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Medically refractory status epilepticus (RSE) causes high morbidity and mortality in children. There are no evidence-based guidelines for treatment. Epilepsy surgery is a treatment option for RSE. We describe a 9-year-old boy treated successfully for RSE with complete corpus callosotomy (CC). Epilepsy surgery should be considered for prolonged RSE. In the absence of evidence of focal epileptogenesis, complete corpus callosotomy may be effective in select cases.

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OBJECT: The operative indications and treatment algorithms for pediatric patients with Chiari Type I malformation (CM-I) vary widely. When an intradural approach and duraplasty are thought necessary at the time of surgery, neurosurgeons may elect to fulgurate or resect a portion of the cerebellar tonsils. Histological analyses of cerebellar tonsils resected during decompression in pediatric patients with CM-I have revealed multiple abnormal findings including extensive ischemic and degenerative changes. The authors describe an interesting phenomenon of cystic degeneration in the distal ends of the cerebellar tonsils in children undergoing operative treatment of CM-I. METHODS: The authors reviewed the clinical database of 440 pediatric patients who underwent surgical decompression for CM-I performed by a single surgeon. The clinical course, preoperative MR imaging and intraoperative ultrasound characteristics, and histological findings in 3 children found to have tonsillar cystic degeneration were analyzed and detailed. RESULTS: Cystic changes were subtle but uniformly evident on preoperative MR imaging and were more readily apparent on intraoperative ultrasonography. In each patient, the tonsillar cyst was resected. Histological examination revealed areas of cystic degenerative change characterized by distortion of the normal cerebellar architecture with absent Purkinje and internal granular cell layers. All children experienced improvement in their symptoms, without complication, postoperatively. CONCLUSIONS: Cystic degeneration of the tonsils in pediatric patients with CM-I is an uncommon pathological process most likely resulting from long-standing and excessive compression. Based on their experience, the authors advocate expeditious surgical treatment, including intradural exploration and capacious duraplasty, for patients in whom there is evidence of this phenomenon on preoperative imaging.

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Recurrent tethered cord syndrome (TCS) has been reported to develop in 5-50% of patients following initial spinal cord detethering operations. Surgery for multiple recurrences of TCS can be difficult and is associated with significant complications. Using a cadaveric tethered spinal cord model, Grande and colleagues demonstrated that shortening of the vertebral column by performing a 15-25-mm thoracolumbar osteotomy significantly reduced spinal cord, lumbosacral nerve root, and terminal filum tension. Based on this cadaveric study, spinal column shortening by a thoracolumbar subtraction osteotomy may be a viable alternative treatment to traditional surgical detethering for multiple recurrences of TCS. In this article, the authors describe the use of posterior vertebral column subtraction osteotomy (PVCSO) for the treatment of 2 patients with multiple recurrences of TCS. Vertebral column resection osteotomy has been widely used in the surgical correction of fixed spinal deformity. The PVCSO is a novel surgical treatment for multiple recurrences of TCS. In such cases, PVCSO may allow surgeons to avoid neural injury by obviating the need for dissection through previously operated sites and may reduce complications related to CSF leakage. The novel use of PVCSO for recurrent TCS is discussed in this report, including surgical considerations and techniques in performing PVCSO.

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Epidural hematoma (EDH) is a common sequela of head trauma in children. An increasing number are managed nonsurgically, with close clinical and imaging observation. We report the case of a traumatic EDH that spontaneously decompressed into the subgaleal space, demonstrated on serial CT scans that showed resolution of the EDH and concurrent enlargement of the subgaleal hematoma.

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We report a case of a skeletally immature achondroplastic adolescent with significant thoracolumbar lordosis who presented with neurogenic claudication and urinary incontinence progressing over a 1-year period. She underwent decompressive lumbar laminectomy from T12 to L5 with preservation of the facet joints. Over the ensuing 4 years of follow-up, she developed a progressive thoracolumbar kyphosis that progressed to 105 degrees. She remained neurologically intact and was fully ambulatory, but had severe back pain in the area of the deformity. Because of the severity and progression of her deformity, a combined anterior and posterior fusion and stabilization was required. We present this case and a review of the literature on spinal stenosis in achondroplasia and the complication of post-laminectomy kyphosis.

