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BACKGROUND: Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection. METHODS: Between October 2003 and April 2005, 42 consecutive patients with refractory epilepsy who underwent endoscopic resection of HH were studied prospectively. The endoscope was held by an articulated pneumatic arm and tracked with a frameless stereotactic neuronavigation system. Data collection and follow-up were performed by personal interview. Five patients were excluded. The remaining 37 patients (22 males, 15 females; median age 11.8 years; range 8 months to 55 years) had frequent and usually multiple types of seizures. RESULTS: Postoperative MRI confirmed 100% resection of the HH from the hypothalamus in 12 patients. At last follow-up (median 21 months; range 13-28 months), 18 (48.6%) patients were seizure free. Seizures were reduced more than 90% in 26 patients (70.3%) and by 50% to 90% in 8 patients (21.6%). Overall, the mean postoperative stay was shorter in the endoscopic patients compared with our previously reported patients who underwent transcallosal resection (mean 4.1 days vs 7.7 days, respectively; p = 0.0006). The main complications were permanent short-term memory loss in 3 patients and small thalamic infarcts in 11 patients (asymptomatic in 9). CONCLUSIONS: Endoscopic resection of hypothalamic hamartoma (HH) is a safe and effective treatment for seizures. Its efficacy seems to be comparable to that of transcallosal resection of HH, but postoperative recovery time is significantly shorter.

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Posttraumatic syringomyelia as a cause of progressive neurologic deterioration has been well described. More recently, the noncystic posttraumatic tethered cord has been associated with identical progressive neurologic deterioration. A retrospective analysis of patients treated surgically with spinal cord untethering and/or cyst shunting to arrest a progressive myelopathy from a posttraumatic tethered and/or cystic cord was performed. Emphasis was on outcome using the American Spinal Injury Association (ASIA) sensory and motor scoring systems. During an 18-month period from May 1993 to December 1994, 70 patients with spinal cord injury were operated upon for tethered and/or cystic spinal cords because of a progressive myelopathy and deteriorating ASIA sensory/motor scores. Fifty-nine patients had follow-up data 1 year postoperatively. At the 1 year follow-up, there was small improvement in light touch sensory scores (0.67 points), pinprick scores (1.3 points), and motor scores (0.41 points) demonstrating that the progression of the myelopathic process was arrested. Thirty-four of these 59 patients had no previous surgery to their spinal cords. At 1 year follow-up, light touch scores improved on average 2.38 points, pinprick scores 3.88 points (p < 0.05), and motor scores 1.47 points, suggesting better outcome with first-time surgery. Of this latter group, 64.3% regained a lost function, 62.5% saw improvement in spasticity, 55.6% had substantial improvement in neurogenic pain, and 95.8% felt that surgery prevented further neurologic deterioration.

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OBJECTIVE: To review the clinical features, radiographic findings, and programming strategies used in our population of patients who developed facial nerve stimulation after cochlear implantation. STUDY DESIGN AND SETTING: Patients referred to our nonprofit, outpatient facility were studied prospectively. PATIENTS: The study consisted of 14 patients with facial nerve stimulation after placement of the Nucleus 22-channel cochlear implant. INTERVENTIONS: Records were reviewed retrospectively, and patients were studied with three-dimensional computed tomographic scanning techniques. Electrical testing was performed, and various cochlear implant programming strategies were evaluated. MAIN OUTCOME MEASURES: Important clinical features were reviewed. The radiographic and anatomical relationships of the facial nerve to the cochlea were evaluated, and the programming strategies used to effectively control facial nerve stimulation were reviewed. RESULTS: Prevalence of facial nerve stimulation in our population was 7%. The most common cause was otosclerosis. Anatomical data confirmed the close proximity of the basal turn of the cochlea and the labyrinthine segment of the facial nerve. There was a high correlation between the electrodes causing symptoms and those found radiographically to be closest to the labyrinthine segment of the facial nerve. We were able to control facial nerve stimulation in all patients through programming mode changes. CONCLUSIONS: Otosclerosis appears to be a risk factor for developing facial nerve stimulation after cochlear implantation, and the site of stimulation appears to be the labyrinthine segment of the facial nerve. Familiarity with more elaborate programming techniques is critical to managing patients with this complication.

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Two patients with congenital dermoids of the eustachian tube presented with recurrent otitis media and chronic otorrhea resistant to antimicrobial therapy. CT demonstrated fat density, homogeneous lesions, filling and expanding the eustachian tube. On MR, signal from the lesions was consistent with fat, and the relationship with the internal carotid artery was better delineated than by CT. Microscopically, the masses consisted of a conglomeration of ectodermal and mesodermal elements.

