May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation

Figure 1.  Preoperative nasopharyngoscopic direct visualization of telangiectasia of the nasal turbinate.

Figure 2.  Noncontrast head CT on postoperative day 3 demonstrates extensive multifocal areas of low attenuation consistent with early signs of infarction involving much of the cerebral hemispheres, most prominently involving the left parietal lobe.

A 54-year-old man with a complex cardiac history, including Tetralogy of Fallot requiring Blalock-Taussig shunt in infancy, infundibular patch repair at age 7, and bioprosthetic tricuspid valve replacement at age 52, had ongoing frequent hospitalizations with decompensated right ventricular heart failure secondary to native pulmonary valve mixed stenosis plus regurgitation and left pulmonary artery stenosis. His case was further complicated by his history of hereditary hemorrhagic telangiectasia (HHT) with recurrent epistaxis and recent GI bleeds with multiple angiodysplastic lesions throughout the stomach, duodenum, and descending colon which were previously treated with argon plasma coagulation.

The patient was admitted to our hospital in NYHA class IV heart failure receiving a continuous dopamine infusion and aggressive diuresis. Upon admission, a right heart catheterization demonstrated severe pulmonary valve regurgitation, left pulmonary artery stenosis, and systemic hypoxemia suggestive of an intrapulmonary shunt. Admission transthoracic echocardiogram demonstrated normal left ventricular ejection fraction of 55-60%, a severely enlarged right ventricle, moderately reduced right ventricular systolic function, severe pulmonary valve regurgitation, and moderate pulmonary valve stenosis.

A multidisciplinary team including congenital cardiology, pulmonary hypertension, interventional pediatric cardiology, and congenital cardiovascular surgery was consulted and after extensive discussions the patient consented to surgical intervention. Prior to his operative date, he underwent cauterization of his bilateral nasal cavity telangiectasias by Otolaryngology (Figure 1). On hospital day sixteen, he underwent a fourth time redo median sternotomy, pulmonary valve replacement with St. Jude Epic 27-mm porcine bioprosthesis, and repair of left pulmonary artery stenosis. Intraoperative transesophageal echocardiogram at the end of the surgical case demonstrated severe right ventricular dilation, severe right ventricular systolic dysfunction, normal pulmonary valve prosthesis, and left ventricular ejection fraction of 55%. The case was technically challenging requiring a cardiopulmonary bypass time of 178 minutes, and massive transfusion (including twelve units packed red blood cells, two packs of platelets, 4 units fresh frozen plasma, and 10 units cryoprecipitate) for a total estimated blood loss of 3.9 L.

Postoperatively, he had persistent right ventricular systolic dysfunction and diffuse mediastinal hemorrhage. By postoperative day two, a repeat transesophageal echocardiogram revealed worsening right ventricular dilation and severe right ventricle systolic dysfunction. The multidisciplinary care team recommended central venoarterial extracorporeal membrane oxygenation (VA ECMO) support for both worsening hypoxemia and continuing severe right ventricular failure.  The aorta was cannulated with a 22 French Bio-Medicus cannula (Medtronic, Minneapolis, USA) and the right atrium cannulated with a 36 French venous cannula (Medtronic, Minneapolis, USA), and full ECMO support was initiated using a Cardiohelp console with a HLS 7.0 oxygenator (Getinge, Goteborg, Sweden) reaching ECMO blood flows of 6 L/minute (an indexed ECMO blood flow of 2.6 L/minute/m²).

On POD 3, bronchoscopy was performed and revealed diffuse thin bloody secretions in the distal airways without a focal source, which was cleared with suction but quickly reaccumulated. Due to the pulmonary hemorrhage and recent mediastinal hemorrhage, systemic anticoagulation was not started at that time. Due to a lack of awakening during a sedation vacation, computed tomography (CT) imaging of his head was obtained and demonstrated a large ischemic stroke affecting the majority of the left MCA territory and part of the right parietal lobe (Figure 2).

HHT (also known as Osler-Weber-Rendu disease) is an autosomal dominant genetic disease with various vascular manifestations (1). In addition to the more common mucocutaneous and gastrointestinal tract telangiectasias, some patients with HHT also have pulmonary arteriovenous malformations (AVMs) with right-to-left shunt that can cause hypoxemia with resultant polycythemia. Cerebral AVMs present a risk of intracranial hemorrhage, ischemia, and hydrocephalus, which correlate with the size of the vascular defect. Given the presence of AVMs and hemorrhagic complications related to telangiectasias, the use of extracorporeal membrane oxygenation (ECMO) in patients with HHT is a potentially high-risk situation.

