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.

Two-dimensional echographic localization of intracardiac cerebrospinal fluid shunt catheters.

This study evaluated the ability of two-dimensional echocardiography (2D echo) to detect and localize the distal end of ventriculoatrial shunt catheters in the cardiac chambers of hydrocephalic patients. Twenty-eight patients were studied, and the performances of 2D echo and standard chest x-ray filming were compared. Although standard chest x-ray filming allowed accurate determination of the catheter position in only half of the patients, 2D echo afforded clear visualization and accurate localization of the catheter in all instances. By its precise definition of intracardiac anatomical landmarks and its excellent time determination, 2D echo can provide valuable information on the movements of the catheter's distal end during the cardiac cycle, during head flexion or rotation, and during deep breathing movements. Some interesting observations were made with 2D echo. A to-and-fro motion of the catheter tip through the tricuspid orifice during the cardiac cycle was visualized in two instances. The combination of head movements and deep respiratory movements induced a variation in the position of the catheter tip. The magnitude of this displacement was 6 cm at the most. Two patients underwent reoperation for lengthening or shortening of the catheter. The operative findings confirmed the ultrasonic localization in both cases where the chest x-ray film had failed to identify or adequately locate the catheter tip. It is concluded that 2D echo is a safe, innocuous, and accurate method with which to locate the distal end of a ventriculoatrial shunt. It is the only available technique that provides visualization of intracardiac structures and cardiac cycle reference.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

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.

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.

[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.

[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.

[Total venous autograft of the superior longitudinal sinus. Experimental study in the dog (author's transl)]. / Autogreffe veineuse totale d'un segment de sinus longitudinal supérieur. Etude expérimentale chez le chien.

The authors describe an original technique for the total replacement of a segment of the superior sagittal sinus in the dog. The graft, by its venous structure lined with a dural wall and by its triangular shape ensured through a certain tension, is close to the anatomical aspect. The authors use an autogenous vein graft. The latter is kept open by a wide suture to a fragment of dura which is fastened to the falx and to the dura of the vault. This fragment forms a "V" gutter in which lies the venous graft, thus protected from post-operative brain oedema. After suturing, a first sinusography is done, and a second one between the 7th and the 15th post-operative day. A study is thus made of the permeability of the graft. This permeability depends on the quality of the sutures and on the reconstruction of the dural wall of the sinus. Twenty operations have been carried out using various techniques. With the technique described, there were 11 permeable sinuses for 11 operations shown by the first sinusography. On second sinusography, 8 sinuses in 11 remained permeable.

[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.

[Practical assessment of brain dysfunction in severe head trauma (author's transl)]. / Evaluation pratique du dysfonctionnement encéphalique chez le traumatisé crânien.

Assessment of brain dysfunction in head injured patients is important as an index of severity of brain damage and forms the basis of monitoring. . The Glasgow Coma scale (G.C.S.) is a practical scale for nurses. The Liege Coma scale contains the three variables of the G.C.S. and provides informations concerning the brain stem reflexes. In a series of 60 head-injured patients, recovery rates were calculated on the basis of either Glasgow scores or Liege scores. The Liege Coma scale provides a more sensitive index of clinical course and a better prognosis.

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.

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.

[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.

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.