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Objective and Importance: Foramen magnum encroachment has been cited as a potential cause for the premature demise of patients afflicted with craniometaphyseal dysplasia (CMD). To our knowledge, the association of Chiari malformation and syringomyelia with CMD has not been previously reported. We describe our evaluation and surgical treatment of a patient presenting with CMD, foramen magnum stenosis, Chiari I malformation, and syringomyelia., Clinical Presentation: A 15-year-old female patient with CMD presented with severe headaches and progressive myelopathy. Evaluation by computed tomography and magnetic resonance imaging revealed mild ventriculomegaly, cervicomedullary compression secondary to Chiari I malformation and foramen magnum stenosis, and C3-T10 syringomyelia., Intervention: Foramen magnum decompression was performed via suboccipital craniectomy, C1-C2 laminectomy, and dural augmentation. Dysplastic bone was revealed to be extremely thick and mineralized. Removal required lengthy and extensive drilling. The postoperative course was complicated by prolonged intubation secondary to airway obstruction and a perforated duodenal ulcer. Airway obstruction was attributed to severe nasopharyngeal bony dysplasia and soft tissue edema. The use of steroids in the treatment of airway edema and delayed enteral feeding was thought to contribute to ulcer development. Nevertheless, neurological symptoms improved dramatically., Conclusion: Foramen magnum decompression can be used to treat life-threatening cervicomedullary compression in patients with CMD. However, caution should be used because surgical intervention may be associated with a higher incidence of complications because of lengthy procedures and the spectrum of craniofacial impairments in patients with CMD.

Serial evaluations were completed after selective dorsal rhizotomy on 90 children with spastic cerebral palsy to analyze whether age, the preoperative gait score, voluntary dorsiflexion at the ankle, the diagnosis (quadriplegia or diplegia), or the length of follow-up correlated with the ability to walk after rhizotomy. The preoperative gait score (P < 0.0001), the diagnosis (diplegia versus quadriplegia, P < 0.0001), unilateral dorsiflexion (P = 0.0029), and bilateral dorsiflexion (P < 0.0001) were significant predictors of the maximal postoperative gait score in the univariate regression analysis, but only the preoperative gait score (P < 0.0001) and the diagnosis (P = 0.0015) retained significant predictive power in the multivariate analysis. These data suggest that the preoperative gait score and the diagnosis are the strongest predictors of ability to walk after selective dorsal rhizotomy. Dorsiflexion demonstrated predictive power only in the univariate model, suggesting that it might have some prognostic value but less than the preoperative gait score or the diagnosis.

The cases of a father and his son who were diagnosed with pathologically confirmed colloid cysts of the third ventricle are presented. The familial occurrence of this tumor is rare and suggests that genetic factors may play a role in its formation. Consistent with the concept that the cyst originates as a developmental abnormality, it is associated with a variety of congenital defects. The conditions that are associated with these tumors are discussed.

We describe a variation of selective dorsal rhizotomy for spastic cerebral palsy that involves sectioning of the dorsal spinal roots immediately caudal to the conus medullaris. The operation entails an L1-L2 laminectomy, ultrasonographic localization of the conus medullaris, and partial deafferentation of the L1-S2 roots with electromyographic testing under an operating microscope. In 66 children with cerebral palsy, the operation reduced spasticity in the lower extremity without complications, e.g., motor weakness, neurogenic bladder, and sensory loss. It offers several important advantages over alternative techniques.

Infection causes major morbidity and mortality in patients with cerebrospinal fluid (CSF) shunts. The prognosis of CSF shunt infections caused by Gram-negative bacteria (GNB) has been thought to be particularly poor. The authors reviewed all GNB shunt infections treated at Children's Memorial Hospital from January 1986 to January 1990 (n = 23). Of these infections 20 (87%) occurred within 4 weeks after shunt revision (median, 10 days). The most frequent symptoms were fever, lethargy, and irritability; the illness was not severe in the majority of these patients. Escherichia coli was isolated from 12 of 23 patients (52%), Klebsiella pneumoniae from 5 (22%), and mixed GNB from 3 (13%) patients. Initial treatment always included immediate shunt removal, externalized ventricular drainage, and intravenous antibiotics. Extraventricular drainage revision and/or intraventricular antibiotics were required in four patients whose CSF cultures were persistently positive for GNB. At admission, these patients had CSF glucose levels of < 10 mg/dl and CSF positive for GNB by Gram's stain. The overall cure rate was 100%, and no recurrence was observed; however, a subsequent infection with a different organism developed in four patients. Only 2 of 19 patients (11%) who were followed up suffered apparent CNS damage. One patient died of unrelated causes shortly after treatment. Our findings indicate that 1) patients with GNB CSF shunt infections often appear relatively well at presentation; 2) CSF positive for GNB by Gram's stain and very low CSF glucose levels predict continued positive CSF cultures, despite appropriate antibiotic therapy; and 3) GNB CSF shunt infections can be successfully treated by prompt shunt removal, extraventricular drainage, and intravenous antibiotics.(ABSTRACT TRUNCATED AT 250 WORDS)

A neurofibroma of the cervical medullary region was accurately delineated using nuclear magnetic resonance (NMR) scanning. The tumor and its compression of the spinal cord were so well visualized that evaluation by myelography and postmyelographic computed tomography (CT) was unnecessary. NMR scanning should be considered as a replacement for myelography with CT in the initial evaluation of cervicomedullary spinal cord tumors.

Magnetic resonance imaging (MRI), computed tomographic (CT) scanning, and angiography or digital subtraction angiography (DSA) were used preoperatively to evaluate 16 surgically proven cases of sellar tumor with extrasellar extension. There were 15 pituitary tumors and 1 schwannoma. The capabilities of MRI in evaluating these tumors were compared with CT scanning and angiography. Bone destruction and tumor calcification were better detected by CT scanning than by MRI. MRI was as effective as CT scanning in detecting a cyst or variation in tumor consistency. Neither MRI nor CT scanning was capable of distinguishing specific tumor types. In every case, MRI was superior to CT scanning for delineating spatial relationships of the tumor to the 3rd ventricle, the optic apparatus, adjacent brain, and parasellar vasculature. Vessel encasement by tumor was clearly seen on MRI when there was no direct indication of this on other studies. Cavernous sinus invasion was not demonstrated by CT scanning, but was indicated by MRI in 5 cases and was surgically confirmed in 3. MRI can provide more precise spatial information on extrasellar tumor extension. Vascular encasement and cavernous sinus invasion may be determined preoperatively. Treatment expectations and operative approaches can be guided by this information. When MRI is available, it is the test of choice for the preoperative evaluation of patients with suspected large pituitary gland (sellar region) tumors. Contrast-enhanced CT scanning and angiography can be used as supplementary studies to add information inherently unique to these techniques.