This case highlights the risks of ECMO in patients with HHT. The causes of this patient’s hemorrhagic and thrombotic events were most likely multifactorial, including contributions from a dilutional and consumptive coagulopathy after cardiopulmonary bypass and hemorrhage, initiation of ECMO, kidney failure, and his underlying HHT. The timing and precise cause of our patient’s cerebral infarction are unclear. However, patients with HHT and clinically significant intrapulmonary AVMs may have an increased risk of paradoxical thromboembolic stroke (2). The international HHT expert guidelines assert that even though HHT is a hemorrhagic disorder, it provides no protection against thrombosis (3). In addition, patients with HHT may  levels of von Willebrand factor and factor VIII, which would potentially increase their risk of thrombosis (4). This case exemplifies the substantial risks of hemorrhagic and thrombotic complications associated with ECMO for patients with HHT. Further study is needed to help determine whether HHT should be considered a contraindication to ECMO.

Theodore O. Loftsgard, APRN, CNP^1,2; Kari A. Wilson, APRN, CNP^1,2; John K. Bohman, MD^2,3

¹Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN

²Critical Care Independent Multidisciplinary Program, Mayo Clinic, Rochester, MN

³Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, MN

References

1. Faughnan ME, Palda VA, Garcia-Tsao G, et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 2011 Feb;48(2):73-87. [CrossRef] [PubMed]
2. Dittus C, Streiff M, Ansell J. Bleeding and clotting in hereditary hemorrhagic telangiectasia. World J Clin Cases. 2015 Apr 16;3(4):330-7. [CrossRef] [PubMed]
3. Faughnan ME, Mager JJ, Hetts SW, et al. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med. 2020 Dec 15;173(12):989-1001. [CrossRef] [PubMed]
4. Shovlin CL, Sulaiman NL, Govani FS, Jackson JE, Begbie ME. Elevated factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism. Thromb Haemost. 2007 Nov;98(5):1031-9. [PubMed]

Cite as: Loftsgard TO, Wilson KA, Bohman JK. May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation. Southwest J Pulm Crit Care Sleep. 2024;28:68-70. doi: https://doi.org/10.13175/swjpccs015-24 PDF

A Finger-Like Projection in the Carotid Artery: A Rare Source of Embolic Stroke Requiring Carotid Endarterectomy

Neal S. Gerstein, MD FASE¹

Liem C. Nguyen, MD²

Omar S. Akbik, MD³

Howard Yonas, MD³

Andrew P. Carlson, MD MS-CR³

¹Department of Anesthesiology and Critical Care Medicine and ³Department of Neurosurgery

School of Medicine

University of New Mexico

Albuquerque, NM USA

²Department of Anesthesiology

University of California – San Diego Medical Center and Sulpizio Cardiovascular Center

San Diego, CA USA

Abstract

Atherosclerotic lesions of the extracranial carotid arteries are one of the most common cases of stroke. Rarely, a stroke may result from isolated non-stenotic carotid disease in the absence of systemic manifestations of cardiovascular disease or significant cardiovascular risk factors. We present an unusual case of multiple strokes resulting from a solitary finger-like projection within the posterior wall of the carotid artery in an otherwise healthy patient. This small finger-like projection has a propensity to act as a nidus for thrombus formation and a potential source of cerebral embolism.

Introduction

Atherosclerotic lesions of the extracranial carotid arteries are one of the most common causes of stroke. Intervention, whether it be via an open or endovascular technique, is typically reserved for symptomatic patients with moderate to severe carotid stenosis while intervention in asymptomatic patients is less clear (1,2). However, in rare cases, a cerebrovascular accident (CVA) may result from isolated non-stenotic carotid disease in healthy patients in the absence of systemic manifestations of cardiovascular disease or significant cardiovascular risk factors. CVA as a result from isolated carotid artery disease has not been previously described in the anesthesiology literature. We present an unusual case of CVA resulting from a solitary finger-like projection within the wall of the carotid artery in an otherwise healthy patient. The etiology associated with a CVA in this context relates to a small and minimal posterior carotid plaque that has a propensity to act as a nidus for thrombus formation and a potential source of cerebral embolism. Our case exemplifies this atypical cause of a CVA and heretofore minimally described entity involving the carotid artery system. 

