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Kenneth R. Curtin, Matthew T. Walker, and Ali Shaibani

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Alejandro J. Lopez, Robert K. Campbell, Omar Arnaout, Yvonne M. Curran, Ali Shaibani, and Nader S. Dahdaleh

The authors report the case of a 28-year-old woman with a spontaneous cerebrospinal fluid leak from the sleeve of a redundant thoracic nerve root. She presented with postural headaches and orthostatic symptoms indicative of intracranial hypotension. CT myelography revealed that the lesion was located at the T-11 nerve root. After failure of conservative management, including blood patches and thrombin glue injections, the patient was successfully treated with surgical decompression and ligation of the duplicate nerve, resulting in full resolution of her orthostatic symptoms.

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Anas Al-Smadi, Tahaamin Shokuhfar, Andrew Johnston, Tord D. Alden, Robin Bowman, and Ali Shaibani

Intraosseous cranial arteriovenous malformations (AVMs) are very rare, challenging entities. The authors report the case of an extracranial parietooccipital vascular lesion. A 12-year-old boy presented with accelerated growth of a right scalp lesion over a few months. Digital subtraction angiography showed a large, right parietooccipital intraosseous AVM with multiple complex arterial feeders. Treatment of these lesions is difficult and can necessitate a multidisciplinary approach. In the featured case, 6 embolization procedures were performed over 1 year, including both transarterial and transvenous approaches, followed by total resection. The authors describe what is thought to be the second case of an AVM originating in the cranial bones and the first case with successful multidisciplinary management.

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Michael C. Hurley, Pamela H. Nguyen, Arthur J. DiPatri, and Ali Shaibani

The authors describe what is, to their knowledge, the first reported case of the anomalous origin of an internal carotid artery from the pulmonary artery. An otherwise asymptomatic 6-year-old girl, who presented with headaches and hypertension, underwent a comprehensive workup that revealed extensive meningeal and cerebral artery anastomoses to the left internal carotid artery—itself arising from the origin of the left pulmonary artery. This unique anatomical anomaly, caused by a disturbed pattern of aortic arch regression, resulted in a right-to-left vascular shunt into the pulmonary artery and a disturbance of intracranial artery flow patterns, complicating the management options.

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Matthew Walker, Saquib Khawar, Ali Shaibani, Sireen Reddy, Aruna Ganju, and Manu Gupta

✓ Intramedullary spinal cord surgery can disrupt the blood—spinal cord barrier and cause intravascular contents to leak into the surgical cavity. Immediate postoperative Gd-enhanced magnetic resonance (MR) imaging can demonstrate leakage of contrast into the surgical bed and complicate the assessment of whether a residual enhancing tumor is present. The authors report a case in which the preoperative lesion was nonenhancing and not expected to enhance on postoperative imaging. A Gd-enhanced MR imaging study obtained less than 24 hours after surgery revealed that the intramedullary surgical cavity was filled with contrast material. Because of the time course and the lesion's preoperative appearance, this “enhancement” was known to be caused by the leakage of medium into the resection cavity rather than of pathological soft-tissue enhancement

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Maryam Soltanolkotabi, Shahram Rahimi, Michael C. Hurley, Robin M. Bowman, Eric J. Russell, Sameer A. Ansari, and Ali Shaibani

The authors report on the case of a 7-year-old boy who presented with a reduced level of activity, macrocephaly, prominent scalp veins, and decreased left-sided visual acuity. Imaging workup demonstrated generalized cerebral volume loss, bilateral chronic subdural hematomas, absent left sigmoid sinus, hypoplastic left transverse sinus, and severe focal weblike stenosis of the right sigmoid sinus. Right sigmoid sinus angioplasty and stent insertion was performed, with an immediate reduction in the transduced intracranial venous pressure gradient across the stenosis (from 22 to 3 mm Hg). Postprocedural diminution of prominent scalp and forehead veins and spinal venous collateral vessels was followed by a progressive improvement in visual acuity and physical activity over a 1-year follow-up period, supporting the efficacy of angioplasty and stent insertion in intracranial venous outflow obstruction.

