Biomedical subjects
Publications and source records attributed to P Bret.
We report a case of metastatic renal carcinoma in a multirecurrent cerebellar haemangioblastoma (HBL) in an adult patient with von Hippel Lindau (VHL) disease. To our knowledge, only two cases of metastases to intracranial HBLs have been reported.
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OBJECTIVE: Evaluate the advantages, results and drawbacks of the Sophy adjustable valve (SV) in the treatment of hydrocephalus in the adult. MATERIAL AND METHODS: One-hundred and forty-seven adult hydrocephalic patients (mean age = 64.5 years) shunted with a SV between 1990 and 1997 were retrospectively reviewed. Etiologies of hydrocephalus were: "normal pressure" chronic hydrocephalus (NPH) in 124 and tumoral hydrocephalus with intracranial hypertension in 23 patients. One-hundred fifty shunts were established in 147 patients: 14 ventriculoatrial and 136 ventriculoperitoneal shunts. The initial pressure settings of the SV were: high pressure: 12 times (8%), medium pressure: 136 times (90.6%) and low pressure: 2 times (1.3%). RESULTS: Sixteen patients were lost to follow-up. Two patients died within hours following the procedure. So, the results were analyzed in the 129 remaining patients, with a follow-up of 16.7 months (+/- 17.8). Three patients died from shunted-related causes (2.3%). Eighty-four patients had good results (65%), 25 had fair results (19.5%) and 20 failed to improve or deteriorated (15.5%) after shunting. Fifty-three reoperations were performed in 44 patients (34%), including 37 procedures for mechanical obstruction and 6 for septic complications. Subdural effusions (SDE) occurred in 16 patients (12.5%): 10 surgical evacuations were required in 7 patients; 8 patients were successfully treated using uprating of the SV pressure alone; one was managed conservatively. Fifty-eight pressure readjustments were performed in 47 patients. The pressure settings were uprated 28 times in patients showing clinical or computerized tomographic (CT) features suggesting overdrainage; 20 of those proved to be successful (71.5%). The pressure settings were lowered 30 times in patients showing a lack of improvement after shunting, attributed to underdrainage; only 12 of those proved to be successful (40%). Spontaneous changes of the pressure settings were observed in 14 patients (10.8%). After a MRI exposure, SV pressure changes were observed in 64% of patients. DISCUSSION: The technological refinements that have been proposed to eliminate the overdrainage which occurs in most usual cerebrospinal fluid shunts are reviewed. The SV valve has allowed non-invasive adjustments of its opening pressure in patients showing complications ascribed to inappropriate drainage. The need for additional surgery was eliminated in 20% to 25% of patients of the present series. Because of frequent spontaneous and magnetic resonance-induced pressure changes, the need for repeated controls of valve pressure is to be reemphasized. CONCLUSION: This experience suggests that the SV represents a substantial improvement over standard fixed-pressure valves.
An unusual case of thoracic spinal cord compression caused by extradural tophaceous deposits is reported in a 59-year-old female with a long-standing history of gout involving the metatarsophalangeal joints. T1 and T2 magnetic resonance images of the spine illustrated an extradural hyperintense signal extending from T2 to T9. A decompressive laminectomy disclosed a white caseum-like material in the extradural space, together with a small organized hematoma. Histologic examination showed areas of amorphous substance containing urate crystals surrounded by inflammatory cells, which was diagnosed as a gouty tophus. The patient made an uneventful recovery after surgery. Fifteen similar cases of the literature are reviewed. Although spinal involvement by gout seems relatively common, a compression of the spinal cord or of the cauda equina in gout patients seems exceptional. The diagnosis should be considered in patients showing a relevant history of gout, but spinal cord compromise may also represent the initial manifestation of the disease.
