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Brian P. Walcott, Jonathan J. Russin, Robin Babadjouni and William J. Mack

This is the case of a man in his 40s who suffered sudden collapse into a deep coma as a result of a ruptured arteriovenous malformation (AVM) feeding artery aneurysm within the lateral ventricle. The ruptured aneurysm was successfully treated with Onyx embolization of the feeding pedicle. The AVM and the feeding artery aneurysm were then removed via a transcallosal approach. This case highlights the utility of interrogating the AVM with microcatheterization of the feeding pedicles in order to define the exact anatomical features necessary for treatment planning. It also reviews the anatomy of the choroidal fissure.

The video can be found here: https://youtu.be/UeqFzhTRU1Q.

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William J. Mack, Louis J. Kim, Demetrius K. Lopes and J Mocco

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Praveen K. Belur, Jason J. Chang, Shuhan He, Benjamin A. Emanuel and William J. Mack

Intracerebral hemorrhage (ICH) is associated with a higher degree of morbidity and mortality than other stroke subtypes. Despite this burden, currently approved treatments have demonstrated limited efficacy. To date, therapeutic strategies have principally targeted hematoma expansion and resultant mass effect. However, secondary mechanisms of brain injury are believed to be critical effectors of cell death and neurological outcome following ICH. This article reviews the pathophysiology of secondary brain injury relevant to ICH, examines pertinent experimental models, and highlights emerging therapeutic strategies. Treatment paradigms discussed include thrombin inhibitors, deferoxamine, minocycline, statins, granulocyte-colony stimulating factors, and therapeutic hypothermia. Despite promising experimental and preliminary human data, further studies are warranted prior to effective clinical translation.

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Omar N. Syed, Todd C. Hankinson, William J. Mack, Neil A. Feldstein and Richard C. E. Anderson

Pediatric neurosurgeons frequently care for children with traumatic scalp and skull injury. Foreign objects are often observed on imaging and may influence the clinician's decision-making process. The authors report on 2 cases of poorly visualized hair beads that had become embedded into the skull during blunt trauma. In both cases, skull radiography and CT scanning demonstrated depressed, comminuted fractures with poorly demonstrated spherical radiolucencies in the overlying scalp. The nature of these objects was initially unclear, and they could have represented air that entered the scalp during trauma. In one case, scalp inspection demonstrated no evidence of the bead. In the other case, a second bead was observed at the site of scalp laceration. In both cases, the beads were surgically removed, the fractures were elevated, and the patients recovered uneventfully. Radiolucent fashion accessories, such as hair beads, may be difficult to appreciate on clinical examination and may masquerade as clinically insignificant air following cranial trauma. If they are not removed, these foreign bodies may pose the risk of an infection. Pediatric neurosurgeons should consider hair accessories in the differential diagnosis of foreign bodies that may produce skull fracture following blunt trauma.

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Peter Adamczyk, Arun Paul Amar, William J. Mack and Donald W. Larsen

Endovascular embolization with Onyx has been increasingly used to treat intracranial and spinal dural arteriovenous fistulas (DAVFs). Several case series have been published in recent years reporting high DAVF cure rates with this technique. Although it is seldom reported, DAVF recurrence may occur despite initial “cure.” The authors present 3 separate cases of a recurrent DAVF after successful transarterial Onyx embolization. Despite adequate Onyx penetration into the fistula and draining vein, these cases demonstrate that DAVF recanalization may reappear with filling from previous or newly recruited arterial feeders. Other published reports of DAVF recurrence are examined, and potential contributory factors are discussed. These cases highlight the need for awareness of this possible phenomenon and suggest that follow-up angiography should be considered in patients treated with catheter embolization.

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Brian Lee, Vivek A. Mehta, William J. Mack, Matthew S. Tenser and Arun P. Amar

Type 1 spinal dural arteriovenous fistula (dAVF) constitute the vast majority of all spinal vascular malformations. Here we present the case of a 71-year-old male with progressive myelopathy, lower-extremity weakness and numbness, and urinary incontinence. MRI imaging of the thoracic spine demonstrated cord edema, and catheter spinal angiography confirmed a type 1 spinal dAVF. The fistula was supplied by small dural branches of the left L-2 segmental artery. Angiographic cure was achieved with a one-stage procedure in which coils were used to occlude the distal segmental vessels, followed by balloon-assisted embolization with Onyx.

