[Adams-Oliver syndrome: a case with minimal expression]. / Syndrome d'Adams-Oliver : à propos d'un cas d'expression minime.

Adams-Oliver syndrome is a rare congenital anomaly characterized by aplasia cutis congenita (ACC) and variable degrees of terminal transverse limb defects. We report on a neonatal case with the sporadic form of the disease with minimal expression, illustrating the wide spectrum of clinical expression in Adams-Oliver syndrome. We also review the literature and highlight the different pathogenetic hypotheses of this syndrome.

[Transverse sacral fracture in children: a case report presenting with neurological injury and review of the literature]. / Fracture transversale du sacrum chez l'enfant.

Transverse fractures of the sacrum are exceptional in children. We report a case in a 10-year-old girl. The patient presented an isolated flexion fracture of the sacrum in Denis zone III (transverse "U" fracture) of S1-S2 with neurological signs at the initial examination: sensorial deficit in the perineum and sphincter dysfunction. Treatment consisted in laminectomy and bone resection to relieve compression causing the neurological injury. Orthopedic treatment led to correct bone healing. Outcome was favorable with complete resolution of the neurological deficit and stability at three years. Eight cases of transverse sacral fracture before the age of 18 years have been reported in the literature. The diagnostic elements are similar to those in adults, but can be missed in children who rarely present sacral fracture. The therapeutic approach has varied, both for children and adults. We advocate surgical treatment in the event of neurological complications and orthopedic treatment of stable bone lesions.

[Intracranial cavernoma. Surgical results of 47 cases]. / Cavernomes intracrâniens. Résultats chirurgicaux de 47 cas opérés.

We report the surgical results in a series of 47 patients with cerebral cavernous malformation who had undergone surgery between 1973 and 1994, with a follow up ranging from 12 months to 24 years (mean: 4 years). They were divided in there groups according to their initial clinical presentation: epilepsy (31 cases), hemorrhage (11 cases) and neurological deficit (5 cases). Surgery consisted of cavernoma resection only (11 cases) or its extension to surrounding gliotic tissue (36 cases). Results are satisfactory: no surgical mortality, low morbidity (4 cases), no recurrent hemorrhage, seizures disappearance with anticonvulsant therapy stop (4 cases) or alleviation (20 cases). Only one patient died far from surgery (6 months) consequently to his initial bleeding, while all the others lead a normal active life. The therapeutic management, compared to the literature, pleads in favour of intentionally surgical attitude and gliotic tissue removal as often as reasonably possible.

[The Codman Medos programmable shunt valve. Evaluation of 53 implantations in 50 patients]. / La valve de dérivation Codman Medos programmable. Bilan de 53 implantations chez 50 patients.

Fifty three Codman Medos programmable valves were implanted in 50 patients (28 men and 22 women) aged 5 to 77 years, from April 1992 to February 1994. They presented with a hydrocephalus 43 times, an arachnoid cyst three times and a CSF leakage four times. The aims of this study were: a) to test the reliability of this equipment, in current use as well as its eventual advantages, inconveniences and restraints, b) to determine its best indications. Follow-up ranged from 12 to 36 months (average = 22 months). Five patients were lost for follow-up. One patient died from a post-operative sepsis. Four died from their disease. Infectious complications concerned 6 patients (11%). A radio-clinical overdrainage syndrome appeared in 9 patients with slit-ventricles, 5 of them associated with sub-dural collections. All these cases were treated with shunt reprogrammings. While 43 pressure settings over 53 were satisfactory in the immediate post-operative period, it appeared that only 38% of shunts had required one only setting when the study period was over. A certain number of pressure adjustments malfunctioned: 6 times, post-operative X-ray controls showed pressures significantly different from the figures which had been selected (difference from -30 mm H2O to + 70 mm H2O); pressure readjustments were effective and accurate for five of them, but one shunt had to be changed; 15 deprogrammings were detected during long term follow-up, 9 of them after MR1. Pressure programming was readjusted only on patients presenting with clinical signs, i-e mainly for those having a pressure difference > +/-30 mm H2O. As a conclusion, the authors consider that such a shunt can be very useful in some precise indications as: NPH, multioperated hydrocephalus, arachnoid and porencephalic cysts, some spontaneous or iatrogenic CSF leakages, temporary shunts necessitating a progressive withdrawal. Because of a) the cost of this equipment, b) the specific restraints (X-rays controls, programmer), and c) the specific incidents (spontaneous and/or post-MR1 deprogramming), it seems difficult to generalize indications to all cases of hydrocephalus. On the other hand, these shunts have the great advantage of simplifying the treatment of overdrainage by avoiding, in all cases, a reoperation for changing the shunt and/or removing a subdural hematoma. Consequent economies due to a significant diminution of the duration of hospitalisation, could justify an enlargement of indications.