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Shih-Shan Lang, Joel A. Bauman, Michael W. Aversano, Matthew R. Sanborn, Arastoo Vossough, Gregory G. Heuer and Phillip B. Storm

Object

Electrolyte and endocrinological complications of endoscopic third ventriculostomy (ETV) are infrequent but serious events, likely due to transient hypothalamic-pituitary dysfunction. While the incidence of diabetes insipidus is relatively well known, hyponatremia is not often reported. The authors report on a series of 5 patients with post-ETV hyponatremia.

Methods

The records of patients undergoing ETV between 2008 and 2010 were reviewed. All ETVs were performed with a rigid neuroendoscope via a frontal bur hole, standard third ventricle floor blunt perforation, Fogarty catheter dilation, and intermittent normal saline irrigation. Postoperative MR images were evaluated for endoscope tract injury as well as the trajectory from the bur hole center to the fenestration site.

Results

Thirty-two patients (20 male and 12 female) underwent ETV. Their median age was 6 years (range 3 weeks–28 years). Hydrocephalus was most commonly due to nontumoral aqueductal stenosis (43%), nontectal tumor (25%), or tectal glioma (13%). Five patients (16%) had multicystic/loculated hydrocephalus. Five patients (16%) developed hyponatremia between 1 and 8 days following ETV, including 2 patients with seizures (1 of whom was still hospitalized at the time of the seizure and 1 of whom was readmitted as a result of the seizure) and 3 patients who were readmitted because of decline in their condition following routine discharge. No hypothalamic injuries were noted on imaging. Univariate risk factors consisted of age of 2 years or less (p = 0.02), presence of cystic lesions (p = 0.02), and ETV trajectory angle 10° or more from perpendicular (p = 0.001).

Conclusions

Endoscopic third ventriculostomy is a well-tolerated procedure but can result in serious complications. Hyponatremia is rare and may be more likely in younger patients or those with cystic loculations. Patients with altered craniometry may be at particular risk with a rigid endoscopic approach requiring greater manipulation of subforniceal or hypothalamic structures.

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Peter J. Madsen, Shih-Shan Lang, Jared M. Pisapia, Phillip B. Storm, Robert W. Hurst and Gregory G. Heuer

Object

Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature.

Methods

A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012.

Results

Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both N-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit.

Conclusions

Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.

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Shih-Shan Lang, Eric L. Zager, Thomas M. Coyne, Raj Nangunoori, J. Bruce Kneeland and Katherine L. Nathanson

In recent literature, there have been case reports of an extremely rare entity characterized by hybrid peripheral nerve tumors consisting of elements of neurofibroma, schwannoma, and/or perineurioma. The authors present a unique case of a patient with multiple painful hybrid tumors with negative genetic testing for neurofibromatosis Type 1 and no clinical evidence of neurofibromatosis Type 2 or schwannomatosis.

A 28-year-old woman presented with tentatively diagnosed schwannomatosis. She had painful bilateral retromastoid scalp tumors as well as multiple other painful tumors in the distribution of the saphenous, femoral, and sciatic nerves. Her family history was significant for a paternal grandfather with a solitary schwannoma. The patient underwent multiple surgical procedures for tumor resection, including tumors in the regions of the retromastoid scalp, bilateral sciatic nerves, left femoral nerve, and left axilla. These tumors were examined and evaluated histologically. Within the tumors, components of both neurofibromas and schwannomas were found, even though these 2 peripheral nerve sheath tumors have been long considered to be distinct entities. This case report suggests a distinct syndrome that has not previously been appreciated.

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Brendan F. Judy, Jordan W. Swanson, Wuyang Yang, Phillip B. Storm, Scott P. Bartlett, Jesse A. Taylor, Gregory G. Heuer and Shih-Shan Lang

OBJECTIVE

Evaluation of increased intracranial pressure (ICP) in the pediatric craniosynostosis population based solely on ophthalmological, clinical, and radiographic data is subjective, insensitive, and inconsistent. The aim of this study was to examine the intraoperative ICP before and after craniectomy in this patient population.

METHODS

The authors measured the ICP before and after craniectomy using a subdural ICP monitor in 45 children. They regulated end-tidal carbon dioxide and the monitoring site under general anesthesia to record consistent ICP readings.

RESULTS

The average age of the patient population was 29 months (range 3.8–180.5 months). Thirty-seven patients (82.2%) were undergoing initial craniosynostosis procedures. All craniosynostosis procedures were categorized as one of the following: frontoorbital advancement (n = 24), frontoorbital advancement with distraction osteogenesis (n = 1), posterior vault distraction osteogenesis (n = 10), and posterior vault reconstruction (n = 10). Nineteen of 45 patients (42.2%) had syndromic or multisuture craniosynostosis. The mean postcraniectomy ICP (8.8 mm Hg, range 2–18 mm Hg) was significantly lower than the precraniectomy ICP (16.5 mm Hg, range 6–35 mm Hg) (p < 0.001). Twenty-four patients (53%) had elevated ICP prior to craniectomy, defined as ≥ 15 mm Hg. Only 4 (8.9%) children had papilledema on preoperative funduscopic examination (sensitivity 17%, specificity 100%, negative predictive value 51%, and positive predictive value 100%). There were no significant differences in elevated precraniectomy ICP based on type of craniosynostosis (syndromic/multisuture or nonsyndromic) or age at the time of surgery. Patients undergoing initial surgery in the first 12 months of life were significantly less likely to have elevated precraniectomy ICP compared with patients older than 12 months (26.3% vs 73.1%, p = 0.005).