Case

In April 2016, a 44-year-old non-smoking woman with a past medical history solely consisting of well-controlled hypertension and hyperlipidemia was exercising when she developed right-sided weakness. She was diagnosed with an ischemic right-sided CVA in the right middle cerebral artery territory. Her symptoms spontaneously resolved. She was managed with aspirin and warfarin for six months followed by aspirin monotherapy. In April 2017, she developed nearly identical symptoms, which again resolved with conservative therapy (aspirin and warfarin) and she was referred for neurosurgery consultation and further evaluation.     

During her most recent evaluation, aside from a body-mass index of 33 kg/m², her physical examination was completely normal including a complete neurologic and cardiac evaluation. Laboratory evaluation revealed no evidence of a hypercoagulable state or sickle-cell disease, autoimmune disease, abnormal erythrocyte sedimentation rate or C-reactive protein levels, and there was no evidence of an intracardiac shunt by transthoracic echocardiography.

Bilateral neck ultrasound duplex scanning revealed normal flow in both the internal carotid and both vertebral arteries. Magnetic resonance angiography of her neck vessels at the time of the initial stroke demonstrated bilateral mild narrowing and a posterior irregularity along with enlargement of the proximal internal carotid artery (ICA) just beyond the bifurcation, which was deemed hemodynamically insignificant (Figure 1).

Figure 1. Preoperative magnetic resonance angiography with contrast; Red arrow indicating defect in posterior right internal carotid artery.

Computed tomographic angiography (CTA) of her neck vasculature performed at the time of the second stroke one year later revealed no significant stenosis of her common carotid, internal carotid, or vertebral arteries but did re-demonstrate a right-sided small finger-like extension in the posterior carotid wall at the level of the bifurcation (Figure 2).

Figure 2. Preoperative computed tomography angiogram. Red arrow indicating right posterior internal carotid artery abnormality.

Anticoagulation was continued for another 4 months and a follow-up CTA did not reveal any change in the previous noted finger-like lesion within the carotid artery.

After evaluation by our neurosurgical colleagues, the decision was made to prepare the patient for a right-sided carotid endarterectomy (CEA). In addition to the routine standard monitors, additional monitoring modalities included invasive arterial blood pressure monitoring, 16-lead electroencephalogram, and bilateral cerebral oximetry monitoring.  Her CEA consisted of a longitudinal arteriotomy from the distal common carotid artery into the proximal ICA. The ‘finger’ of firm, irregular plaque was seen on the posterior ICA wall and could be easily dissected off the wall, ruling out a congenital web. The plaque was neither soft nor ruptured at the time of surgery; it was an irregular finger-like extension from the underlying plaque that was presumably the focus of thrombus formation (Figure 3).

Figure 3. Panel A: blue arrow indicating finger-like abnormal projection from posterior wall of right internal carotid artery. Panel B: excised projection specimen.

Pathological examination of the plaque revealed no evidence of gross calcifications, no signs of microscopic ulceration, or intra-plaque hemorrhage that are associated with an unstable plaque. The ICA clamp was then released temporarily to allow backflow of blood and with it washout of plaque that might have migrated upstream. Once the arteriotomy closure was complete, vascular clamps were removed and satisfactory pulsations were noted in the common carotid artery and external carotid artery, as well as the ICA. Following an uneventful emergence from anesthesia, the patient was extubated and brought to ICU in stable condition. Postoperative CTA demonstrated a normal caliber and lumen in the surgically treated right carotid artery (Figure 4).

Figure 4. Postoperative computed tomography angiogram demonstrating normal right internal carotid artery lumen.

Discussion

Stroke is a leading cause of death in developed nations with a majority of those ischemic in nature. Extracranial carotid artery atherosclerotic disease is the third leading cause of ischemic stroke in the general population (3). While medical management including antiplatelet therapy, treatment of hypertension, hyperlipidemia, diabetes, and smoking cessation have been shown to decrease the risk of stroke, surgical intervention in the form of CEA has been widely investigated in several randomized control studies and has proven efficacy in the appropriate patient population. A 2017 Cochrane systematic review along with other robust reviews found CEA most effective in symptomatic patients with >70% and is of some benefit for patients with 50-69% symptomatic stenosis (4,5). Surgery plays a limited role in complete or near complete occlusion (6).

