[Central nervous system dysgerminoma: a clinicopathological study of 3 cases]. / Dysgerminomes du système nerveux central: étude anatomo-clinique de 3 cas.

BACKGROUND: Intracranial germ cell tumors are rarely seen and typically localize in the pineal or suprasellar region. The largest category of germ cell tumors is dysgerminoma. AIM: to describe clinicopathological features and immunohistochemical profile of dysgerminomas. CASE REPORT: We report three cases of central nervous system dysgerminomas. There were two young women and a man who were 6, 11 and 23-year-old. They presented with symptoms of insipidus diabetes (n=3) with association to visual field defects in the third case. Radiological findings showed a supra seller lesion in two cases. Double localization in the pineal and suprasellar regions was seen in the third case. Histologic examination and immunohistochemical study of surgical specimen were consistent with primary central nervous system dysgerminoma.

Transoral protrusion of a peritoneal catheter: a rare complication of ventriculoperitoneal shunt.

Ventriculoperitoneal (VP) shunt surgery is the most used technique for the treatment of hydrocephalus. This procedure is associated with a large amount of complications. Bowel perforation caused by a peritoneal shunt catheter is one of these complications, sometimes fatal, and is usually difficult to recognize, except when protrusion of the peritoneal catheter through a natural orifice occurs. This report presents the case of a 2-year-old boy who had undergone a VP shunt and later presented with protrusion of the peritoneal catheter through his mouth. The shunt device was removed and an external shunt procedure was achieved, using the original ventricular catheter kept in place. The diagnosis of bacterial meningitis was retained and an antibiotic therapy was started. The evolution was fatal in 15 days secondary to a bacterial ventriculitis. Through the reported cases of bowel perforation, many risk factors were individualized, such as age, congenital etiology of the hydrocephalus, silicon allergy or the length of the peritoneal catheter. Bowel perforation is a serious complication of VP shunt surgery, leading sometimes to a fatal outcome.

Transcortical approach to a huge pineal mature teratoma.

Intracranial teratomas are rare germ cell neoplasms occurring more often during childhood. We report the case of a huge mature teratoma of the pineal region in a 10-year-old patient that was not correctly diagnosed preoperatively by stereotactic biopsy. The tumor was revealed by intracranial hypertension and a Parinaud syndrome. The tumor markers were within normal levels in the serum. A left transcortical parietal approach was used to completely resect the tumor. No adjuvant treatment was given. A complete neurological recovery was observed after the surgical procedure. Follow-up at 2 years did not show any recurrence. Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor. The treatment is exclusively surgical.

Descriptive epidemiology of childhood central nervous system tumours in Tunisia. experience of a single institution over a 15-year period (1990-2004).

INTRODUCTION: Central nervous system tumours represent 20% of all childhood cancers, and are the second most common group of neoplasms after leukaemias. OBJECTIVE: To describe epidemiological characteristics of central nervous system tumours in a paediatric Tunisian population. PATIENTS AND METHODS: A retrospective study of 492 childhood central nervous system tumours operated between 1990 and 2004 was undertaken. We investigated the age-related location, gender distribution and the histology of all tumours, and adopted the latest WHO classification (2007) in grouping all the tumours. RESULTS: There were 488 primary and 4 secondary tumours; 426 (86.6%) were intracranial and 66 (13.4%) were intraspinal. Of the 426 intracranial tumours, 214 (50.24%) were supratentorial and 212 (49.76%) were infratentorial. The median age at diagnosis was 8 years, with a male:female ratio of 1.14:1. Low-grade tumours (WHO I/II) constituted 67.3% of all lesions and the rest (32.7%) were high-grade tumours (WHO III/IV). The most common tumour found in our series was astrocytoma (38%), followed by medulloblastoma (16.2%), then ependymoma (6.9%), cystic tumours (6.3%) and craniopharyngioma (5.3%). The overall 5-year survival rate was 45% with a mean follow-up period of 36 months. CONCLUSION: In our patient population, the incidence and distribution of central nervous system tumours were similar to those reported in literature. Overall survival rates varied according to tumour location and histopathology.

Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion-hemiplegia-epilepsy syndrome.

A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling right hemisphere with marked temporal herniation. The baby underwent a decompressive right hemicraniectomy with temporal cortical biopsies. The post-operative course was favourable. The histological findings were unspecific, showing a gliotic spongiosis with disseminated granular cells. The post-operative MRI depicted a right hemisphere atrophy. To our knowledge, a space-occupying presentation of HHE syndrome requiring surgical decompression has never been described before while only a few reports dealt with the neuropathological aspects of this syndrome.

[Intramedullary epidermoid cyst: report of five cases]. / Kystes ipidermoïdes intramédullaires: a propos de cinq cas.

BACKGROUND: Spinal epidermoid cysts are rare tumors and they are exceptionally intramedullary. AIM: Report of news cases CASE REPORT: The diagnosis is established by magnetic resonance imaging and the treatment is surgical. The prognosis is generally good, with possible recurrence after many years. The author report five new cases of intramedullary epidermoide cysts operated between 1996 and 2001.