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OBJECT: In this retrospective analysis, the authors report a prospective study in which intraoperative ultrasonography was used to determine the extent of surgery necessary during posterior fossa decompression surgery for Chiari malformation Type I (CM-I) in children. METHODS: Between 1995 and 2003, posterior fossa decompression was performed in 149 patients (mean 5.9 years of age, range 9 months-18 years of age) with CM-I. Of these, 130 underwent intraoperative ultrasonographic evaluation of the craniocervical junction (CCJ) and 15 did not. Four patients with craniosynostosis were excluded from the study. Duraplasty and tonsillar shrinkage were performed when ultrasonographic evidence showed significant decreases in cerebrospinal fluid (CSF) or abnormal tonsillar piston action. Surgical success was determined on the basis of clinical outcome and need for reoperation. One hundred and twenty-four (95.5%) of the children had successful outcomes following surgery and six (4.5%) experienced continued or worsening symptoms requiring reoperation. Forty patients did not undergo duraplasty because the ultrasonography evidence showed adequate decompression with bone removal alone. Of 90 patients with significant compression, decreased CSF dynamics, and/or abnormal tonsillar piston-like action at the CCJ, 85 underwent duraplasty and tonsillar shrinkage and five did not for various reasons. One patient in whom the dura mater was violated accidentally during bone decompression subsequently underwent duraplasty. Hospital stays lasted 6.4 +/- 3.9 days (mean +/- standard deviation) when duraplasty was performed compared with 4.3 +/- 1.1 days when it was not (p < 0.0003). After bone decompression alone, no patient experienced complications. After duraplasty, 12 patients experienced complications and had headaches, nausea, and pain more often than patients who underwent bone decompression alone. Mean tonsillar descent was 11 +/- 4 mm after bone decompression only and 13.9 +/- 4.9 mm after duraplasty, with tonsillar shrinkage (p < 0.0003) seen on magnetic resonance imaging. CONCLUSIONS: In patients who undergo decompressive surgery for CM-I, intraoperative ultrasonography may be a useful tool to aid the surgeon in deciding whether to opt for bone removal only or bone removal plus duraplasty and tonsillar shrinkage.

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OBJECTIVE: Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the formation of hamartomas in multiple organs. Five to 15% of affected individuals display subependymal giant cell astrocytomas, which can lead to substantial neurological and postoperative morbidity due to the production of hydrocephalus, mass effect, and their typical location adjacent to the foramen of Monro. We sought to see whether therapy with oral rapamycin could affect growth or induce regression in astrocytomas associated with TSC. METHODS: Five subjects with clinically definite TSC and either subependymal giant cell astrocytomas (n = 4) or a pilocytic astrocytoma (n = 1) were treated with oral rapamycin at standard immunosuppressive doses (serum levels 5-15 ng/ml) from 2.5 to 20 months. All lesions demonstrated growth on serial neuroimaging studies. Magnetic resonance imaging scans were performed before and at regular intervals following initiation of therapy. RESULTS: All lesions exhibited regression and, in one case, necrosis. Interruption of therapy resulted in regrowth of subependymal giant cell astrocytomas in one patient. Resumption of therapy resulted in further regression. Treatment was well tolerated. INTERPRETATION: Oral rapamycin therapy can induce regression of astrocytomas associated with TSC and may offer an alternative to operative therapy of these lesions.

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OBJECT: The authors identified eight patients with Rubinstein-Taybi syndrome (RTS) and examined how the underlying conditions of this syndrome can impede the detection of symptoms associated with a tethered spinal cord. They also reviewed the literature and did not find any published series describing such an association with this syndrome. METHODS: In this retrospective review, the authors report the diagnosis, treatment, and outcome of tethered spinal cord in eight children (age range 2-14 years) with RTS. The patients presented between 1995 and 2003 with neurogenic bladder, decreased activity, gait disturbance, and low-back pain. The children were examined using whole-spine magnetic resonance (MR) imaging and, in recent years, their spinal cord movement was assessed using cine MR imaging. After undergoing lumbar laminectomy and spinal cord untethering procedures, six patients experienced improvement in symptoms associated with tethered spinal cord such as pain, urinary tract infection, incontinence, spasticity, and neurogenic bladder. CONCLUSIONS: Although some underlying conditions of RTS may mask the symptoms of a tethered spinal cord in children, careful attention to the patient's medical history as provided by his or her parents may aid in early diagnosis, evaluation, and treatment, thus preventing further neurological deterioration and leading to improved outcomes.