While the above trials deal primarily with symptomatic carotid stenosis, a different pathology known as free-floating thrombus (FFT) can exist with or without carotid stenosis. As in our case, these patients are typically younger patients without established peripheral vascular disease or other systemic cardiovascular diseases. They typically have underlying atherosclerotic disease that predisposes them to thromboembolic events and as such are at a high risk for recurrent ischemic strokes. Most studies show that patients with FFT who are treated medically with anticoagulation have complete dissolution of the FFT without any further neurologic progression (7,8).

In contrast to FFT, our case patient was found to have a finger-like projection from the posterior wall of the right ICA just distal to its bifurcation without evidence of luminal thrombus. Our patient had persistent ischemic CVAs despite therapeutic anticoagulation and antiplatelet therapy with warfarin and aspirin, respectively. She continued to have persistent imaging findings of a small finger like projection on repeat neck CTAs. Intraoperatively, no thrombus was identified but rather a small plaque was resected which appeared to be similarly shaped to the finger-like projection seen on her CTA with an irregular intraluminal surface thought to be the nidus for thrombus formation. Upon further examination of the plaque by pathology, no gross calcifications were identified. Histologically no signs of microscopic ulceration or intraplaque hemorrhage were identified to indicate an unstable plaque, which is more commonly seen in advanced atherosclerotic disease. Critical differences in plaque morphology have been found to highly correlate with whether a patient has symptomatic or asymptomatic carotid disease (9).

There is a single similar case from 2011 from our institution, describing a 48-year-old woman who presented with intermittent hand numbness, facial weakness, and dysarthria (10). CTA of her head and neck demonstrated a several millimeter protrusion from her posterior ICA just distal to the bifurcation.  The patient had recurrent neurologic symptoms attributed to ongoing cerebral emboli despite anticoagulation and antiplatelet requiring CEA, during which organized thrombus was found in continuity with her isolated thin (1 mm) posterior carotid artery atherosclerotic plaque. It was concluded that the development of significant neurologic symptoms in the context of minimal stenosis is due to carotid endothelial hyperplasia with organizing thrombus on top of a small preexisting carotid atherosclerotic plaque. Similarly, our case report illustrates a patient receiving maximal medical therapy in the form of warfarin anticoagulation and antiplatelet therapy with persistent ischemic CVAs and an intraluminal plaque. The previous case had evidence of organizing thrombus while our case demonstrated only irregular plaque. This could either be because any adherent thrombus was washed out during the opening or that the thrombus itself had resolved with prolonged anticoagulation, leaving the finger-like plaque in the lumen. This speaks to a different pathology than what is typically observed in patients with FFT in that this intraluminal plaque morphology itself likely places the patient at risk for recurrent thrombus formation.

In summary, our rare etiology of stroke is heretofore unreported in the perioperative medicine literature. This case illustrates that in an otherwise healthy patient without systemic cardiovascular disease, the possibility of significant but minimal isolated carotid disease may be a nidus for thrombus and ultimately an embolic etiology for a significant neurologic injury. This report, along with the similar case described by Tran and Yonas (10), do not indicate a clear causal relationship. However, it is plausible that the described recurrent ipsilateral strokes are related to these uncommon and characteristic carotid morphologic findings. These assertions are further substantiated by the lack of any new symptoms during all patient follow-up visits. Nonetheless, a detailed study to document morphology involving a large sample of similar plaques of otherwise similar size and composition would be needed in order to make a definitive conclusion regarding the association between this finger-like carotid projection and recurrent CVAs. Perioperative and critical care physicians need to be aware that advanced radiological imaging is required to identify this isolated carotid pathology. Its association with cerebral emboli should be considered when presented with recurrent CVA events in the context of minimal evidence of atherosclerotic disease on routine carotid screening studies.

