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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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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.

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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 bothN-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, 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.

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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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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.

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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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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

评估增加颅内压(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.

开云体育世界杯赔率

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

在这项研究中,作者报告st 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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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.

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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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张Shih-Shan朗,琥珀Valeri冰清,菲尔lip B. Storm, Gregory G. Heuer, Lauren Leavesley, Richard Bellah, Chong Tae Kim, Heather Griffis, Todd J. Kilbaugh, and Jimmy W. Huh

OBJECTIVE

床(滚刀)海拔30°后严重的traumatic brain injury (TBI) has become standard positioning across all age groups. This maneuver is thought to minimize the risk of elevated ICP in the hopes of decreasing cerebral blood and fluid volume and increasing cerebral venous outflow with improvement in jugular venous drainage. However, HOB elevation is based on adult population data due to a current paucity of pediatric TBI studies regarding HOB management. In this prospective study of pediatric patients with severe TBI, the authors investigated the role of different head positions on intracranial pressure (ICP), cerebral perfusion pressure (CPP), and cerebral venous outflow through the internal jugular veins (IJVs) on postinjury days 2 and 3 because these time periods are considered the peak risk for intracranial hypertension.

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Patients younger than 18 years with a Glasgow Coma Scale score ≤ 8 after severe TBI were prospectively recruited at a single quaternary pediatric intensive care unit. All patients had an ICP monitor placed, and no other neurosurgical procedure was performed. On the 2nd and 3rd days postinjury, the degree of HOB elevation was varied between 0° (head-flat or horizontal), 10°, 20°, 30°, 40°, and 50° while ICP, CPP, and bilateral IJV blood flows were recorded.

RESULTS

Eighteen pediatric patients with severe TBI were analyzed. On each postinjury day, 13 of the 18 patients had at least 1 optimal HOB position (the position that simultaneously demonstrated the lowest ICP and the highest CPP). Six patients on each postinjury day had 30° as the optimal HOB position, with only 2 being the same patient on both postinjury days. On postinjury day 2, 3 patients had more than 1 optimal HOB position, while 5 patients did not have an optimal position. On postinjury day 3, 2 patients had more than 1 optimal HOB position while 5 patients did not have an optimal position. Interestingly, 0° (head-flat or horizontal) was the optimal HOB position in 2 patients on postinjury day 2 and 3 patients on postinjury day 3. The optimal HOB position demonstrated lower right IJV blood flow than a nonoptimal position on both postinjury days 2 (p = 0.0023) and 3 (p = 0.0033). There was no significant difference between optimal and nonoptimal HOB positions in the left IJV blood flow.

CONCLUSIONS

In pediatric patients with severe TBI, the authors demonstrated that the optimal HOB position (which decreases ICP and improves CPP) is not always at 30°. Instead, the optimal HOB should be individualized for each pediatric TBI patient on a daily basis.

Open access

克里斯托弗·l·卡马尔乔丹w . Swanson, Sameer Shakir, Alexander M. Tucker, Benjamin C. Kennedy, Phillip B. Storm, Gregory G. Heuer, Scott P. Bartlett, Jesse A. Taylor, and Shih-Shan Lang

Spring-mediated cranioplasty is a useful treatment modality for correcting scaphocephalic head shape in sagittal craniosynostosis because it is less invasive than whole-vault cranioplasty and offers durable morphologic outcomes. Herein, the authors provide a multimedia demonstration of alternative operative approaches for spring-mediated cranioplasty for sagittal craniosynostosis.

The video can be found here:https://vimeo.com/511256259

Open access

克里斯托弗·l·卡马尔乔丹w . Swanson, Sameer Shakir, Alexander M. Tucker, Benjamin C. Kennedy, Phillip B. Storm, Gregory G. Heuer, Scott P. Bartlett, Jesse A. Taylor, and Shih-Shan Lang

Cranial spring hardware is generally removed 3 months after placement for spring-mediated cranioplasty. Spring removal is performed as an outpatient procedure under general anesthesia in approximately 15 minutes through the incision locations of the index procedure. Herein, the authors provide a multimedia demonstration of cranial spring hardware removal after spring-mediated cranioplasty for sagittal craniosynostosis.

The video can be found here:https://vimeo.com/511179695

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