Surgical resection of a sphenoid wing meningioma in a patient with Glanzmann thrombasthenia.

Glanzmann thrombasthenia (GT) is a rare autosomal recessive disorder characterized by a deficiency or functional defect of platelet glycoprotein (GP) IIb/IIIa. Physiologically, this platelet receptor mediates aggregation of activated platelets by binding the adhesive proteins, fibrinogen, von Willebrand factor (VWF) and fibronectin. This facilitates attachment and aggregation of platelets at sites of vascular injury. We reported the management of a pterional meningioma resection in a patient with Glanzmann thrombasthenia, with recombinant factor VIIa (rFVIIa - NovoSeven) as haemostatic agent. A 48-year-old woman suffering from Glanzmann thrombasthenia was scheduled for spheno-orbital meningioma en plaque surgery. Because of repeated platelet transfusions, this patient developed isoantibodies against missing GPIIbIIIa and alloantibodies against Human Leukocyte Antigen (HLA) leading to refractoriness to platelet transfusions. We observed that Novoseven offered sufficient haemostasis conditions. Therefore, we noticed a deep vein thrombosis. This imposed us to use low weight molecular heparin despite recent surgery.

Primary intraventricular brain abscess.

We report a primary intraventricular brain abscess in a 13-year-old boy. We discuss possible explanations for this rare occurrence.

Juxtafacet cyst of the lumbar spine. Clinical, radiological and therapeutic aspects in 28 cases.

OBJECT: A consecutive series of 28 "operated" juxtafacet cysts is reported. We emphasize the clinical and radiological aspects leading to diagnosis. We also discuss the results of the surgical treatment. MATERIAL AND METHODS: Medical information and radiological studies involving 28 patients were analyzed. Each patient has been operated on by decompressive laminectomy and resection of the cyst. The diagnosis was always confirmed by a pathological examination. The cyst most frequently occurred at the L4-L5 level (n = 18), and seldom at the L5-S1 (n - 6) or L3-L4 (n - 4) levels. RESULTS: The differential diagnosis from other pathological causes responsible for a radicular compression could not be done by physical examination. Spine X-rays or myelogram were nonspecific. Computed Tomography or CT-myelography could help in the diagnosis but MR imaging was the most sensitive. In our series, the respective sensitivities of these techniques are 56, 42 and 77%. The preoperative diagnosis was correct in 18 patients (64%). The cyst was sometimes adherent to the underlying dura, then significantly increasing the risk of dural tear and spinal fluid leak, especially when located at L3-L4 level. Surgical ablation lead to a complete recovery or an important improvement in 26 patients. CONCLUSIONS: The diagnosis of the juxtafacet cyst of the lumbar spine is better achieved by MRI. Surgery is the gold standard treatment, safe and long-term effective. When a total cyst removal with an internal facetectomy are performed, recurrence is exceptional.

Posterior epidural migration of sequestered lumbar disc fragments. Report of two cases.

The posterior epidural migration of sequestered lumbar disc fragments is an uncommon event. The authors report two such cases in which patients presented with either intense radicular pain or cauda equina syndrome. The radiological characteristics were the posterior epidural location and the ring enhancement of the mass after injection of contrast material. The major diagnostic pitfalls are discussed.

Comparative evaluation of cerebral aneurysms with selective arterially enhanced CT and DSA.

The purpose of our study was to compare selective arterially enhanced spiral computed tomographs (ACT) with digital subtraction angiographies (DSA) in the presurgical assessment of cerebral aneurysms. A total of 24 aneurysms in 18 patients were explored in a prospective study by ACT and DSA, using an interactive combined CT-angiography suite. Dimensions of the aneurysm, its relation to the parent vessel, and the aneurysmal index were defined on DSA and on surface-shaded display of 3D reformatted images obtained from ACT. Results were correlated with surgical findings. Three aneurysms suspected on DSA were not confirmed by ACT. One fusiform aneurysm suspected on DSA corresponded to a sacciform aneurysm on ACT. Surgical findings confirmed 20 sacciform aneurysms. The aneurysmal index could be measured in all 20 cases of sacciform aneurysms on ACT and could not be determined with confidence in 55 % of the cases on DSA. DSA and ACT gave identical results in 35 % of cases. In 10 %, the index measured by ACT was superior to that determined by DSA for aneurysms which had a diameter of less than 3 mm. In conclusion, the combination of DSA and ACT improved the results of DSA alone. ACT is a reliable method to measure the aneurysmal index in aneurysms with a diameter superior to 3 mm.

