Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a rare, recently described WHO Grade I neoplasm. The authors report 6 examples of RGNT arising primarily from the cerebellar vermis. All the patients were female, and the mean age of presentation was 24.8 years. The original diagnoses included pilocytic astrocytoma, ependymoma, cerebellar dysembryoplastic neuroepithelial tumor (DNT), and oligodendroglioma. The cases showed classic pathological characteristics, although in 2 cases the lesions included DNT-like “floating neurons” involving Purkinje cells, a feature which has not been previously reported to the authors' knowledge. The clinical outcome was excellent with no recurrences after complete resection. These cases expand the known clinical and histological spectrum of this rare tumor type. Given the lack of fourth ventricle involvement in most of these cases, the authors suggest revising the name to RGNT of the posterior fossa.
Report of 6 cases
Manish N. Shah, Jeffrey R. Leonard and Arie Perry
Justin M. Brown, Manish N. Shah and Susan E. Mackinnon
Peripheral nerve injuries can result in devastating numbness and paralysis. Surgical repair strategies have historically focused on restoring the original anatomy with interposition grafts. Distal nerve transfers are becoming a more common strategy in the repair of nerve deficits as these interventions can restore function in months as opposed to more than a year with nerve grafts. The changes that take place over time in the cell body, distal nerve, and target organ after axotomy can compromise the results of traditional graft placement and may at times be better addressed with the use of distal nerve transfers. A carefully devised nerve transfer offers restoration of function with minimal (if any) detectable deficits at the donor site. A new understanding of cortical plasticity along with patient reeducation allow for good return of strength and function after nerve transfer.
Manish Kuchakulla, Ashish H. Shah, Valerie Armstrong, Sarah Jernigan, Sanjiv Bhatia and Toba N. Niazi
Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature.
The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007–2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications.
In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors’ series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors’ illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner’s syndrome after embolization.
Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children.
Manish N. Shah, Alex A. Kane, J. Dayne Petersen, Albert S. Woo, Sybill D. Naidoo and Matthew D. Smyth
This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis.
Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed.
There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery.
Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.
Manish N. Shah, Ivan T. Stoev, Dominic E. Sanford, Feng Gao, Paul Santiago, David P. Jaques and Ralph G. Dacey Jr.
The goal of this study was to examine the reasons for early readmissions within 30 days of discharge to a major academic neurosurgical service.
A database of readmissions within 30 days of discharge between April 2009 and September 2010 was retrospectively reviewed. Clinical and administrative variables associated with readmission were examined, including age, sex, race, days between discharge and readmission, and insurance type. The readmissions were then assigned independently by 2 neurosurgeons into 1 of 3 categories: scheduled, adverse event, and unrelated. The adverse event readmissions were further subcategorized into patients readmitted although best practices were followed, those readmitted due to progression of their underlying disease, and those readmitted for preventable causes. These variables were compared descriptively.
A total of 348 patients with 407 readmissions were identified, comprising 11.5% of the total 3552 admissions. The median age of readmitted patients was 55 years (range 16–96 years) and patients older than 65 years totaled 31%. There were 216 readmissions (53% of 407) for management of an adverse event that was classified as either preventable (149 patients; 37%) or unpreventable (67 patients; 16%). There were 113 patients (28%) who met readmission criteria but who were having an electively scheduled neurosurgical procedure. Progression of disease (48 patients; 12%) and treatment unrelated to primary admission (30 patients; 7%) were additional causes for readmission. There was no significant difference in the proportion of early readmissions by payer status when comparing privately insured patients and those with public or no insurance (p = 0.09).
The majority of early readmissions within 30 days of discharge to the neurosurgical service were not preventable. Many of these readmissions were for adverse events that occurred even though best practices were followed, or for progression of the natural history of the neurosurgical disease requiring expected but unpredictably timed subsequent treatment. Judicious care often requires readmission to prevent further morbidity or death in neurosurgical patients, and penalties for readmission will not change these patient care obligations.
