[Vulvar mass in neonatal period : spot the difference]. / Diagnostic différentiel d'une masse vulvaire en période néonatale.

We report the cases of two female newborns who present, at the first day, a interlabial mass. In both cases, the antenatal scans were normal, and the clinical examination in the delivery room didn't reveal any other abnormality. Similar features may suggest the same diagnosis. However, in the first case, it is a paraurethral cyst which causes the bulging of vaginal introitus whereas, in the second infant, it is an imperforated hymen. Paraurethral cysts are uncommon and even more so among infants. The diagnosis is primarily clinical, and ultrasound confirms the diagnosis and verifies the absence of other associated anomalies. These cysts usually resolve spontaneously. Rarely, they can lead to urinary retention. The second diagnosis, imperforate hymen, is the most common congenital malformation of the female genital tract. It can lead to hydrocolpos with the upstream accumulation of vaginal secretion and sometimes induce a hydrometrocolpos, with vaginal and uterine dilatation. The resulting mass can cause obstruction of surrounding urinary or digestive structures. Surgical management is generally necessary.

Nous rapportons le cas de deux nouveau-nés de sexe féminin qui présentaient une masse au niveau vulvaire, dans les premiers jours de vie. Dans les deux cas, le reste de l'examen clinique était sans particularité et aucune anomalie n'avait été décelée en anténatal. Les présentations cliniques semblables peuvent faire évoquer le même diagnostic. Pourtant, dans le premier cas, c'est un kyste para-urétral qui cause la masse alors que, dans le second, c'est une imperforation de l'hymen. Ces deux pathologies sont peu fréquentes chez les nourrissons. Leur diagnostic est avant tout clinique et l'échographie permet à la fois de confirmer celui-ci et de vérifier l'absence d'autres anomalies. La plupart du temps, les kystes para-urétraux régressent spontanément. Cependant, ils peuvent parfois être la cause d'une rétention urinaire. En cas d'imperforation de l'hymen, la réalisation d'un acte chirurgical est, par contre, nécessaire afin d'éviter des complications plus tardives. Un hydrocolpos peut y être associé, il est dû à la rétention de sécrétions vaginales en amont. La masse engendrée peut provoquer une obstruction des structures avoisinantes.

Surfactant use in late preterm infants: a survey among Belgian neonatologists.

Specific recommendations on surfactant administration in late preterm (LPT) infants with pulmonary disease are lacking. We performed an online-based, nationwide survey amongst all (n = 102) Belgian neonatologists to identify the use of surfactant in LPT infants suffering from several respiratory pathologies. The survey used clearly defined clinical cases and resulted in a 86% response rate. Neonatologists adhere to the 200 mg/kg initial surfactant dosing scheme. Surfactant is widely used in respiratory distress syndrome (70.1%), but there is less unanimity on its use in meconium aspiration syndrome (58.0%), transient tachypnoea of the newborn (30.6%), congenital pneumonia (27.2%) and congenital diaphragmatic hernia (8.6%). Respondents adhere to the European guideline of a timely referral to a newborn intensive care unit (non-invasive ventilation and FiO2 > 0.30 at 12 h of age), in order to minimise the risk of deterioration.Conclusion: We demonstrate a wide variety in the use of surfactant within LPT infants. The majority of Belgian neonatologists therefore urge for an investment in multi-centre trials on surfactant administration in LPT infants, in order to create an evidence-based practice as well as to reduce the strain on health care budgets.Trial registration: https://clinicaltrials.gov What is Known: • Any late preterm (LPT) infant with respiratory distress needs a timely referral to a neonatal intensive care unit in case of non-invasive ventilation and FiO2 > 0.30 at 12 h of life, in order to minimise the risk of acute deterioration as well as chronic lung disease. • Any modest increase in morbidity in the sizeable group of LPT infants exerts a significant strain on health care budgets. What is New: • We report the attitudes and opinions of Belgian neonatologists about the use of surfactant in LPT infants suffering from several respiratory diseases. • Our survey demonstrates a significant variability in practice between neonatologists during treatment of respiratory pathologies in LPT infants. This highlights an urgent need for univocal therapeutic lines.