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Hemimegalencephaly (HME) is a congenital brain malformation characterized by unilateral enlargement of the cerebral hemisphere. Clinically, HME is typically associated with hemiparesis, psychomotor retardation, and intractable seizures usually apparent soon after birth. HME is often an isolated finding, but it has been described as an occasional feature of a large number of syndromes, many of which may not be readily identified at birth. There are a multitude of case series and reports of HME in the English literature; however, there is no comprehensive, unbiased, detailed survey characterizing the proportion of cases of HME that are associated with a syndrome. We performed a retrospective study of all cases of HME seen at our institution from 1990 to 2003. Of the 15 cases of HME identified, 53% (8/15) were non-syndromic and 47% (7/15) of the cases were associated with a known or suspected genetic syndrome. In patients with syndromic HME, many of the syndromic features were not readily discernible at birth or in early infancy. It is, therefore, imperative to continually evaluate any infant with HME for signs and symptoms of these and other syndromes. Knowing the relative differential diagnosis will lead to a more comprehensive evaluation, improvement in expectant management, and appropriate counseling of families before considering radical surgical options such as hemispherectomy.

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Based on our review of the literature, we present the first use of coiling in an infant with a traumatic artery aneurysm that resulted from shaken baby syndrome. Computed tomography (CT) scans showed a skull fracture, hemorrhagic subdural collections, multiple parenchymal contusions, and intraventricular and subarachnoid hemorrhages in a 3-week-old infant who presented with lethargy, poor feeding, and seizure. These multiple injuries were consistent with shaken baby syndrome. After closed-head injury medical management, including subdural taps, the baby was discharged home. When increasing seizures and hydrocephalus developed 8 months later, CT angiographic scans showed a pseudoaneurysm of the anterior cerebral artery. We successfully occluded the aneurysm with pushable coils placed via a microcatheter and treated the obstructive hydrocephalus with endoscopic third ventriculostomy. We show that minimally invasive radiological and surgical techniques may be effective in managing the sequelae of trauma in children.

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BACKGROUND: Angiography is considered the standard to assess the treatment success of cerebral arteriovenous malformations (AVMs). When postoperative angiograms show neither residual nidus nor early draining veins, patients are considered cured, and the risks of hemorrhage are eliminated. However, this notion is challenged by the development of recurrent AVMs in children. In our report, 2 children developed recurrent AVMs after undergoing complete resection, which was documented by postoperative angiography. We review other similar cases reported in the literature to help establish guidelines for postoperative monitoring. METHODS: In this retrospective review, 36 children underwent complete surgical resection of cerebral AVMs that were documented by intra- or postoperative angiography. After a follow-up period ranging from 1 to 17 years, rates of recurrence were assessed. RESULTS: Although angiographic documentation showed complete resection, 2 children developed recurrences 3 and 5 years later. Among these 36 children, the recurrence rate was 5.5% at follow-up (mean 9 years). CONCLUSIONS: Based on our data and review of the literature, intra- or early postoperative angiography is essential to exclude the presence of a residual nidus but does not ensure cure of the AVMs in children. If postoperative angiographic scans are obtained too early, postoperative changes may prevent the detection of a residual nidus. Therefore, we recommend that angiographic scans be obtained intraoperatively or early postoperatively to document complete resection and again at 1 and 5 years after surgical resection.

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BACKGROUND AND PURPOSE: We herein present our experience in diagnosing and treating 13 children with vein of Galen aneurysmal malformations (VGAM), with an emphasis on possible prognostic indicators, endovascular strategies, factors affecting treatment during the neonatal period, and long-term follow-up. With this review, we hope to identify those factors that have the most significant prognostic value in determining long-term outcomes in children with VGAM. METHODS: We retrospectively reviewed the radiology studies, hospital charts, and outpatient clinic chart notes (when applicable) of 13 children evaluated and treated for VGAM at a single tertiary care pediatric hospital. Clinical presentation, diagnostic methods, treatment strategies, and outcome were documented for each child. The present neurologic status and level of function of each patient was determined by review of the outpatient charts and direct contact with the clinicians who were conducting the follow-up. Outcome was graded on a 5-point scale, ranging from 0 (death) to 4 (normal), taking into account only neurologic and developmental characteristics. RESULTS: Eight of 13 patients presented as neonates with congestive heart failure. The other five patients ranged in age from 4 months to 13 years at the time of presentation. The five patients presenting outside of the neonatal period achieved normal or near-normal outcomes. Two of the eight patients presenting during the neonatal period achieved normal or near-normal outcomes, one experienced significant impairment, and the other five died. We were unable to identify significant differences in outcome on the basis of differences in treatment strategies. CONCLUSION: Our experience confirms that children with VGAM presenting during the neonatal period have a generally much worse prognosis than do those presenting later in childhood. Complicating factors in the management and treatment of these children are discussed in light of their impact on outcome.

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