CONCLUSIONS

In this study, the authors report the largest cohort of syndromic and nonsyndromic craniosynostosis patients (n = 45) who underwent precraniectomy and postcraniectomy ICP evaluation. A craniectomy or completed craniotomy cuts for distractors effectively reduced ICP in 43/45 patients. The authors’ findings support the notion that papilledema on funduscopy is a highly specific, however poorly sensitive, indicator of increased ICP, and thus is not a reliable screening method. These findings indicate that even nonsyndromic patients with craniosynostosis are at risk for increased ICP. Furthermore, patients who present prior to 12 months of age appear less likely to have elevated ICP on presentation. Further studies with other noninvasive imaging of the retina may be useful as an adjunct tool for determining elevated ICP.

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Shih-Shan Lang, Lauren A. Beslow, Robert L. Bailey, Arastoo Vossough, Joanna Ekstrom, Gregory G. Heuer and Phillip B. Storm

Object

The true postoperative incidence of arteriovenous malformation (AVM) recurrence in the pediatric population remains largely unreported. Some literature suggests that delayed imaging studies should be obtained at 6 months to 1 year after negative findings on a postoperative angiogram. The aim of this study was to describe the timing of AVM recurrences after resection and the neuroimaging modalities on which the recurrences were detected.

Methods

This study was performed in a retrospective cohort of all pediatric patients treated surgically for AVM resection by a single neurosurgeon between 2005 and 2010. Patients were followed after resection with MR angiography (MRA) or conventional angiography, when possible, at various time points. A visual scale for compactness of the initial AVM nidus was used, and the score was correlated with probability of recurrence after surgery.

Results

A total of 28 patients (13 female, 15 male) underwent an AVM resection. In 18 patients (64.3%) an intraoperative angiogram was obtained. In 4 cases the intraoperative angiogram revealed residual AVM, and repeat resections were performed immediately. Recurrent AVMs were found in 4 children (14.3%) at 50, 51, 56, and 60 weeks after the initial resection. Recurrence risk was 0.08 per person-year. No patient with normal results on an angiogram obtained at 1 year developed a recurrence on either a 5-year angiogram or one obtained at 18 years of age. All patients with recurrence had a compactness score of 1 (diffuse AVM); a lower compactness score was associated with recurrence (p = 0.0003).

Conclusions

All recurrences in this cohort occurred less than 15 months from the initial resection. The authors recommend intraoperative angiography to help ensure complete resection at the time of the surgery. Follow-up vascular imaging is crucial for detecting recurrent AVMs, and conventional angiography is preferred because MRA can miss smaller AVMs. One-year follow-up imaging detected these recurrences, and no one who had negative results on an angiogram obtained at 1 year had a late recurrence. However, not all of the patients have been followed for 5 years or until 18 years of age, so longer follow-up is required for these patients. A lower compactness score predicted recurrent AVM in this cohort.

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Fabio A. Frisoli, Shih-Shan Lang, Arastoo Vossough, Anne Marie Cahill, Gregory G. Heuer, Hisham M. Dahmoush, Phillip B. Storm and Lauren A. Beslow

Object

Cerebral arteriovenous malformations (AVMs) have a higher postresection recurrence rate in children than in adults. The authors' previous study demonstrated that a diffuse AVM (low compactness score) predicts postresection recurrence. The aims of this study were to evaluate the intra- and interrater reliability of the AVM compactness score.

Methods

Angiograms of 24 patients assigned a preoperative compactness score (scale of 1–3; 1 = most diffuse, 3 = most compact) in the authors' previous study were rerated by the same pediatric neuroradiologist 9 months later. A pediatric neurosurgeon, pediatric neuroradiology fellow, and interventional radiologist blinded to each other's ratings, the original ratings, and AVM recurrence also rated each AVM's compactness. Intrarater and interrater reliability were calculated using the κ statistic.

Results

Of the 24 AVMs, scores by the original neuroradiologist were 1 in 6 patients, 2 in 16 patients, and 3 in 2 patients. Intrarater reliability was 1.0. The κ statistic among the 4 raters was 0.69 (95% CI 0.44–0.89), which indicates substantial reliability. The interrater reliability between the neuroradiologist and neuroradiology fellow was moderate (κ = 0.59 [95%CI 0.20–0.89]) and was substantial between the neuroradiologist and neurosurgeon (κ = 0.74 [95% CI 0.41–1.0]). The neuroradiologist and interventional radiologist had perfect agreement (κ = 1.0).