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We describe the clinical manifestations, associated abnormalities, MRI appearances and pathologic significance of a case of hypophyseal duplication. A 16-year-old girl presented with delayed sexual development and history of midline craniofacial anomalies. MRI revealed paired infundibula extending inferiorly to two small pituitary glands, a midline hypothalamic mass, and a midline cleft in the basisphenoid. Twelve cases of pituitary duplication have previously been described. The suggested pathogenesis is duplication of the prechordal plate and anterior end of the notochord during early embryologic development.

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The case is reported of meningioangiomatosis of the brain stem in a 3 1/2-year-old girl who suffered from vomiting, left facial weakness, difficulty in swallowing, and ataxia. This is believed to be the first reported case of meningioangiomatosis in the brain stem. Computerized tomography showed an intensely enhancing hyperdense mass in the left restiform body. Magnetic resonance imaging revealed that the lesion was isointense to gray matter on the T1-weighted image and hypointense on the T2-weighted image, with a surrounding zone of high T2 signal and intense enhancement. Angiography was normal. Surgical exploration demonstrated an intramedullary firm mass that was partially resected. Histologically, the mass consisted of a low-grade lesion of meningeal origin with spindle cells in a whorling pattern that were occasionally focused around small vessels. On 2-year follow-up imaging, the lesion remains unchanged in size. Certain particularities of this lesion are discussed in the context of the literature.

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To evaluate T2-weighed fast spin-echo (FSE) and conventional spin-echo (CSE) magnetic resonance (MR) techniques in the assessment of brain myelination, 100 consecutive pediatric patients were imaged prospectively with both CSE and FSE sequences. All patients underwent a routine MR examination that included T2-weighted CSE imaging (imaging time, 10 minutes 21 seconds) and T2-weighted FSE imaging (imaging time, 2 minutes 5 seconds). The two techniques were compared for estimating the degree of myelination (using normal anatomic landmarks) by blind review. With T2-weighted CSE images as the "gold standard" for estimation of normal myelination, FSE images were evaluated to determine if they showed the degree of myelination similarly to CSE images. There was a strong correlation (P < .01) between CSE and FSE images in the estimation of myelination over a wide range of patient ages.

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Cystic or cyst-like malformations of the posterior fossa represent a spectrum of disorders, including the Dandy-Walker malformation, vermian-cerebellar hypoplasia, mega cisterna magna, and arachnoid cyst. Differentiation of these lesions may be difficult with routine cross-sectional imaging; however, an accurate diagnosis is essential for proper treatment planning and genetic counseling. Dandy-Walker malformation is easily diagnosed on the basis of the classic triad: complete or partial agenesis of the vermis, cystic dilatation of the fourth ventricle, and enlarged posterior fossa. Vermian-cerebellar hypoplasia is a general classification that describes congenital malformations with a normal-sized posterior fossa, varying degrees of vermian and cerebellar hypoplasia, and a prominent retrocerebellar cerebrospinal fluid space that communicates freely with a normal or dilated fourth ventricle. Mega cisterna magna can be asymmetric and can manifest apparent mass effect, simulating the appearance of an arachnoid cyst; therefore, ventriculography or cisternography may be needed to demonstrate communication of the cystic mass with the subarachnoid space. A careful review of the embryologic development is essential in understanding these malformations and in making a more accurate radiologic diagnosis.

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In the literature, clinical descriptions of sigmoid sinus thrombosis occurring after closed head injury in children are rare. One to 5 days after trauma to the back of the head, five children (aged 1 to 7 yr) presented with gait ataxia, vomiting, and headache. Trauma was mild in four children. Computed tomography of all the children, performed within 5 days after the injury, showed focal hyperdensity in the region of the left sigmoid sinus. Four children had extra-axial hyperdense collections along the left transverse sinus, and three had skull fractures adjacent to the left sigmoid sinus. Magnetic resonance imaging (MRI) of all the children, performed 2 to 6 days after injury, showed left sigmoid-sinus thrombosis and decreased flow or thrombosis within the lateral third of the left transverse sinus. All the children had MRI scans 4 to 6 weeks after their diagnosis and were followed up for 1 to 12 months. In four children whose symptoms subsided completely within 2 to 10 weeks, MRI showed recanalization of the sigmoid sinus within 4 to 6 weeks after injury. In one child whose symptoms resolved after 6 months, sigmoid-sinus thrombosis persisted with the formation of collateral flow. We conclude that traumatic sigmoid-sinus thrombosis should be suspected when a child has persistent or delayed gait ataxia and vomiting after injury to the back of the head. Computed tomography characteristically demonstrated focal hyperdensity within the sigmoid sinus that we term the dense sigmoid-sinus sign. Because the sinus recanalized and the symptoms subsided in most children within 6 weeks, we conclude that prophylactic medical or surgical intervention is not indicated.