Shunt-related abdominal metastasis of cerebral teratocarcinoma: report of an unusual case and review of the literature.

OBJECTIVE AND IMPORTANCE: Internal drainage of cerebrospinal fluid to the abdominal cavity via a ventriculoperitoneal shunt (VPS) is a common procedure for therapy of obstructive hydrocephalus; because this condition is often caused by brain tumors blocking the natural cerebrospinal fluid pathways, the VPS as an artificial anastomosis can provide the means for the spreading of tumor cells by the cerebrospinal fluid. We report the case of a VPS-related abdominal metastasis of a teratocarcinoma and review the pertaining literature. CLINICAL PRESENTATION AND INTERVENTION: A 24-year-old man with a history of three brain tumors that were operated on when the patient was 14, 21, and 23 years of age developed an acute ileus 7 months after VPS insertion for cerebral teratocarcinoma. Intraoperatively, a massive abdominal tumor was observed, which turned out to be a peritoneal metastasis of the aforesaid brain tumor. The patient died as a result of his illness 1 month later. RESULTS: To date, 58 VPS-related metastases of brain tumors have been described. The male-to-female ratio is 1.6:1, the mean age at shunt insertion is 12.2 years, and the interval between shunt operation and diagnosis of metastases is 16.8 months. During the observation time, 69.2% of the patients died as a result of their illness or abdominal metastases. The most common sources of the metastases were germinomas (27.7%), medulloblastomas (19.1%), and endodermal sinus tumors (10.3%). CONCLUSION: The presented case is only the second VPS-related abdominal spreading of a cerebral teratocarcinoma. Metastases via VPS are rare but should be considered as a possible complication and mode of systemic spread in patients with primary intracranial malignancy.

Radiation-induced myelopathy and vertebral necrosis.

Radiation-induced myelopathy is often a diagnosis of exclusion. In addition to the classic criteria needed to support the diagnosis, the presence of another radiation-induced lesion, such as aseptic vertebral necrosis, is useful to confirm the cause of the spinal cord lesion.

[Multifocal germ cell tumors]. / Tumeurs germinales multicentriques.

A 16-year old male presented with a mediastinal germ cell tumor (seminoma) treated by combined surgery, radiotherapy and chemotherapy. Nine years later, he presented with an intracerebral germ cell tumor affecting both the suprasellar ventricles and the pineal area. After partial removal of the intraventricular tumor, the patient received radiotherapy and chemotherapy. No metastases could be found. He died 8 months later, probably from secondary cardiovascular disturbances. This case seems to be the first one to illustrate the possibility of multifocal extragonadal seminoma.

[Pseudotumoral medullary syndrome manifesting a probably beginning multiple sclerosis. Clinical, radiological and neuropathological correlations]. / Syndrome médullaire pseudo-tumoral révélant une sclérose en plaques probable débutante. Corrélations cliniques, radiologiques et neuropathologiques.

A 19-y old sportsman was admitted because he complained of ascending paresthesia since two weeks, muscular strength diminution in both arms, then respiratory difficulties without fever. Clinical examination confirmed an asymmetric bilateral pyramidal syndrome affecting the four limbs, a hyposensitive level up to C5, without meningeal symptoms. MRI detected a widening of the cervical (C2-C5) spinal cord, with gadolinium enhancement. CSF demonstrated lymphocytic pleocytosis and total protein of 530 mg/l with an oligoclonal IgG pattern. With a diagnosis of multiple sclerosis, a corticoid therapy was started but without clinical improvement. Six weeks later, a new MRI revealed an increased enlargement of the cervical spinal cord suggesting a tumoral process. No tumor could be detected at neurosurgery. Biopsy specimen (from C3-C4) demonstrated an inflammatory demyelinating disease with astrocytic hypertrophy. Electron microscopy confirmed the active demyelinating disorder with persistently denuded axons. Several macrophages were observed, but no lymphocytic invasion. Astrocytes were prominent in some areas with "en plaque" gliosis. MRI failed to discover any other demyelinating lesions. Further clinical evaluation was inconspicuous, the patient regaining full activities after 6 months. MRI after 9 months demonstrated no spinal cord lesion. This observation contributes to suggest the influence of the blood-brain barrier breakdown on the occurrence of clinical and radiological signs of new lesions in suspected multiple sclerosis.

