[Very preterm birth less than 33 weeks' gestation: how setting-up of a perinatal network does influence the activity of the neonatal tertiary care unit? The experience of the Poitou-Charentes region, France]. / Grande prématurité: impact de la mise en place d'un réseau périnatal sur l'activité du centre de néonatalogie de niveau 3 de la région Poitou-Charentes.

OBJECTIVES: To evaluate the impact of regional perinatal network setting on very preterm neonates (gestational age<33 weeks) referral and activity of regional level 3 NCIU, and short-term outcome of infants cared for. POPULATION AND METHODS: Comparison of data from medical records of hospital days and hospital outcome of very preterm neonates born before and after the setting-up of regional perinatal network (2002-2005). RESULTS: The setting-up of the Poitou-Charentes perinatal network has led to a 45% rise in number of very preterm neonates admitted to the level 3 neonatal care (114 in 2002, 166 in 2005), number of hospitalisation days has also increased by 31% in neonatology unit (2181 days in 2002, 2864 days in 2005) but remained stable in intensive care unit. A transient rise in neonatal mortality was observed, although the incidence of severe ultrasonographic cerebral abnormalities and that of bronchopulmonary dysplasia were lowered. CONCLUSION: Setting-up of perinatal network in Poitou-Charentes (France) has led to improved access to level 3 neonatal care, with rise in very preterm neonates survival and low incidence of short-term sequelae.

[Cardiac hamartomas in Bourneville's tuberous sclerosis. Perinatal echographic diagnosis and spontaneous evolution]. / Hamartomes cardiaques de la sclérose tubéreuse de Bourneville: diagnostic échographique périnatal et évolution spontanée.

UNLABELLED: Cardiac hamartomas are frequently the earliest revelation of Bourneville's tuberous sclerosis. They sometimes allow to consider the diagnosis during the antenatal period, and to plan therefore genetic and CNS investigations. After the neonatal period, the evolution of hamartomas is frequently favourable, characterised with at least partial involution. OBSERVATION: Antenatal equivocal cardiac echographic images did not allow the diagnosis in a fetus whose father had Bourneville's disease. Other antenatal investigations (genetics, central nervous system MRI) were not helpful. The diagnosis was carried out at birth as cardiac ultrasound reliably showed two cardiac hamartomas. CONCLUSION: Even when suspected, the antenatal diagnosis of Bourneville's disease is difficult. The presence of cardiac hamartomas at birth is a reliable mean to make the diagnosis.

Fatal adenovirus type 7b infection in a child with Smith-Lemli-Opitz syndrome.

Adenovirus type 7 causes worldwide respiratory tract infections, mainly in children. Severe systemic infections can occur, especially in immunocompromised patients and in patients with underlying chronic diseases. This report describes the first case of a fatal disseminated adenovirus type 7 infection in a child with Smith-Lemli-Opitz syndrome, a rare autosomal recessive disorder due to a primary enzymatic defect in cholesterol metabolism. Nasopharyngeal secretions and autopsy specimens including liver, lung, pleural fluid, and rectum were collected for viral culture. Adenovirus serotype 7 strains were obtained from all anatomic sites, except the liver. All these clinical isolates were analyzed using restriction endonuclease digestion of the genome, identifying them as genome type 7b, a virulent type. In this case, the fatal evolution could have been accelerated by the presence of an immunodeficiency although immunodeficiency is not included in the definition of Smith-Lemli-Opitz syndrome. The frequent recurrent banal infections in Smith-Lemli-Opitz syndrome could be prevented by a cholesterol supplementation regimen. Finally, this report emphasizes the need for efficient therapy for disseminated adenovirus infections, especially for virulent genome types.

[Pneumopericardium with favorable outcome in a ventilated premature infant]. / Pneumopéricarde d'évolution favorable chez un prématuré ventilé.

