day 42, patient underwent intrathoracic autologous blood patch with persistence of large air leak. After interdisciplinary conference with cardiothoracic surgery, pulmonary, and the ICU team, it was decided that patient is not a surgical candidate hence interventional pulmonology was consulted for EBV placement to facilitate chest tube removal and ventilator weaning.

Patient underwent fiberoptic bronchoscopy on hospital day 52; pulmonary balloon was used to sequentially block the right mainstem, bronchus intermedius, and basilar segments. The air leak was recognized to be coming from right middle lobe (RML) and the apex of the right upper lobe (RUL) status post placement of two endobronchial valves in the medial and lateral segments of the RML (Figure 2).

Figure 2. Bronchoscopic view of endobronchial valves.

The RUL could not be entered secondary to angulation and technical inability of the instruments to achieve a sharp bend. Post-bronchoscopy, patient had 50 mL reduction in air leak resulting in improvement of his ventilator settings such that a tracheostomy could be safely performed. Left-sided chest tube was removed with resolution of pneumothorax. Repeat CT chest on hospital day 115 demonstrated persistent right bronchopleural fistula (Figure 3).

Figure 3. Computed tomography chest showing bronchopleural fistula in the right middle lobe and collapsed and shrunken right middle lobe with endobronchial occlusion stents at the central airway. Yellow arrow showing endobronchial valves and red arrows showing bronchopleural fistula

The patient is currently pending transfer to a long-term acute care hospital for aggressive physical therapy and eventual transfer to a tertiary center for lung transplantation evaluation.

Discussion

Scientific research has moved at an unprecedented speed in an attempt to shed light on the manifestations of COVID-19. The most common presentation of COVID-19 includes cough, fever, shortness of breath, and new onset anosmia and ageusia (6).

Common complications include coagulopathy, pulmonary emboli, and in severe cases, acute respiratory distress syndrome (7). Bronchopleural fistulae have emerged as a rare but known complication of COVID-19. This pathology is traditionally seen as a post-surgical complication arising from lobectomy or pneumonectomy (8). All cause mortality secondary to bronchopleural fistulae are high; with mortality rates ranging from 18-67% (8).

A relatively novel therapeutic modality for bronchopleural fistulae are endobronchial valves, which have been used in patients who are not candidates for surgery, such as our patient (9). They work as a one-way valve that allow the pathologically trapped air to exit the respiratory system, but not enter (4).

Differential diagnoses for bronchopleural fistulae include alveolar pleural fistulas and empyema (11). Alveolar pleural fistulas are abnormal communications between the pulmonary parenchyma, distal to a segmental bronchus, and the pleural space, while bronchopleural fistulas are more proximal; representing abnormal connections between a mainstem, lobar, or segmental bronchus and the pleural space (12). These pathologies are differentiated with direct visualization on bronchoscopy, as was demonstrated in our patient (12).

There are currently no official statistics on the epidemiology of bronchopleural fistulae in COVID-19. A disappointing aspect of our case was the lack of complete resolution of the patient’s air leak after the placement of the endobronchial valve. While the patient’s condition did improve after the valve was placed, he continued to suffer from respiratory illness related to his bronchopleural fistula. Although complete remission was not achieved, the endobronchial valve placement did facilitate respiratory recovery sufficient enough to facilitate a tracheostomy. The patient was then stabilized for eventual transfer to a long-term acute care facility, where he will undergo physical therapy and await lung transplantation. It is important to emphasize that while the endobronchial valve was not curative, it stabilized the patient for possible future curative treatments.  

Conclusion

Despite their rarity, bronchopleural fistulas are a pulmonary complication of COVID-19. Although the insertion of the endobronchial valve in our patient resulted in a reduction of the air leak as opposed to complete resolution, this case still emphasizes a therapeutic benefit of endobronchial valves in such instances. Overall, our case demonstrates the importance of clinical vigilance in the face of unusual pulmonary complications related to COVID-19, and that treatment of these complications requires flexibility and creativity.

References

1. WHO Coronavirus (COVID-19) Dashboard [Internet]. World Health Organization. World Health Organization; [cited 2021May31]. Available from: https://covid19.who.int/ 
2. Hopkins C, Surda P, Kumar N. Presentation of new onset anosmia during the COVID-19 pandemic. Rhinology. 2020 Jun 1;58(3):295-298. [CrossRef] [PubMed]
3. Miesbach W, Makris M. COVID-19: Coagulopathy, Risk of Thrombosis, and the Rationale for Anticoagulation. Clin Appl Thromb Hemost. 2020 Jan-Dec;26:1076029620938149. [CrossRef] [PubMed]
4. Talon A, Arif MZ, Mohamed H, Khokar A, Saeed AI. Bronchopleural Fistula as a Complication in a COVID-19 Patient Managed With Endobronchial Valves. J Investig Med High Impact Case Rep. 2021 Jan-Dec;9:23247096211013215. [CrossRef] [PubMed]
5. Donatelli P, Trenatacosti F, Pellegrino MR, et al. Endobronchial valve positioning for alveolar-pleural fistula following ICU management complicating COVID-19 pneumonia. BMC Pulm Med. 2021 Sep 27;21(1):307. [CrossRef] [PubMed]
6. Salik I, Vashisht R, Abramowicz AE. Bronchopleural fistula. StatPearls [Internet]. 2020 Aug 27. [CrossRef]
7. Cardillo G, Carbone L, Carleo F, Galluccio G, Di Martino M, Giunti R, Lucantoni G, Battistoni P, Batzella S, Dello Iacono R, Petrella L, Dusmet M. The Rationale for Treatment of Postresectional Bronchopleural Fistula: Analysis of 52 Patients. Ann Thorac Surg. 2015 Jul;100(1):251-7. [CrossRef] [PubMed]
8. Sarkar P, Chandak T, Shah R, Talwar A. Diagnosis and management bronchopleural fistula. Indian J Chest Dis Allied Sci. 2010 Apr-Jun;52(2):97-104. [PubMed]
9. Pathak V, Waite J, Chalise SN. Use of endobronchial valve to treat COVID-19 adult respiratory distress syndrome-related alveolopleural fistula. Lung India. 2021 Mar;38(Supplement):S69-S71. [CrossRef] [PubMed]
10. Musani AI, Dutau H. Management of alveolar-pleural fistula: a complex medical and surgical problem. Chest. 2015 Mar;147(3):590-592. [CrossRef] [PubMed]
11. Mehta HJ, Malhotra P, Begnaud A, Penley AM, Jantz MA. Treatment of alveolar-pleural fistula with endobronchial application of synthetic hydrogel. Chest. 2015 Mar;147(3):695-699. [CrossRef]  [PubMed]

Acknowlegements

This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities. 

Cite as: Sheikhan N, Benge EJ, Kaur A, Hruska JK, McWhorter Y, Chung A. Utility of Endobronchial Valves in a Patient with Bronchopleural Fistula in the Setting of COVID-19 Infection: A Case Report and Brief Review. Southwest J Pulm Crit Care. 2021;23(4):109-14. doi: https://doi.org/10.13175/swjpcc046-21 PDF 

October 2021 Critical Care Case of the Month: Unexpected Post-Operative Shock

Sharanyah Srinivasan MBBS

Sooraj Kumar MBBS

Benjamin Jarrett MD

Janet Campion MD

University of Arizona College of Medicine, Department of Internal Medicine and Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Tucson, AZ USA

History of Present Illness 

A 55-year-old man with a past medical history significant for endocarditis secondary to intravenous drug use, osteomyelitis of the right lower extremity was admitted for ankle debridement. Pre-operative assessment revealed no acute illness complaints and no significant findings on physical examination except for the ongoing right lower extremity wound. He did well during the approximate one-hour “incision and drainage of the right lower extremity wound”, but became severely hypotensive just after the removal of the tourniquet placed on his right lower extremity. Soon thereafter he experienced pulseless electrical activity (PEA) cardiac arrest and was intubated with return of spontaneous circulation being achieved rapidly after the addition of vasopressors. He remained intubated and on pressors when transferred to the intensive care unit for further management.

PMH, PSH, SH, and FH

• S/P Right lower extremity incision and drainage for suspected osteomyelitis as above
• Distant history of endocarditis related to IVDA
• Not taking any prescription medications
• Current smoker, occasional alcohol use
• Former IVDA
• No pertinent family history including heart disease

Physical Exam

• Vitals: 100/60, 86, 16, afebrile, 100% on ACVC 420, 15, 5, 100% FiO2
• Sedated well appearing male, intubated on fentanyl and norepinephrine
• Pupils reactive, nonicteric, no oral lesions or elevated JVP
• CTA, normal chest rise, not overbreathing the ventilator
• Heart: Regular, normal rate, no murmur or rubs
• Abdomen: Soft, nondistended, bowel sounds present
• No left lower extremity edema, right calf dressed with wound vac draining serosanguious fluid, feet warm with palpable pedal pulses
• No cranial nerve abnormality, normal muscle bulk and tone

Clinically, the patient is presenting with post-operative shock with PEA cardiac arrest and has now been resuscitated with 2 liters emergent infusion and norepinephrine at 70 mcg/minute.

What type of shock is most likely with this clinical presentation?

1. Cardiogenic shock
2. Hemorrhagic shock
3. Hypovolemic shock
4. Obstructive shock
5. Septic / distributive shock

Cite as: Srinivasan S, Kumar S, Jarrett B, Campion J. October 2021 Critical Care Case of the Month: Unexpected Post-Operative Shock. Southwest J Pulm Crit Care. 2021;23(4):93-7. doi: https://doi.org/10.13175/swjpcc041-21 PDF 

A Case and Brief Review of Bilious Ascites and Abdominal Compartment Syndrome from Pancreatitis-Induced Post-Roux-En-Y Gastric Remnant Leak

Marissa A. Martin, MD¹

Michael H. Lee, MD²

Anna Neumeier, MD³

Tristan J. Huie, MD³

¹ University of Colorado Department of Internal Medicine

² University of California, San Francisco Division of Pulmonary and Critical Care Medicine

³ University of Colorado Division of Pulmonary Sciences and Critical Care Medicine

Abstract

This is a case of a 55-year-old man with Roux-en-Y gastric bypass surgery 15 years prior who presented with acute pancreatitis and developed distributive shock, bacteremia, acute respiratory distress syndrome, anuric acute renal failure, and a distended abdomen with increasing ascitic fluid on imaging. An elevated bladder pressure, lactic acidosis, and anuria raised concern for abdominal compartment syndrome. Paracentesis was done and four liters of bilious ascitic fluid were drained. Intra-abdominal pressure was measured and improved from 27 cmH₂O to 13 cmH₂O with paracentesis. Mean arterial pressure and urine output also improved. The patient developed recurrent loculated intra-abdominal fluid collections, though ultrasound, CT scans with and without contrast, MRCP, ERCP, upper GI fluoroscopy, and small bowel enteroscopy failed to reveal a source of the bilious output. Ultimately, a gastrostomy tube was placed and delivery of contrast material through the tube revealed active extravasation from the remnant stomach. This case underscores the importance of considering post-surgical leak regardless of how remotely a Roux-en-Y surgery took place, confirms the importance of pursuing early gastrostomy tube placement and contrast administration when post-Roux-en-Y gastric remnant leaks are suspected, and demonstrates the role of paracentesis in critically ill patients with abdominal compartment syndrome.

Background

Post-surgical leaks complicate up to 7% of Roux-en-Y gastric bypass procedures and they result in greater than 50% morbidity and mortality (1,2). Most leaks (between 69% and 77%) occur at the gastrojejunal anastomosis, and on average, they become symptomatic three days after surgery (3,4). Rare leaks from the gastric remnant, which is the larger portion of the stomach that during a Roux-en-Y surgery is bypassed with the gastrojejunal anastomosis, have been reported and have been said to have delayed presentations, though this has typically only been weeks after surgery, not years (1,5). This is a case of post-Roux-en-Y gastric remnant leak that occurred 15 years after the original surgery, underscoring the importance of considering post-surgical leak as a diagnostic possibility regardless of how remotely a Roux-en-Y surgery took place. This case discusses a possible provoking factor, illustrates the clinical presentation, and suggests a diagnostic and treatment approach for these leaks. As morbid obesity becomes more prevalent in today’s society and Roux-en-Y gastric bypass procedures become even more mainstream, knowledge of delayed complications, such as the one discussed in this case, is crucial.