Gregory J. Zipfel, Manish N. Shah, Daniel Refai, Ralph G. Dacey Jr. and Colin P. Derdeyn
This article presents a modification to the existing classification scales of intracranial dural arteriovenous fistulas based on newly published research regarding the relationship of clinical symptoms and outcome. The 2 commonly used scales, the Borden-Shucart and Cognard scales, rely entirely on angiographic features for categorization. The most critical anatomical feature is the identification of cortical venous drainage (CVD; Borden-Shucart Types II and III and Cognard Types IIb, IIa + b, III, IV, and V), as this feature identifies lesions at high risk for future hemorrhage or ischemic neurological injury. Yet recent data has emerged indicating that within these high-risk groups, most of the risk for future injury is in the subgroup presenting with intracerebral hemorrhage or nonhemorrhagic neurological deficits. The authors have defined this subgroup as symptomatic CVD. Patients who present incidentally or with symptoms of pulsatile tinnitus or ophthalmological phenomena have a less aggressive clinical course. The authors have defined this subgroup as asymptomatic CVD. Based on recent data the annual rate of intracerebral hemorrhage is 7.4–7.6% for patients with symptomatic CVD compared with 1.4–1.5% for those with asymptomatic CVD. The addition of asymptomatic CVD or symptomatic CVD as modifiers to the Borden-Shucart and Cognard systems improves their accuracy for risk stratification of patients with high-grade dural arteriovenous fistulas.
Scott L. Zuckerman, Ilyas M. Eli, Manish N. Shah, Nadine Bradley, Christopher M. Stutz, Tae Sung Park and John C. Wellons III
Axillary nerve palsy, isolated or as part of a more complex brachial plexus injury, can have profound effects on upper-extremity function. Radial to axillary nerve neurotization is a useful technique for regaining shoulder abduction with little compromise of other neurological function. A combined experience of this procedure used in children is reviewed.
A retrospective review of the authors' experience across 3 tertiary care centers with brachial plexus and peripheral nerve injury in children (younger than 18 years) revealed 7 cases involving patients with axillary nerve injury as part of an overall brachial plexus injury with persistent shoulder abduction deficits. Two surgical approaches to the region were used.
Four infants (ages 0.6, 0.8, 0.8, and 0.6 years) and 3 older children (ages 8, 15, and 17 years) underwent surgical intervention. No patient had significant shoulder abduction past 15° preoperatively. In 3 cases, additional neurotization was performed in conjunction with the procedure of interest. Two surgical approaches were used: posterior and transaxillary. All patients displayed improvement in shoulder abduction. All were able to activate their deltoid muscle to raise their arm against gravity and 4 of 7 were able to abduct against resistance. The median duration of follow-up was 15 months (range 8 months to 5.9 years).
Radial to axillary nerve neurotization improved shoulder abduction in this series of patients treated at 3 institutions. While rarely used in children, this neurotization procedure is an excellent option to restore deltoid function in children with brachial plexus injury due to birth or accidental trauma.
Manish N. Shah, Jeffrey R. Leonard, Gabrielle Inder, Feng Gao, Michael Geske, Devon H. Haydon, Melvin E. Omodon, John Evans, Diego Morales, Ralph G. Dacey, Matthew D. Smyth, Michael R. Chicoine and David D. Limbrick
This study describes the pediatric experience with a dual-multifunction-room IMRIS 1.5-T intraoperative magnetic resonance imaging (iMRI) suite and analyzes its impact on clinical variables associated with neurosurgical resection of intracranial lesions, including safety and efficacy.
Since the inception of the iMRI–guided resection program in April 2008 at both Barnes-Jewish and St. Louis Children's Hospital, a prospective database recorded the clinical variables associated with demographics and outcome with institutional review board approval. A similarly approved retrospective database was constructed from February 2006 to March 2010 for non–iMRI resections. These databases were retrospectively reviewed for clinical variables associated with resection of pediatric (age 20 months–21 years) intracranial lesions including brain tumors and focal cortical dysplasia. Patient demographics, operative time, estimated blood loss, additional resection, length of stay, pathology, and complications were analyzed.
