Craniopharyngioma in children.

PURPOSE: The treatment regimens at presentation and recurrence, the related morbidity, and survival rates were studied for children with craniopharyngioma who were treated at King Faisal Specialist Hospital & Research Center (KFSH&RC). METHODS & MATERIALS: From 1975 to 1996, a total of 56 children younger than 18-years-old at diagnosis underwent treatment with surgery and/or radiotherapy for craniopharyngiomas at KFSH&RC. There was evidence that these patients had advanced disease at diagnosis; 36% of 51 patients whose visual status pretreatment was known were either blind or had major bilateral visual defects prior to treatment, and 36% of the 56 patients had diabetes insipidus. This report concentrates on 44 patients who had first definitive resection at KFSH&RC. Treatment policy was total resection if possible (17 patients), if not, subtotal resection (17 patients) or lesser procedures (10 patients). Five patients received postoperative irradiation after first definitive resection. RESULTS: Ten-year-survival, and progression-free and event-free survival rates were 65%, 39%, and 29%, respectively. There were 9 postoperative deaths; 7 following the first procedure and 2 following resection for relapse, and 3 early deaths at home prior to relapse, following the first resection. None of the 22 patients who underwent less than total resection without postoperative radiation treatment were progression-free at 5 years, whereas the 5 patients who were irradiated remain progression-free. CONCLUSION: A more selective approach to total resection, and the routine use of postoperative irradiation following lesser surgical procedures requires evaluation.

Grade zero removal of supratentorial convexity meningiomas.

Although meningiomas are benign intracranial tumors, their frequency of recurrence after surgery has not been as low as expected. The recurrence rate of meningiomas is clearly related to the degree of tumor removal. Simpson Grade I removal, which entails excising the tumor and its dural and sinus attachments, is associated with the lowest rate of recurrence. To further minimize the recurrence of convexity meningiomas, we removed an additional dural margin of about 2 cm around the tumor (Grade 0 removal). For tumors involving bone, we removed the hyperostotic bone with a healthy margin and pericranium in en bloc resection. Between 1982 and 1992, 37 patients (15 men, 22 women) with an average age of 52.1 years were operated on by the above technique. Nineteen had a follow-up period of more than 5 years. To date, no tumors have recurred and no morbid incidences have occurred with this maneuver. We believe that the recurrence rate of convexity meningiomas can be diminished by including in the resection a margin of dura that might harbor a foci of tumor cells.

Sphenoid sinus brown tumor of secondary hyperparathyroidism: case report.

OBJECTIVE AND IMPORTANCE: Brown tumor is a misnomer for a reparative cellular process caused by primary or, less commonly, secondary or tertiary hyperparathyroidism. Atypical involvement of the base of the cranium in the area of the sphenoid sinus and the orbit is exceedingly rare and may produce a neurological deficit as a primary clinical presentation. Those neurosurgeons dealing with cranial base and pituitary lesions should be aware of this disease entity, and it should be listed in the differential diagnosis. CLINICAL PRESENTATION: A 21-year-old woman with end-stage renal failure on chronic dialysis was referred to the Department of Neurosciences at King Faisal Specialist Hospital and Research Centre with a 4-month history of progressive impairment of vision, associated with headache and nausea. Computed tomography and magnetic resonance imaging demonstrated an expansive mass lesion in the sphenoid sinus with erosion of the sellar floor and posterior medial wall of the orbit causing compression of the optic nerves. The biochemical laboratory studies showed elevation of parathyroid hormone and confirmed the diagnosis of hyperparathyroidism. INTERVENTION: The transnasal-transsphenoidal approach was used for extensive microsurgical removal of the lesion. The postoperative course was uneventful, and the improvement in vision was dramatic. CONCLUSION: Brown tumor, although rare, should be listed in the differential diagnosis of expansive mass lesions in the area of the sphenoid sinus and cranial base. The management is multidisciplinary, and therapeutic options should target the underlying cause.