Case Report

A 55-year-old man with a past medical history of atrial fibrillation, previous alcohol-induced acute pancreatitis, and Roux-en-Y gastric bypass surgery 15 years prior presented with three days of abdominal pain and pre-syncope. He was drinking four to five alcoholic drinks daily. On presentation to the emergency department, the patient was in atrial fibrillation with a heart rate greater than 160 beats/min and was hypotensive to 77/53 mmHg. He was afebrile and mildly leukopenic with a white blood cell count of 4.4 k/mL. He had a lactate level of 12.5 mmol/L and a lipase of 1756 U/L with clinical and radiographic evidence of acute pancreatitis (Figure 1).

Figure 1. CT scan showing an enlarged pancreatic head and proximal body (arrow) with peripancreatic fat stranding (arrowhead), consistent with acute pancreatitis.

He was admitted to the medical intensive care unit, where over the next two days his distributive shock was complicated by Enterobacter cloacae bacteremia, acute respiratory distress syndrome, and acute anuric renal failure. For the management of his multi-organ failure, the patient was placed on mechanical ventilation, paralytic therapy, and infusions of norepinephrine, vasopressin, and phenylephrine. He was also started on continuous renal replacement therapy.

On hospital day three, the patient developed increasing abdominal distention with CT showing an interval increase in the size of ascites. An elevated bladder pressure of 21 mmHg, measured following the administration of rocuronium, along with a lactate of 12.3 mmol/L and anuria raised the concern for abdominal compartment syndrome. Paracentesis was done and four liters of bilious ascitic fluid were drained (Figure 2).

Figure 2. Paracentesis drained four liters of bilious fluid. Using a manometer, intra-abdominal pressure was measured first prior to fluid removal and subsequently after each liter was drained. The intra-abdominal pressure was 27 cmH₂O initially and decreased to 13 cmH₂O.

Using the manometer from a lumbar puncture kit, intra-abdominal pressure was measured first prior to fluid removal and subsequently after each liter was drained. With fluid removal, the initial intra-abdominal pressure of 27 cmH₂O improved to 13 cmH₂O (Figure 2), and the mean arterial pressure increased by 16 mmHg (from 70 mmHg to 86 mmHg). The norepinephrine, which had been infusing at 0.1 mcg/kg/min, was discontinued over the subsequent hour and a half, and the patient maintained a mean arterial pressure of 85 mmHg. Over the subsequent 12 hours, the patient’s urine output increased, and continuous renal replacement therapy was discontinued. Analysis of the ascitic fluid showed significantly elevated total bilirubin (17 mg/dL), lactate dehydrogenase (3545 U/L), and amylase (1481 U/L). Serum ascites albumin gradient was 1.1.

Over the next two weeks, the patient developed recurrent loculated intra-abdominal fluid collections (Figure 3) and leukocytosis (as high as 31.9 k/mL) refractory to two additional paracenteses with large volume ascitic fluid removal and broad-spectrum antibiotic treatment.

Figure 3. CT scan showing recurrent loculated intra-abdominal fluid collections (arrow) despite broad spectrum antibiotics and repeated paracenteses.

For definitive management of the recurrent ascites, two intra-abdominal drains were placed with fluid cultures growing Candida albicans. Intravenous micafungin was started, which was later narrowed to oral fluconazole. Continued high bilious output from the drains (as high as 3 L daily) raised the suspicion for biliary perforation or a post-Roux-en-Y leak. Multiple imaging studies including ultrasound, CT scans with and without contrast, and magnetic resonance cholangiopancreatography (MRCP), however, did not reveal a source of the bilious output. Although a hepatobiliary iminodiacetic acid (HIDA) scan showed a large leakage at the gastrojejunal anastomotic site, subsequent endoscopic retrograde cholangiopancreatography (ERCP), upper GI fluoroscopy, and small bowel enteroscopy did not demonstrate an overt contrast leak. Ultimately, a gastrostomy tube was placed by interventional radiology and delivery of contrast material through the tube revealed an active extravasation from the remnant stomach (Figure 4).

Figure 4.  CT scan showing extravasated contrast material (arrows) from the patient’s remnant stomach.

The patient was eventually discharged home on hospital day 28 with one remaining intra-abdominal drain in addition to the gastric tube to allow for gastric decompression and spontaneous healing of the post-Roux-en-Y leak.

Discussion

As discussed in the introduction, post-surgical leaks are a known complication of Roux-en-Y gastric bypass procedures and they have great morbidity and mortality. They most commonly occur at the gastrojejunal anastomosis and are typically detected within days of the original surgery. In our patient, it is likely that his alcohol-induced acute pancreatitis triggered the release of activated proteolytic pancreatic enzymes, which resulted in the gastric remnant leak and infected bilious ascites, a pathophysiologic mechanism previously suggested by one case series (6). Our patient’s delayed presentation 15 years after his Roux-en-Y gastric bypass surgery underscores the importance of considering post-surgical leak as a diagnostic possibility regardless of how remotely the surgery took place.

Diagnosing post-Roux-en-Y gastric remnant leaks can remain challenging even when they are suspected. Our patient’s gastric remnant leak was identified only after contrast delivery through the gastrostomy tube; previous diagnostic studies, including ultrasound, CT scans with and without contrast, MRCP, ERCP, upper GI fluoroscopy, and small bowel enteroscopy were all non-diagnostic. Similar diagnostic difficulty was described in another case of gastric remnant leak also complicated by the formation of amylase-containing dark ascitic fluid, in which the correct diagnosis was made only with CT-guided percutaneous gastrostomy followed by administration of contrast material (5). We hypothesize that this diagnostic difficulty is due to the inability of enteral contrast to reach the decompressed gastric remnant in adequate volume to detect a perforation, since it would be required to move against the typical flow of gastric secretions after a Roux-en-Y procedure. Our case confirms the importance of pursuing early gastrostomy tube placement and contrast administration when post-Roux-en-Y gastric remnant leak is suspected in order to allow for definitive diagnosis and appropriate treatment.

This case also highlights the diagnostic utility of paracentesis in abdominal hypertension or abdominal compartment syndrome, defined as an intra-abdominal pressure ≥ 12 mmHg or an intra-abdominal pressure > 20 mmHg with new organ dysfunction, respectively (7). Although our patient’s distended abdomen, elevated bladder pressure, and anuria collectively raised the concern for abdominal compartment syndrome, his abdomen remained soft. We therefore pursued paracentesis rather than exploratory laparotomy to both achieve an accurate assessment of the intra-abdominal pressure and drain the ascitic fluid. Our patient’s initial intra-abdominal pressure was 27 cmH2O (equivalent to 20 mmHg, similar to the patient’s paralyzed bladder pressure of 21 mmHg), which decreased to 13 cmH2O (or 9.6 mmHg) after four liters of fluid were removed. There was also clear evidence of improvement in end-organ perfusion and function after the paracentesis. We demonstrated a diagnostic as well as therapeutic role of paracentesis in critically ill patients with abdominal compartment syndrome. We showed that paracentesis is a viable alternative to surgical laparotomy, particularly when objective data such as bladder pressure does not correspond with physical examination findings.

References

1. Strobos E, Bonanni F. Asymptomatic gastric remnant leak after laparoscopic Roux-en-Y gastric bypass. Surg Obes Relat Dis. 2009 Sep-Oct;5(5):630-2. [CrossRef] [PubMed]
2. Madan AK, Lanier B, Tichansky DS. Laparoscopic repair of gastrointestinal leaks after laparoscopic gastric bypass. Am Surg. 2006 Jul;72(7):586-90; discussion 590-1. [PubMed]
3. Levine MS, Carucci LR. Imaging of bariatric surgery: normal anatomy and postoperative complications. Radiology. 2014 Feb;270(2):327-41. [CrossRef] [PubMed]
4. Lim R, Beekley A, Johnson DC, Davis KA. Early and late complications of bariatric operation. Trauma Surg Acute Care Open. 2018 Oct 9;3(1):e000219. [CrossRef] [PubMed]
5. Karmali S, Azer N, Sherman V, Birch DW. Computed tomography-guided percutaneous gastrostomy for management of gastric remnant leak after Roux-en-Y gastric bypass. Surg Obes Relat Dis. 2011 Mar-Apr;7(2):227-31. [CrossRef] [PubMed]
6. Schein M, Saadia R, Decker GA. Postoperative pancreatitis--a cause of anastomotic leaks? A report of 4 cases. S Afr Med J. 1988 May 7;73(9):550-1. [PubMed]
7. Kirkpatrick AW, Roberts DJ, De Waele J, et al. Intra-abdominal hypertension and the abdominal compartment syndrome: updated consensus definitions and clinical practice guidelines from the World Society of the Abdominal Compartment Syndrome. Intensive Care Med. 2013 Jul;39(7):1190-206. [CrossRef] [PubMed]

Cite as: Martin MA, Lee MH, Neumeier A, Huie TJ. A case and brief review of bilious ascites and abdominal compartment syndrome from pancreatitis-induced post-Roux-en-Y gastric remnant leak. Southwest J Pulm Crit Care. 2021;23(1):18-22. doi: https://doi.org/10.13175/swpcc018-21 PDF

July 2021 Critical Care Case of the Month: When a Chronic Disease Becomes Acute

Kara Calhoun MD, MPH

Division of Pulmonary Sciences & Critical Care Medicine

University of Colorado

Denver, CO USA

History of Present Illness

A 32-year-old woman with no known past medical history presented with progressive shortness of breath for the past 2 weeks. She denied having a cough, fever, or chills, but she did have a one-month history of fatigue, weakness, and painful rashes on her hands.

PMH, SH, and FH

• No known past medical history
• Former tobacco user (quit 2 years prior to admission)
• No drug use
• Worked as an office assistant
• Has two pet dogs and four pet macaws
• No family history of lung disease
• Not taking any prescription medications

Physical Exam

• BP: 116/65, Pulse: 105, T: 37°C, RR: 28, SpO2: 89% on HHFNC (60L; 100%)
• Pulmonary: Tachypneic, in respiratory distress, crackles throughout
• Cardiovascular: Tachycardic but regular, no murmurs
• Extremities: No edema
• Skin: Palms with purplish discoloration and erythematous papules

Radiography

Figure 1. Initial portable chest x-ray.

Which of the following should be done next?

1. CT Chest
2. COVID-19 testing
3. Sputum gram stain and culture
4. 1 and 3
5. All of the above

Cite as: Calhoun K. July 2021 Critical Care Case of the Month: When a Chronic Disease Becomes Acute. Southwest J Pulm Crit Care. 2021;23(1):1-4. doi: https://doi.org/10.13175/swjpcc023-21 PDF

Acute Type A Aortic Dissection in a Young Weightlifter: A Case Study with an In-Depth Literature Review

Stella C. Pak, MD¹

Edinen Asuka, MD²

¹Department of Medicine,

Orange Regional Medical Center

Middletown, NY USA

²All Saints University School of Medicine

Dominica

Abstract

In spite of the continuing efforts of researchers and practitioners, the mortality rate for acute type A aortic dissection remains relatively high at about 20-50%. Conventional risk factors associated with acute type A aortic dissection include a family history or prior history of aortic disease, connective tissue disease, smoking, alcohol use, substance abuse, diabetes mellitus type II, and age of 40 or greater. With the growing awareness for fitness in our society, vigorous exercise is emerging as a novel risk factor for acute type A Aortic dissection. Herein, we present a non-trauma related acute type A aortic dissection secondary to weight-lifting in a young man. We also reviewed several articles in order to provide a comprehensive literature overview for readers, clinicians and future researchers.

