Bénédict Rilliet

Early surgery for brainstem cavernomas

SummaryBackground. The purpose was to review our experience with the surgical management of brainstem cavernomas (BSCs) and especially the impact of the surgical timing on the clinical outcome.Method. We retrospectively reviewed 22 patients harboring a BSC, who underwent 23 procedures.Findings. Surgery was carried out during the early stage after the last haemorrhage, with a mean delay of 21.6 days (range 4–90 days). Sixteen procedures were performed after a first bleeding event while seven after multiple bleedings. Complete resection was achieved in 19 patients (86.4%). Early after surgery, 12 patients (52.2%) improved neurologically, 5 (21.7%) were stable and 6 (26.1%) worsened. New postoperative deficits were noted after 9 procedures (39.1%). Statistically significant factors for postoperative aggravation were: late surgery (P = 0.046) and multiple bleedings (P = 0.043). No patient operated on within the first 19 days after bleeding did worsen (n = 11), as opposed to 6 out of 12 who did when operated on later. After a mean follow-up of 44.9 months, 20 patients (90.9%) were improved, 1 patient (4.6%) was worse and 1 patient was lost to follow-up (4.6%), after reoperation for rebleeding of a previously completely resected cavernoma. Late morbidity was reduced to 8.6%. The mean Glasgow Outcome Scale (GOS) at the end of the follow-up period was 4.24, compared to a mean preoperative GOS of 3.22 (P<0.001). Complete neurological recovery of motor deficits, sensory disturbances, cranial nerves (CNs), internuclear ophtalmoplegia and cerebellar dysfunction were respectively 41.7%, 38.5%, 52.6%, 60.0% and 58.3%. Among the most affected CNs: CN 3, CN 5 and CN 7 were more prone to completely recover, respectively in 60.0%, 70.0% and 69.2%.Conclusions. Surgical removal of BSCs is feasible in experienced hands with acceptable morbidity and good outcome. Early surgery and single bleeding were associated with better surgical results.

Dynamic MR angiography (MRA) of spinal vascular diseases at 3T

Spinal magnetic resonance angiography (MRA) is difficult to perform because of the size of the spinal cord vessels. High-field MR improves resolution and imaging speed. We examined 17 patients with spinal vascular diseases with dynamic contrast-enhanced three-dimensional MR sequences. In three patients, the artery of Adamkievicz could be seen; we could also detect all arteriovenous malformations and dural fistulas. MRA has the potential to replace diagnostic spinal angiography and the latter should be used only for therapeutic purposes.

Cefuroxime Prophylaxis Is Effective in Noninstrumented Spine Surgery : A Double-Blind, Placebo-Controlled Study

Study Design. Double-blind, placebo-controlled randomized clinical trial. Objective. To assess the efficacy of 1 preoperative 1.5 g dose of cefuroxime in preventing surgical site infection after surgery for herniated disc. Summary of Background Data. Antibiotic prophylaxis was only tested in nonconclusive trials in this setting. Methods. The study was conducted in 2 university hospitals in Switzerland. Patients were assessed for occurrence of surgical site infection (defined by the criteria of the Centers for Diseases Control and Prevention), other infections, or adverse events up to 6 months after surgery. Outcome measures were compared in a univariate, per-protocol analysis. Results. Baseline characteristics were similar in patients allocated to cefuroxime (n = 613) or placebo (n = 624). Eight (1.3%) patients in the cefuroxime group and 18 patients (2.8%) in the placebo group developed a surgical site infection (P = 0.073). A diagnosis of spondylodiscitis or epidural abscess was made in 9 patients in the placebo group, but none in the cefuroxime group (P < 0.01), which corresponded to a number necessary to treat of 69 patients to prevent one of these infections. There were no significant adverse events attributed to either cefuroxime or placebo. Conclusion. A single, preoperative dose of cefuroxime significantly reduces the risk of organ-space infection, most notably spondylodiscitis, after surgery for herniated disc.

Introduction of a urodynamic score to detect pre- and postoperative neurological deficits in children with a primary tethered cord

ObjectAn increasing number of asymptomatic children are diagnosed with occult spinal dysraphism, raising the question of their optimal management. Urodynamic study (UDS) is the most reliable method of detecting neuro-urological abnormalities in these children. The rate of postoperative retethering ranges from 10 to 20% and is not always immediately clinically significant. The aim of this prospective study was to develop a reliable method that could be used in the preoperative assessment and postoperative follow-up of children with a tethered cord syndrome (TCS).MethodsFrom 1989 to 1997, 15 children underwent spinal cord untethering for TCS. Preoperatively, patients were assessed with MRI and UDS. Postoperative UDS were repeated at 6- to 12-month intervals. Four UDS parameters were identified, graded, and added to obtain a UDS score. A group of 38 children without dysraphic condition was used as control and allowed the calculation of a normal score.ConclusionsThere was a statistically significant difference in the preoperative UDS scores between the control group and the study group (p<0.001). Postoperatively, there was a statistically significant improvement (p<0.001) in UDS scores. UDS score is a reliable tool for identifying and quantifying neuro-urological disorders in patients with TCS. Postoperatively, this score was useful in the early diagnosis of spinal cord retethering.

Gliomas in children: a review.

Abstract The management and prognosis of gliomas are significantly different in children and in adults. Fortunately, the proportion of gliomas that are malignant is smaller in children than in the adult population. The different types of gliomas encountered in the pediatric population are reviewed, taking account of the most recent contributions on this subject. The importance of considering both localization and histological classification for better definition of the prognostic factors of each subgroup is emphasized. A brief review of the possible causes of gliomas is presented. Unfortunately, the information obtained by molecular and genetic study of these tumors has still not resulted in anything that can help the children suffering from gliomas in a concrete way. Surgery, with all its recent refinements, remains the best treatment for the majority of benign gliomas providing they can be removed without unacceptable sequelae. The role of chemotherapy has emerged recently for the treatment of nonresectable low-grade gliomas, such as hypothalamic- chiasmatic tumors, especially for infants, in whom the adverse effects of radiotherapy can be severe and irreversible. On the eve of the new millennium, there is renewed hope that the problem of malignant gliomas will be solved in the not-too-distant future.