The purpose of the present study is to evaluate retrospectively the effects of several intra-operative manipulations on the results of foramen magnum decompression (FMD) in patients having syringomyelia associated with type I Chiari malformation. Seventy-five patients having syringomyelia associated with Chiari I malformation were operated on between 1975 and 1996. This population was grouped into 4 subgroups according to the surgical protocol: group I = 42 patients with FMD alone; group II = 16 patients with FMD and third ventricle shunting; group III = 9 patients with FMD and syringosubarachnoid shunting (SSS); group IV = 8 patients with FMD and cerebellar tonsils resection. Pre- and postsurgical magnetic resonance imaging (MRI) studies were analyzed (and compared). Nine patients were lost to follow-up. The results were evaluated in the 66 remaining patients (mean follow-up: 52 months), using the Bidzinski's outcome scale (ref). Two patients (3%) died postoperatively, 31 (47%) had very good results (after additional surgery in 7), 16 (24.2%) had good results (after additional surgery in 7) and 17 (25.7%) had poor results despite further surgery in 9. A total of 27 reoperations were undertaken after primary FMD in 23 patients (35%). Thirty-nine patients (59%) had both pre- and postsurgical MRI evaluation. In 28 (72%) the syrinx had markedly decreased whereas it had remained stable in 11 (28%). Clinical results were not significantly different between the patients of groups I, II and III. Very good or good results were obtained in 24 patients (64.8%) of group I (after additional surgery in 10), in 8 (61.5%) of group II (after additional surgery in 1) and in 7 (87.5%) of group III (after additional surgery in 3). Results in group IV were as follows: 7 patients (87%) had very good results and one had a good result. With a mean follow-up of 28 months, no patient required additional surgery. Postsurgical MRI syrinx reduction was observed in all 8 patients either in the early postoperative course or on delayed followup. It is suggested that tonsils resection might enhance the results of FMD in individuals having Chiari I-related syringomyelia.
A series of 25 adult patients surgically treated for a tethred cord syndrome is reported. Preoperatively 19 patients presented with a sensorimotor deficit in their lower limbs, 17 with sphincter disturbances, 12 with pain and/or neuroorthopedic symptoms and 9 with cutaneous lumbar anomalies. At surgery, an isolated anomaly (lipoma, anomalous or adherent filum terminale) was disclosed in 18 patients. In the remaining 7, a more complex form of dysraphism was disclosed. Follow-up ranges from 3 months to 20 years (mean: 6.5 years). Ten patients improved, 6 were stabilized and 9 showed continuous worsening. The best results were obtained in patients in whom the cord tethering resulted from an anomalous filum terminale. Results were significantly worse in patients suffering long standing symptomatology and showing either radiologically or surgically mixed mechanisms of cord tethering. Early surgical correction should be idealy undertaken in patients suffering from minor neurological deficits and in whom magnetic resonance imaging illustrates a low conus medullaris attached by a short thickened filum terminale.
BACKGROUND: The management of bile leaks has evolved in the laparoscopic era. This study characterizes risk factors for their developmental and their clinical course and management. METHODS: Data on a cohort of patients who developed bile leaks after cholecystectomy in the laparoscopic era were gathered prospectively and retrospectively from an ongoing surgical database and following a review of hospital charts. RESULTS: Sixty-four patients (mean age 56 +/- 17.1 years, 72% women) were included over a 5-year study period. The incidence of leaks was 1.1% among patients entered in a laparoscopic cholecystectomy database. Intraoperative complications were encountered in 36%. Rates of intraoperative complication and conversion to open surgery were greater among patients who developed leaks (5.2% vs 0.6% and 33% vs 6.3%, respectively, p < 0.00001). Patients presented 5.3 +/- 4.2 days following surgery with abdominal pain (89%), fever (74%), and tenderness (81%). Ultrasound diagnosed a suspected leak in 73%, which ERCP showed as originating from the cystic duct stump in 77%. Biliary obstruction was noted in 20 (31%) patients (14 with stones). Treatments included percutaneous (13%), endoscopic (28%), primary or secondary operative procedures (14%), or a combination thereof (45%). CONCLUSION: A complication at laparoscopic cholecystectomy increases the likelihood of a subsequent bile leak. Most patients present early with a patent cystic duct stump in the absence of biliary obstruction. Endoscopic therapy is successful in the majority of cases, but otherwise percutaneous or operative procedures may be needed.
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Mucinous cystic neoplasms of the pancreas may present in a "ductectatic" form, which parallels a distinct clinical presentation. We describe six patients with this entity termed mucinous ductal ectasia, or intraductal papillary mucinous neoplasm. All six patients presented with typical clinical and endoscopic findings and subsequently, almost all were found to have mucinous ductal cystadenocarcinomas. The endoscopic and pancreatographic findings associated with an intraductal papillary mucinous neoplasm are characteristic, unique, and yield a high diagnostic accuracy. It is important to recognize these features of intraductal papillary mucinous neoplasm since the tumor has a lower malignant potential than adenocarcinoma of the pancreas, and surgical resection is curative in many cases.
