Faculty Bibliography

OBJECTIVE: Transient paraplegia in the immediate postoperative period after lipomyelomeningocele repair is uncommon and is not discussed in the literature. We present the unique case of a patient who developed transient paraplegia 48 hours after lipomyelomeningocele repair attributable to the acute development of a thoracic syrinx. CLINICAL PRESENTATION: At birth, the patient was noted to have a large skin-covered mass in the lumbosacral region. On neurological examination, both iliopsoas and quadriceps exhibited 3/5 motor function, and the plantar flexors and dorsiflexors exhibited 1/5 motor function. Urodynamic studies were normal. Magnetic resonance imaging demonstrated the presence of a lipomyelomeningocele associated with tethering of the spinal cord in the lumbosacral region. INTERVENTION: At 5 months of age, the patient underwent repair of the lipomyelomeningocele. After surgery, the patient developed progressive paraplegia along with bowel and bladder dysfunction. Given the concern about a postoperative hematoma resulting in cauda equina syndrome, the patient returned to the operating room for a wound exploration. No compressive lesion such as a hematoma was found at surgery. A postoperative magnetic resonance imaging scan obtained afterward, however, demonstrated the presence of a large thoracic syrinx. CONCLUSION: Syrinx formation can occur as early as 48 hours after lipomyelomeningocele repair, leading to progressive lower extremity weakness and bowel and bladder incontinence. In the immediate postoperative period, an acute syrinx can mimic cauda equina syndrome, and a magnetic resonance imaging scan is necessary to distinguish between these two entities. In this patient, the syrinx was transient and resolved without a shunting procedure

OBJECT: Tectal gliomas are a distinct form of pediatric brainstem tumor that present in patients with symptoms related to increased intracranial pressure due to obstructive hydrocephalus. The natural history of these lesions is often uniquely indolent. Thus, initial surgical therapies are directed at treatment of hydrocephalus, usually with ventricular shunt placement. Recently, third ventriculostomy has been used in patients with tectal gliomas, both as an initial procedure and after shunt failures. In this report the authors review their experience with the treatment of hydrocephalus in patients with tectal gliomas. METHODS: The authors reviewed 31 consecutive cases of tectal gliomas and compared the success rates of ventricular shunt placement with the success rates of endoscopic third ventriculostomy (ETV). Shunt placement procedures were associated with a significant number of malfunctions, and most patients required shunt revisions. The ETV procedure was attempted both as an initial treatment and after shunt malfunction. Overall, ETV was attempted in 18 patients and was performed successfully in all cases. At the time of follow-up evaluation, 16 patients (89%) were shunt free. CONCLUSIONS: The authors found that ETV could be performed with good long-term success both as an initial treatment and after shunt failure. Overall, ETV was found to be superior to ventricular shunt placement in the management of hydrocephalus associated with tectal gliomas.

PURPOSE: Clinicians often assume that children with posterior fossa tumors are at minimal risk for cognitive or adaptive deficits if they do not undergo cranial irradiation. However, small case series have called that assumption into question, and have also suggested that nonirradiated cerebellar tumors can cause location-specific cognitive and adaptive impairment. This study (1) assessed whether resected but not irradiated pediatric cerebellar tumors are associated with cognitive and adaptive functioning deficits, and (2) examined the effect of tumor location and medical complications on cognitive and adaptive functioning. PATIENTS AND METHODS: The sample was composed of 103 children aged 3 to 18 years with low-grade cerebellar astrocytomas, who underwent only surgical treatment as part of Children's Cancer Group protocol 9891 or Pediatric Oncology Group protocol 9130. The sample was divided into three groups based on primary tumor location: vermis, left hemisphere, or right hemisphere. Data were collected prospectively on intelligence, academic achievement, adaptive skills, behavioral functioning, and pre-, peri-, and postsurgical medical complications. RESULTS: The sample as a whole displayed an elevated risk for cognitive and adaptive impairment that was not associated consistently with medical complications. Within this group of children with cerebellar tumors, tumor location had little effect on cognitive, adaptive, or medical outcome. CONCLUSION: We did not replicate previous findings of location-specific effects on cognitive or adaptive outcome. However, the elevated risk of deficits in this population runs contrary to clinical lore, and suggests that clinicians should attend to the functional outcomes of children who undergo only surgical treatment for cerebellar tumors