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A phase III open-label clinical trial was conducted at 11 institutions to determine the safety and efficacy of gadoteridol in children suspected of having neurologic disease. One hundred three children were included in the safety analysis; 92 were evaluated for efficacy (76 intracranial and 16 spinal examinations). Three adverse events were reported in two children. All adverse events were considered minor and resolved spontaneously without treatment or sequelae. In a comparison of enhanced T1-weighted magnetic resonance images with unenhanced T1- and T2-weighted images, enhancement of disease was noted in 70% of the intracranial and 38% of the spinal examinations. Additional diagnostic information was reported in 82% of the postcontrast intracranial examinations and 62% of the spinal examinations. Use of this additional information contributed to a potential modification of patient diagnosis in 48% of intracranial and 20% of spinal cases with additional information. These results indicate excellent safety and efficacy for use of gadoteridol in children with suspected intracranial or spinal disease.

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Measurement of the atlas-dens interval is the radiographic standard for identification of patients with Down syndrome at high risk for neurologic injury from spinal cord compression. In 17 pediatric patients with Down syndrome, measurements of the atlas-dens interval, distance between the clivus and posterior odontoid process, and width of the neural canal obtained with plain radiographs were compared to predict the width of the subarachnoid space on magnetic resonance (MR) images. In all patients, cervical radiography and craniocervical MR imaging were prospectively performed during lateral flexion and extension. The width of the subarachnoid space was measured with MR imaging. When the 95% confidence interval for correlation coefficients of regression was used, subarachnoid space width on MR images correlated with neural canal width on radiographs better than with either atlas-dens interval or clivus-posterior odontoid process distance (P = .05). Measurement of neural canal width is a better predictor of potential spinal cord compression than the atlas-dens interval or clivus-posterior odontoid process distance and should be emphasized in screening examinations performed with plain radiography.

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MR imaging of a 2-year-old girl with Kniest dysplasia revealed abnormally large cartilaginous femoral heads. Although ossification of the femoral head is delayed in Kniest dysplasia, it is nonetheless also a megaepiphyseal growth center with respect to the cartilage model.

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Experience with color Doppler imaging (CDI) during 16 pediatric intraoperative and one percutaneous neuro-radiologic procedures was reviewed to assess whether CDI increased the success rate or decreased the procedure time, thus contributing substantially to procedure performance. Intraoperative CDI was used to rapidly identify abnormal vessels or displacement of normal vessels and correlated with preoperative studies. In six cases (four vascular malformations, one mycotic aneurysm, and one hamartoma), surgical resection was altered on the basis of flow information obtained. In one case of percutaneous embolization of a Galenic malformation, CDI provided information contributing to the cessation of the procedure. In six cases (debulking of three gliomas, resection of one vascular malformation, and two biopsies of nonneoplastic conditions), information was added but did not alter the surgical approach. In the remaining four cases (three cerebrospinal fluid drainage procedures, one posterior fossa decompression), no additional information was obtained. Consultation among the ultrasound staff, neuroradiologists, and neurosurgeons before the operative procedure maximized the usefulness of CDI, thus aiding in the success of surgery.

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Choroid plexus neoplasms (CPN) are rare tumors occurring with a relative incidence of 0.5% of intracranial neoplasms in all age groups and 1.5-6.4% of all pediatric brain tumors. In children, the most common site of origin is the atria of the lateral ventricles where the CPN may represent either a carcinoma or a benign papilloma. CPN arising in the fourth ventricle are more common in adults, and are more likely to represent a benign papilloma. We present 2 children with CPN arising from the inferior fourth ventricle with similar features on MRI, one of which represented a benign papilloma and the other a malignant carcinoma. CPN should be included, even in young children, in the differential diagnosis of any infratentorial, intraventricular tumor.

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Ten cases of traumatic atlanto-occipital disruption in pediatric patients are reported. All injuries resulted from motor vehicle accidents, the majority of which were pedestrian/automobile. Three patients survived their injury for a period greater than one year. The importance of recognizing atlanto-occipital disruption is stressed because of its relative frequency in severely traumatized pediatric patients, particularly pedestrian/vehicle incidents, and because of the potential for survival. Diagnosis, in most instances, is based on the lateral cervical spine radiograph. The most applicable diagnostic features in children, as demonstrated on the lateral cervical spine radiograph, are reviewed.

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