A neonatal specialist with recurrent methicillin-resistant Staphylococcus aureus (MRSA) carriage implicated in the transmission of MRSA to newborns.

This study reports an investigation of outbreaks of methicillin-resistant Staphylococcus aureus (MRSA) infection and colonization involving 17 newborns in the neonatal unit of a teaching hospital. A neonatal specialist colonized with MRSA that eventually became mupirocin-resistant was implicated as a recurrent source of transmission of MRSA to newborns.

[End of life in neonatal medicine under the direction of French law]. / Fin de vie en médecine néonatale à la lumière de la loi.

Two recent laws have significantly reformed the French Public Health Code: the law of March 4th 2002, related to the patient's rights and the quality of the health care system and the law of April 22nd 2005, related to the patient's rights and the end of life. These changes have prompted health care professionals involved in perinatal and neonatal medicine to update their considerations on the ethical aspects of the end of life in neonatal medicine. Therefore, the authors examined the clauses of the law related to the patient's rights and to the end of life, confronting them with the distinctive features of neonatal medicine. In this paper, the medical practices, which are either prohibited or authorized in the course of end of life are considered: prohibition of euthanasia, authorization for alleviating pain at the risk of shortening life, authorization for restricting, withholding or withdrawing treatments. Next, the justifications provided by the legislation to authorize these practices are analysed: prohibition of unreasonable obstinacy and respect for individual wishes. Then, the conditions required by the law to determine and to implement these acts are discussed: consultation with the healthcare staff and justified advice from a consulting physician, consideration of parental opinion, registration of the decision and its justifications into the patient's medical file, protection of the dying patient's dignity and preservation of his life quality by providing palliative care. Lastly, we report the terms of the ethical dilemma which may occur in the area of neonatal medicine in spite of genuine and persevering efforts in order to conciliate legal requirement and ethical responsibility.

[Pseudo-"isolated" intestinal perforation in a very low birth weight infant: exceptional presentation of Hirschsprung's disease]. / Perforation intestinale du grand prématuré en apparence "isolée": mode de révélation exceptionnel de la maladie de Hirschsprung.

Intestinal perforations in preterm newborn are characterized with high morbidity and mortality rates. They often are associated with necrotizing enterocolitis and seldom correspond to idiopathic spontaneous intestinal perforation. Perforations upstream of an intestinal organic obstruction (atresia), or of a functionnal obstruction (meconium-ileus, Hirschsprung disease) have been considered to be rare in preterm newborns. We report a case of caecal perforation with a pneumoperitoneum which occurred at 5 days of life, in a 28-week gestational age infant, that was treated by cecostomy. There were no signs of necrotizing enterocolitis. At 43 days of life, the preoperative contrast enema study revealed a left colon transition zone, suggesting a Hirschsprung disease, which was confirmed by rectal biopsies. Transanal pull-through was performed. There were no postoperative complication. This case demonstrates that an isolated intestinal perforation in very preterm newborn can reveal a Hirschsprung disease, especially if it occurs in the caecum.

[Aneurysm of the vein of Galen and cardiac insufficiency. Current therapeutic approach]. / Anévrisme de l'ampoule de Galien et insuffisance cardiaque. Attitude thérapeutique actuelle.

Aneurysm of the ampulla of Galien is a rare but serious cause of cardiac failure in the neonate or child. The diagnosis is suspected on finding a continuous murmur on auscultation of the skull. It is an intracranial arterio-venous malformation which is sometimes responsible for a very important shunt between the arterial (carotid or vertebral) and venous systems. The vital and neurological prognosis of these children is classically very poor. The authors report the cases of the last three consecutive children aneurysms of the ampulla of Galien followed up at Grenoble Hospital in the last five years. The clinical presentations were very different, corresponding to the usual three forms described in the literature: a reputedly very severe form with cardiac failure at the 3rd day of life requiring multiple percutaneous embolisations; a neonatal form revealed by an isolated macrocranium; and a form diagnosed late (4.5 years) in the investigation of effort dyspnoea which regressed after two sessions of embolisation. All three children are alive and have normal psychomotor development for their age. A multidisciplinary approach involving neonatal physicians, paediatric cardiologists and neurologists, with complementary non-invasive investigations such as transfontanellar ultrasonography, electroencephalography, MRI and echocardiography are necessary to optimise the management and limit the neurological sequellae in children with this type of malformation. Cerebral arteriography and percutaneous embolisation under general anaesthesia by a trained team gave very good medium-term results in two of these cases, lightening the usual pessimism surrounding this condition.