Case Report

A 45-year-old man who was otherwise healthy presented to the Emergency Department after having a “popping” sensation in his chest while weight-lifting with an 80-lbs (36.3 kg) dumbbell at a gym. He is an avid weight-lifter. This chest discomfort was immediately followed by a sensation of electric shock from his chest down to his legs and a transient loss of bilateral vision. He then developed an acute episode of lightheadedness, diaphoresis, throbbing headache, and a heavy-pressure in his neck, chest, and back. He denied any recent trauma or injury. He denied the use of tobacco, recreational drugs, or anabolic steroid. He denied the history of connective tissue diseases or cardiovascular diseases.

He was hypotensive with blood pressure of 99/45 mmHg. However other vital signs were within the normal limit: a temperature of 98.2 °F, a heart rate of 74/min, and a respiration rate of 15/min, an oxygen saturation of 97% at room air. His physical examination was remarkable for diminished pulses on his right upper and lower extremities. He did not have any marfanoid traits, such as tall stature, elongated face, or dolichostenomelia. His height and weight measured at the time of admission were 181cm and 95.7kg respectively (BMI 29.2).

His white blood count was elevated at 12.1 x 10⁹/L, but his hemoglobin remained stable at 15.6 g/dL. His troponin I was 0.26. He was found to have acute renal injury with BUN of 26 and creatinine of 1.7. His ECG, Chest X-ray, and CT of head and neck were unremarkable. He subsequently underwent a diagnostic cardiac catheterization, which revealed a swirling pattern and delayed washout of the contrast, findings suggestive of a false lumen. CT angiography displayed type A aortic dissection from the aortic root all the way down to the abdomen (Figure 1).

Figure 1.  CT angiography demonstrating ascending aortic dissection (arrow). The area with the arrow is the ascending portion of the aorta.

TEE visualized a rupture in the left coronary cusp at the aortic valve was visualized with the ejection fraction of 40% to 45%. Histopathological examination of the aortic wall and the aortic valve cusps revealed myxoid degeneration. There was no evidence of cystic medial necrosis.

He underwent an emergent repair of aorta with aortic root replacement, using a Dacron aortic graft and a mechanical aortic valve (25-On-X). Heparin bridging was initiated once his post surgically bleeding risk was low. Warfarin was later started with therapeutic INR goal of 2.0 to 3.0. On postoperative day 10, he was discharged on Aspirin 81 mg, warfarin and metoprolol 50 mg daily. 

At 1 month post-discharge follow-up, his distal pulse was strong and equal in all four extremities. He was asymptomatic with no complaints of chest pain, dyspnea, headache, or lightheadedness.

Literature Review

Aortic dissection occurs when the tunica intima of the aorta develops a tear that extends into the inner two-third layer of its tunica media which consists of collagen, smooth muscle and elastic fibers. The above pathological changes lead to the formation of a true lumen and a false lumen separated by an intimal flab (1-14). This causes blood to escape into the false lumen and incite a cascade of events. The external elastic lamina separates the tunica media from the adventitial layer, which serves as an external scaffolding. The tunica adventitia consists of fibroblast cells, collagen and elastic fibers. On the other hand, the tunica intima is made of endothelial cells on basement membrane; separated from the tunica media by the internal elastic lamina (2-14). As blood enters the false lumen, retrograde or anterograde propagation of blood occurs due to pressure changes. If a retrograde propagation takes place within the false lumen, it can extend into the aortic root through the sinotubular junction, eventually causing damage to the aortic root and its content or escape into the pericardial space; consequently leading to aortic insufficiency, acute coronary syndrome or cardiac tamponade (10-34). The contents of the aortic root in question include sinuses of Valsalva where the coronary sinuses and the orifices of the coronary arteries are located, or other structures such as aortic annulus, commissures, leaflets (cusps) and ventriculo-aortic junction. In the case of an anterograde propagation, the blood collection within the false lumen can extend distally from the site of initial tear to the branches of the aorta such as brachiocephalic trunk (innominate artery), left subclavian artery, renal arteries and mesenteric arteries thereby leading to stroke, limb ischemia, renal insufficiency and bowel ischemia. Involvement of the brachiocephalic trunk or the left subclavian artery can also cause pseudohypotension (35-41). In some cases, distal extension can reach the site of aortic bifurcation and recanalize into the intravascular compartment; thereby, creating a double barrel aorta. This in effect, reduces the risk of aortic rupture (10,34-44). In a clinical scenario where there is no preceding intimal tear, the most likely causes are always connective tissue diseases such as Marfan syndrome (FBN1 gene mutation), Ehlers-Danlos syndrome (vascular type-COL3A1 gene mutation), familial thoracic aortic aneurysm and dissection (TGFBR1 and TGFBR2, FBN1, MYH11, and ACTA 2 genetic mutations), and Leoys-Dietz aneurysm syndrome (TGFBR1 or TFGBR2 gene mutations) (12,14,34-44). In such cases, there is an initial formation of intramural hematoma, which may occur secondary to rupture of the aortic vasa vasorum. Disruption of the vaso vasorum can also occur due inflammatory response generated from vasculitides or infectious causes like syphilis.

Aortic dissection is relatively rare when compared to other cardiovascular diseases such as ruptured aortic aneurysm, acute coronary syndrome and abdominal aortic aneurysm. The true incidence of aortic dissection is hard to determine because most case approximations are made from autopsy reports (34,39,40-45). Although, the estimated incidence is 5 to 30 cases per million people yearly. Aortic dissections are known to occur more in males compared to females with men constituting about 65% of cases. Peak age of onset is between 50-65 years. In a population-based study of all Olmsted County, Minnesota, residents with aortic dissection between 1995 to 2015, it was noted that age- and sex-adjusted incidence of aortic dissection for men was 10.2 per 100,000 person-years versus 5.7 per 100,000 person-years for women (1,11-14,46). Aortic dissection is commonly classified based on time of presentation and structural variations. With regards to time of presentation, it can be acute (less than 2 weeks) or chronic (greater than 2 weeks) (1,46). Chronic aortic dissections tend to have better prognosis.

There are two main anatomic classifications, DeBakey (Figure 2) and Stanford (Figure 3). Most aortic dissections originate mainly from the ascending aorta with the rest emanating from the aortic arch and the descending aorta (1,46).

The DeBakey classification is divided into three main types:

• Type I- emerges from the ascending aorta, extends to the aortic arch and often involving the distal segment of the aorta. Most common in the younger population (less than 40 years). It is also the most serious form of aortic dissection.
• Type II- Emerges from the ascending aorta and is restricted to this section of the aorta.
• Type III- Emerges from the descending aorta extending distally above the diaphragm (Type IIIa) or beyond the diaphragm into the abdominal aorta (Type IIIb) (34,46).

Figure 2. Illustrations of DeBakey classification (Type I, II, and III). T Paul Tran and Ali Khoynezhad. Dove Medical Press Limited. 2009. Available at: https://www.dovepress.com/articles.php?article_id=2444 (accessed 8/7/20).

The Stanford classification is broken down into:

• Type A- Involvement of the ascending aorta irrespective of the origin of intimal tear. A composite of DeBakey Type I and II.
• Type B- Involvement of the descending aorta (distal to the origin of the left subclavian artery) and its distal component. An analogy of type III DeBakey (1,34,46).

Figure 3. Stanford classification of aortic dissection (Type A and B). T Paul Tran and Ali Khoynezhad. Dove Medical Press Limited. 2009. Available at: https://www.dovepress.com/articles.php?article_id=2444 (accessed 8/7/20).

Etiology. There are several risk factors for aortic dissection. The main predisposing risk factors most commonly reported include:

• Hypertension (Associated with about 70%-80% of cases).
• Connective tissue diseases and genetic disorders such as Marfan syndrome, Ehlers-Danlos syndrome, Familial thoracic aortic aneurysm and dissection, Leoys-Dietz aneurysm syndrome, Turner syndrome, and bicuspid aortic valve (5% likelihood of aortic dissection).
• Age greater than 40 years (75% of cases occur in patients between 40-70 years)
• Use of illicit substances such as cocaine, and ecstasy.
• Pre-existing aortic aneurysm
• Previous history of aortic dissection
• Family history of aortic dissection
• Pregnancy
• Vasculitides and autoimmune diseases such as Giant cell arteritis, Takayasu’s arteritis, polyarteritis nodosa, and Behcet’s disease.
• Iatrogenic causes such as cardiac catheterization, aortic valve replacement, coronary artery bypass graft and intra-aortic balloon pump.
• Tertiary syphilis
• Use of anabolic steroids
• Penetrating atherosclerotic ulcer secondary to infiltration of the tunica media by an atherosclerotic plaque. Meaning, risk factors for atherosclerosis such as smoking, hypercholesterolemia, and diabetes are implicated in aortic dissection.
• Penetrating chest trauma
• Chronic alcohol use
• Weight-lifting is a novel risk factor for aortic dissection even in individuals without connective tissue diseases or cardiovascular risk factors. The existence of other risk factors only makes it more likely to occur (17-20, 40-46).

Signs and Symptoms: The diagnosis of aortic dissection is greatly missed by most physicians in the emergency department upon presentation. Delay in treatment can lead to an increase in mortality to about 50% within the first 48 hours. It is highly crucial the diagnosis is made quickly and treatment is initiated promptly to decrease the risk of mortality (1,41-46). With respect to clinical presentation, patients present with following symptoms:

• Severe tearing chest pain of sudden onset. Pain may be located in the anterior chest wall, interscapular region and in the abdomen. Anterior chest wall pain is often due to involvement of the ascending aorta while interscapular back pain and abdominal pain are associated with involvement of the distal segments of the aorta due to anterograde extension of the false lumen. Note that about 10% of patients present with painless aortic dissection; which is more common in patients with connective tissue diseases such as Marfan syndrome. Some patients present with pleuritic chest pain secondary to pericardial involvement.  Overall, chest pain is the most common symptom; occurring in about 80-96% of patients, with anterior chest pain being the most reported. About 71.4% of painless aortic dissection present with a normal ECG reading. Coronary malperfusion may result in cardiac arrest (1,14,26-46).
• Sweating, nausea and vomiting (may occur due to autonomic changes)
• Headache
• Lightheadedness
• Back pain
• Abdominal pain
• Neck or jaw pain (aortic arch involvement)
• Neurologic deficits (hemiparesis, hemiplegia hemianesthesis and loss of vision) and syncope as a result of hypovolemia, arrhythmia, acute coronary syndrome, increase vagal tone or involvement of the innominate artery and its branches (such as the internal carotid artery) (1,40-46).
• Horner syndrome (Ptosis, miosis and anhidrosis) secondary to obstruction of sympathetic outflow tract.
• Hoarseness due to vagus nerve compression.
• Exertional leg and gluteal pain may occur if the iliac artery is involved.
• Paresthesia, and extremity pain may occur due to limb ischemia.
• Dyspnea
• Dysphagia
• Hemoptysis
• Anxiety and palpitations

Common signs observed in patients with aortic dissection include:

• Differential blood pressure measurements in the upper extremities
• High blood pressure (More common in Type B aortic dissection)
• Hypotension (More common in Type A aortic dissection)
• Wide pulse pressure measurement (signifying aortic valve involvement)
• Diastolic murmur (secondary to aortic insufficiency)
• Muffled heart sounds
• Weak peripheral pulses
• ECG changes indicating acute coronary syndrome
• Decreased breath sounds, dullness to percussion if pleural effusion is present. Pleural effusion may be as result of inflammatory response, aneurysm leakage or eventual rupture of the dissected aorta. 
• Horner syndrome
• Changes in mental status

Patients may experience a wide range of complications if they are not managed early. Some of which include stroke, paraplegia, life threatening arrhythmia with cardiac arrest, paraplegia, limb amputation, multiple organ failure, severe cardiac tamponade, renal failure, bowel ischemia, myocardial infarction, aortic regurgitation, superior vena cava syndrome and even death (1,22,14,46).  

Diagnostic modalities and findings.