Conclusions

Intrarater and interrater reliability of the AVM compactness score were excellent and substantial, respectively. These results demonstrate that the AVM compactness score is reproducible. However, the neuroradiologist and interventional radiologist had perfect agreement, which indicates that the compactness score is applied most accurately by those with extensive angiography experience.

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Shih-Shan Lang, Bingqing Zhang, Hugues Yver, Judy Palma, Matthew P. Kirschen, Alexis A. Topjian, Benjamin Kennedy, Phillip B. Storm, Gregory G. Heuer, Janell L. Mensinger and Jimmy W. Huh

OBJECTIVE

External ventricular drains (EVDs) are commonly used in the neurosurgical population. However, very few pediatric neurosurgery studies are available regarding EVD-associated infection rates with antibiotic-impregnated EVD catheters. The authors previously published a large pediatric cohort study analyzing nonantibiotic-impregnated EVD catheters and risk factors associated with infections. In this study, they aimed to analyze the EVD-associated infection rate after implementation of antibiotic-impregnated EVD catheters.

METHODS

A retrospective observational cohort of pediatric patients (younger than 18 years of age) who underwent a burr hole for antibiotic-impregnated EVD placement and who were admitted to a quaternary care ICU between January 2011 and January 2019 were reviewed. The ventriculostomy-associated infection rate in patients with antibiotic-impregnated EVD catheters was compared to the authors’ historical control of patients with nonantibiotic-impregnated EVD catheters.

RESULTS

Two hundred twenty-nine patients with antibiotic-impregnated EVD catheters were identified. Neurological diagnostic categories included externalization of an existing shunt (externalized shunt) in 34 patients (14.9%); brain tumor (tumor) in 77 patients (33.6%); intracranial hemorrhage (ICH) in 27 patients (11.8%); traumatic brain injury (TBI) in 6 patients (2.6%); and 85 patients (37.1%) were captured in an “other” category. Two of 229 patients (0.9% of all patients) had CSF infections associated with EVD management, totaling an infection rate of 0.99 per 1000 catheter days. This is a significantly lower infection rate than was reported in the authors’ previously published analysis of the use of nonantibiotic-impregnated EVD catheters (0.9% vs 6%, p = 0.00128).

CONCLUSIONS

In their large pediatric cohort, the authors demonstrated a significant decline in ventriculostomy-associated CSF infection rate after implementation of antibiotic-impregnated EVD catheters at their institution.

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Peter J. Madsen, Vivek P. Buch, Jennifer E. Douglas, Arjun K. Parasher, David K. Lerner, Erin Alexander, Alan D. Workman, James N. Palmer, Shih-Shan Lang, Benjamin C. Kennedy, Arastoo Vossough, Nithin D. Adappa and Phillip B. Storm

OBJECTIVE

Craniopharyngioma represents up to 10% of pediatric brain tumors. Although these lesions are benign, attempts at gross-total resection (GTR) can lead to serious complications. More conservative approaches have emerged but require adjuvant radiation. Endoscopic endonasal surgery (EES) aimed at GTR has the potential to result in fewer complications, but there has been limited comparison to open surgery. The authors performed a review of these two approaches within their institution to elucidate potential benefits and complication differences.

METHODS

The authors performed a retrospective review of pediatric patients undergoing resection of craniopharyngioma at their institution between 2001 and 2017. Volumetric analysis of tumor size and postoperative ischemic injury was performed. Charts were reviewed for a number of outcome measures.

RESULTS

A total of 43 patients with an average age of 8.2 years were identified. Open surgery was the initial intervention in 15 and EES in 28. EES was performed in patients 3–17 years of age. EES has been the only approach used since 2011. In the entire cohort, GTR was more common in the EES group (85.7% vs 53.3%, p = 0.03). Recurrence rate (40% vs 14.2%, p = 0.13) and need for adjuvant radiation (20.0% vs 10.7%, p = 0.71) were higher in the open surgical group, although not statistically significant. Pseudoaneurysm development was only observed in the open surgical group. Volumetric imaging analysis showed a trend toward larger preoperative tumor volumes in the open surgical group, so a matched cohort analysis was performed with the largest tumors from the EES group. This revealed no difference in residual tumor volume (p = 0.28), but the volume of postoperative ischemia was still significantly larger in the open group (p = 0.004). Postoperative weight gain was more common in the open surgical group, a statistically significant finding in the complete patient group that trended toward significance in the matched cohort groups. Body mass index at follow-up correlated with volume of ischemic injury in regression analysis of the complete patient cohort (p = 0.05).

CONCLUSIONS

EES was associated with similar, if not better, extent of resection and significantly less ischemic injury than open surgery. Pseudoaneurysms were only seen in the open surgical group. Weight gain was also less prevalent in the EES cohort and appears be correlated with extent of ischemic injury at time of surgery.