References

1. Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2014;45(7):2160-236. [CrossRef] [PubMed]
2. Meschia JF, Bushnell C, Boden-Albala B, et al. Guidelines for the primary prevention of stroke: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2014;45(12):3754-832. [CrossRef] [PubMed]
3. Ooi YC, Gonzalez NR. Management of extracranial carotid artery disease. Cardiol Clin. 2015;33(1):1-35. [CrossRef] [PubMed]
4. Orrapin S, Rerkasem K. Carotid endarterectomy for symptomatic carotid stenosis. Cochrane Database Syst Rev. 2017;6:CD001081. [CrossRef] [PubMed]
5. Meschia JF, Klaas JP, Brown RD, et al. Evaluation and Management of Atherosclerotic Carotid Stenosis. Mayo Clin Proc. 2017;92(7):1144-57. [CrossRef] [PubMed]
6. Rothwell PM, Eliasziw M, Gutnikov SA, et al. Endarterectomy for symptomatic carotid stenosis in relation to clinical subgroups and timing of surgery. Lancet. 2004;363(9413):915-24. [CrossRef] [PubMed]
7. Vellimana AK, Kadkhodayan Y, Rich KM, et al. Symptomatic patients with intraluminal carotid artery thrombus: outcome with a strategy of initial anticoagulation. J Neurosurg. 2013;118(1):34-41. [CrossRef] [PubMed]
8. Bhatti AF, Leon LR, Jr., Labropoulos N, et al. Free-floating thrombus of the carotid artery: literature review and case reports. J Vasc Surg 2007;45(1):199-205. [CrossRef] [PubMed]
9. Virmani R, Ladich ER, Burke AP, et al. Histopathology of carotid atherosclerotic disease. Neurosurgery. 2006;59(5 Suppl 3):S219-27; discussion S3-13. [CrossRef] [PubMed]
10. Tran H, Yonas H. Small carotid thrombus and minimal stenosis causing repeated embolic strokes. J Neuroimaging. 2011;21:266-8. [CrossRef] [PubMed]

Cite as: Gerstein NS, Nguyen LC, Akbik OS, Yonas H, Carlson AP. A finger-like projection in the carotid artery: A rare source of embolic stroke requiring carotid endarterectomy. Southwest J Pulm Crit Care. 2018;16(3):150-5. doi: https://doi.org/10.13175/swjpcc022-18 PDF 

Medical Image of the Week: Moyamoya Disease

Figure 1. Cerebral angiogram of the brain demonstrating bilateral high-grade stenosis of the anterior and middle cerebral arteries, worse on the left.

Figure 2. Magnetic resonance imaging showing multiple punctate infarcts in the frontal and parietal lobes on the left side.

A 52-year-old, right-handed, Caucasian woman with a history of hypertension and morbid obesity presented with acute onset of word-finding difficulty and slurred speech. Her medical and family history was negative for cerebral vascular event, coronary artery disease or smoking. Computed tomography of the patient's brain showed narrow caliber middle cerebral artery vasculature bilaterally. This abnormal finding prompted further investigation with cerebral angiogram. The angiogram showed bilateral high-grade stenosis of the anterior and middle cerebral arteries, worse on the left (Figure 1). Magnetic resonance imaging revealed multiple left sided punctate infarcts in the frontal and parietal lobes (Figure 2). Diagnosis of ischemic stroke secondary to moyamoya disease was established. This patient was not a candidate for fibrinolytic therapy since it had been more than 4 hours from initial presentation. She was treated with aspirin, clopidogrel, and atorvastatin for secondary prevention of ischemic stroke. Two months after her discharge date, the patient had a middle cerebral artery to superior temporal artery bypass on the left side, followed by a middle cerebral artery to superior temporal artery bypass on the right two months after initial bypass. Patient progressed to an uneventful recovery. Discharge planning included the continuation of aspirin, clopidogrel, and atorvastatin indefinitely.

Moyamoya disease (MMD) is an uncommon vasculopathy of unknown origin associated with diverse risk factors (1). It was first discovered in a Japanese population, and reported more commonly in this sub-population. However, numerous cases were reported across the globe (2). Moyamoya disease associated with other systemic condition such as neurofibromatosis type 1, trisomy 21, thyroid cranial irradiation or thyroid disease is termed moyamoya syndrome (MMS) (1,2). Moyamoya syndrome is a cerebrovasculopathy originating from collateral flow that develops secondary to occlusion of the internal carotid artery and the proximal afferent vessels at the circle of Willis (3). MMS can have abrupt or insidious onset and may progress to diversifying cerebral ischemic stroke or to intracranial hemorrhage, which is a worse prognosis and the primary cause of death in patients with MMD (4). It has been shown that ischemic stroke associated with MDD or MMS usually occurs when compensatory collateral vessels are unable to supply sufficient blood to the brain after occlusion or stenosis of the internal carotid arteries or its tributary vessels (5,6). On the other hand, intracranial hemorrhage occurs secondary to the rupture of abnormal moyamoya vessels (7,8).