• ECG and cardiac enzyme (troponin) level must be checked to exclude myocardial involvement. ECG findings are usually non-specific with nearly 1-2% showing ST-elevation (1,39,41-46).
• Baseline blood work such as CBC, electrolytes, Blood urea nitrogen (BUN), and creatinine level must be established. D-dimer may be used it low risk patients to exclude diagnosis. Although, due to lack of evidence to validate its use, it is not strongly recommended (40,46).
• Chest x-ray- findings on may include widened mediastinum (present in greater than 80%), calcium sign, apical cap (left); loss of paratracheal stripe; involution of mainstem bronchus; pleural effusion, tracheal and esophageal deviation. Normal x-ray findings occur in about 20% cases (1,41,46).
• Computed Tomography (CT)-chest and abdomen with iodinated contrast- fast, noninvasive and available in most emergency departments. It is used to detect the region of tear and aids surgical planning. Not recommended for patients with contrast allergy, older patients (greater than 65 years), poor renal function and history of renal insufficiency.
• Transesophageal echocardiography (TEE): It is relatively available, noninvasive and best for ascending aortic dissections to detect changes or damages structures within the aortic root. It can be done at bedside and does not require contrast media. Although, it is operator dependent and discouraged in patients with esophageal varices, masses or strictures (14,39,46).
• Magnetic resonance Imaging (MRI): It is used for detection of site of tear, assessment of dissection and involvement of branches of aorta, ascertain the presence and degree of aortic insufficiency. Iodinated contrast is not needed. It also aids surgical planning but it is time consuming, expensive, not readily available in some hospitals and not advisable for use in patients with metallic implants such as pacemakers and implantable cardioverter defibrillator.
• Doppler ultrasound: This can be useful in patients presenting with signs of limb hypoperfusion to assess for diminished blood flow on the extremities involved (22,46).

Management: Aortic dissection can be managed surgically or conservatively with medications. Type A aortic dissections often require surgical management while type B aortic dissection can be managed conservatively with medications. Medical management is necessary at presentation to help stabilize patient’s vitals. The mean arterial blood pressure goal is often between 60 to 75mm Hg (1,14,23,46). Medical management is started by administration of intravenous short and fast acting beta-blockers (esmolol, propanolol and labetalol) and morphine for pain management (14,23,46). Sodium nitroprusside is then given to the patient to enhance vasodilation and ensure adequate visceral perfusion. Patients with contraindications to beta-clockers (2^nd or 3^rd degree heart block, decompensated heart failure, severe asthma, and sinus bradycardia) should be given non-dihydropyridine calcium channel blockers (verapamil and diltiazem) as an alternative (1,14,46).

Surgical approach to management:

• Open heart (aortic) surgery-Mainly used in the absence of aortic valve defect (12,46).
• Minimally invasive endovascular aortic repair- it can be done with endovascular composite consisting of a Dacron stent graft and a transcatheter aortic valve (if aortic valve is compromised) (2,42,45,46).
• Valve sparing aortic root replacement (David procedure) (10,12,14,46).
• Bentall procedure (10,12,41,44,46).
• Sutureless vascular-ring connector with Dacron graft aortic repair.  
• Hybrid technique- a combination of stent graft and visceral bypass grafting (10,14,46).

Aortic fenestration has been reported to be used as an interim measure to prevent organ ischemia in cases of organ involvement (22,46). Aortoiliac bypass can also be used when circulation through the iliac vessels are severely compromised to avoid limb ischemia. A case report by John S. Schor, Michael D. Horowitz, et al. (29) detailed a case about a patient with type III aortic dissection (anterograde propagation) and iliac involvement complicated by a clot at the site of aortic bifurcation; which was treated with aortic fenestration and aortoiliac bypass using a knitted Dacron graft. In this case, nonthoracic approach was employed to salvage the limbs and prevent further damage (22,46). When employing surgical management, it is important to evaluate patient’s eligibility for surgery by checking for comorbid conditions and contraindications such as renal insufficiency, advanced age, ischemic cardiomyopathy, diabetes, shock, existing cardiac tamponade and bleeding diathesis.

Prognosis: Approximately 30-40% of patients with acute aortic dissection die after reaching the emergency room. The mortality rate for type A dissections treated medically is estimated to be about 20% within the first 24 hours and 50% at 30 days after initial onset (11,14,46). If surgically managed, Type A dissections incur a mortality rate of 10% after 24 hours and close to 20% at 30 days after repair. On the other hand, for Type B dissections, the 30-day mortality can be as high as 10% for uncomplicated cases. Mortality rate is 1-2% per hour for the first day in patients who do not qualify for surgery. The presence of comorbidities and complications further increases the risk of mortality (1,10,16,18,46).

Follow-up: After the initial management, patients should undergo cardiac rehabilitation, lifestyle modification (smoking cessation, weight loss and avoidance of illicit drugs) and physical therapy if movement is limited (6,46). All patients should be educated on the need for adequate blood pressure control and medication compliance. Serial imaging is recommended with CT scan or MRI at 3-6 months interval to monitor disease progression and check for the emergence of new aneurysms or recurrent dissections (14,34,46). For patients requiring valve replacement with bioprosthetic valve, antiplatelet such as aspirin should be prescribed to prevent clot formation (7,8,46). Although, anticoagulation with warfarin should be added for patients with risk factors such as atrial fibrillation, hypercoagulable state, severe left ventricular systolic dysfunction, history of thromboembolic events; and in patients with subclinical valve thrombosis and no underlying risk factors. Patients with mechanical aortic valve require both aspirin and anticoagulation with warfarin irrespective of their risk stratification (1,22,34,46). For patients requiring anticoagulation with warfarin, early bridging with intravenous unfractionated heparin or subcutaneous heparin should be initiated and target INR should be maintained at 2.0 to 3.0 for 3-6 months or indefinitely depending on the case and type of valve used. For patients with mechanical aortic valve and underlying risks for valve thrombosis, therapeutic INR can be extended to 2.5 to 3.5 (37,38,46). If any contraindication for warfarin exist, aspirin dosage can be increased. Direct oral anticoagulants (dabigatran, rivaroxaban, apixaban, and edoxaban) should be avoided in mechanical valves (37,46).

Discussion

Exercise is known to be one of the most effective means of controlling blood pressure. Although all sports have both dynamic and static components, sports requiring a high static demand, such as weight lifting are thought to be associated with a risk of triggering acute aortic dissection (20,46). It is normal for blood pressure to rise to about 200/110 mm Hg during exercise but once it surpasses that level, there is risk of negative cardiovascular outcome (22,30,40,46). Sudden change in blood pressure during weight lifting can predispose the patient to aortic dissection. They have been several cases of aortic dissection reported in weightlifters and individuals who engaged in strenuous exercises prior to their dissection event (17,19,21,22,35,46).  It is crucial to note that all types of aortic dissection have been reported to occur in these patients; and that includes type A, and type B aortic dissections (22,46). On the contrary, blood pressure is known to reduce following a short exercise session and more so in physically active individuals that are not premeditated with antihypertensive.(34,45,46) A systematic review and meta-analysis done by Elizabeth Carpio-Rivera, José Moncada-Jiménez, et al. (3) on an heterogeneous sample population, showed that there was a significant reduction in blood pressure irrespective of the participant's initial blood pressure level, gender, physical activity level, antihypertensive drug intake, type of blood pressure measurement, time of day in which the blood pressure was measured, type of exercise performed, and exercise training program with a p value of less than 0.05 for all parameters.

In this particular case report, the patient is an avid weightlifter who developed a type A aortic dissection while weightlifting at the gym. His initial presentation was a popping sensation in the chest, which later evolved into a neurologic sequence of transient bilateral visual loss, paresthesia and other symptoms such as headache, lightheadedness, diaphoresis, pressure-like sensation in his neck, chest and back. He reported no underlying cardiovascular risk factors, use of tobacco, recreational drugs or anabolic steroid use and denies any family history of connective tissue or genetic diseases. There was no report of any recent trauma or injury to the chest wall. Upon evaluation of his vitals, he was hypotensive with diminished pulse on his right upper and lower extremities and no marfanoid features were noted. Lab values were indicative of leukocytosis with acute renal injury secondary to inflammatory response and hypotension respectively. CT angiography of the chest and abdomen showed type A aortic dissection with anterograde propagation of the false lumen to the abdominal aorta. This finding was also supported by cardiac catheterization findings of swirling pattern and with delayed contrast washout. No radiologic chest x-ray findings were noted; head and neck CT scan result came back unremarkable with no ischemic changes seen in the brain. It is crucial to note that a negative chest x-ray does not necessarily exclude aortic dissection as shown in this case. TEE revealed rupture of the left coronary cusp with an ejection fraction of 40% to 45%. Histopathological findings showed no cystic medial necrosis but myxoid degeneration was noted on the aortic wall and cusps. Subsequently, the aortic valve was replaced with a mechanical aortic valve, with a Dacron graft used to replace the aortic root. Post-operatively, the patient was discharged on day 10 with antiplatelet and antihypertensive medications with complete recovery noted at one month follow up. This patient displayed a classic presentation of type A aortic dissection and due to prompt management complications such as aortic rupture, multiple organ failure, cardiac ischemia and renal failure were avoided. This is a clear evidence of type A aortic dissection in a young weightlifter with no underlying traditional risk factors.

Hatzaras I, Tranquilli M, et al. (18) state that “as an initial rule of thumb, it appears that lifting up to one half the individual's body weight is relatively safe, not exceeding a blood pressure of 200 mm Hg, even during the effort cycle of the lifting exercise.” This connotes that weight lifting is safe as long as the patient is educated not to cause too much cardiovascular stress. In Selena Pasadyn, et al. (45) 295 patients were given an online survey to elaborate more about their experience with type A aortic dissection. The eventual response rate on athletic component was 48% (141). Out of 132 patients, 18% stated their doctor did not talk to them about post recovery exercise regimen while 31% (40/129) stated their physicians were uncertain about the types of exercises they should or should not engage in (24). Out of 123 patients, 99 (81%) patients stated they wanted specific recommendations about what exercise regimens were safe. Due to paucity of data on specific exercise recommendations post-event (after an aortic dissection); it is clear that physicians find it difficult to educate their patients on the type and degree of exercise regimens their patients should participate in during their recovery phase. This ambiguity has caused increased isolation among patients post-event; substantial decrease in physical activity and has negatively affected the quality of life. This can also lead to recurrence of dissection if the patient exceeds the required exercise level after prior dissection event. Conversely, preceding the dissection event, out of 80 patients who exercised, 33 (41%) participated in strength work, such as weightlifting or resistance training, and 28.9% (22/76) did engage post-event. 35% (47/136) of patients also reported lifting heavy objects on a regular basis before their dissection, and 9.2% (11/119) did after their dissection. After a successful surgery, only one patient returned to competitive athletics (cycling). This shows that an association exists between strenuous activities such as weightlifting and aortic dissection. Engagement in physical exercise was reduced after dissection as noted.  For post-dissection patients, it may be beneficial to take a cautious approach and limit activities that require extreme or maximal exertion extensive sprinting or running, snow shoveling, and mowing the lawn with a non–self-propelled mower. Systolic blood pressure while running at 8 mph may increase by 108 to 162 mm Hg above resting levels but by 26 to 40 mm Hg during brisk walking at 3 mph. Squeezing a hand grip maximally for about 1 minute has shown to increase systolic blood pressure by 50mm Hg and diastolic by 30mm Hg.(6,34,46) With regards to weightlifting, it is important for the post-aortic dissection patients to use a low amount of weight and to stop several repetitions before exhaustion. They should minimize lifting heavy objects, with heavy being defined as objects that require a lot of effort and straining (such as a Valsalva maneuver) to lift (4,6,9,27-28,46). Research by De Souza Nery S, Gomides RS, et al. (46) has shown that blood pressure increased to about 230/165 mm Hg (from 130/80 mm Hg resting blood pressure) when a biceps curl was carried out with heavy weights for the maximum amount of repetitions possible.

