Jeffrey H. Wisoff, Rick Abbott, and Fred Epstein
✓ Sixteen children underwent 18 operations for radical resection of chiasmatic-hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with macrocephaly, failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had precocious puberty with mild visual deficits; and older children (> 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive tumors in spite of low-grade histology, and died from progressive tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of disease progression, 4 months to 4½ years following surgery. Six of these patients, with a follow-up period of 10 months to 4½ years (mean 27 months), have had no adjuvant therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-hypothalamic tumors of infancy are aggressive neoplasms that require multimodality therapy.
George I. Jallo, Karl F. Kothbauer, and I. Rick Abbott
The traditional treatment for all forms of hydrocephalus has been the implantation of ventricular shunt systems; however, these systems have inherent tendencies toward complications such as malfunction and infection. A significant advance in the treatment of hydrocephalus has been the evolution of endoscopy. The recent technological advances in this field have led to a renewed interest in endoscopic third ventriculostomy as the treatment of choice for obstructive hydrocephalus. Although several different endoscopes are available, the authors favor a rigid one to perform a blunt fenestration of the third ventricle floor. This description of the technique stresses the nuances for successful completion of this procedure.
Lauren Weintraub, Todd Miller, Ilana Friedman, Rick Abbott, and Adam S. Levy
The screening and detection of recurrent medulloblastoma presents the clinician with significant diagnostic challenges, including the risk of misdiagnosis. The authors present the case of a young girl with a history of a treated standard-risk medulloblastoma that highlights the risk of assuming recurrence has occurred when clinical and/or imaging changes are observed. This girl developed both new clinical deficits and had radiographic evidence of recurrence. She subsequently experienced a complete resolution of symptoms and radiographic findings with steroids alone.
Mark Lee, Ali R. Rezai, Rick Abbott, Daniel H. Coelho, and Fred J. Epstein
✓ Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.
George I. Jallo, Karl Kothbauer, Vikas Mehta, Rick Abbott, and Fred Epstein
Meningioangiomatosis is an uncommon clinical entity. This lesion has been reported at time of autopsy in patients with neurofibromatosis (NF) and in case reports of patients without NF Type 2 (NF2). The authors report a series of six patients with meningioangiomatosis who do not have NF2 and describe the clinical presentation, diagnosis of disease, and treatment. They also review the literature concerning this entity.
Six patients with meningioangiomatosis were treated at the authors' institutions from 1994 to 2001. The mean age of the patients was 10.7 years (range 5–14 years). All of the children presented with a seizure disorder. Surgery was performed in all children, and a gross-total resection was accomplished.
All patients exhibited clinical improvement. At last follow up (mean 6.3 years) all patients are free of seizures and are not taking anticonvulsant medications. No signs of recurrence have been noted on imaging studies.
The authors advocate a gross-total resection of meningioangiomatosis for the treatment of seizure disorder in this population.
Sean M. Lew, Joseph N. Morgan, Estee Psaty, Daniel R. Lefton, Jeffrey C. Allen, and Rick Abbott
The goal of this study was to determine the incidence of radiation-induced cavernomas in children treated for medulloblastoma.
A retrospective chart and film review was performed for all patients treated for medulloblastoma at the Insitute for Neurology and Neurosurgery/Beth Israel Medical Center between August 1996 and the present. The clinical and radiographic histories of pediatric patients (ages 3–21 years at diagnosis) with a histologically confirmed diagnosis of medulloblastoma who received craniospinal radiation therapy were reviewed.
Fifty-nine patients were identified, with a mean age at radiation treatment of 7.7 years and a mean follow-up time of 7.2 years. The dose to the craniospinal axis was 24 Gy (31 patients) or 36 Gy (28 patients). The radiation energy in the craniospinal axis was provided by photons in 55 patients and protons in four. All patients received a posterior fossa boost of 54 Gy (46 patients) or 72 Gy (13 patients). Twenty-six lesions developed in 18 patients (31%) during the observation period. The cumulative incidence of lesion development was 5.6, 14, and 43%, at 3, 5, and 10 years, respectively. The sites of occurrence were cerebral (20 cases) and cerebellar (six cases). There was no significant correlation between age at diagnosis, sex, craniospinal radiation dose or energy source, and lesion development. Only one patient required surgical intervention for a symptomatic hemorrhagic lesion in the frontal lobe. Histological analysis in this case was consistent with cavernoma.
Cavernomas are common after cranial irradiation in children, and their incidence increases over time. Most of these lesions follow a benign course and do not require intervention.
Pulak Ray, George I. Jallo, R. Y. H. Kim, Bong-Soo Kim, Sean Wilson, Karl Kothbauer, and Rick Abbott
Endoscopic third ventriculostomy (ETV) has become a common alternative for managing hydrocephalus in select patients. Nevertheless, there is still controversy regarding the indications for ETV as the primary procedure, given its variable success rates. The purpose of this study is to review the authors' experience with ETV for a variety of patients.
A total of 43 children underwent ETV between July 1992 and June 2003. Their medical records, operative reports, and imaging studies, when available, were retrospectively reviewed with regard to outcome, complications, and patency rate. Treatment failure was defined as the need to place a shunt within 4 weeks of performing ETV in the patient.
There were 20 male and 23 female patients with a mean age of 9.6 years (range 8 weeks–21 years). The overall success rate was 69.8%, and the mean follow-up duration was 24.6 months. Six patients underwent eight repeated ETVs at a mean interval of 25 months, with a patency rate of 62.5% after the second procedure. Only two surgeries were aborted for anatomical reasons. The highest success rates (100% in each instance) were achieved for obstructive hydrocephalus resulting from midbrain/tectal tumor (four patients) and pineal tumor (three patients).
The ETV procedure is an effective management tool for obstructive hydrocephalus in children. It should be considered the primary procedure, rather than ventriculoperitoneal shunts, in carefully selected children. The success rate is dependent on the origin of the hydrocephalus.