Effective chemotherapy using PCV (procarbazine, lomustine and vincristine) has been documented in anaplastic oligodendrogliomas and oligoastrocytomas. A pilot study using PCV was conducted for relapsing patients with anaplastic oligodendrogliomas and oligoastrocytomas. Preliminary results are reported. Fourteen patients were enrolled. All received at least two courses of PCV and were evaluable for response. Eleven patients (78%) responded to chemotherapy with complete responses in 2 patients. Response was more obvious regarding contrast enhanced areas than volumes changes (11 responses versus 7). A story of seizure was the main clinical prognostic factor for response. All toxicities were manageable and no treatment related death occurred. Chemotherapy is an effective treatment in aggressive oligodendrogliomas. Further studies must assess the role of chemotherapy in the multidisciplinary management of oligodendroglioma.
We report a series of 8 patients with syringomyelia and associated Chiari I malformation treated between 1992 and 1996 using a cranio-vertebral decompression with tonsil resection. The follow up period ranged from 6 months through 4 years (mean: 28 months). Neurological symptoms and signs improved in 7 patients (87%), and were stabilized in 1 (13%). At 6 months, postoperative MRI showed that the syringe has decreased in all patients. We emphasize the respect of the different stages of surgical procedure (limited foramen magnum opening, arachnoid preservation, tonsillar subpial aspiration, arachnoid suture and dural closure). Other of treatment and their results are reviewed.
A case of intramedullary subependymoma (SE) of the cervical spinal cord is reported. A 35 year-old female patient developed paresthesiae in her right upper and lower limbs for approximately two years. Magnetic resonance imaging (MRI) of the cervical spinal cord disclosed an intrisic medullary tumor at the C2-C3 levels, predominantly involving the right hemicord. At surgery, a subpial ependymoma was totally removed using the ultrasonic surgical aspirator with no resulting anomalies of the intraoperative somatosensory evoked responses. The patient made a good recovery. At one-year follow-up, she had resumed her previous occupation and complained only from minor sequelae. Twenty-nine spinal cord SE of the literature are detailed. It is likely that they account for less than two per cent of all spinal cord tumors. A majority of cases involved the cervical cord or the cervicothoracic junction. Signs and symptoms are similar to those of other spinal intramedullary tumors. In 17 cases explored by MRI, no specific pattern could distinguish SE from astrocytomas or ependymomas, except perhaps for a more marked tendency for SE to grow excentrically within the cord or to show an exophytic component. Spinal cord SE show pathological features grossly comparable to those described in intracranial SE with clusters of small glial cells scattered among densely packed fibers. Histogenesis of SE has been a matter of debate over years, whereas it is currently admitted that they may represent a variant of ependymomas. In conclusion, most clinical and imaging features of spinal cord SE are similar to those of other pathological varieties. Radical removal is the most appropriate treatment modality, despite inherent risk of postoperative deterioration and significant sequelae on long term follow-up.
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The cases of 15 children operated on for cerebral pure oligodendroglioma were studied. Two groups of children were distinguished. Group I children presented with epilepsy (7 cases); their tumor was histologically benign in all cases. These children are all alive after a median follow-up of 72 months: however, 2 of them presented with a local recurrence, which was operated on, and are actually disease-free. Group 2 children presented with intracranial hypertension (8 cases); the tumor was anaplastic in 7 cases. Despite postoperative radiotherapy and chemotherapy 6 children died; their median survival time was 17 months. The present series shows the existence of a clear correlation between clinical presentation, histological grading and survival in childhood cerebral oligodendrogliomas.
Radiosurgery (RS) was initiated in Lyon in October 1989. The technique was adapted from that described by Lutz and Saunders in Boston (BRW stereotactic frame). Irradiation is delivered with 18-MV photons produced by a LINAC. From December 1989 to December 1992, 41 patients with arteriovenous malformations were treated by RS; the median age was 33 years. The largest lesion diameter was 11.2-38.5 mm. Fifteen to 20 Gy were delivered on the 70% isodose line. Angiography was performed at 2 years post-treatment in 32 patients demonstrating an overall complete thrombosis rate of 81.3%. This incidence was significantly correlated with the Spetzler and Martin grade before RS (P = 0.0055). Two patients (4.9%) experienced haemorrhage after radiosurgical treatment and one died from an intracerebral-intraventricular haemorrhage. Four patients (9.7%) experienced permanent radiation-induced neurological complications.
We report for the first time in the recent North American literature, the case of a patient with rupture of a hepatic hydatid cyst into the gallbladder, with subsequent obstruction of the cystic duct by a daughter cyst acting as a ball-valve and causing acute acalculous cholecystitis.