Conclusion

Weight-lifting has been demonstrated to improve cardiorespiratory endurance and muscular strength. However, weight-lifting with more than half of the individual’s body weight may be associated with a risk of triggering aortic complications such as aortic dissection. With the growing number of individuals taking up weight training in this era, patient education to minimize cardiovascular stress should be paramount. Although, aortic dissection is less common in the younger population, Physicians need to prioritize it as one of the differentials in young weightlifters without underlying risk factors due to its high mortality. Patients with or without history of connective tissue or genetic disorders and with moderate to high risk for acute aortic dissection may need pre-assessment with an imaging modality such as echocardiography before they start weightlifting or participating in high intensity sports. And individuals with confirmed aortic root dilation should be strongly advised to refrain from strenuous exercises such as weightlifting. These patients may also benefit from blood pressure and heart rate monitoring during their exercise sessions. Exercise recommendations should be made by putting into consideration patient’s age, body mass index, underlying comorbidities and existing risk factors. The duration of exercise should also be modest to avoid unnecessary prolonged cardiovascular stress. For post-event patients (after dissection), it is important that these patients are educated on the type and level of exercise to engage in, and blood pressure should be maintained to avoid recurrence of aortic dissection or even rupture. Regardless of patient’s current health status, it is advisable not to exceed a blood pressure of 200mm/110Hg during peak exercise. Current guidelines and recommendations suggest that patients with prior history of aortic dissection should lift very low weights (less than 50 lbs.) and at submaximal levels; avoid exercise maneuvers that elicit excessive straining (Valsalva) and stop weightlifts several repetitions before fatigue. In addition, recent exercise guideline for the general population stipulates that engaging in aerobic exercise at moderate intensity (such as slow jogging, cycling at a mild pace, walking) at least 30 minutes most days of the week for about 150 minutes per week tend to yield good  cardiovascular outcomes with minimal risk for aortic dissection and other cardiovascular complications. Most maximum heart rate prediction equations have shown to overestimate the actual value and some have shown variations with respect to age, gender, physical status and body mass index of participants. Although, the recommended target heart rate regardless of age is 50% to 85% of maximum heart rate; for patients with Marfan syndrome, it is much safer to follow the Marfan foundation physical activity recommendations such as maintaining heart rate at less than 100 bpm for patients not on beta-blockers, and less than 110 bpm for patients on beta-blockers (at moderate intensity).These patients are also encouraged to avoid high intensity exercises such as weightlifting, steep climbing, and activities requiring rapid pressure changes like scuba diving.

References

1. David Levy; Amandeep Goyal; Yulia Grigorova; Jacqueline K. Le. Aortic Dissection. (Updated 2019 Dec 16). In: StatPearls (Internet). Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441963/
2. Columbia University Irving Medical Center. Aortic dissection. (Accessed April 7^th, 2020). Available at: https://columbiasurgery.org/conditions-and-treatments/aortic-dissection (accessed 8/7/20).
3. Carpio-Rivera E, Moncada-Jiménez J, Salazar-Rojas W, Solera-Herrera A. Acute Effects of Exercise on Blood Pressure: A Meta-Analytic Investigation. Arq Bras Cardiol. 2016;106(5):422-433. [CrossRef] [PubMed]
4. Sarzynski MA, Rankinen T, Earnest CP, et al. Measured maximal heart rates compared to commonly used age-based prediction equations in the Heritage Family Study. Am J Hum Biol. 2013;25(5):695-701. [CrossRef] [PubMed]
5. BMJ best practices. Aortic dissection. Available at: https://bestpractice.bmj.com/topics/en-us/445 (accessed 8/7/20).
6. Chaddha A, Kline-Rogers E, Woznicki EM, et al. Cardiology patient page. Activity recommendations for postaortic dissection patients. Circulation. 2014;130(16):e140-e142. [CrossRef] [PubMed]
7. American College of Cardiology. Anticoagulation Strategies After Bioprosthetic Valve Replacement: What Should We Do? Available at:  https://www.acc.org/latest-in-cardiology/articles/2016/12/19/08/44/anticoagulation-strategies-after-bioprosthetic-valve-replacement (accessed 8/7/20).
8. Türk UO, Alioğlu E, Nalbantgil S, Nart D. Catastrophic cardiovascular consequences of weight lifting in a family with Marfan syndrome. Turk Kardiyol Dern Ars. 2008;36(1):32-34. [PubMed]
9. American Heart Association. Target Heart Rates. Available at:  https://atgprod.heart.org/HEARTORG/HealthyLiving/PhysicalActivity/GettingActive/Target-Heart-Rates_UCM_434341_Article.jsp (accessed 8/7/20).
10. El-Hamamsy I, Ouzounian M, Demers P, et al. State-of-the-Art Surgical Management of Acute Type A Aortic Dissection. Can J Cardiol. 2016;32(1):100-109. [CrossRef] [PubMed]
11. DeMartino RR, Sen I, Huang Y, et al. Population-Based Assessment of the Incidence of Aortic Dissection, Intramural Hematoma, and Penetrating Ulcer, and Its Associated Mortality From 1995 to 2015. Circ Cardiovasc Qual Outcomes. 2018;11(8):e004689. [CrossRef] [PubMed]
12. Cirino AL, Harris S, Lakdawala NK, et al. Role of Genetic Testing in Inherited Cardiovascular Disease: A Review. JAMA Cardiol. 2017;2(10):1153-1160. [CrossRef] [PubMed]
13. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2017;70(2):252-289. [CrossRef] [PubMed]
14. Science Direct. Aortic Dissection. (Accessed April 6th, 2020). Available at: https://www.sciencedirect.com/topics/medicine-and-dentistry/aortic-dissection (accessed 8/7/20).
15. Wasfy MM, Hutter AM, Weiner RB. Sudden Cardiac Death in Athletes. Methodist Debakey Cardiovasc J. 2016;12(2):76-80. [CrossRef] [PubMed]
16. Fukui T. Management of acute aortic dissection and thoracic aortic rupture. J Intensive Care. 2018;6:15. Published 2018 Mar 1. [CrossRef] [PubMed]
17. Gwan-Nulla DN, Davidson WR Jr, Grenko RT, Damiano RJ Jr. Aortic dissection in a weight lifter with nodular fasciitis of the aorta. Ann Thorac Surg. 2000;69(6):1931-1932. [CrossRef] [PubMed]
18. Hatzaras I, Tranquilli M, Coady M, Barrett PM, Bible J, Elefteriades JA. Weight lifting and aortic dissection: more evidence for a connection. Cardiology. 2007;107(2):103-106. [CrossRef] [PubMed]
19. Hogan CJ. An aortic dissection in a young weightlifter with non-Marfan fibrillinopathy. Emerg Med J. 2005;22(4):304-305. [CrossRef] [PubMed]
20. Itagaki R, Kimura N, Itoh S, Yamaguchi A, Adachi H. Acute type a aortic dissection associated with a sporting activity. Surg Today. 2017;47(9):1163-1171. [CrossRef] [PubMed]
21. Ragucci MV, Thistle HG. Weight lifting and type II aortic dissection. A case report. J Sports Med Phys Fitness. 2004;44(4):424-427. [PubMed]
22. de Virgilio C, Nelson RJ, Milliken J, et al. Ascending aortic dissection in weight lifters with cystic medial degeneration. Ann Thorac Surg. 1990;49(4):638-642.[CrossRef] [PubMed]
23. White A, Broder J, Mando-Vandrick J, Wendell J, Crowe J. Acute aortic emergencies--part 2: aortic dissections. Adv Emerg Nurs J. 2013;35(1):28-52. [CrossRef] [PubMed]
24. Pacini D, Di Marco L, Fortuna D, et al. Acute aortic dissection: epidemiology and outcomes. Int J Cardiol. 2013;167(6):2806-2812. [CrossRef] [PubMed]
25. Kim HJ, Lee HK, Cho B. A case of acute aortic dissection presenting with chest pain relieved by sublingual nitroglycerin. Korean J Fam Med. 2013;34(6):429-433. [CrossRef] [PubMed]
26. Chen CH, Liu KT. A case report of painless type A aortic dissection with intermittent convulsive syncope as initial presentation. Medicine (Baltimore). 2017;96(17):e6762. [CrossRef] [PubMed]
27. Nikolaidis PT, Rosemann T, Knechtle B. Age-Predicted Maximal Heart Rate in Recreational Marathon Runners: A Cross-Sectional Study on Fox's and Tanaka's Equations. Front Physiol. 2018;9:226. Published 2018 Mar 15. [CrossRef] [PubMed]
28. Heinzmann-Filho JP, Zanatta LB, Vendrusculo FM, et al. Maximum heart rate measured versus estimated by different equations during the cardiopulmonary exercise test in obese adolescents. Frequência cardíaca máxima medida versus estimada por diferentes equações durante o teste de exercício cardiopulmonar em adolescentes obesos. Rev Paul Pediatr. 2018;36(3):309-314. [CrossRef] [PubMed]
29. Schor JS, Horowitz MD, Livingstone AS. Recreational weight lifting and aortic dissection: case report. J Vasc Surg. 1993;17(4):774-776. [CrossRef] [PubMed]
30. Morentin Campillo B, Molina Aguilar P, Monzó Blasco A, et al. Sudden Death Due to Thoracic Aortic Dissection in Young People: A Multicenter Forensic Study. Rev Esp Cardiol (Engl Ed). 2019;72(7):553-561.[CrossRef] [PubMed]
31. Erbel R, Eggebrecht H. Aortic dimensions and the risk of dissection. Heart. 2006;92(1):137-142. [CrossRef] [PubMed]
32. Chaddha A, Eagle KA, Braverman AC, et al. Exercise and Physical Activity for the Post-Aortic Dissection Patient: The Clinician's Conundrum. Clin Cardiol. 2015;38(11):647-651. [CrossRef] [PubMed]
33. Hagan PG, Nienaber CA, Isselbacher EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283(7):897-903. [CrossRef] [PubMed]
34. Braverman AC. Acute aortic dissection: clinician update. Circulation. 2010;122(2):184-188. [CrossRef] [PubMed]
35. Singh B, Treece JM, Murtaza G, Bhatheja S, Lavine SJ, Paul TK. Aortic Dissection in a Healthy Male Athlete: A Unique Case with Comprehensive Literature Review. Case Rep Cardiol. 2016;2016:6460386. [CrossRef] [PubMed]
36. Jacobs JE, Latson LA Jr., Abbara S, et al. Acute chest pain — suspected aortic dissection. American College of Radiology. 1995. Available at: https://acsearch.acr.org/docs/69402/Narrative/ (accessed 8/7/20).
37. William H Gaasch, Barbara A Konkle. Antithrombotic therapy for surgical prosthetic heart valves and surgical valve repair: Indications. UpToDate. Available at: https://www.uptodate.com/contents/antithrombotic-therapy-for-surgical-prosthetic-heart-valves-and-surgical-valve-repair-indications (accessed 8/7/20).
38. von Kodolitsch Y, Wilson O, Schüler H, et al. Warfarin anticoagulation in acute type A aortic dissection survivors (WATAS). Cardiovasc Diagn Ther. 2017;7(6):559-571. [CrossRef] [PubMed]
39. Spittell PC, Spittell JA Jr, Joyce JW, et al. Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). Mayo Clin Proc. 1993;68(7):642-651. [CrossRef] [PubMed]
40. Hagiwara A, Shimbo T, Kimira A, Sasaki R, Kobayashi K, Sato T. Using fibrin degradation products level to facilitate diagnostic evaluation of potential acute aortic dissection. J Thromb Thrombolysis. 2013;35(1):15-22. [CrossRef] [PubMed]
41. Patel PD, Arora RR. Pathophysiology, diagnosis, and management of aortic dissection. Ther Adv Cardiovasc Dis. 2008;2(6):439-468. [CrossRef] [PubMed]
42. Spanos K, Tsilimparis N, Kölbel T. Exercise after Aortic Dissection: to Run or Not to Run. Eur J Vasc Endovasc Surg. 2018;55(6):755-756. [CrossRef] [PubMed]
43. Shu C, Wang T, Li QM, et al. Thoracic endovascular aortic repair for retrograde type A aortic dissection with an entry tear in the descending aorta. J Vasc Interv Radiol. 2012;23(4):453-460.e1. [CrossRef] [PubMed]
44. Hughes GC. Management of acute type B aortic dissection; ADSORB trial. J Thorac Cardiovasc Surg. 2015;149(2 Suppl):S158-S162. [CrossRef] [PubMed]
45. Pasadyn S, Roselli E, Blackstone E, Phelan D. Abstract 11126: Acute type a aortic dissections: disruption of lifestyle in competitive athletes. Circulation. 2018;138:A11126. [Abstract]. Available at: https://www.ahajournals.org/doi/10.1161/circ.138.suppl_1.11126 (accessed 8/7/20).
46. de Souza Nery S, Gomides RS, da Silva GV, de Moraes Forjaz CL, Mion D Jr, Tinucci T. Intra-arterial blood pressure response in hypertensive subjects during low- and high-intensity resistance exercise. Clinics (Sao Paulo). 2010;65(3):271-277. [CrossRef] [PubMed]

Cite as: Pak SC, Asuka E. Acute type A aortic dissection in a young weightlifter: a case study with an in-depth literature review. Southwest J Pulm Crit Care. 2020;21(2):39-53. doi: https://doi.org/10.13175/swjpcc025-20 PDF 

April 2020 Critical Care Case of the Month: Another Emerging Cause for Infiltrative Lung Abnormalities

Henry W. Luedy, MD¹

Sandra L. Till, DO²

Robert A. Raschke, MD¹

¹HonorHealth Scottsdale Osborn Medical Center

²Banner University Medical Center-Phoenix

Phoenix, AZ USA

Editor’s Note: the following case presentation represents a compilation of several patients.

