[Microsurgical experiments in 12 cases of intramedullary hemangioblastomas].
12 patients (8 males and 4 females) operated on for intramedullary hemangioblastomas, using microsurgical techniques are presented. Special consideration is given to the clinical and pathological aspects. The results of surgery are discussed. The family history is positive in 4 cases. Clinical aspect : a) the initial symptoms is usually pain (9 patients) motor deficits were present in 2 cases. Only 1 patient presented visual disturbances at the beginning of the disease. b) the interval of time between the first symptom and the fully developped clinical picture is shorter than 1 year in 4 patients and longer (up to 18 years) in 8 patients. c) the full clinical picture consists of motor deficit, sensory disturbances (especially dorsal columns), cerebellar signs, raised intracranial pressure, sphincter disturbances, retinal angiomas, arterial hypertension and psychoorganic syndrome in decreasing order of frequence. Pathological findings at opsurgery : There are. a) 3 cases of hemangioblastomas at the bulbo-cervical junction b) 5 cases of cerebello-medullary hemangoblastomas. c) 4 cases of medullary hemangioblastomas. 1 patient had renal and pancreatic cysts. 2 patients had pheochromocytomas with arterial hypertension. Operative technique. Microsurgical techniques, bipolar coagulation, and continuous irrigation of the operative field are of primordial importance. The tumor must be approached from the periphery and never from the center. One should coagulate the afferent vessels first and the efferent vessels only at the end. Results of surgery. In 1 case, only a partial removal was possible and the patient did not improve. A complete removal was possible in 11 patients with the following results : -- 4 patients are in excellent condition and have a normal socio-professional life. -- 5 patients improved and are independant. The are able to perform partial time-work. -- 1 patient, with bulbar extension of the tumor improved from the motor point of view, but he presents a permanent. deficit of the IX, X, XI cranial nerves. He has a permanent tracheal canula. -- 1 patient improved following surgery, but he died 12 months later (stress ulcer and cardiac decompensation). The autopsy revealed a recurrence of the cerebellar tumor.
D000328:Adult; D002531:Cerebellum / Q000473:pathology; D005260:Female; D006394:Hemangiosarcoma / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology; D008866:Microsurgery / Q000379:methods; D009069:Movement Disorders / Q000209:etiology; D010375:Pedigree; D013120:Spinal Cord Neoplasms / Q000473:pathology / Q000601:surgery*; D014786:Vision Disorders / Q000209:etiology
Expériences microchirurgicales dans 12 cas d'hémangioblastomes intramédullaires