UNLABELLED: Pneumopericardium is a rare and severe complication of artificial ventilation in neonates. CASE REPORT: A preterm neonate born after 29 weeks of gestation was placed under ventilatory support for bronchopulmonary dysplasia. At 63 days of life, just after a severe bronchospasm which required bag ventilation with high pressures, she collapsed and required immediate cardiopulmonary resuscitation with epinephrine infusion. The diagnosis of pneumopericardium was deduced from the chest X-ray obtained in emergency, on which there was also a right pneumothorax. Cardiac recovery with return of spontaneous circulation was only obtained after evacuation of the pneumopericardium with a 23-gauge needle via the sub-xiphoid route. The pneumothorax was drained and the long-term evolution was favorable. CONCLUSION: In the case of cardiopulmonary compromise, the early diagnosis of pneumopericardium should lead to the immediate evacuation of the pneumopericardium in order to improve the prognosis.

[A cause of acute encephalitis: primary infection due to Epstein-Barr virus]. / Une cause d'encéphalite aiguë: la primo-infection à virus d'Epstein-Barr.

UNLABELLED: The neurologic complications of Epstein-Barr virus (EBV) primary infection are frequently overlooked, yet EBV is responsible for some cases of acute encephalitis. We report on a case of an infant with acute brain-stem encephalitis during EBV primary infection. OBSERVATION: A 22-month-old boy was admitted to the hospital for acute neurological symptoms. Initially, he presented ataxia and dysphagia. During the first days, his neurological status worsened with loss of consciousness requiring ventilatory support. A progressive improvement appeared after a 1 week course. One month later, a moderate ataxia was still noted, but 3 months later, the clinical examination was normal. The neuroradiological explorations revealed damage of the white matter, with high T2-weighed signal on the first brain magnetic resonance imaging. One month later, the control magnetic resonance imaging was normal. The diagnosis of EBV encephalitis was based upon the exclusion of other possible causes and positive serology for EBV, consistent with acute primary infection. CONCLUSION: EBV infection should be looked for during the etiologic work-up of an acute encephalitis. The different cases reported in the literature show a favorable initial outcome, but neurological relapse and sequelae are possible. No specific treatment is effective.

[Bacteroides fragilis meningitis revealing a meningorectal fistula]. / Méningite à Bacteroides fragilis révélatrice d'une fistule rectoméningée.

UNLABELLED: Cases of meningitis due to Bacteroides fragilis are rare; we report a case revealing a meningorectal fistula. CASE REPORT: A 2-month-old infant developed a severe sepsis syndrome following a rectosigmoidoscopy for rectal bleeding. Lumbar puncture diagnosed bacterial meningitis. Cerebrospinal fluid (CSF) culture evidenced B fragilis with betalactamase. The initial antibiotherapy was changed for imipenem-metronidazole, which is at present the recommended antibiotherapy. Malformation including pre-spinal tumor and meningorectal fistula was evoked on magnetic resonance imaging (MRI) and confirmed by surgery. The outcome was favorable after surgery and antibiotherapy. CONCLUSION: B fragilis meningitis are usually associated with sepsis, whose origin is obvious. In our case, meningitis was isolated, revealing a meningorectal fistula.

[Intracranial hypertension in the infant: from its physiopathology to its therapeutic management]. / Hypertension intracrânienne de l'enfant: de la physiopathologie à la prise en charge thérapeutique.

The pathophysiology of elevated intracranial pressure (ICP) is assessed from a three cerebral compartment model and from brain compliance. The mechanisms leading to elevated ICP (expanding process, cerebral edema, brain swelling, hydrocephalus) and their consequences (brain herniation, ischemia-anoxia phenomenon, Cushing reaction and neurogenic pulmonary edema) are overviewed. The causes of elevated ICP in children are reported with emphasis on traumatology. Diagnostic procedures include clinical assessment, fundoscopy, cerebral computerized tomography scan and specific problems of cerebrospinal fluid investigation. Methods and results of intracranial pressure monitoring are reported. The treatment of elevated ICP is based upon clinical follow-up and monitoring of ICP. General therapeutic rules consist of adequate position, suppression of any neck, skull and abdominal compression, stimuli limitation and fluid restriction. Specific treatments include mechanical ventilation, sedation and analgesia, barbiturates, anticonvulsant drugs, mannitol, corticosteroids, hypothermia, enteral nutrition, and antibiotics.