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Frederick A. Boop

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

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W. Jerry Oakes

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


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.


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.


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


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.


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%.


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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Gyang Markus Bot, Jan-Karl Burkhardt, Nalin Gupta and Michael T. Lawton


Revascularization is indicated in the management of moyamoya disease (MMD), with options that include direct and indirect techniques. Indirect bypass is popular in young children because the diminutive caliber of donors and recipients makes direct bypass difficult. The authors reviewed a series of patients treated with direct superficial temporal artery (STA)–to–middle cerebral artery (MCA) bypass in combination with encephalomyosynangiosis (EMS) in children 3 years or younger to demonstrate feasibility and safety.


A retrospective review of all surgeries for MMD over a 19-year period identified 11 procedures in 6 patients. Surgical results, angiographic outcomes, and clinical outcomes were analyzed.


Patients had a mean age of 22.4 months. The symptomatic hemisphere was revascularized first, and the contralateral hemisphere was revascularized on average 2.8 months later in 5 patients. All direct bypasses were patent postoperatively and remained patent at late follow-up (mean 4.1 years), with both STA and MCA diameters increasing significantly (n = 5, p < 0.03). At last follow-up (mean follow-up duration, 5.0 years), favorable outcomes (modified Rankin Scale scores 0–2) were observed in 5 of the 6 patients (83%), with 1 dependent patient remaining unchanged postoperatively.


Direct STA-MCA bypass in combination with EMS for MMD is feasible and safe in patients 3 years or younger, based on favorable clinical and radiological outcomes in this patient cohort. Direct bypass should be considered when immediate revascularization is needed, without the biological delay associated with indirect bypass.

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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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Ronit Agid and Karel Terbrugge