Brain biopsy in tuberculoma: the risks and benefits.

In developing countries, 5 to 8% of the space-occupying lesions of the central nervous system are tuberculomas. Diagnosis can be difficult in the absence of extracranial tuberculosis; computed tomography is suggestive only. To assess the value of brain biopsies in tuberculomas, the records of 15 patients aged 6 to 80 years were reviewed. Histological confirmation was obtained in 15 patients, and acid-fast bacilli were cultured from 12 patients. Intracranial hypertension was the principal sign in 11 patients; other neurological signs were related to the location of the tuberculoma. One patient had evidence of extracranial tuberculosis. Biopsy-related complications consisted of an epidural hematoma in 1 patient and hydrocephalus in another; both required additional surgery. One case of tuberculous meningitis was probably related to surgery and poor drug compliance. There was no postoperative mortality. Thirteen patients (2 were lost to follow-up) were cured after an average of 16 months of antituberculous therapy. It was concluded that the brain biopsy is useful in diagnosing tuberculoma but that there is some associated risk.

Combined oral isoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis.

Eighteen patients, 16 boys and 2 girls, aged 5-14 years, with subacute sclerosing panencephalitis (SSPE) were treated with oral isoprinosine (100 mg/kg/day) and intraventricular alpha-interferon 2b (Intron A, Schering Corp.), starting at 500,000 U twice a week and later increasing to 3 million U biweekly. Minimal follow-up of living patients is 12 months; maximal 40 months. On the basis of the Neurological Disability Index (NDI) scores and staging, 8 have treatment-induced remissions (3 improved, 5 arrested), 4 are worse and 6 died. This 44% (8/18) rate of remission/improvement compares well with the 9% (1/11) remission in historical controls in the same institution (p = < 0.05) and 5% spontaneous remission in the literature. Combined oral isoprinosine-intraventricular alpha-interferon appears to be an effective treatment for SSPE.

Medulloblastoma in adults.

Seventeen adult patients (16 years of age and over) with medulloblastoma were managed at King Faisal Specialist Hospital and Research Centre between 1981 and 1992. Nine patients were males and eight were females. The average age at diagnosis was 26.7 years; the females were younger than the males. The average duration of history before diagnosis was 14.5 weeks, the females presenting earlier than the males. The tumors were located in the midline (9) and the hemispheres (8). Nine patients had the desmoplastic variant and eight had standard primitive neuroectodermal tumor. Hydrocephalus developed in 14 of the patients only seven of whom required a shunting procedure. All patients had either subtotal or gross total removal of the tumor. Sixteen patients had postoperative radiation therapy; one patient refused radiation therapy. Two patients had chemotherapy following recurrence of the tumor. The clinical presentation, radiological studies, histological variants, treatment and outcome are discussed in this report.

Large meningioma of the foramen magnum in a 4-year-old child.

The case of a 4-year-old boy with 4 months' history of progressive quadriplegia is presented. The neuroradiological work-up, which included computed tomography scanning and magnetic resonance imaging, showed changes consistent with the diagnosis of a large mass lesion in the area of the foramen magnum. At operation a meningioma was completely excised. There was a gradual and almost complete neurological recovery.

Suprasellar meningioma and blindness: a unique experience in Saudi Arabia.

BACKGROUND: The natural history of intracranial meningioma is no different in the Arabian peninsula than in other countries. The extent of the total loss of vision in one or both eyes, however, is unique to this area. METHODS: A retrospective study was conducted at King Faisal Specialist Hospital and Research Centre, a tertiary care institution in Saudi Arabia, on all cases of intracranial meningioma seen over a period of 9 years. The following data were collated: clinical presentation, site and size of tumor, radiologic features, surgical methods, and preoperative and postoperative visual status. RESULTS: Seventy of 232 cases (30.1%) of intracranial meningioma presented in the suprasellar area; the majority of these were more than 5 cm in diameter. Because of the large size it was not always possible to identify the exact point of origin or to achieve complete tumor removal. Deteriorating vision was the cardinal presenting feature and the incidence of blindness in one or both eyes at presentation was high (42.2%); 12.8% were totally blind on admission. None of these patients showed improvement after surgery (complete or subtotal removal of tumor). CONCLUSION: Lack of understanding of the symptoms and cultural influences are direct contributing factors to this complication of a benign intracranial lesion. This represents a serious public health concern in the Arabian peninsula.

