Cystoperitoneal shunt as alternative treatment for a giant thoracic meningocele in a patient with neurofibromatosis.

BACKGROUND: Thoracic meningoceles are associated with neurofibromatosis 1 in 60% to 85% of all cases. Usually, these meningoceles remain asymptomatic, but back pain, headache, cough, and dyspnea are possible manifestations. Often, there is an associated kyphoscoliotic deformity of the thoracic spine. CASE DESCRIPTION: A 60-year-old woman known in our department after a fossa posterior decompression for an Arnold-Chiari malformation was admitted through the emergency department because of progressive dyspnea. A giant intrathoracic meningocele was already diagnosed earlier but was left untreated because the patient was asymptomatic at that time. She now had dyspnea, and on chest x-ray and CT scan, there was an obvious shift of the mediastinum to the right. Because of the long-existing hemithoracic meningocele, we assumed that this patient actually had only 1 functional lung, and so, left-sided thoracotomy with resection of the meningocele and closure of the defect included a high operative mortality. Instead, we chose to obtain a permanent drainage of the meningocele by putting a shunt between the meningocele and the peritoneum. Postoperatively, the patient recovered well and became oxygen-independent. CONCLUSION: Treatment of giant intrathoracic meningoceles in patients with progressive dyspnea can be challenging, and different options can be found in the literature. Treatment with a cystoperýtoneal shunt, as in our case, can be a less invasive alternative in patients with a high operative mortality risk. To our knowledge, this is the first report of a patient with neurofibromatosis 1 treated in this way.

Dual occurrence of pineal germinoma and testicular seminoma. Case report and review of the literature.

A 16-year-old male presented with obstructive hydrocephalus secondary to pineal germinoma. There have been many reported cases of abdominal metastasis of pineal germinoma after ventriculoperitoneal shunting. Endoscopic ventriculostomy was preferred in our case, thus avoiding iatrogenic peritoneal seeding, but spinal metastasis was unavoidable. Metastatic infiltration of the ventricular system and spinal meninges is the commonest mode of spread. Later, the patient underwent orchidectomy for an asymptomatic left testicular seminoma. He responded to chemotherapy, and had a complete recurrence-free remission for more than 10 years. As far as we know there are only a handful of reported cases of dual occurrence of two primary germ cell tumors (GCT), i.e. gonadal seminoma and pineal germinoma with spinal seeding. We also addressed the controversial subject of radiation versus chemotherapy in the management of patients with pineal germinomas. A review of the relevant literature and recommendations for future treatment of similar cases are discussed.

Manifestations of Neuro-Behcet's disease. Report of two cases and review of the literature.

Behcet's disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10-25% of the patients with BD. We report two cases with Neuro-Behcet's disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behcet's disease are focal necrotic lesions in the brain. However, the association of Behcet's disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behcet's disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behcet's disease associated with an aneurysm of the superior cerebellar artery.

Navigated dorsolateral suboccipital transcondylar (NADOSTA) approach for treatment of hypoglossal schwannoma. Case report and review of the literature.

Hypoglossal schwannoma is a rare skull base neoplasm, which lies in the midst of extremely complex anatomical structures. We report a 39-year-old man who presented with a history of right glossal hemiatrophy, dysesthesia and weakness of the left extremities. These symptoms were caused by a unilateral hypoglossal schwannoma located at the cranial base. This lesion was exposed through a dorsolateral suboccipital transcondylar approach, which was navigated with the frameless stereotaxy (NADOSTA). We also describe the epidemiology, symptomatology and the diagnosis of hypoglossal schwannoma as well as literature review of the operative approach. By introducing this minimally invasive dorsal surgical approach with neuronavigation in treating hypoglossal schwannoma, we believe that safe exposure and complete tumor resection can be achieved with minimal rate of morbidity.