The video can be found here: http://youtu.be/8aehJbueH0U.

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Brian Lee, Vivek A. Mehta, Arun P. Amar, Matthew S. Tenser and William J. Mack

Open surgical disconnection has long been the treatment of choice for dural arteriovenous fistulas (dAVFs) of the anterior cranial fossa. However, advanced patient age and the presence of medical comorbidities can substantially increase the risk of craniotomy and favor a less invasive endovascular approach. Optimal positioning within the distal ophthalmic artery, beyond the origin of the central retinal branch, is achievable using current microcatheter technology and embolic materials. Here we present the case of an 88-year-old female with an incidentally discovered dAVF of the anterior cranial fossa. Angiographic cure was achieved with one-stage Onyx embolization.

The video can be found here: http://youtu.be/KVE0fUIECQM.

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Shuhan He, Martin H. Pham, Matthew Pease, Gabriel Zada, Steven L. Giannotta, Kai Wang and William J. Mack

Object

A more comprehensive understanding of the epigenetic abnormalities associated with meningioma tumorigenesis, growth, and invasion may provide useful targets for molecular classification and development of targeted therapies for meningiomas.

Methods

The authors performed a review of the current literature to identify the epigenetic modifications associated with the formation and/or progression of meningiomas.

Results

Several epigenomic alterations, mainly pertaining to DNA methylation, have been associated with meningiomas. Hypermethylation of TIMP3 inactivates its tumor suppression activity while CDKN2 (p14[ARF]) and TP73 gene hypermethylation and HIST1H1c upregulation interact with the p53 regulation of cell cycle control. Other factors such as HOX, IGF, WNK2, and TGF-β epigenetic modifications allow either upregulation or downregulation of critical pathways for meningioma development, progression, and recurrence.

Conclusions

Genome-wide methylation profiling demonstrated that global hypomethylation correlates with tumor grades and severity. Identification of additional epigenetic changes, such as histone modification and higher-order chromosomal structure, may allow for a more thorough understanding of tumorigenesis and enable future individualized treatment strategies for meningiomas.

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Eisha A. Christian, Diana L. Jin, Frank Attenello, Timothy Wen, Steven Cen, William J. Mack, Mark D. Krieger and J. Gordon McComb

OBJECT

Even with improved prenatal and neonatal care, intraventricular hemorrhage (IVH) occurs in approximately 25%–30% of preterm infants, with a subset of these patients developing hydrocephalus. This study was undertaken to describe current trends in hospitalization of preterm infants with posthemorrhagic hydrocephalus (PHH) using the Nationwide Inpatient Sample (NIS) and the Kids’ Inpatient Database (KID).

METHODS

The KID and NIS were combined to generate data for the years 2000–2010. All neonatal discharges with ICD-9-CM codes for preterm birth with IVH alone or with IVH and hydrocephalus were included.

RESULTS

There were 147,823 preterm neonates with IVH, and 9% of this group developed hydrocephalus during the same admission. Of patients with Grade 3 and 4 IVH, 25% and 28%, respectively, developed hydrocephalus in comparison with 1% and 4% of patients with Grade 1 and 2 IVH, respectively. Thirty-eight percent of patients with PHH had permanent ventricular shunts inserted. Mortality rates were 4%, 10%, 18%, and 40%, respectively, for Grade 1, 2, 3, and 4 IVH during initial hospitalization. Length of stay has been trending upward for both groups of IVH (49 days in 2000, 56 days in 2010) and PHH (59 days in 2000, 70 days in 2010). The average hospital cost per patient (adjusted for inflation) has also increased, from $201,578 to $353,554 (for IVH) and $260,077 to $495,697 (for PHH) over 11 years.

CONCLUSIONS

The number of neonates admitted with IVH has increased despite a decrease in the number of preterm births. Rates of hydrocephalus and mortality correlated closely with IVH grade. The incidence of hydrocephalus in preterm infants with IVH remained stable between 8% and 10%. Over an 11-year period, there was a progressive increase in hospital cost and length of stay for preterm neonates with IVH and PHH that may be explained by a concurrent increase in the proportion of patients with congenital cardiac anomalies.