There are multiple potential causes of intracranial venous hypertension in children. Development of dural sinus stenosis in infancy may be one such cause, mimicking the clinical presentation of other causes such as vein of Galen malformations. This condition can be ameliorated by early endovascular revascularization.

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Richard J. Parkinson, Bernard R. Bendok, Christopher C. Getch, Parham Yashar, Ali Shaibani, William Ankenbrandt, Issam A. Awad, and H. Hunt Batjer

✓ The treatment of large and giant paraclinoid carotid artery (CA) aneurysms often requires the use of suction decompression for safe and effective occlusion. Both open and endovascular suction decompression techniques have been described previously. In this article the authors describe a revised endovascular suction decompression technique that provides several advantages in the treatment of large and giant paraclinoid and CA aneurysms.

A 51-year-old woman presented with a relatively brief history of progressive visual loss in the right eye, nonspecific headache, and an afferent pupillary defect. After angiography studies had been obtained, it was determined that she had a giant right paraclinoid internal CA aneurysm with a dome size of approximately 26 mm on the right and a neck diameter of 10 mm.

A modified technique was performed in which suction decompression was used. With the aid of a No. 7 French Concentric balloon guide catheter (Concentric Medical, Inc., Mountain View, CA) and application of a temporary clip distal to the aneurysm, the aneurysm was trapped and decompressed using retrograde suction through the guide catheter when the balloon was inflated. After satisfactory placement of three permanent clips, an intraoperative angiogram obtained through the same guide catheter confirmed CA patency. The aneurysm was then punctured and aspirated, ensuring complete occlusion of the aneurysm sac and reconstruction of the parent vessel. The patient made an excellent recovery and did not suffer any complications. She did not experience worsening in her vision.

This technical modification to endovascular suction decompression allows several potential advantages, including higher volume decompression and the ability to deliver endovascular devices to distal arterial locations.

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Maryam Soltanolkotabi, Samantha E. Schoeneman, Tord D. Alden, Michael C. Hurley, Sameer A. Ansari, Arthur J. DiPatri Jr., Tadanori Tomita, and Ali Shaibani


The authors undertook this study to assess the safety and efficacy of Onyx embolization in the treatment of intracranial arteriovenous malformations (AVMs) in pediatric patients.


All pediatric Onyx embolization of intracranial AVM cases performed consecutively at a single children's hospital over a 5-year period were collected and evaluated.


Twenty-five patients (mean age 10.5 years) underwent a total of 38 procedures. An aggregate of 56 pedicles were embolized (mean 1.47 per session). The Spetzler-Martin grade was determined in all cases. Onyx embolization resulted in complete obliteration of the AVM in 3 cases (12%) and partial obliteration in 22 cases (88%). A total of 23 patients underwent surgical treatment. The mean preoperative AVM devascularization in these cases was 72%. One patient was treated with radiosurgery following Onyx embolization. Overall, 10 complications occurred in a total of 38 procedures (26.3%). None of the complications resulted in permanent neurological morbidity. The rate of transient neurological complications was 10.5% (4 of 38 procedures) and the rate of transient nonneurological complications was 5.3% (2 of 38 procedures). The remaining 4 complications were clinically silent (rate of 10.5%). There were no procedure-related deaths in this study population. There was no significant difference in patients with and without complications in terms of demographic characteristics, AVM grade, or embolization features (p ≥ 0.2). Deep venous drainage was associated with higher complication rates (p = 0.03).


Onyx utilization is feasible for preoperative or primary embolization in the treatment of pediatric intracranial AVMs; however, the spectrum of complications encountered is broad, and attention must be paid to the technical nuances of and indications for its use to avoid many potential dangerous effects. Although the overall complication rates were higher than expected, all were either clinically silent or had only transient clinical effects. Thus, this experience suggests that Onyx embolization can be performed safely with a low rate of permanent morbidity in pediatric patients harboring these difficult lesions.