[Left pulmonary artery sling associated with right pulmonary hypoplasia and ventricular septal defect]. / Artère pulmonaire gauche aberrante associée à une hypoplasie pulmonaire droite et á une communication interventriculaire.

The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.

[Fetomaternal hemorrhage: a series of 9 cases]. / Hémorragie foetomaternelle: une série de neuf observations.

AIM: This study was designed to stress the importance of early diagnosis of fetomaternal hemorrhage (FMH) in attempt to prevent the subsequent adverse outcome on the fetus and the newborn. PATIENTS AND METHODS: Nine newborns were admitted because of neonatal anemia to our neonatal unit from October 1989 through September 1995. The diagnosis of FMH was made by the sigma diagnostic fetal hemoglobin that is the Kleihauer test in our hematologic laboratory. Other causes of neonatal anemia have been ruled out. RESULTS: Seven out of the nine cases have expressed early signs of fetal distress in term of abnormal fetal monitoring and/or thick meconium associated with decreased fetal movements. At birth, a wide clinical spectrum depending on the amount of the hemorrhage was seen, ranging from mild anemia with no symptoms (four cases), hypovolemic shock (one case), respiratory distress syndrome (two cases) and maladjustment to extra-uterine life (one case). There was one death at 48 hours after birth; one infant survived with severe encephalopathy. CONCLUSION: These results indicate that it is mandatory to carry out a Kleihauer test whenever a high suspicious index of FMH is faced or an unexplained neonatal anemia is found.

[Congenital long QT syndrome in newborns]. / Syndrome du QT long congénital de révélation néonatale.

UNLABELLED: The perinatal manifestations of the long QT syndrome are rare, but early diagnosis and therapy are necessary to prevent sudden death. CASE REPORTS: A long QT syndrome was diagnosed in two neonates who presented with foetal bradycardia. In one case, a mutation in the gene KCNQ1 was identified, and a long QT syndrome was diagnosed in the mother and two brothers of the neonate. On beta-blocker therapy, one infant became free of long QT syndrome related symptoms, but a sudden death of the second infant occurred. CONCLUSION: The long QT syndrome should be considered in the differential diagnosis of foetal bradycardia. Early treatment of the neonate and his family may prevent ventricular arrhythmias and sudden death.

[Cardiorespiratory arrest in full term newborn infants: six case reports]. / Malaises graves et morts subites après une naissance normale à terme: à propos de six cas.

Cardiorespiratory arrest occurring within the first two hours of life of a perfectly normal newborn is a very seldom event hitherto unreported. Six infants born after an uneventful pregnancy by normal vaginal delivery, with a normal Apgar score and physical examination, were found with unexpected cardiorespiratory arrest requiring cardiac and respiratory resuscitation early after birth. All were lying in the prone position, their face covered up while facing mother's abdomen, breast or neck. All mothers were primipara. All newborns but one died. Biological and bacteriological samples were normal and early onset neonatal sepsis was ruled out. Autopsy, performed in five infants, was not contributive. We hypothesize that the sudden and unexpected cardiorespiratory arrest occurring in these normal newborns was secondary to acute upper airway obstruction. To prevent this life threatening post-natal asphyxic episode, it is essential to ensure that the face of a newborn lying down upon mother's breast and abdomen is properly and continuously cleared.

[Roles of iron overload and chelating treatment in Yersinia infectious complicated by major thalassemia]. / Rôles de la surcharge martiale et du traitement chélateur dans les infections à Yersinia survenant sur une thalassémie majeure.

A study of 3 personal and 32 published cases showed that Yersinia septicaemia in subjects under 21 presents as digestive disorders with fever and occurs in one half of the cases in thalassaemia patients. Among the factors which may be blamed for this predisposition the most important are excessive tissue iron levels and treatment with chelating agents. Iron overload is present in 70% of patients with Yersinia septicaemia. The risk of infection in such subjects requires appropriate therapeutic measures.