Frontosphenoidal synostosis: a rare cause of unilateral anterior plagiocephaly

IntroductionWhen a child walks in the clinic with a unilateral frontal flattening, it is usually associated in our minds with unilateral coronal synostosis. While the latter might be the most common cause of anterior plagiocephaly, it is not the only one. A patent coronal suture will force us to consider other etiologies, such as deformational plagiocephaly, or synostosis of another suture. To understand the mechanisms underlying this malformation, the development and growth of the skull base must be considered.Materials and methodsThere have been few reports in the literature of isolated frontosphenoidal suture fusion, and we would like to report a series of five cases, as the recognition of this entity is important for its treatment.ConclusionFrontosphenoidal synostosis must be searched in the absence of a coronal synostosis in a child with anterior unilateral plagiocephaly, and treated surgically.

Occipital Condyle Fractures in Children

Occipital condyle fractures (OCF) are seldom described in children, with only 14 cases reported in the literature. We report the observation of a 13-year-old child in whom such a fracture was diagnosed. A review of the paediatric cases allowed us to identify the clinical features of OCF in children. The average age was found to be 10 years, and the causative traumatism was most often related to road traffic or sporting accidents with associated head injury. The level of consciousness was depressed in 67% of the cases, and in 53%, an injury to the cranial nerves, brain stem or high spinal cord was present. Neck pain with reduction of head mobility was reported in 27% of the cases. Treatment was generally conservative, i.e. a hard collar or halo vest for an average of 8 weeks. The course was favourable with respect to osseous consolidation, mobility and pain. On the other hand, the neurological deficits improved, but sequelae remained. Early diagnosis and treatment is mandatory since the secondary displacement of fractured fragments can injure the cranial nerves in a delayed fashion or even be fatal due to compression of the brain stem.

Association of multiple vertebral hemangiomas and severe paraparesis in a patient with a PTEN hamartoma tumor syndrome. Case report.

The PTEN hamartoma tumor syndrome, manifestations of which include Cowden disease and Bannayan-Riley-Ruvalcaba syndrome, is caused by various mutations of the PTEN gene located at 10q23. Its major criteria are macrocephaly and a propensity to develop breast and thyroid cancers as well as endometrial carcinoma. Minor diagnostic criteria include hamartomatous intestinal polyps, lipomas, fibrocystic disease of the breasts, and fibromas. Mutations of PTEN can also be found in patients with Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). The authors report the case of a 17-year-old girl who had a severe cyanotic cardiac malformation for which surgery was not advised and a heterozygous missense mutation (c.406T>C) in exon 5 of PTEN resulting in the substitution of cysteine for arginine (p.Cysl36Arg) in the protein, which was also found in her mother and sister. The patient presented in the pediatric emergency department with severe spastic paraparesis. A magnetic resonance imaging study of the spine showed vertebral hemangiomas at multiple levels, but stenosis and compression were maximal at level T5-6. An emergency T5-6 laminectomy was performed. The decompression was extremely hemorrhagic because the rapid onset of paraparesis necessitated prompt treatment, and there was no time to perform preoperative embolization. The patients postoperative course was uneventful with gradual recovery. This represents the first report of an association of a PTEN mutation and multiple vertebral angiomas. The authors did not treat the remaining angiomas because surgical treatment was contraindicated without previous embolization, which in itself would present considerable risk in this patient with congenital cyanotic heart disease.

Leptomeningeal cyst in newborns due to vaccum extraction: report of two cases

Two new cases of leptomeningeal cysts subsequent to vacuum extraction are reported. Both children presented with a huge, nonpulsating, transilluminating subgaleal collection over the anterior fontanel that appeared soon after instrument delivery. Plain X-rays, computed tomography, and magnetic resonance imaging confirmed that the subgaleal collection was cerebrospinal fluid and showed the presence of a diastatic coronal suture in both cases. Treatment consisted of duraplasty with periosteal flaps and application of fibrin glue. In one case, an associated porencephalic cyst was treated with a cystoperitoneal shunt. Surgical treatment of leptomeningeal cyst due to vaccum extraction is simple and should not be postponed, despite the tendency for the extracrainal cyst to regress, because of the potential risk of continuous growth of an underlying porencephalic cyst and risk of neurological damage.

The Geneva and Lausanne (French-speaking Switzerland) experience: in favor of the transsphenoidal approach when feasible

BackgroundThe authors present their current attitude towards management of craniopharyngiomas in children. Radical surgery cannot be performed when one is not sure about its potential danger to the visual pathways and the hypothalamus.MethodsMost of the surgeries that have been performed in our institution via an intracranial approach were incomplete and followed by radiotherapy, in the last 10 years with stereotactic conformational radiotherapy.DiscussionConsidering our past and present experience concerning the transphenoidal approach for treatment of craniopharyngiomas, we make a plea for the consideration of this approach in children whenever feasible (around 25% of the cases in children older than 5 years). Craniopharyngiomas that can be removed by this approach represent a milder form of the disease and the results concerning the visual, hypothalamic functions and quality of life are significantly better than that obtained via an intracranial approach. When the craniopharyngioma cannot be removed by this approach because of tight hypothalamic adherences, the technique of cystosphenoidostomy with a custom-made shunt plus adjuvant stereotactic conformational radiotherapy is an alternative for its total removal.