[Decompressive craniectomy in the management of sylvian infarction]. / Interet de l'hemicraniectomie decompressive dans la prise en charge des ramollissements Sylviens pseudo-tumoraux.

Space-occupying middle cerebral artery infarction represents about 10 to 15% of supratentorial ischemic strokes. This syndrome carries a high rate of mortality and requires aggressive surgical decompression. The authors present 6 patients with signs of trans-tentorial herniation operated on between February 2001 and August 2003. Neurological preoperative status was evaluated with Glasgow coma scale score and postoperatively with Barthel index. Three patients had excellent recovery (Barthel Index up to 70), one remained dependant and two died. Younger patients had better prognosis. Decompressive surgery, when done early, should improve mortality rate and even functional outcome. Optimal selection of patients, with the help of Diffusion-Weighted imaging, could vouch good results.

Thoracic myelopathy caused by ossification of the ligamentum flavum: a report of 18 cases.

OBJECT: Thoracic myelopathy caused by ossification of the ligamentum flavum (OLF) is a rare entity, most evident in Japan. The authors studied the clinical manifestations, radiological aspects, surgical treatment, and pathogenesis of this disease. METHODS: Eighteen patients with OLF-induced thoracic myelopathy underwent laminectomy. The severity of myelopathy varied. Complete paraplegia was seen in three cases. Compression of the upper and middle third of the thoracic spine was evident in six cases and of the lower third in 12 cases. Multilevel OLF was demonstrated in 13 cases. In most cases, the ossified ligamentum flavum appears as a V-shaped lesion on computerized tomography and magnetic resonance images. In all patients the diameter of the posterior spinal canal, already narrowed, was further exacerbated by the OLF. Laminectomy was limited to the levels of compression, and the ligamentum flavum was resected in all cases. The symptoms and signs improved in 13 cases and stabilized in four cases. In one case symptoms recurred as a result of ossified lesions forming at other sites. Histological examination showed that the mode of development of the ossified ligaments was endochondral ossification. CONCLUSIONS: Reports of OLF-induced myelopathy are rare and mainly described in Japan. The incidence also seems high in North Africa. An early laminectomy limited to the level of compression is recommended. Ossified ligamentum flavum is different from the calcification of the ligamentum flavum, which is due to crystal deposits.

Primary orbital hydatid cyst in an elderly patient.

BACKGROUND: Orbital hydatidosis is rare, accounting for only 1% of all hydatid cysts. Herein we report a case and review the sparse literature. METHODS: Case report and review of pertinent literature. RESULTS: A 74-year-old farmer presented with progressive proptosis and loss of vision of the left eye. Evaluation revealed a hydatid cyst of the orbit. Orbital hydatidosis is usually unilateral and unaccompanied by cysts elsewhere in the body. Most affected are children and young adults. Although there is no gender predominance, orbital hydatidosis may affect the left eye preferentially. Onset is usually insidious over several months. Serologic testing is unreliable, and imaging by computed tomography or magnetic resonance imaging is now standard. Surgical resection is curative, but albendazole must be administered postoperatively if there is spillage intraoperatively. CONCLUSIONS: Hydatid cysts should be included in the differential diagnosis of unilateral proptosis in patients from regions where echinococcosis is endemic.

Primary hydatidosis of the central nervous system: a retrospective study of 39 Tunisian cases.

OBJECTIVE: To analyze epidemiological characteristics, clinical symptoms, radiological aspects, treatment and outcome of central nervous system hydatidosis and compare our results with those reported in literature. PATIENTS AND METHODS: In our retrospective study, we reviewed 39 cases of primary central nervous system hydatid cysts operated on in our hospital between 1998 and 2007. RESULTS: There were 20 male and 19 female patients (sex-ratio M/F=1.05) between 2 and 68 years of age (mean=26.5 years). Thirteen of the patients were children (33.3%) with a mean age of 6.8 years and 26 were adults (66.7%) with a mean age of 36.3 years. The location of hydatid cysts was intracranial in 27 cases (69.2%) and spinal in 12 cases (30.8%). Headache and motor deficits were the predominant symptoms in patients with intracranial hydatidosis whereas back pain and spinal cord compression syndrome were the most frequent clinical presentations in patients with spinal hydatidosis. All patients underwent surgical resection of the cyst. Pathologic findings were consistent with hydatid cyst in all cases. During the follow-up period which ranged between 12 months and 5 years, 12 patients had recurrence (30.7%). Only one patient with intracranial hydatid cyst died postoperatively due to anaphylactic shock. CONCLUSION: Despite all the advances in imaging techniques and therapeutic methods, central nervous system hydatidosis remains difficult to cure and patient outcomes are not satisfactory especially in case of spinal involvement due to the high incidence of recurrence.