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Frederick A. Boop

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John H. Chi, Heather J. Fullerton and Nalin Gupta

Object

Congenital hydrocephalus has an estimated population incidence of 0.2 to 0.8/1000 live births. With improvements in techniques for cerebrospinal fluid shunting, treatment of hydrocephalus has become safe and routine, yet data describing mortality from congenital hydrocephalus or demonstrating improvements in mortality with the advent of modern treatment are scarce. The authors' analysis sought to rectify this situation.

Methods

The authors performed an electronic search of National Center for Health Statistics death certificate databases to identify deaths from 1979 to 1998 attributed to congenital hydrocephalus, spina bifida with hydrocephalus, and acquired hydrocephalus (both obstructive and communicating) in all children in the US aged 1 day to 20 years. Mortality rates were defined as deaths per 100,000 person-years and were analyzed for differences on the basis of age, race, sex, and year.

The authors identified 10,406 deaths attributed to childhood hydrocephalus within the 20-year study period. The overall mortality rate was 0.71 per 100,00 person-years. Mortality rates were highest in infants, with 3979 deaths; they were similar between girls and boys. Compared with white infants, black infants had higher relative risk (RR) for death caused by congenital hydrocephalus (RR 1.46, p value < 0.0001) and acquired hydrocephalus (RR 2.58, p value < 0.0001) but not for that caused by hydrocephalus with spina bifida (RR 0.65, p value < 0.0001). From 1979 to 1998, the mortality rate due to congenital hydrocephalus declined 66.3%, from spina bifida with hydrocephalus it declined by 30.4%, and from acquired hydrocephalus it declined by 67.5%.

Conclusions

Mortality rates from childhood hydrocephalus have declined in US children over the previous 20 years. Black race is associated with higher mortality rates in infants for both congenital and acquired hydrocephalus, whereas sex has no effect.

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Mark W. Kieran, Liliana C. Goumnerova, Michael Prados and Nalin Gupta

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Marcus L. Ware, Soonmee Cha, Nalin Gupta and Victor L. Perry

✓ This case illustrates the potential growth rate of an atypical meningioma in a pediatric patient, emphasizes one of the potential risks after therapeutic radiation, and underscores the importance of clinical evaluation and follow up of the symptomatic patient after tumor resection and radiation therapy. The authors report a case of a radiation-induced atypical meningioma of the olfactory groove in a 13-year-old girl who received 36 Gy of radiation to the craniospinal axis and 72 Gy to the primary site of a primitive neuroectodermal epithelial tumor of the left parietooccipital lobe when she was 4 years of age. This tumor was not present on routine magnetic resonance imaging performed 13 months prior to the discovery of this lesion or on computerized tomography scanning obtained 6 months prior to the discovery of this tumor. At the time of its discovery, the tumor was 5 × 5 × 4 cm. This tumor was resected and the patient's symptoms improved. This case illustrates the importance of continued close follow up after cranial irradiation in the pediatric population.

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Paul Steinbok, Hugh J. L. Garton and Nalin Gupta

Object

Tethered cord syndrome (TCS) is associated with a number of congenital anomalies involving early development of the spinal cord. These include myelomeningocele, spinal cord lipoma, low-lying conus medullaris, and a fibrofatty terminal filum. Occult TCS occurs in patients when clinical features indicate a TCS but the typical anatomical abnormalities are lacking. It is controversial whether surgical release of the terminal filum leads to clinical improvement in a patient who does not have a previously identified anatomical abnormality. To assess the clinical standard used by practicing pediatric neurosurgeons, a practice survey was conducted at the 2004 Annual Meeting of the Joint Section for Pediatric Neurological Surgery of the American Association of Neurological Surgeons/Congress of Neurological Surgeons.

Methods

The survey examined clinical decision making for a same-case scenario with differing appearance on imaging studies. There was a clear consensus regarding diagnosis and treatment in the patient with symptoms, a low-lying conus medullaris, and a fatty terminal filum. The vast majority of respondents (85%) favored surgical untethering for this patient. A majority of respondents (67%) also favored treatment for the patient having symptoms and a fatty terminal filum. There was, however, significant disagreement regarding the diagnosis and treatment of disease in one patient with symptoms and an inconclusive magnetic resonance imaging study. Some respondents clearly favored surgery, whereas others believed that this patient did not meet the diagnostic criteria for TCS.

Conclusions

The results of this survey support the development of a randomized clinical trial to address the benefit of surgery for occult TCS.