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Daphne Li, Tahaamin Shokuhfar, Julia Pantalone, Brian Rothstein, Tord D. Alden, Ali Shaibani, and Amanda M. Saratsis

Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associated with genetic abnormalities, particularly involving chromosome 9. Due to CSF overproduction, patients with DVHCP often fail management with shunting alone and may require adjuvant interventions. The authors present the case of a child with partial trisomy 9p and delayed diagnosis of hydrocephalus with radiographic evidence of DVHCP who was successfully managed with ventriculoperitoneal shunt (VPS) placement, adjuvant bilateral endoscopic choroid plexus coagulation (CPC), and the novel application of anterior choroidal artery embolization. In addition, a systematic MEDLINE search was conducted using the keywords “diffuse villous hyperplasia,” “choroid plexus hypertrophy,” and “idiopathic cerebrospinal fluid overproduction.” Clinicopathological characteristics and outcomes of the present case were reviewed and compared to those in the literature.

A 14-month-old girl with partial trisomy 9p presented with macrocephaly and radiographic evidence of communicating hydrocephalus and DVHCP. Ventriculoperitoneal shunting resulted in distal failure due to inadequate CSF absorption, and ventriculoatrial shunt (VAS) placement was not possible due to multiple cardiac anomalies. Daily CSF production was reduced via endoscopic third ventriculostomy and bilateral CPC, followed by distal choroidal artery embolization, enabling VPS re-internalization. The embolization was complicated by radiographic evidence of an iatrogenic cerebral infarct, but this was clinically occult. Thirty-two additional cases of communicating hydrocephalus due to DVHCP are reported in the literature: 27 pediatric, 3 adult, and 2 postmortem. Genetic abnormalities were noted in 14, with 7 (50%) involving chromosome 9. Twelve patients underwent plexectomy (9 bilateral, 2 unilateral, 1 partial), and 10 underwent CPC (4 bilateral, 3 unilateral, and 3 unspecified), with or without shunting. Eight patients were successfully managed with shunting alone (6 VASs, 2 VPSs), and none underwent arterial embolization.

DVHCP is a rare cause of communicating hydrocephalus that may be associated with genetic abnormalities. A thorough review of the literature highlights diagnostic criteria and interventional options involved in managing this cause of CSF overproduction. The present case demonstrates that angiographic confirmation of prominent choroidal arteries may contribute to the diagnosis DVHCP. In addition, embolization of the distal choroidal arteries may be considered as a potential adjuvant treatment in patients for whom conventional treatments have failed or are not feasible.

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Brian A. O'Shaughnessy, Bernard R. Bendok, Richard J. Parkinson, Ali Shaibani, Matthew T. Walker, Ebrahim Shakir, and H. Hunt Batjer

✓ Chiari malformation Type I (CM-I), a condition defined by caudal descent of the cerebellar tonsils through the foramen magnum, is generally considered a congenital lesion. Several authors, however, have described an acquired form that appears identical to the congenital lesion on neuroimages. The most commonly reported cause of an acquired CM-I is cerebrospinal fluid diversion through a lumboperitoneal shunt. In this paper, the authors report the case of a patient in whom an acquired CM-I developed in association with a supratentorial arteriovenous malformation (AVM) of the brain. Development of the acquired CM was documented on serial magnetic resonance images. Moreover, the CM was seen to originate and worsen in concert with the clinicoradiological progression of the AVM. The underlying mechanism responsible for the acquired CM in this case is thought to be a high-flow venopathy of the transverse and sigmoid sinuses causing occlusion on the right and redirection of venous outflow into posterior fossa veins, with consequent venous congestion and swelling of the posterior fossa structures.