It is imperative to differentiate between non-hemorrhagic and hemorrhagic moyamoya. Neuroimaging is the preferred method of diagnosis after high clinical suspicion of MMD or MMS. Intracranial hemorrhage and cerebral infarction can be diagnosed with computed tomography and magnetic resonance imaging/ cerebral angiogram, respectively (8,9). Recent use of magnetic resonance perfusion imaging has been shown to be crucial in diagnostics and medical-surgical decision making. There is no common consensus when it comes to treatment of moyamoya at this time. Initial management is symptomatic with anticoagulants, antiplatelet and corticosteroids (10). Treatment options may also include direct or indirect surgical revascularization as optimal therapy (11,12).

Stella Pak MD, Kokou Adompreh-Fia MD, Damian Valencia MD, Adam Fershko MD, and Jody Short DO.

Department of Medicine

Kettering Medical Center

Kettering, OH USA

References

1. Phi JH, Wang KC, Lee JY, Kim SK. Moyamoya Syndrome: A window of moyamoya disease. J Korean Neurosurg Soc. 2015 Jun;57(6):408-14. [CrossRef] [PubMed]
2. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969 Mar;20(3):288-99. [CrossRef] [PubMed]
3. Yamamoto, S, Koh M, Kashiwazaki D, Akioka N, Kuwayama N, Noguchi K, Kuroda S. Is Quasi-moyamoya disease a uniform disease entity? A three-dimensional constructive interference in steady state imaging study. J Stroke Cerebrovasc Dis. 2016 Jun;25(6):1509-16. [CrossRef] [PubMed]
4. Baba, T., Houkin, K. Kuroda. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry. 2008 Aug;79(8):900-4. [CrossRef] [PubMed]
5. Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Ihara I, Yamagata S. Study of the posterior circulation in moyamoya disease. Part 2: Visual disturbances and surgical treatment. J Neurosurg. 1986 Oct;65(4):454-60. [CrossRef] [PubMed]
6. Kuroda S, Ishikawa T, Houkin K, Iwasaki Y. Clinical significance of posterior cerebral artery stenosis/occlusion in moyamoya disease. No Shinkei Geka. 2002 Dec;30(12):1295-300. [PubMed]
7. Kang K, Lu J, Zhang D, Li Y, Wang D, Liu P, Li B, Ju Y, Zhao X. Difference in cerebral circulation time between subtypes of moyamoya disease and moyamoya syndrome. Sci Rep. 2017;7(1):2587. [CrossRef] [PubMed]
8. Lui, P, Han C, Li DS, Lv XL, Li YX, Duan L. Hemorrhagic moyamoya disease in children: Clinical, angiographic features, and long-term surgical outcome. Stroke. 2016 Jan;47(1):240-3. [CrossRef] [PubMed]
9. Kellner CP, McDowell MM, Phan MQ, Connolly ES, Lavine SD, Meyers PM, Sahlein D, Solomon RA, Feldstein NA, Anderson RC. Number and location of draining veins in pediatric arteriovenous malformations: association with hemorrhage. J Neurosurg Pediatr. 2014 Nov;14(5):538-45. [CrossRef] [PubMed]
10. Whitaker J. Management of moyamoya syndrome [comment]. Arch Neurol. 2001;58:132. [CrossRef] [PubMed]
11. Golby AJ, Marks MP, Thompson RC, Steinberg GK. Direct and combined revascularization in pediatric moyamoya disease. Neurosurg. 1999;45:50-8. [PubMed]
12. Mizoi K, Kayama T, Yoshimoto T, Nagamine Y. Indirect revascularization for moyamoya disease: is there a beneficial effect for adult patients? Surg Neurol. 1996;45:541-8. [CrossRef] [PubMed] 

Cite as: Pak S, Adompreh-Fia K, Valencia D, Fershko A, Short J. Medical image of the week: moyamoya disease. Southwest J Pulm Crit Care. 2017;15(5):227-9. doi: https://doi.org/10.13175/swjpcc136-17 PDF

Medical Image of the Week: Intraventricular Hemorrhage Casting

Figure 1. Panel A: Computerized tomography of the head without contrast taken at an outlying facility displayed a right thalamic intraparenchymal hematoma, measuring 3.4 x 4.2 cm, with vasogenic edema and intraventricular rupture (blue arrow). Intraventricular hemorrhage casting is visualized in the right lateral ventricular causing obstructive hydrocephalus (red arrow). Panel B: Repeat non-contrast CT of the head 6 hours later revealed an increase in size of thalamic hematoma to 4.3 x 5.2 x 4.8 cm, an increase in amount of Intraventricular hemorrhage, progression of hydrocephalus from cast obstruction, and worsening vasogenic edema causing 5 mm left midline shift.

