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Encephalocele represents one end of the spectrum of open neural tube defects. We report an infant with Chiari type 3 malformation manifesting as infratorcular occipital meningoencephalocele with dermoid cyst. To our knowledge, this is the first time that this association is described. The pathogenesis, surgical treatment, prognosis and management difficulties are also being discussed., (Copyright 2010 S. Karger AG, Basel.)

Benign osteoblastoma is an uncommon primary bone tumor frequently found in the vertebral column and long tubular bones, and rarely occurring in the calvarium. A case of a massive benign osteoblastoma of the suboccipital bone and foramen magnum region in a 9-year-old boy is reported. He presented with progressively worsening nuchal pain and headaches secondary to a bony lesion in the suboccipital and foramen magnum region. Computed tomography (CT) of the brain showed a large midline occipital/suboccipital bony lesion extending to either side (R > L) and extending from the torcula till the foramen magnum region, causing moderate obstructive hydrocephalus. The atlas was uninvolved by the tumor. In addition, the cerebellum was pushed anteriorly squashing the fourth ventricle. The tumor was completely resected with wide margins via a suboccipital route. At follow-up after 7 years, the patient was asymptomatic, and CT imaging demonstrated no recurrence. The occurrence of benign osteoblastoma in the suboccipital bone and foramen magnum region has not been reported earlier in the pediatric population. Surgical extirpation of the lesion with wide margins is advocated and can produce an excellent long-term outcome. Serial vigilant follow-up along with sequential imaging is advocated even in cases with complete resection to detect early recurrence and possible malignant transformation., (Copyright © 2010 S. Karger AG, Basel.)

Tuberculous brain abscess is a rare manifestation of central nervous system tuberculosis. We report the case of a tuberculous temporal lobe abscess in a 14-year-old female child that mimicked an otogenic pyogenic brain abscess. The patient had no prior history of tuberculosis. She had chronic otitis media and presented with signs of raised intracranial tension. Radiological imaging was suggestive of an acute pyogenic left temporal lobe abscess. A left temporal craniotomy was performed and the abscess was completely excised. Histological examination was consistent with a chronic abscess, and bacterial cultures were negative. A left radical mastoidectomy was also carried out. However, she presented with repeated abscess formation at the same site over the next 8 weeks, which was refractory to surgical therapy and broad-spectrum antibiotic administration. Furthermore, the purulent exudate showed strong positivity in the PCR test for tubercular bacilli. After administration of antituberculous treatment, she showed gradual clinical and radiological improvement. At follow-up after 2 years, she is asymptomatic. CT imaging at 2 years showed total resolution of abscess. Tuberculous abscess in the temporal lobe following otogenic infection has not been reported in the pediatric population. Although rare, the possibility of tuberculous etiology should be borne in mind as a differential diagnosis of acute abscess of otogenic origin, especially in endemic areas where the incidence of chronic otitis media as well as tuberculosis is high. The pathogenesis and treatment of tuberculous brain abscess in children is reviewed in light of the current literature on the subject., (Copyright 2009 S. Karger AG, Basel.)

The occurrence of tonsillar herniation and syringomyelia in association with tumor-induced obstructive hydrocephalus has been described in literature. In midline tumors, the symptomatology is predominantly due to hydrocephalus and resultant tonsillar herniation, but the treatment is primarily directed towards the tumor. A case of a pineal region epidermoid in a 17-year-old boy - in whom postoperative meningitis resulted in hydrocephalus, symptomatic tonsillar herniation and cervical syringomyelia - is presented here. Although the tumor was resected and cerebrospinal pathways were established, a ventriculoperitoneal shunt insertion was required for persistent symptomatic hydrocephalus. At serial follow-up after 2 years, the patient was asymptomatic and MR imaging showed a reduction in size of the cervical syrinx, but there was no significant ascent of the tonsils. The occurrence of tonsillar herniation and syringomyelia following postoperative meningitis in a case of pineal region epidermoid is uncommon and has not been reported till now. Cerebrospinal diversion procedure will most often be required in such a situation for symptomatic relief. The resolution of tonsillar herniation and syringomyelia may not always be complete on serially documented neuroimaging in such a situation. Stabilization of the previously progressive neurological course is the realistic goal. The need for an additional surgical procedure for associated tonsillar herniation and syringomyelia is advocated only if there is progressive neurological worsening.