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Daniel C. Lu, Nalin Gupta and Praveen V. Mummaneni

Surgical decompression of the vertebral artery (VA) between the suboccipital area and C-1 is typically performed via a large midline incision or a far-lateral approach. Such traditional open approaches are often associated with significant muscle dissection and blood loss. In this case, a 12-year-old boy suffered a stroke related to a VA rotational occlusion (bow hunter syndrome) and dissection due to a prominent suboccipital bone mass. Successful decompression of the VA was performed via a minimally invasive 22-mm tubular retractor. This is the first reported case report of a minimally invasive decompression of the VA between the skull base and C-1.

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Lynn M. Myles, Nalin Gupta, Derek Armstrong and James T. Rutka

✓ Symptomatic arachnoid cysts of the spine are uncommon in children and have only rarely been reported to occur extradurally. The authors report a case of multiple extradural spinal arachnoid cysts in a 9-year-old child who presented with signs of spinal cord compression. The extent of the disease, which affected the thoracic, lumbar, and sacral spine, and the number of independent cysts make this case unique and suggest an underlying defect in the dura of the spinal canal that is predisposed to the formation of cysts. The investigations of choice, surgical planning, and surgical technique are considered. The literature is reviewed and mechanisms of cyst formation discussed.

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James M. Drake

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Phiroz E. Tarapore, Michael E. Sughrue, Lewis Blevins, Kurtis I. Auguste, Nalin Gupta and Sandeep Kunwar

Object

Pituitary adenomas are uncommon in childhood. Although medical treatment can be effective in treating prolactinomas and some growth hormone (GH)–secreting tumors, resection is indicated when visual function is affected or the side effects of medical therapy are intolerable. The authors of this report describe their 10-year experience in managing pituitary adenomas via the microscopic endonasal transsphenoidal approach in a pediatric population.

Methods

They performed a retrospective review of a surgical case series based at a single institution and consisting of 34 consecutive pediatric patients with endocrine-active (32 patients) and endocrine-inactive (2 patients) adenomas. These patients were surgically treated via an endonasal transsphenoidal approach between 1999 and 2008. Patient charts were reviewed, and clinical data were compiled and analyzed using the chi-square and Kaplan-Meier tests.

Results

The patient cohort consisted of 20 girls and 14 boys, with ages ranging from 9 to 18 years and a median age of 16 years. Thirty-two patients (94%) underwent surgery for endocrine-active tumors, including 10 (29%) with Cushing disease, 21 (62%) with prolactinomas, and 1 (3%) with GH-secreting tumors. Two patients with nonsecreting adenomas underwent surgery for apoplexy. The mean tumor volume was 5.4 cm3, and 13 patients (38%) had suprasellar extension and 7 (21%) had cavernous sinus invasion. Gross-total resection was achieved in 26 patients (76%), although it was significantly less likely to be achieved in the setting of cavernous sinus invasion (p < 0.001) but was unaffected by suprasellar extension. Residual tumor was treated with radiation therapy in 6 patients (18%). The average duration of hospital stay was 1.6 days. The median follow-up time was 18 months. After surgery, 19 patients (56%) had normal hormone function without adjuvant therapy, 8 (24%) had normal function with adjuvant therapy, and 5 (15%) had persistently elevated hormone levels. Patients with a macroprolactinoma were significantly more likely to require postoperative adjuvant therapy than were those with a microprolactinoma (p < 0.03).

Conclusions

Endonasal transsphenoidal resection is a safe, well-tolerated, and potentially curative treatment option for pituitary adenomas in children. Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity. Endocrine function is usually preserved, and the majority of patients will not require lifelong medical therapy.

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Nalin Gupta, Jody A. Farrell, Larry Rand, Charles B. Cauldwell and Diana Farmer

In a recently completed randomized, prospective clinical trial, fetal repair for myelomeningocele was shown to result in reduced rates of hydrocephalus requiring placement of a ventriculoperitoneal shunt, improvement in Chiari malformation Type II, and improvement in neurological function compared with standard postnatal repair. Successful fetal surgery requires the active participation and interaction of several clinical teams. Each group has a specific role, and overlap is often required at different points of the treatment plan. Extensive multispecialty discussions with the patient and family are necessary before informed consent can be obtained. Fetal surgery carries significant risks to the mother and fetus and these must be carefully considered prior to a final treatment decision. This review will summarize the evaluation and treatment of patients undergoing fetal repair for myelomeningocele at one institution.