An 80-year-old woman with a past medical history of hypertension and hypercholesterolemia presented to an outlying hospital at 11:00 hours with slurred speech, left arm drift, and headache. A non-contrast CT of the head revealed an intraparenchymal hematoma in the right thalamus measuring 3.4 x 4.2 cm with an associated intraventricular rupture (Figure 1A, blue arrow). An intraventricular hemorrhage cast with secondary hydrocephalus was also noted on initial imaging (Figure 1A, red arrow). She was placed on a nicardipine drip for blood pressure control and subsequently transferred to OSF St. Francis Medical Center (OSFMC) for a higher level of care.

Upon arrival to OSFMC, the patient was poorly responsive, non-verbal, and could not follow commands.  She was directly admitted to the Neuroscience Intensive Care Unit for further management. Vitals signs were stable on presentation. Neurologic examination revealed a comatose patient with asymmetric and minimally reactive pupils, absent gag reflex, right gaze preference, brisk corneal reflex on the right and absent response on the left, absent deep tendon reflexes on the left upper and lower extremity, with absent response to painful stimuli on the left upper and lower extremity. Patient had a Glasgow Coma Scale score of 6, NIH stroke scale score of 23, and an Intracerebral Hemorrhage Score of 5. A repeat non-contrast CT scan of the head was performed at 17:00 hours to monitor for expansion of hematoma and progression of secondary hydrocephalus. Imaging revealed an interval increase in the size of the acute intraparenchymal hematoma, measuring 4.3 x 5.2 x 4.8 cm. In addition, there was an increase in amount of intraventricular hemorrhage, progression of hydrocephalus, and worsening vasogenic edema causing a mass effect with a 5 mm left midline shift (Figure 1B). At the request of the patient’s family members, her code status changed to DNR and she was made comfort care. No interventions were pursued and patient entered hospice care. 

Intracerebral hemorrhage (ICH) occurs in about 15% of strokes per year (1). The most common cause of spontaneous ICH is rupture of micro-aneurysms of small blood vessels in brain tissue, secondary to chronic hypertension. Hypertensive hemorrhages typically occur in the basal ganglia and thalamus, which are in close proximity to the cerebral ventricular system. Blood can accumulate at these sites forming an acute intraparenchymal hematoma, which can expand and exert mechanical pressure on the ventricular walls leading to intraventricular rupture and secondary intraventricular hemorrhage (IVH) (2). Intraventricular rupture occurs in approximately 45% of spontaneous ICH, which results in an expected mortality of 50-80% (1). Blood in the ventricular system can clot forming a “cast” (Figure 1A, red arrow). Ventricular casts are especially troublesome because the cast can block the outflow of cerebrospinal fluid causing an acute obstructive hydrocephalus, which can lead to increased intracerebral pressure (ICP), mass effect, and brain herniation (2). In Figure 1A, the intraventricular cast formation likely represents the patient’s normal ventricular size prior to ventriculomegaly from hydrocephalus. Figure 1B shows the typical progression of the intraparenchymal hematoma and obstructive hydrocephalus. There are several treatment options for management of an intraparenchymal hematoma with intraventricular rupture; they include reduction of ICP via ventriculostomy and medical therapy, surgical evacuation of the hematoma, and intraventricular thrombolytics to reduce casting and secondary obstructive hydrocephalus (2,3). Despite these interventions, the prognosis remains poor (3).

Melvin Parasram MS OMS4,¹ Mangala Gopal OMS4,² Lee Raube DO MS,³ Editha Johnson DO,⁴ Deepak Nair MD^4,5

¹Midwestern University, Arizona College of Osteopathic Medicine, Glendale, AZ USA

²Des Moines University, College of Osteopathic Medicine, Des Moines, IA USA

³Departments of Emergency Medicine and ⁴Neurology, University of Illinois College of Medicine at Peoria, Peoria, IL USA  