History of Present Illness

The patient is a 27-year-old man who presented to the Emergency Department in late February 2020 with fever, cough, and green sputum production. He was recently in Hawaii where he meant his Asian girlfriend and was “partying hard”. He was intoxicated and had recent nausea and vomiting.

PMH, SH and FH

No significant PMH or FH. He does admit to smoking, marijuana use, THC use, and vaping. 

Physical Examination

• Vital Signs: BP 111/54 (BP Location: Right arm)  | Pulse 74  | Temp 98.7 °F (37.1 °C) (Oral)  | Resp 18  | Ht 5' 11" (1.803 m)  | Wt 72.6 kg (160 lb)  | SpO2 99%  | BMI 22.32 kg/m²
• General:  Awake, alert, interactive, no acute distress
• HEENT:  Anicteric, moist mucosa, trachea midline
• CV:  RRR
• Lungs: bilateral lower lobe rhonchi, no wheezing, symmetric expansion
• Abdomen: Soft, non-tender, non-distended, positive bowel sounds
• Extremities: no Lower extremity edema, no clubbing, no cyanosis
• Neuro:  No focal deficits, moves all extremities.
• Psych:  Appropriate

Which of the following are appropriate at this time? (Click on the correct answer to be directed to the second of six pages.)

1. CBC
2. Chest X-ray
3. Electrolytes
4. 1 and 3
5. All of the above

Cite as: Luedy HW, Till SL, Raschke RA. April 2020 critical care case of the month: another emerging cause for infiltrative lung abnormalities. Southwest J Pulm Crit Care. 2020;20(4):119-23. doi: https://doi.org/10.13175/swjpcc018-20 PDF 

January 2020 Critical Care Case of the Month: A Code Post Lung Needle Biopsy

Sarika Savajiyani MD and Clement U. Singarajah MBBS

 Phoenix VA Medical Center

Phoenix, AZ USA

A 67-year-old man with a history of stage IIA rectal adenocarcinoma post neoadjuvant chemoradiation presented with a near code event after elective CT guided biopsy of an enlarging left lower lobe lung nodule. The patient became bradycardic and profoundly hypotensive immediately after the CT guided biopsy with the following vital signs: Systolic BP < 90 mmHg, HR 40/min sinus bradycardia, SpO₂ on 100% oxygen non rebreather was 90%. Telemetry and EKG showed ST elevation in the anterior leads. He complained of vague arm and leg weakness and tingling, but did not lose consciousness or suffer a cardiac arrest. 

A CT scan was performed about 2-3 minutes after the patient deteriorated (Figure 1).

Figure 1. A-E: Representative images from CT scan in soft tissue windows. Lower: Video of CT scan in soft tissue windows.

What radiographic finding likely explains the patient’s clinical deterioration?  (Click on the correct answer to be directed to the second of six pages)

1. Arterial air embolism
2. Pneumothorax
3. Pulmonary edema
4. Pulmonary Embolism
5. Venous air embolism

Cite as: Savajiyani S, Singarajah CU. January 2020 critical care case of the month: a code post lung needle biopsy. Southwest J Pulm Crit Care. 2020;20(1):1-6. doi: https://doi.org/10.13175/swjpcc042-19 PDF

Ultrasound for Critical Care Physicians: Characteristic Findings in A Complicated Effusion

Emilio Perez Power MD, Madhav Chopra MD, Sooraj Kumar MD, Tammy Ojo MD, and James Knepler MD

Division of Pulmonary, Allergy, Critical Care and Sleep

University of Arizona College of Medicine

Tucson, AZ USA

Case Presentation

A 60-year-old man with right sided invasive Stage IIB squamous lung carcinoma, presented with a one week history of progressively worsening shortness of breath, fever, and chills. On admission, the patient was hemodynamically stable on 5L nasal cannula with an oxygen saturation at 90%. Physical exam was significant for a cachectic male in moderate respiratory distress using accessory muscles but able to speak in full sentences. His pulmonary exam was significant for severely reduced breath sound on the right along with dullness to percussion. His initial laboratory finding showed a mildly elevated WBC count 15.3 K/mm3, which was neutrophil predominant and initial chest x-ray with complete opacification of the right hemithorax. An ultrasound of the right chest was performed (Figure 1).

Figure 1. Ultrasound of the right chest, mid axillary line, coronal view.

Based on the ultrasound image shown what is the likely cause of the patient’s opacified right hemithorax?

1. Consolidation
2. Exudative pleural effusion
3. Pneumothorax
4. Transudative pleural effusion

Cite as: Power EP, Chopra M, Kumar S, Ojo T, Knepler J. Ultrasound for critical care physicians: characteristic findings in a complicated effusion. Southwest J Pulm Crit Care. 2018;17(6):150-2. doi: https://doi.org/10.13175/swjpcc122-18 PDF

Ultrasound for Critical Care Physicians: Who Stole My Patient’s Trachea?

Monika Kakol MD, Connor Trymbulak MSc, and Rodrigo Vazquez Guillamet MD

Department of Internal Medicine Department

University of New Mexico School of Medicine

Albuquerque, NM USA

A 73-year-old man with a past medical history of asthma-chronic obstructive pulmonary disease overlap syndrome and coronary artery disease presented to the emergency department with acute on chronic respiratory failure. The patient failed to respond to initial bronchodilator treatment and non-invasive positive pressure ventilation. A decision was made to proceed with endotracheal intubation and mechanical ventilation. Upper airway ultrasonography was used to confirm positioning of the endotracheal tube and the following images were obtained:

Figure 1. Longitudinal view of the trachea.

Figure 2. Transverse view of the trachea at the level of the tracheal rings.

What does the ultrasound depict (see Figures 1 & 2)? (Click on the correct answer for an explanation)

1. Endotracheal intubation
2. Esophageal intubation
3. Calcified tracheal rings
4. Thyroid

Cite as: Kakol M, Trymbulak C, Guillamet RV. Ultrasound for critical care physicians: Who stole my patient’s trachea? Southwest J Pulm Crit Care. 2018;17(2):72-5. doi: https://doi.org/10.13175/swjpcc102-18 PDF

April 2018 Critical Care Case of the Month

Clement U. Singarajah, MD

Phoenix VA Medical Center

Phoenix, AZ USA

History of Present Illness

A 70-year-old man was admitted for shortness of breath (SOB) secondary to a “COPD exacerbation/ILD”. A pulmonary consult was placed for possible interstitial lung disease (ILD). A thoracic CT scan for pulmonary embolism showed no embolism and no obvious ILD. He was treated for a COPD exacerbation with the usual therapy of antibiotics, steroids, nebulized bronchodilators and oxygen. He started to improve.

A few days later as he was preparing for discharge, the patient suddenly decompensated becoming more SOB (once more proving that this a dangerous time for patients in hospital). There were reports that this began after he choked and perhaps aspirated on some food and drink. His blood pressure remained stable, but he became tachycardic to 130 beats/min, hypoxic on 100% non-rebreathing mask with saturations of 92%. Obvious clinical acute respiratory failure was present. The patient was started on non-invasive ventilation but continued to deteriorate.  He was deemed too unstable to obtain a CT scan. EKG showed sinus tachycardia. The patient was transferred to the ICU for respiratory failure. A chest x-ray was obtained (Figure 1).

Figure 1. Panel A: Admission chest x-ray which was interpreted as not different from the patient’s previous chest x-ray. Panel B: Portable chest x-ray taken shortly after initiation of non-invasive ventilation just after arrival in the intensive care unit.

The portable chest x-ray taken in the ICU shows a new right-sided consolidation and which of the following? (Click on the correct answer to proceed to the second of six pages)

1. Left pleural effusion and a loculated pneumothorax with tension physiology
2. Left pneumothorax with tension physiology
3. Left pneumothorax without tension physiology
4. Left sided tension hydropneumothorax
5. None of the above

Cite as: Singarajah CU. April 2018 critical care case of the month. Southwest J Pulm Crit Care. 2018;16(4):183-91. doi: https://doi.org/10.13175/swjpcc042-18 PDF

March 2018 Critical Care Case of the Month

Babitha Bijin MD

Jonathan Callaway MD

Janet Campion MD

University of Arizona

Department of Medicine

Tucson, AZ USA

Chief Complaints

• Shortness of breath
• Worsening bilateral LE edema

History of Present Illness

A 53-year-old man with history of multiple myeloma and congestive heart failure presented to the emergency department with complaints of worsening shortness of breath and bilateral lower extremity edema for last 24 hours. In the last week, he has had dyspnea at rest as well as a productive cough with yellow sputum. He describes generalized malaise, loss of appetite, possible fever and notes new bilateral pitting edema below his knees. Per patient, he had flu-like symptoms one week ago and was treated empirically with oseltamivir.

Past Medical History

• Multiple myeloma-IgG kappa with calvarial and humeral metastases, ongoing treatment with cyclophosphamide, bortezomib and dexamethasone
• Community acquired pneumonia 2016, treated with oral antibiotics
• Heart failure with echo 10/2017 showing moderate concentric left ventricular hypertrophy, left ventricular ejection fraction 63%, borderline left atrial and right atrial dilatation, diastolic dysfunction, right ventricular systolic pressure estimated 25 mm Hg
• Hyperlipidemia
• Chronic kidney disease, stage III

Home Medications: Aspirin 81mg daily, atorvastatin 80mg daily, furosemide 10mg daily, calcium / Vitamin D supplement daily, oxycodone 5mg PRN, chemotherapy as above

Allergies: No known drug allergies

Social History:

• Construction worker, not currently working due to recent myeloma diagnosis
• Smoked one pack per day since age 16, recently quit with 30 pack-year history
• Drinks beer socially on weekends
• Married with 3 children

Family History: Mother with hypertension, uncle with multiple myeloma, daughter with rheumatoid arthritis

Review of Systems: Negative except per HPI

Physical Exam

• Vitals: T 39.3º C, BP 80/52, P121, R16, SpO2 93% on 2L
• General: Alert man, mildly dyspneic with speech
• Mouth: Nonicteric, moist oral mucosa, no oral erythema or exudates
• Neck: No cervical neck LAD but JVP to angle of jaw at 45 degrees
• Lungs: Bibasilar crackles with right basilar rhonchi, no wheezing
• Heart: Regular S1 and S2, tachycardic, no appreciable murmur or right ventricular heave
• Abdomen: Soft, normal active bowel sounds, no tendernesses, no hepatosplenomegaly
• Ext: Pitting edema to knees bilaterally, no cyanosis or clubbing, normal muscle bulk
• Neurologic: No focal abnormalities on neurologic exam

Laboratory Evaluation

• Complete blood count: WBC 15.9 (92% neutrophils), Hgb/Hct 8.8/27.1, Platelets 227
• Electrolytes: Na⁺ 129, K⁺ 4.0, Cl^- 100, CO2 18, blood urea nitrogen 42, creatinine 1.99 (baseline Cr 1.55)
• Liver: AST 35, ALT 46, total bilirubin1.7, alkaline phosphatase 237, total protein 7.4, albumin 2.
• Others: troponin 0.64, brain naturetic peptide 4569, venous lactate 2.6

Chest X-ray

Figure 1. Admission chest x-ray.