Brain tumors in pediatric patients at King Faisal Specialist Hospital and Research Centre.

We report our experience with brain tumors in pediatric patients, patients who came to our attention when they were aged 14 years or less. In general, the pattern of brain tumors at this Institution is similar to that reported in the neurosurgical literature. Fortunately we have modern diagnostic technology and modern operating instrumentation available to use for dealing with such lesions, which are often in their late stages.

Craniocervical junction tuberculosis: a rare but dangerous disease.

BACKGROUND: Mycobacterium tuberculosis of the cervical spine is a rare but dangerous manifestation of extra-pulmonary tuberculosis. The clinical picture ranges from early, nonspecific, insidious symptoms to severe neurological complications and death, attributed to craniocervical junction instability and cervicomedullary compression. The different lines of management include antituberculous medication with traction and external fixation or adjunctive surgery (debridement and stabilization) in patients with severe or persistent neurological complications and/or vertebral instability. METHODS: We describe two patients with advanced craniocervical junction tuberculosis. The early clinical picture was nonspecific in Case 1 and obscured by psychiatric illness in Case 2. The detailed clinical and radiological findings, and the management, will be described. Involvement of the occipital condyles and foramen magnum, which has not been reported previously, will be demonstrated. RESULTS: Both cases underwent transoral biopsy, aspiration, and debridement of retropharyngeal abscess (granuloma). Histological and tissue culture studies proved the abscesses were tuberculous and anti-tuberculous medications were started. Case 1 showed complete resolution of the clinical and radiological findings. Case 2 developed cardiorespiratory arrest while in a halo jacket. He was resuscitated but remained quadriplegic and semiconscious; he developed nosocomial gram negative pneumonia. He was referred back to his local hospital where he died 1 year later. CONCLUSIONS: Tuberculosis is an infrequent but notable cause of cervicomedullary compression. It should be suspected in patients with infective spondylitis who are immunocompromised or reside in an area highly endemic for tuberculosis. Management strategies include antituberculosis medication, transoral biopsy and drainage of the abscess, traction and external fixation, posterior decompression, and internal fixation, according to the clinical and radiological findings.

Extra-axial cavernous hemangioma: two case reports.

Two patients with extra-axial cavernous hemangioma who presented with headache and oculovisual disturbances were investigated with computed tomography and magnetic resonance imaging. The lesions masqueraded as basal meningioma, but this diagnosis was not supported by magnetic resonance spectroscopy in one patient. Cerebral angiography with embolization was indicated in one patient, but embolization was not justified in the other. Both patients underwent a pterional craniotomy. The lesions were extradural and highly vascular, necessitating excessive transfusion in one patient in whom gross total resection was achieved, and precluding satisfactory removal in the other. There was no mortality. Transient ophthalmoplegia, the only complication in one patient, was due to surgical manipulation of the cavernous sinus; it resolved progressively over 3 months. Extra-axial skull base cavernous hemangiomas are distinct entities with clinical and radiological characteristics that differ from those of intraparenchymal cavernous malformations. They can mimic meningiomas or pituitary tumors. In some cases, magnetic resonance spectroscopy may narrow the differential diagnoses. Surgical resection remains the treatment of choice, facilitated by preoperative embolization to reduce intraoperative bleeding and by the application of the principles of skull base surgery. Fractionated radiotherapy is an alternative in partial or difficult resections and in high-risk and elderly patients.

Minimally invasive approach to management of pituitary adenomas.