[An unrecognized neonatal emergency: extensive hematoma of the scalp. 5 cases]. / Une urgence néonatale méconnue: l'hématome extensif du cuir chevelu. 5 observations.

BACKGROUND: Subgaleal hemorrhage results from a pericranial effusion of blood subsequent to neonatal trauma. This exceptional situation compared with other pericranial effusion conditions in the neonate may be life-threatening. CASE REPORTS: We report the obstetrical and neonatal data in 5 cases of subgaleal hemorrhage observed in our unit over an 8-year 8-month period. We detail one particularly demonstrative case which illustrates the potentially serious course of certain clinical presentations. DISCUSSION: Subgaleal hemorrhage is a clinical diagnosis. Signs of hemorrhagic shock are associated with hemostasis disorders in the more severe forms of the condition. The main risk factor is instrumental delivery with suction. Careful monitoring is required.

[Are mydriatic eyedrops dangerous for pre-term infants?]. / Les collyres mydriatiques sont-ils dangereux chez le nouveau-né prématuré ?

The funduscopic examination is essential in neonatology to screen for retinopathy in the pre-term infant. Mydriatic eyedrops, which are used for this examination, are known to induce digestive side effects. We present a case of necrotizing enterocolitis developing in a pre-term infant as a complication of mydriatics. This infant was a girl born at 28 weeks gestation and 5 days, with Down's syndrome, who died on the 44th day of life, due to necrotizing enterocolitis, after instillation of 1 drop of atropine 0.3% in each eye. The chronology of events, the application method, and the clinical symptoms of atropine impregnation argue in favor of a causal relationship between atropine and necrotizing enterocolitis. The review of the literature made on the basis of this observation shows that side effects of mydriatic eyedrops are frequent in pre-term infants and raise the question of atropine hypersensitivity in pre-term infants with Down's syndrome.

[Assessment of a hypothermia protocol implementation for hypoxic-ischemic encephalopathy in term newborns]. / Évaluation d'un protocole de prise en charge de l'encéphalopathie anoxo-ischémique du nouveau-né par hypothermie.

BACKGROUND: A whole-body hypothermia protocol for term infants with hypoxic-ischemic encephalopathy (HIE) was implemented in our network in May 2004. The main objective of this study was to assess the feasibility of the protocol. The impact on the outcome was also assessed. METHODS: Monocentric retrospective study of all term infants admitted for HIE after the implementation of the protocol. The feasibility of the protocol was evaluated by its observance during the first 72 h. The neurodevelopmental outcome at 12 months of age of this population was compared with a historical control group. RESULTS: Twenty-five children were included. The protocol was not correctly applied for 8 children. The target temperature (33-34°C) was not reached for 3 infants. Four infants were admitted after 6h of age. In 1 infant, HIE was not diagnosed at admission. In the 17 patients with a good protocol observance, rectal temperature fell to the target temperature on average at 6.4h of age. The long-term follow-up rate was improved after the implementation of the protocol (100 % versus 92 % before protocol implementation). Death or neurodevelopmental disability occurred in 40 % during the protocol period versus 87 % before protocol implementation (p<0.01). CONCLUSIONS: The main limiting factor for implementation of whole-body hypothermia in infants with HIE is admission delay. The follow-up and the rate of death or disability in infants with HIE improved after implementing the protocol.

[Very premature births: Dilemmas and management. Part 1. Outcome of infants born before 28 weeks of postmenstrual age, and definition of a gray zone]. / Naissances très prématurées : dilemmes et propositions de prise en charge. Première partie : pronostic des naissances avant 28 semaines, identification d'une zone grise.