Thoracic CT (2 views)

Figure 2. Representative images from the thoracic CT scan in lung windows.

What is most likely etiology of CXR and thoracic CT findings? (Click on the correct answer to proceed to the second of seven pages)

1. Coccidioidomycosis pneumonia
2. Pulmonary edema
3. Pulmonary embolism with infarcts
4. Staphylococcus aureus pneumonia
5. Streptococcus pneumoniae infection 

Cite as: Bijin B, Callaway J, Campion J. March 2018 critical care case of the month. Southwest J Pulm Crit Care. 2018;16(3):117-25. doi: https://doi.org/10.13175/swjpcc035-18 PDF 

February 2018 Critical Care Case of the Month

Robert A. Raschke, MD

University of Arizona College of Medicine-Phoenix

Phoenix, AZ

History of Present Illness

A 25-year-old was admitted to an outside hospital with an acute episode of nausea and vomiting and chronic progressive weakness.  He smoked 2 cigarettes per day and drank a 12-pack of beer per month.  He had a history of undefined chronic liver disease.

Physician Examination

Physical examination was reported as showing a chronically ill appearing man who was “weak” using crutches to ambulate.

The patient was made NPO and was rehydrated with intravenous normal saline.

Which of the following are indicated at this time? (Click on the correct answer to proceed to the second of four pages)

1. Creatinine phosphokinase (CPK)
2. Serum potassium
3. Thyroid studies
4. 1 and 3
5. All of the above

Cite as: Raschke RA. February 2018 critical care case of the month. Southwest J Pulm Crit Care. 2018;16(2):62-6. doi: https://doi.org/10.13175/swjpcc009-18 PDF

December 2017 Critical Care Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale AZ USA

Clinical History: A 57-year-old man with no known previous medical history was brought to the emergency room via ambulance and admitted to the intensive care unit with a compliant of severe chest pain in the substernal region and epigastrium. The patient was awake and alert and did not complain of shortness of breath.

Physical examination was largely unremarkable and the patient’s oxygen saturation was 98% on room air. The patient’s vital signs revealed tachycardia (105 bpm) and his blood pressure was 108 mmHg / 60 mmHg.

Laboratory evaluation showed a slightly elevated white blood cell count (13 x 10⁹ cells/L), but his hemoglobin and hematocrit values were with within normal limits, as was his platelet count. 

Which of the following diagnoses are appropriate considerations for this patient’s condition? (Click on the correct answer to proceed to the second of nine pages)

1. Acute pericarditis
2. Aortic dissection
3. Community-acquired pneumonia
4. Myocardial infarction
5. All of the above

Cite as: Gotway MB. December 2017 critical care case of the month. Southwest J Pulm Crit Care. 2017;15(6):241-52. doi: https://doi.org/10.13175/swjpcc145-17 PDF 

November 2017 Critical Care Case of the Month

Stephanie Fountain, MD

Pulmonary and Critical Care Medicine

Banner University Medical Center Phoenix

Phoenix, AZ USA

History of Present Illness

A 56-year-old man presented with “food stuck in throat” since eating steak 18 hours prior to presentation. He is unable to eat or drink and has a sore throat. He is able to speak but has a “hoarse voice.” He denied drooling.

Past Medical History, Family History, and Social History

• He described himself as “healthy” and had not sought medical care in years.
• Former smoker but quit 2 years ago.
• He uses alcohol daily.
• He denied illicit drug use.

Physical Exam

• Afebrile, blood pressure 137/74 mm HG, heart rate 74 beats/min, SpO2 98% on room air.
• Physical exam was normal

Which of the following should be done next? (Click on the correct answer to proceed to the second of six pages)

1. Esophagogastroduodenoscopy (EGD)
2. Papain (Adolph’s Meat Tenderizer®) administration
3. Tracheostomy
4. 1 and 3
5. All of the above

Cite as: Fountain S. November 2017 critical care case of the month. Southwest J Pulm Crit Care. 2017;15(5):191-8. doi: https://doi.org/10.13175/swjpcc130-17 PDF

Tumor Lysis Syndrome from a Solitary Nonseminomatous Germ Cell Tumor

Brandon T. Nokes, MD¹

Rodrigo Cartin-Ceba, MD²

Joseph Farmer, MD²

Alyssa B. Chapital, MD, PhD²

¹Hospital Internal Medicine and ²Division of Critical Care

Mayo Clinic Arizona

Phoenix, AZ USA

Abstract

Spontaneous tumor lysis syndrome is a rare clinical entity, which typically occurs in the context of rapidly proliferating hematologic malignancies. Tumor lysis syndrome in solid organ malignancies is even rarer, and typically provoked by cytotoxic treatment regimens. We describe a case of spontaneous tumor lysis of a solitary metastatic brain lesion from a nonseminomatous germ cell tumor. This case is unique in that spontaneous tumor lysis from a brain metastasis of a solid organ malignancy has never been reported, and spontaneous tumor lysis in a nonseminomatous germ cell tumor is exceedingly rare.

Case Report

A 31-year-old gentleman was admitted to our facility after developing status epilepticus and consequently, being involved in a MVA. Imaging revealed a 3.5cm right frontal brain lesion with surrounding edema, but no other acute intracranial pathology. The patient was intubated, sedated, and transferred to critical care for further treatment. His past medical history was notable for primary surgical resection of a T1N0M0 nonseminomatous germ cell tumor in March 2015, followed by detection of a 2.5cm lung nodule in September 2015, with concurrent beta-human chorionic gonadotropin (HCG) and alpha-fetoprotein (AFP) biochemical recurrence. He underwent 4 cycles of bleomycin, etoposide, and cisplatin (BEP).

A head CT revealed a 4cm x 3.5cm right frontal lesion with surrounding edema (Figure 1).

Figure 1. T2 Axial MRI showing 4 cm x 3.5 cm lesion with associated vasogenic edema.

Dexamethasone 4mg every 6 hours was initiated for treatment of vasogenic edema. Laboratory studies were significant for a white blood cell count elevated at 19.3 x10⁹/L, international normalized ratio (INR) 1.34, partial thromboplastin time (PTT) 26.2 seconds, and prothrombin time (PT) 16.1 seconds. Plasma lactate was elevated at 30.6mmol/L. Bicarbonate was 6mmol/L with an anion gap of 45, glucose 186mg/dL, BUN 15.2mg/dL, and creatinine was 2.0mg/dL. Urine drug screen was negative. His AFP was 7.4ng/mL and beta-HCG was 13IU/L. Over the following 24 hours, the patient experienced decreased urine output. A bedside ultrasound reveals normal IVC collapse. Further lab assessment revealed a CK within normal limits and a urinalysis showed the presence of 11 to 20 RBCs, 4 to 10 WBCs and some granular casts as well as trace protein. His phosphorus was 8.9, calcium 8.1, and uric acid was 13mg/dL. His lactate dehydrogenase levels were also elevated at 271 U/L.

Due to concern of tumor lysis syndrome, the patient was initiated on rasburicase, which was followed by maintenance allopurinol 300mg daily. However, due to worsening renal failure, the patient was started on hemodialysis. He was taken to the operating room the following morning for immediate surgical resection of his brain metastasis; no evidence of residual disease was seen on follow-up imaging (Figure 2).

Figure 2. T2 Axial MRI status post a right frontal craniotomy and gross total resection of the previously noted mass. Small amount of blood noted within the resection cavity. Residual vasogenic edema persists in the white matter surrounding the operative bed.

Repeat chest, abdomen and pelvis imaging did not show any additional metastatic lesions.

In the following days, he was subsequently extubated, transferred to the floor, and continued hemodialysis, eventually fully recovering his renal function. Ultimately, he was discharged with outpatient follow-up for additional chemotherapy planning after physical rehabilitation.

Discussion

Tumor lysis syndrome (TLS) can be subdivided into laboratory TLS and clinical TLS, as defined by the Cairo-Bishop diagnostic criteria (1). Spontaneous TLS can occur in solid organ malignancies (1). TLS in solid organ malignancies is provoked by chemotherapy or radiation therapy, which creates massive cell lysis and elaboration of intracellular potassium, phosphate, and uric acid as well as hypocalcemia, which can lead to renal failure and cardiac dysrhythmias (1). LDH is also elevated. TLS can also be thought of as being provoked, either by ongoing chemotherapy or a decrease in effective circulating volume, or unprovoked. It is rare for TLS to occur in nonseminomatous germ cell tumors. Only 2 case reports have been published regarding spontaneous TLS in nonseminomatous germ cell tumors (2,3). Our case is most likely a spontaneous TLS. To date, no reports have been published regarding spontaneous TLS from a solitary brain metastasis from a nonseminomatous germ cell tumor. Further, no cases have been reported regarding tumor lysis from a solitary brain metastasis of any solid organ malignancy.

The occurrence of TLS in solid organ malignancies is thought to occur secondary to rapid cellular proliferation that exceeds the available blood supply for a tumor, leading to tumor ischemia and diffuse tumor cell necrosis. The biochemical milieu elaborated from these necrotic cells can result in end-organ pathology.

The treatment of TLS is contingent upon the rate of cancer progression and whether there is evidence of end-organ damage. Importantly and ideally, patients can be stratified into intermediate, moderate, or high-risk of developing TLS based on their malignancy type and rate of cancer progression, such that TLS may be prevented with prophylactic hydration, electrolyte monitoring and allopurinol or rasburicase (4,5). Biochemical TLS alone can be treated with IV hydration and allopurinol, a xanthine oxidase inhibitor which potentially halts TLS progression. When there is end-organ damage, rasburicase (a recombinant urate oxidase) is the first-line treatment along with aggressive hydration (5). Additional therapies are directed towards minimizing sequelae of TLS (i.e. calcium gluconate for hyperkalemia associated EKG changes or emergent dialysis for acute renal failure). There is no role for urinary alkalinization.

We were fortunate in that our patient had a great outcome, owing to early detection and aggressive intervention, and we implore our fellow physicians to be mindful of TLS as a possible clinical outcome in all malignancies, irrespective of its clinical rarity.

References

1. Mirrakhimov AE, Ali AM, Khan M, Barbaryan A. Tumor lysis syndrome in solid tumors: an up to date review of the literature. Rare Tumors. 2014;6(2):5389. [CrossRef] [PubMed]
2. D'Alessandro V, Greco A, Clemente C, et al. Severe spontaneous acute tumor lysis syndrome and hypoglycemia in patient with germ cell tumor. Tumori. 2010;96(6):1040-3. [PubMed]
3. Pentheroudakis G, O'Neill VJ, Vasey P, Kaye SB. Spontaneous acute tumour lysis syndrome in patients with metastatic germ cell tumours. Report of two cases. Support Care Cancer. 2001;9(7):554-7. [CrossRef] [PubMed]
4. Feres GA, Salluh JI, Ferreira CG, Soares M. Severe acute tumor lysis syndrome in patients with germ-cell tumors. Indian J Urol. 2008;24(4):555-7. [CrossRef] [PubMed]
5. Coiffier B, Altman A, Pui CH, Younes A, Cairo MS. Guidelines for the management of pediatric and adult tumor lysis syndrome: an evidence-based review. J Clin Oncol. 2008;26(16): 2767-78. [CrossRef] [PubMed]

Cite as: Nokes BT, Cartin-Ceba R, Farmer J, Chapital AB. Tumor lysis syndrome from a solitary nonseminomatous germ cell tumor. Southwest J Pulm Crit Care. 2017;15(4):148-50. doi: https://doi.org/10.13175/swjpcc107-17 PDF

Carotid Cavernous Fistula: A Case Study and Review

Iaswarya Ganapathiraju, OMS-IV¹

Douglas T Summerfield, MD²

Melissa M Summerfield, MD²

¹Des Moines University College of Osteopathic Medicine

Des Moines, IA USA

²Mercy Medical Center North Iowa and North Iowa Eye Clinic

Mason City, IA USA

Abstract

Carotid cavernous fistulas are rare complications of craniofacial trauma, resulting in abnormal connections between the arterial and venous systems of the cranium. The diagnosis of carotid cavernous fistulas and other injuries as a result of trauma can be confounded by the traumatized patient’s inability to communicate their symptoms to their physician. The following case study demonstrates the importance of a thorough physical exam in caring for such patients and serves to remind physicians to have a low threshold for consultation when managing numerous injuries following trauma.

