Cerebrospinal fluid hydrothorax without ventriculoperitoneal shunt migration in an infant.

Cerebrospinal fluid (CSF) hydrothorax is a rare complication of a ventriculoperitoneal shunt (VPS), and even rarer in the absence of shunt migration into the thoracic compartment. Because of the limited volume within the thoracic cavity, hydrothorax in infants can rapidly cause severe respiratory distress. The case of an infant with recurrent CSF hydrothorax despite a well-positioned VPS is presented. A ventriculoatrial shunt was successfully performed as the definitive treatment. The absence of ascites or predisposing factors for decreased peritoneal absorption and a false-negative ß(2)-transferrin CSF marker are some of the factors that can add to the diagnostic challenge in these patients. This is a potentially life-threatening condition in infants, which should be closely considered in patients with VPS who develop hydrothorax, although diagnosis is not always straight forward. The etiology and pathophysiology of this very rare disease continue to be elusive, and treatment with ventriculoatrial shunt provides good results.

Immediate resolution of tonsillar herniation and severe cervicothoracic syringomyelia after third ventriculostomy for hydrocephalus caused by a brainstem tumor. Case report.

The authors report the case of a patient who harbored a large intrinsic brainstem tumor associated with tonsillar herniation, hydrocephalus, and severe cervicothoracic syringomyelia. This 15-year-old girl had a 2-month history of progressively worsening headaches. Magnetic resonance imaging showed a large tumor in the dorsolateral brainstem accompanied by hydrocephalus as well as a cervicothoracic syrinx extending to the level of T-8. She underwent endoscopic third ventriculostomy that resulted in a marked decrease in the ventricle size as well as complete resolution of the syrinx and tonsillar herniation within 4 days. Clinical and neuroimaging findings of the case along with a pertinent literature review are presented.

Extraventricular neurocytoma in neurofibromatosis Type 1: case report.

Neurocytomas are rare tumors of the central nervous system that are typically located in the ventricular system. The authors report a case of a child with neurofibromatosis Type 1 (NF1) who had a tumor of the optic nerves and chiasm with signal abnormality extending through the diencephalon, as well as an occipital lobe mass, which was presumed to be part of the visual pathway neoplasm. Because the occipital lobe lesion slowly increased in size over time, while the other areas remained stable, a biopsy was performed. Pathological evaluation revealed an extraventricular neurocytoma of extraventricular neurocytoma. To the authors' knowledge, neurocytomas have not been previously reported in patients with NF1. Because visual pathway gliomas are extremely common in children with NF1, they are often treated empirically as low-grade gliomas without histological confirmation. The importance of obtaining a biopsy in lesions that have atypical imaging features is highlighted.

Brainstem lesions in neurofibromatosis type 1.

OBJECTIVE: The presence of multiple, nonenhancing areas of hyperintensity without mass effect are well recognized on magnetic resonance imaging scans in children with neurofibromatosis type 1 (NF1). Focal regions of brainstem enlargement with or without contrast enhancement are considerably less frequent; the neuroimaging characteristics and natural history of these lesions in patients with NF1 are poorly understood. The objective of this study was to define the clinical and radiographic course of brainstem lesions in children with NF1. METHODS: We retrospectively reviewed the neuroimaging studies of all patients with NF1 between 2000 and 2006 to determine the prevalence of brainstem lesions. Clinical features, previous treatments, and neuroimaging studies of the brainstem lesions were evaluated. RESULTS: A total of 125 patients underwent neuroimaging studies; of these, 23 patients (18.4%) showed evidence of brainstem mass lesions and had follow-up magnetic resonance imaging scans available for review. Eight patients in this cohort received additional treatment with surgery, radiation, or chemotherapy. Of these, two patients underwent surgery for lesions distant from the brainstem, and six patients underwent treatment that included the brainstem and were thought to potentially affect the natural history or progression of the brainstem abnormality. With a median follow-up period of 67 months for untreated patients (17 out of 23) and 102 months for patients who received therapy (six out of 23), only one previously untreated patient experienced radiographic and clinical progression. All patients but one remain alive. CONCLUSION: We conclude that brainstem lesions in NF1 are prevalent and behave in a biologically indolent nature; most do not require therapeutic intervention.

Obliteration of carotid-cavernous fistulas using direct surgical and coil-assisted embolization: technical case report.

OBJECTIVE AND IMPORTANCE: Carotid cavernous fistulas (CCF) type-D are often refractory to endovascular treatment. Surgery for these lesions is a well-described alternative option. A case of combined pretemporal approach to the cavernous sinus and direct coiling of CCF is presented as well as treatment options in cases of recurrent and intractable fistulas. CLINICAL PRESENTATION: A 58-year-old woman with three years' history of double vision, headache, and numbness of the right upper extremity. On clinical examination she was noted to have congestion in the left eye with neurological examination without any deficits. Angiography revealed a type-D fistula.. INTERVENTION: Patient underwent craniozygomatic pretemporal approach and direct coiling of the fistula. Intraoperative angiogram was done to confirm adequate obliteration of the fistula and preservation of flow in the parent artery.. CONCLUSION: Although the first-line treatment option for symptomatic cavernous sinus fistulas is endovascular embolization, surgery is indicated in cases where it fails to achieve satisfactory results. This is especially important when the patient has progressive neurological deficit, and in cases of compromised venous drainage. Direct coil-assisted obliteration of the fistula is a successful treatment option for these lesions..