With very preterm deliveries, the decision to institute intensive care, or, alternatively, to start palliative care and let the baby die, is extremely difficult, and involves complex ethical issues. The introduction of intensive care may result in long-term survival of many infants without severe disabilities, but it may also result in the survival of severely disabled infants. Conversely, the decision to withhold resuscitation and/or intensive care at birth, which is an option at the margin of viability, implies allowing babies to die, although some of them would have developed normally if they had received resuscitation and/or intensive care. Withholding intensive care at birth does not mean withholding care but rather providing palliative care to prevent pain and suffering during the time period preceding death. The likelihood of survival without significant disabilities decreases as gestational age at birth decreases. In addition to gestational age, other factors greatly influence the prognosis. Indeed, for a given gestational age, higher birth weight, singleton birth, female sex, exposure to prenatal corticosteroids, and birth in a tertiary center are favorable factors. Considering gestational age, there is a gray zone that corresponds to major prognostic uncertainty and therefore to a major problem in making a "good" decision. In France today, the gray zone corresponds to deliveries at 24 and 25 weeks of postmenstrual age. In general, babies born above the gray zone (26 weeks of postmenstrual age and later) should receive resuscitation and/or full intensive care. Below 24 weeks, palliative care is the only option offered in France at the present time. Decisions within the gray zone will be addressed in the 2nd part of this work.

[Very premature births: Dilemmas and management. Second part: Ethical aspects and recommendations]. / Naissances très prématurées : dilemmes et propositions de prise en charge. Seconde partie : enjeux éthiques, principes de prise en charge et recommandations.

In the first part of this work, the outcome following very premature birth was assessed. This enabled a gray zone to be defined, with inherent major prognostic uncertainty. In France today, the gray zone corresponds to deliveries occurring at 24 and 25 weeks of postmenstrual age. The management of births occurring below and above the gray zone was described. Withholding intensive care at birth for babies born below or within the gray zone does not mean withholding care but rather providing palliative care to prevent pain and suffering during the time period preceding death. Given the high level of uncertainty, making good decisions within the gray zone is problematic. Decisions should be based on the infant's best interests. Decisions should be reached with the parents, who are entitled to receive clear and comprehensive information. Possible decisions to withhold intensive care should be made following the procedures described in the French law of April 2005. Guidelines, based on gestational age and the other prognostic elements, are proposed to the parents before birth. They are applied in an individualized fashion, in order to take into account the individual features of each case. At 25 weeks, resuscitation and/or full intensive care are usually proposed, unless unfavorable factors, such as severe growth restriction, are associated. A senior neonatologist will attend the delivery and will make decisions based on both the baby's condition at birth and the parents' wishes. At 24 weeks, in the absence of unfavorable associated factors, the parents' wishes should be followed in deciding between initiating full intensive care or palliative care. Below 24 weeks, palliative care is the only option to be offered in France at the present time.

[Pseudo precocious puberty in the McCune-Albright syndrome]. / Pseudo-puberté précoce dans le syndrome de McCune-Albright.

McCune Albright Syndrome is characterized by the triad of polyostostic fibrous dysplasia of bones, skin pigmentation (café au lait-spots) and sexual precocity. The authors report a case of a 7 years girl with this syndrome in whom the sexual precocity is due to an autonomous hyperfunctioning of luteinized follicular cysts of ovary. The pathogenic mechanisms of this autonomy are debate.

[Skin aplasia of the trunk in the newborn. Apropos of 2 cases]. / Aplasies cutanées du tronc chez le nouveau-né. A propos de deux cas.

Report of two cases of truncular dermoepidermal skin defect in newborn. The defect concerned the lateral thoracoabdominal area, on both sides of the umbilicus, jointed with a fine linear communication, and have the classical butterfly wind-like shape. Recovery was achieved by centripetal spontaneous cicatrization, only managed with protective vitamin dressing, in about 45 days. There was no clinical evidence of other anomaly. In this occasion, an exhaustive review of congenital skin defects is done. This disease is not frequent, 500 cases are published, principally in the scalp area (vertex). Thoracoabdominal localisation (10%) are symmetrical, spontaneously healing without surgery, in spite of a large defect. Skin defect of extremities (25% of cases) have also a good issue, except in case of associated congenital diseases; on the other hand scalp defects are sometimes complicated with sepsis or bleeding. Discussion emphasize the characteristics of skin defects, the possible pathological association (rare in case of thoracoabdominal defect) and pathogenic hypothesis, not yet definite at all.