Introduction

Carotid cavernous fistulas (CCFs) are aberrant connections between the carotid arterial system and the cavernous sinus, which form as complications of craniofacial trauma, or are congenital or spontaneous in nature (1). They occur in up to 3.8% of patients with basilar skull fractures and are more common with middle fossa fracture (2). Prompt diagnosis and treatment of CCF is necessary as approximately 20 – 30% of carotid cavernous fistulas lead to vision loss if not addressed appropriately (3)/\The following is a case study of a patient who presented with multiple traumatic injuries including CCF with subsequent discussion of the typical presentation, diagnosis, and treatment of direct CCF.  

Case Presentation

A 64-year-old woman with a therapeutic INR on Coumadin for atrial fibrillation sustained a fall down a flight of stairs. She was found unresponsive the next day by her relatives and was subsequently brought to the emergency department for evaluation. A maxillofacial CT showed a nondisplaced right maxillary wall fracture and nondisplaced zygomatic arch fracture, as well as a subtle inferotemporal orbital fracture, none of which was determined to require immediate treatment by the otolaryngology service. Further imaging included a CT of the head which revealed a large subdural hematoma, a superotemporal hematoma, and subfalcine herniation. She was taken to the OR for emergent craniotomy and evacuation of the hematoma before transfer to the critical care unit. In the CCU, she remained intubated and sedated but her condition improved until extubation on hospital day 3. She continued to have swelling surrounding both eyes during this time, but physical exam showed pupils which were equal, round, and reactive to light.

On day 6 of her stay, the patient was noted to have waxing and waning confusion and slightly increased oxygen requirement. Thus, she was re-intubated and sedated for “agitation” and “hypoxic respiratory failure.” Physical exam on the next day was notable for pupillary anisocoria with the right pupil at 1 mm diameter and left at 2.5 mm. There was a poor pupillary light reaction bilaterally. Neurology was consulted and recommended repeat imaging and EEG. Repeat CT and MRI of the brain showed no evidence of herniation, and EEG was negative for seizure-like activity. The anisocoria was thought to be from mass effect of the temporal lobe on cranial nerve III. The patient’s condition continued to deteriorate; physical exam elicited grimace to painful stimuli and the patient was able to open her eyes but did not track movement or follow commands. She was subsequently noted to have a left orbit that became harder to compress with ballottement test compared to the right, so Ophthalmology was consulted.

An ophthalmologic exam showed extensive chemosis of the left eye compared to the right with conjunctival hemorrhage in bilateral eyes (Figure 1).

Figure 1. Ophthalmologic exam revealed chemosis, exophthalmos, and a mid-dilated, fixed pupil of left eye compared to right.

Ocular tonometry revealed a pressure of 14 mmHg in the right eye and 53 mmHg in the left. There was a mid-dilated, fixed pupil on the left. The differential at this point included traumatic acute angle closure glaucoma versus a retroorbital process. The patient was started on timolol, pilocarpine, and dorzolamide eye drops for intraocular pressure control. An orbital CT was obtained, which showed an engorged superior ophthalmic vein on the left with a new 4 mm proptosis of the left eye (Figure 2) when compared to previous imaging.

Figure 2. A: CT scan showed proptosis of 4 mm of left eye compared to right eye. B: Enlarged left ophthalmic vein also noted on CT scan (arrow).

This raised concern for traumatic carotid cavernous fistula. A CTA obtained the following morning confirmed this suspicion (Figure 3).

Figure 3. A: Reconstructed coronal CT coronal angiogram showing enlarged left cavernous sinus, confirming diagnosis of carotid cavernous fistula. B-E: Static coronal images from CT angiogram with major arteries labeled. F: Video of CT angiogram.

The patient was transferred to an outside facility for surgical management, which consisted of angiography and embolization via coiling of her CCF.

Discussion

Carotid cavernous fistulas are abnormal connections that form between the cavernous sinus and the internal or external carotid arteries, or branches of the internal or external carotid arteries. They are divided into direct and indirect variants per Barrow classification (Table 1, Figure 4).

ICA = Internal carotid artery ECA = External carotid artery

Figure 4. A: The normal eye: superior ophthalmic vein draining into cavernous sinus and internal and external carotid arteries traversing the cavernous sinus. B: Barrow Classifications for types of carotid cavernous fistulas: Type A: direct connection between internal carotid artery and cavernous sinus. Type B: connection between dural branches of internal carotid artery and cavernous sinus. Type C: connection between dural branches of external carotid artery and cavernous sinus. Type D: connection between dural branches of both internal carotid artery and external carotid artery and the cavernous sinus.

Types B through D are commonly termed ‘indirect’ or ‘dural’ fistulas. These can develop spontaneously as a result of hypertension and are the more common presentation of CCF. More specifically, type B is a connection between the dural branches of the ICA and the cavernous sinus, type C is a connection between the dural branches of the external carotid artery (ECA) and the cavernous sinus, and type D connects the dural supply of both the ICA and ECA and the cavernous sinus (1). Type A, or a ‘direct’ CCF, is a connection between the intracavernous internal carotid artery (ICA) and the cavernous sinus. Direct CCF is a rare ocular complication that forms most commonly as a result of craniofacial trauma, but can also be due to aneurysmal rupture or spontaneous development. This is also the most dramatic presentation of CCF and was the case in our patient.

Prompt identification and management of CCF is necessary to prevent associated morbidity and mortality. The presentation of CCF depends mainly on the drainage of the fistula. Anterior-drainage of fistulas through the superior ophthalmic vein produces symptoms of exophthalmos, proptosis, acute chemosis or swelling/edema of conjunctiva, and headache, all of which are more common in direct CCFs. The backup of drainage can result in a secondary angle closure with extremely high intraocular pressure. Posterior-drainage of fistulas into the superior and inferior petrosal sinuses tend to lack the aforementioned features of orbital congestion, but can produce painful cranial neuropathy of the trigeminal, facial, or ocular motor nerves. Failure to identify and appropriately treat posterior-draining fistulas can lead to eventual reversal of flow and development of anterior drainage (4).

The signs of CCF are not visible on neuroimaging at a patient’s presentation and generally develop over the first week a patient is admitted.  Clinical signs which may prompt further investigation and repeat imaging include chemosis, increasing exophthalmos, pain, and increased intraocular pressure. Often, the tools for checking intraocular pressure are not available in an ICU setting. In the absence of signs of a ruptured globe, an intensivist could palpate the orbit over a closed eye (as occurred in this case). If there is asymmetry in resistance to palpation, this should incite an ophthalmologic consult to consider a retro-orbital process.

Repeat neuroimaging is likely to be done in these cases, but it is important to order the right test. Radiologic signs of CCF include proptosis and asymmetric enlargement of a cavernous sinus or superior ophthalmic vein and would be noted on an orbital or maxillofacial CT. A head CT might miss these signs, so it is important to obtain imaging dedicated to examining the retro-orbital space. To confirm the diagnosis of CCF, one must then obtain a CT angiogram, which will show the aberrant connections between the intracranial vessels. Upon confirming a diagnosis of CCF, the preferred mode of management is endovascular obliteration using an arterial or venous approach as it has been shown to be safe and effective, and confers long-term cure in most cases (5).

A previous review of 16 cases of carotid cavernous fistulas treated with transarterial embolization with detachable balloon show satisfactory results, defined as resolution of CCF without residual disability, in 11 cases and resolution but with residual disability in 5 cases. The most common of the disabilities in these cases was vision impairment, as seen in 4 out of the 5 cases. In addition, 14 out of the 16 cases resolved with preserved internal carotid artery flow (1). As a result, transarterial embolization with detachable balloon (TAEDB) has been established as the preferred method of treatment for carotid cavernous fistulas (6). Other options for treatment include neurosurgery and stereotactic radiosurgery when endovascular approach is not feasible.

Our patient presented with several traumatic injuries following a fall down a flight of stairs and was unable to contribute to history-taking. Detection and treatment of the CCF that she later developed was complicated by several factors. The true exophthalmos of the affected eye was partially masked by the fact that she had an inferotemporal orbital fracture of the opposite eye, which was incorrectly thought to be enophthalmic. Additionally, her altered mental status and subsequent re-intubation limited her ability to vocalize the pain which would have been present in her affected eye due to tremendously increased intraocular pressure.

From a critical care physician perspective, part of the key to her diagnosis was her re-intubation. The patient developed severe agitation requiring sedation without other more typical reasons for intubation such as hypoxia, tachypnea, or dyssynchronous breathing. We suspect this agitation was likely secondary to pain from the rapidly increasing pressure in her affected eye which became symptomatic just prior to her worsening mental status. Her physical exam was ultimately crucial to the detection of her CCF, specifically chemosis, exophthalmos, and increased intraocular pressure in the affected eye. These signs led to the subsequent ophthalmologic consultation, imaging, and eventually the diagnosis of CCF.

An important lesson learned from this patient’s management is having a low threshold for consultation when the clinical picture does not match diagnostic workup. In our case, the patient’s clinical condition changed but repeat workup including EEG and MRI of the head was negative. Previous imaging had revealed right-sided facial fractures, yet her new findings, including increased resistance to palpation of the orbit and chemosis, were largely left-sided. In situations when the cause of a patient’s deteriorating condition is unclear and there is incongruity between the physical exam and diagnostic workup, it is imperative to obtain further consultation. In our case, the ophthalmic exam gave the clues for further workup and the ultimate diagnosis.

In conclusion, this patient’s case is a good study in the classic presentation of direct CCF in association with craniofacial trauma, and also illuminates the difficulty in detection of orbital injuries in a trauma patient who cannot vocalize the symptoms they are experiencing. The lesson learned from her presentation is to have a low threshold for ophthalmologic consultation for unexplained changes in ophthalmic condition and discrepancies between clinical presentation and diagnostic findings.

References 

1. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985 Feb;62(2):248-56. [CrossRef] [PubMed]
2. Liang W, Xiaofeng Y, Weiguo L, Wusi Q, Gang S, Xuesheng Z. Traumatic carotid cavernous fistula accompanying basilar skull fracture: a study on the incidence of traumatic carotid cavernous fistula in the patients with basilar skull fracture and the prognostic analysis about traumatic carotid cavernous fistula. J Trauma. 2007 Nov;63(5):1014-20. [CrossRef] [PubMed]
3. Doran M. Carotid-Cavernous Fistulas: Prompt Diagnosis Improves Treatment. American Academy of Ophthalmology. https://www.aao.org/eyenet/article/carotid-cavernous-fistulas-prompt-diagnosis-improv. Published March 18, 2016. Accessed July 11, 2017.
4. Miller NR. Diagnosis and management of dural carotid-cavernous sinus fistula. Neurosurg Focus. 2007;23(5):E13. [PubMed]
5. Gupta AK, Purkayastha S, Krishnamoorthy T, Bodhey NK, Kapilamoorthy TR, Kesavadas C, Thomas B. Endovascular treatment of direct carotid cavernous fistulae: a pictorial review. Neuroradiology. 2006 Nov;48(11):831-9. [CrossRef] [PubMed]
6. Lewis AI, Tomsick TA, Tew JM Jr, Lawless MA. Long-term results in direct carotid-cavernous fistulas after treatment with detachable balloons. J Neurosurg. 1996 Mar;84(3):400-4. [CrossRef] [PubMed]

Cite as: Ganapathiraju I, Summerfield DT, Summerfield MM. Carotid cavernous fistula: a case study and review. Southwest J Pulm Crit Care. 2017:15(1):32-8. doi: https://doi.org/10.13175/swjpcc083-17 PDF