The authors found that 42 iMRI–guided resections were performed, whereas 103 conventional resections had been performed without the iMRI. The mean patient age was 10.5 years (range 20 months–20 years) in the iMRI group and 9.8 years (range 2–21 years) in the conventional group (p = 0.41). The mean duration of surgery was 350 minutes in the iMRI group and 243 minutes in the conventional group (p < 0.0001). The mean hospital stay was 8.2 days in the iMRI group, and 6.6 days in the conventional group, and this trended toward significance (p = 0.05). In the first 2 weeks postoperatively, there were 8 reoperations (7.77%) in the conventional group compared with none in the iMRI group, which was not significant in a 2-tailed test (p = 0.11) but trended toward significance in a 1-tailed test (p = 0.06). The significant complications included reoperation for hydrocephalus or infection: 6.8% (conventional) versus 4.8% (iMRI).
Intraoperative MR imaging–guided resections resulted in a trend toward reduction in the need for repeat surgery in the immediate 2-week postoperative period compared with conventional pediatric neurosurgical resections for tumor or focal cortical dysplasia. Although there is an increased operative time, the iMRI suite offers a comparable safety and efficacy profile while potentially reducing the per-case cost by diminishing the need for early reoperation.
Manish N. Shah, James A. Botros, Thomas K. Pilgram, Christopher J. Moran, DeWitte T. Cross III, Michael R. Chicoine, Keith M. Rich, Ralph G. Dacey Jr., Colin P. Derdeyn and Gregory J. Zipfel
The goal of this study was to determine the clinical course of Borden-Shucart Type I cranial dural arteriovenous fistulas (DAVFs) and to calculate the annual rate of conversion of these lesions to more aggressive fistulas that have cortical venous drainage (CVD).
A retrospective chart review was conducted of all patients harboring DAVFs who were seen at the authors' institution between 1997 and 2009. Twenty-three patients with Type I DAVFs who had available clinical follow-up were identified. Angiographic and clinical data from these patients were reviewed. Neurological outcome and status of presenting symptoms were assessed during long-term follow-up.
Of the 23 patients, 13 underwent endovascular treatment for intolerable tinnitus or ophthalmological symptoms, and 10 did not undergo treatment. Three untreated patients died of unrelated causes. In those who were treated, complete DAVF obliteration was achieved in 4 patients, and palliative reduction in DAVF flow was achieved in 9 patients. Of the 19 patients without radiographic cure, no patient developed intracranial hemorrhage or nonhemorrhagic neurological deficits (NHNDs), and no patient died of DAVF-related causes over a mean follow-up of 5.6 years. One patient experienced a spontaneous, asymptomatic obliteration of a partially treated DAVF in late follow-up, and 2 patients experienced a symptomatic conversion of their DAVF to a higher-grade fistula with CVD in late follow-up. The annual rate of conversion to a higher-grade DAVF based on Kaplan-Meier cumulative event-free survival analysis was 1.0%. The annual rate of intracranial hemorrhage, NHND, and DAVF-related death was 0.0%.
A small number of Type I DAVFs will convert to more aggressive DAVFs with CVD over time. This conversion to a higher-grade DAVF is typically heralded by a change in patient symptoms. Follow-up vascular imaging is important, particularly in the setting of recurrent or new symptoms.
Grant M. Fischer, Elmira Vaziri Fard, Manish N. Shah, Rajan P. Patel, Gretchen Von Allmen, Leomar Y. Ballester and Meenakshi B. Bhattacharjee
Although rare, hyaline cytoplasmic inclusions isolated to astrocytes of the cerebral cortex have been identified in a spectrum of diseases ranging from intractable epilepsy in pediatric patients with only mild to moderate developmental delays to Aicardi syndrome. These inclusions classically stain positive for filamin A, giving rise to the term “filaminopathies.” The authors report on 2 pediatric patients with intractable epilepsy and developmental delay who uniquely displayed filamin A–negative hyaline astrocytic inclusions in resected brain tissues. Additionally, these inclusions stained positive for S100 and negative for glial fibrillary acidic protein, chromogranin, neurofilament, CD34, vimentin, periodic acid–Schiff (PAS), and Alcian blue. These are the first reported cases of filamin A–negative hyaline astrocytic inclusions, providing a novel variation on a previously reported entity and justification to further investigate the pathogenesis of these inclusions. The authors compare their findings with previously reported cases and review the literature on hyaline astrocytic inclusions in intractable pediatric epilepsy.