⁵Illinois Neurological Institute, OSF St. Francis Medical Center, Peoria, IL USA

References

1. Hinson HE, Hanley DF, Ziai WC. Management of intraventricular hemorrhage. Curr Neurol Neurosci Rep. 2010 Mar;10(2):73-82. [CrossRef] [PubMed]
2. Hanley DF. Intraventricular hemorrhage: severity factor and treatment target in spontaneous intracerebral hemorrhage. Stroke. 2009 Apr;40(4):1533-8. [CrossRef] [PubMed]
3. Nieuwkamp DJ, de Gans K, Rinkel GJ, Algra A. Treatment and outcome of severe intraventricular extension in patients with subarachnoid or intracerebral hemorrhage: a systematic review of the literature. J Neurol. 2000 Feb;247(2):117-21. [CrossRef] [PubMed] 

Cite as: Parasram M, Gopal M, Raube L, Johnson E, Nair D. Medical image of the week: intraventricular hemorrhage casting. Southwest J Pulm Crit Care. 2016;13(5):220-3. doi: http://dx.doi.org/10.13175/swjpcc094-16 PDF 

January 2015 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ

Clinical History: A 68-year-old woman with a history of myelodysplastic syndrome associated with transfusion-dependent anemia and thrombocytopenia presented with recent onset left chest pain and fever. The patient had a remote history of total right knee arthroplasty, hypertension, asthma, and schizoaffective disorder. Several months earlier the patient was hospitalized with methicillin-sensitive Staphylococcus aureus infection involving the right knee arthroplasty, associated with bacteremia and a septic right elbow. This infection was treated with incision and drainage of the elbow, antibiotic bead placement about the right knee arthroplasty with an antibiotic-impregnated spacer, and antibiotics (6 weeks intravenous cefazolin followed by chronic doxycycline suppression therapy, the former later switched to nafcillin and rifampin). The patient had been discharged from the hospital with only compression hose for deep venous thrombosis prophylaxis, owing to her episodes of epistaxis in the setting of transfusion-dependent anemia.

Upon presentation, the patient was hypotensive, tachycardic, and hypotensive. Laboratory data showed a white cell count of 3.9 cells x 10⁹ / L, a platelet count of 7000 x 10⁹ / L, and a hemoglobin level of 7 g/dL.

Frontal chest radiography (Figure 1A) was performed (a baseline chest radiograph- Figure 1B- is presented for comparison).

Figure 1. Panel A: Frontal chest radiography Panel B: Frontal chest radiograph obtained 3 months to presentation.

Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the 2nd of 7 panels)

1. The frontal chest radiograph shows bilateral hilar and mediastinal lymph node enlargement
2. The frontal chest radiograph shows enlarged lung volumes with numerous cystic foci
3. The frontal chest radiograph shows linear and reticular abnormalities with architectural distortion associated with fibrotic lung disease
4. The frontal chest radiograph shows lingular and left lower lobe consolidation and left pleural effusion
5. The frontal chest radiograph shows multiple poorly defined bilateral pulmonary nodules

Reference as: Gotway MB. January 2015 imaging case of the month. Southwest J Pulm Crit Care. 2015;10(1):21-31. doi: http://dx.doi.org/10.13175/swjpcc003-15 PDF

Medical Image of the Week: Massive Cerebral Infarction

 

Figure 1. Movie of head CT scan.

 

Figure 2. Movie of head MRI.

A 77 year old man with a history of chronic heart failure was admitted to the hospital complaining of left sided hemiparesis for about an hour. He was oriented but had slurred speech and was unable to move his left arm or leg. His pulse was irregular and ECG showed atrial fibrillation. A CT scan of the head (Figure 1) was interpreted as relatively unremarkable. Magnetic resonance imaging (MRI) of the head (Figure 2) showed massive right brain infarction. These studies illustrate the higher sensitivity of MRI in comparison to CT in the detection of stroke, especially early after the onset on symptoms (1).

Nijamudin Samani, MD; Yong-Jie Yin, MD; Sanjaya Karki, MD; and Jing-Xiao Zhang, MD

Department of Emergency and Critical Care

Second Hospital of Jilin University

Norman Bethune College of Medicine

Changchun, China

Reference

1. Chalela JA, Kidwell CS, Nentwich LM, Luby M, Butman JA, Demchuk AM, Hill MD, Patronas N, Latour L, Warach S. Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison. Lancet. 2007;369(9558):293-8. [CrossRef] [PubMed]

Reference as: Samani N, Yin YJ, Karki S, Zhang JX. Medical image of the week: massive cerebral infarction. Soutwest J Pulm Crit Care. 2013;7(1):25-6. doi: http://dx.doi.org/10.13175/swjpcc084-13 PDF