Soft cervical disc herniation: a retrospective study of 100 cases.

During the period from January 1975 to January 1991, 100 patients with soft cervical disc herniation were operated on by either the anterior (84 patients) or posterior (16 patients) approach. There were 33 women and 67 men, aged from 25 to 67 years (average: 41.5%); 97% presented with radicular symptoms and/or signs while 15% presented with long tract symptoms and/or signs. The patients were evaluated by computed tomography and/or myelography. Thirty-six of the lesions occurred at the C 5/6 level and 54% at the C 6/7 level. Seventy-seven % reported total or partial relief of preoperative symptoms and signs postoperatively. Patients presenting with radiculopathy had better outcome than those presenting with combined radicular and spinal cord involvement.

MRI-pathological correlations in acute traumatic central cord syndrome: case report.

Ante- and post-mortem MRI and detailed pathological examination were performed in a patient with a typical acute traumatic central cord syndrome (ATCCS) after a minor hyperextension injury to the neck who died 60 h later from heart failure. T2-weighted MRI showed a central hyperintense area at C3-4. There were disc protrusions, but no vertebral fracture or displacement. Histopathology disclosed severe axonal swelling and oedema in the dorsolateral fasciculi and, to a lesser degree, in the dorsal columns. In addition, an area of recent necrosis was found in the right anterior horn at C4-5. These findings suggest that the pathological hallmark of typical ATCCS is mechanical axonal disruption at a segmental level, but that more severe trauma may be accompanied by tissue destruction.

Traction diverticulum of the hypopharynx following anterior cervical spine surgery. Case report and review.

Pharyngeal complications due to anterior cervical spine surgery (ACSS) are not rare. We describe the case of a traction diverticulum of the hypopharynx after ACSS, a complication that has not yet been reported. We discuss the possible mechanism. A review of the complications of ACSS that are of interest to the otolaryngologist is included.

Primary intracerebral osteosarcoma.

A 64-year-old man was admitted because of a rapidly progressive left side hemiparesis. A computed tomography scan showed a right side intrathalamic mass, partially calcified. Surgical specimen proved to be an osteosarcoma. The patient died 3 days later from an acute myocardial infarct. Autopsy failed to discover any other significant lesion. A review of the literature disclosed that this case might be the second published primary intracerebral osteosarcoma and the first with immunocytochemical analysis.

[Facio-craniostenosis]. / Les facio-craniosténoses.

Facio-craniostenosis. Facio-craniostenosis is associated with premature stenosis of one or several cranial sutures. The clinical variety is defined by the affected suture. In the complex forms, major disturbances of the anterior fossa of the cranial base are observed. Intracranial hypertension sometimes observed may lead to impairment of the function of the brain or the eyes. The authors have observed 73 children with craniostenosis and have operated upon 49 of them. Three types of methods have been used. The stress is made on the latest one combining advancement of the orbito-frontal "bandeau" and the regularization of the vault. Operating early is essential for prevention of functional sequellae. The optimal age for surgical treatment seems to be between 3 and 6 months. Complications have been remarkable benign.

[Ablation by the anterior transcorporeal approach of an ante-spinal cord cervical meningioma. Apropos of a case]. / Ablation par voie transcorporéale antérieure d'un méningiome cervical anté-médullaire. A propos d'un cas.

The authors report a case of an intradural spinal meningioma ventrally located just rear to the 5th cervical body. The patient (a 46-year-old woman) suffered from a spasmodic quadriplegia, walking was nearly impossible. Because of the strictly median and ventral localization the authors have chosen an anterior approach passing through the body of C5 after discectomy of C4-C5 and C5-C6 discs. The tumor and its dural attachment was totally removed with respect to the spinal cord and all rootlets. Reconstruction of cervical spine was performed with an iliac bone graft. The neurological recuperation was complete and quickly achieved. Bone fusion was obtained within 3 months with a good result. The authors emphasize the rarity of ventrally located meningiomas in the cervical spine and the advantages of anterior route which alone enables a total surgical removal of such lesions.