Pituitary adenomas are the third most common benign intracranial tumor seen in neurosurgical practice. They represent >or= 15 % of all primary intracranial tumors with 25 % prevalence as reported in autopsy series. Advances in biomedical assays, imaging studies support their diagnosis and tailor their management. The direct endonasal transsphenoidal surgery is the recommended intervention for adenoma resection in more than 95 %. The safety and efficacy of this intervention was enhanced by microsurgery and more recently by the introduction of neuronavigation, assisted endoscopy and intraoperative MRI. Anticipation of clinical, biochemical, radiological and surgical pitfalls by a multidisciplinary team is of paramount importance in improving treatment and preventing potential complications.

Trans-eyebrow mini-orbitozygomatic pterional approach for minimally invasive skull base surgery.

Surgical treatment of skull base pathologies remains one of the most challenging tasks to neurosurgeons. Advances in neuroimaging as well as the evolution of modern techniques and neurosurgical equipment have paved the way for a more precise diagnosis, a better selection of the surgical approach and have prompted the adaptation of minimally invasive concepts to different aspects of modern neurosurgical practice. The trans-eyebrow mini orbitozygomatic pterional approach is a tailored approach that provides multiple avenues to the target, minimizing retraction of the brain and affords better exposure of the pathology. It was performed on 16 patients with frontobasal or suprasellar tumors. The post-operative clinical outcome was excellent with smooth and fast recovery as well as satisfactory cosmetic results. It should be considered as part of the armamentarium to manage skull base lesions when indicated and not to replace other well recognized skull base approaches.

Lymph node metastasis in medulloblastoma.

A 14-year-old boy was diagnosed as having a medulloblastoma in the posterior fossa. Radiation therapy was given and the patient made a good recovery. Three months later he presented with supraclavicular lymph node metastasis, in the absence of other foci of metastases. He was treated with 60Co and the metastasis decreased. Two months later a bone scan and bone marrow aspiration showed bone metastasis. He was started on chemotherapy. The different possible mechanisms of metastasis are discussed.

Atypical course of carotid cavernous sinus fistula in a six-year-old child.

A case of post-traumatic carotid cavernous sinus fistula complicated by coma-producing intraventricular tamponade in a 6-year-old boy is reported. Urgent surgical treatment included external ventricular draining, trapping and muscle embolization by the Jaeger-Hamby procedure. Follow-up cerebral angiography showed complete obliteration of the fistula. The child made an excellent recovery and now leads a normal life.

The spectrum of intracranial aneurysms in pediatrics.

Intracranial aneurysms are rare in children, accounting for merely 0.5-4.6% of all aneurysms. Several characteristics distinguish them from aneurysms in adults: male predominance; higher incidence of unusual location, such as peripheral or posterior circulation; and a greater number of large and giant aneurysms. These unique features can be attributed to the higher incidence of traumatic, infectious, developmental, and congenital lesions. Subarachnoid hemorrhage is not the exclusive mode of presentation; neuro-compressive signs and symptoms are also frequently seen. The treatment of intracranial aneurysms in pediatrics is dictated by their unusual pathology and liability to rehemorrhage. Although direct clipping has been the standard surgical technique, certain obsolete or innovative procedures should also be considered, such as entrapment, proximal occlusion, and endovascular embolisation, or even, in some cases, the non-invasive treatment of "watch and wait" for a spontaneous thrombosis. Six representative cases from King Faisal Specialist Hospital and Research Centre are reviewed. They reflect the diversity of the problem and the multi-disciplinary approach to the treatment.

Osteoplastic laminotomy as a minimally invasive spinal procedure.

A minimally invasive surgical technique is demonstrated to perform osteoplastic laminotomy. It consists of protection of the dural sac and the nerve roots with a rubber or silicone tube, cutting of the laminae with an oscillating saw and bilateral refixation of the laminae. This simple and safe technique, which has been used by the authors for six years, provides good stability of the spine postoperatively and helps to avoid post-laminectomy membrane formation.