A case of a convexity dural-based cavernous hemangioma in a 15-year-old male child is reported. The child presented only with headaches and had no neurological deficits. MRI features were suggestive of a meningioma. The tumor was completely resected and histology was conclusive of a hemangioma. At follow-up after 2 years, the child is asymptomatic and MRI showed no evidence of the lesion. Supratentorial convexity dural-based hemangioma in a child is rare and hitherto only 1 case has been reported in the world literature. Complete resection of the lesion is curative and long-term prognosis is excellent. The uniqueness of the present case is the supratentorial convexity dural-based location, innocuous presentation and unusual radiological features on MRI. The clinical profile, radiological features and management of cavernous hemangiomas in a child are discussed and the current literature is briefly reviewed., (Copyright (c) 2009 S. Karger AG, Basel.)

We describe a cervical intramedullary neurenteric cyst in a 12-year-old male patient who presented with gradual onset and progressively worsening neck pain, spastic quadriparesis and impaired sensation in the C(2) dermatome. MR imaging revealed a well-defined peripherally enhancing cystic intramedullary lesion with a posteroinferior enhancing nodule at the C(2)-C(3) level mimicking an abscess. There was no evidence of spinal dysraphism. The lesion was completely resected through a posterior approach and the patient showed radical improvement in his symptomatology. At follow-up after 3 years, he was asymptomatic and the MR imaging showed no evidence of any residual or recurrent cyst. The case presented here is unique, since a spinal neurenteric cyst showing intense peripheral contrast enhancement mimicking an abscess is unusual. The radiological features, pathogenesis and surgical considerations in cervical intramedullary neurenteric cysts are discussed and the relevant literature is briefly reviewed., ((c) 2008 S. Karger AG, Basel.)

The most common secondary neoplasms which occur following cranial radiation therapy are sarcoma and meningioma. The occurrence of glioblastoma multiforme following radiation and chemotherapy in acute lymphocytic leukaemia (ALL) is rare. We report 3 cases of glioblastoma multiforme in children developing 11-72 months following completion of chemotherapy/radiotherapy for ALL. The exact cause for the development of glioblastoma multiforme following therapy for ALL is not clear. A genetic predisposition may be essential for the occurrence of such a highly malignant primary brain tumour in leukaemia patients, irrespective of radiation and/or chemotherapy. The pathogenesis and surgical management are discussed, and the literature on the subject is reviewed., ((c) 2007 S. Karger AG, Basel.)

A 7-year-old boy with Goldenhar syndrome was diagnosed to have a posterior cranial fossa dermoid cyst. The presence of such a combination of clinical entities has not been reported earlier. The embryonic dysgenesis causing midline posterior fossa dermoid and other anomalies observed in Goldenhar syndrome occur between the third and fifth week of intrauterine life and are probably interrelated. This report emphasizes the need for a high index of suspicion and the value of cranial imaging in a case with Goldenhar syndrome., ((c) 2007 S. Karger AG, Basel.)

We report a characteristic pattern of 'waist-like' syringomyelia observed in three infants having mobile and reducible congenital atlantoaxial dislocation. The syringomyelia in at least two of these cases was diagnosed to be an intramedullary tumor. Following atlantoaxial fixation, all three patients had long-term recovery of their symptoms., (Copyright (c) 2007 S. Karger AG, Basel.)

Two patients, a 12-year-old girl and an 8-year-old boy, with congenital craniovertebral anomaly and Klippel-Feil syndrome also had a posterior cranial fossa dermoid. The association of these two discrete pathological lesions in the same individual is extremely rare. As both lesions are related to an embryological disorder, issues regarding the possible stage of dysgenesis are analyzed. The treatment options in such cases are discussed., (Copyright 2001 S. Karger AG, Basel)