[A case of severe congenital nephrotic syndrome secondary to NPHS1 mutation]. / Syndrome néphrotique congénital sévère avec mutation NPH S1.

The congenital nephrotic syndrome is a rare and severe pathology, and its management represents a real challenge for pediatric nephrologists. We report the case of a congenital nephrotic syndrome secondary to a homozygous mutation of the NPHS1. The young patient has a severe clinical course, and benefits of a management by anti-proteinuric treatment and a unilateral nephrectomy. This clinical case illustrates the difficulties of the management of a severe congenital nephrotic syndrome. To date, it is difficult to identify these patients beforehand because there is a poor correlation between the genotype and the phenotype of the NPHS1 mutation. There are two managements described in the literature: an early bilateral nephrectomy at 7 kg of weight with a renal transplant around 10 kg, versus a conservative management via an anti-proteinuric treatment and/or an unilateral nephrectomy. Current evidence is based on retrospective studies and the choice of a conservative approach versus early bilateral nephrectomy should take into account the severity of protein loss and its complications.

Le syndrome néphrotique congénital est une pathologie rare et sévère, dont la prise en charge représente un défi pour les néphrologues pédiatriques. Nous rapportons le cas d'un jeune patient présentant cette pathologie secondaire à une mutation homozygote du gène NPHS1. Il présente un tableau clinique sévère et bénéficie d'un traitement anti-protéinurique et d'une néphrectomie unilatérale. Ce cas clinique illustre les difficultés de la prise en charge des cas sévères, dont l'identification préalable est difficile à ce jour car la corrélation entre le génotype et le phénotype de la mutation NPHS1 est pauvre. Il existe deux prises en charges décrites dans la littérature : une néphrectomie bilatérale précoce vers 7 kg de poids et une greffe rénale vers 10 kg, ou bien une prise en charge conservative via un traitement anti-protéinurique et/ou une néphrectomie unilatérale permettant de postposer la greffe. Les données actuelles n'étant basées que sur des études rétrospectives, le choix entre une approche conservative et une néphrectomie bilatérale précoce doit prendre en compte la sévérité de la déperdition protéique et ses complications.

[Therapeutic advances in neonatology]. / Actualités thérapeutiques en néonatologie.

In this article, we will review major therapeutic advances in neonatology over the past ten years. We will discuss the antenatal administration of magnesium sulfate, the interest of hypothermia in the context of hypoxic ischaemic encephalopathy, the benefits and modalities of placental transfusion, less invasive techniques for ventilation and administration of the surfactant, possibilities to fortify breast milk and the concept of developmental care. These therapeutic advances are sometimes based on new therapeutics, sometimes on new concepts and, sometimes, on new less invasive techniques. They have made it possible to optimize the care of premature babies but also of term newborns.

Dans cet article, nous allons passer en revue les grandes avancées thérapeutiques dans le domaine de la néonatologie au cours des dix dernières années. Nous traiterons de l'administration anténatale du sulfate de magnésium, de l'intérêt de l'hypothermie contrôlée dans le cadre de l'encéphalopathie d'origine hypoxo-ischémique, des bénéfices et modalités de la transfusion placentaire, des techniques moins invasives pour la ventilation et pour l'administration du surfactant, de la fortification du lait maternel et du concept de soins de développement. Ces avancées thérapeutiques reposent tantôt sur des nouveautés thérapeutiques, tantôt sur de nouveaux concepts et, parfois, sur de nouvelles techniques moins invasives. Elles ont permis d'optimiser la prise en charge des prématurés, mais aussi des nouveau-nés à terme.

[Late preterm : high risk newborns despite appearances]. / La prématurité tardive : des nourrissons fragiles malgré les apparences.

Late preterm infants are born between 34 weeks of amenorrhea and 36 weeks 6 days. Late preterms represent the largest proportion of premature infants (about 75 %). Late prematurity is increasing in recent decades. While studies initially focused on mortality and morbidity related to very preterm birth, the late preterms have been the subject of increased attention over the past 15 years. Late preterm infants have an increased risk of respiratory complications, infections, feeding problems, hypothermia and hypoglycemia. Neonatal, infant and during adulthood mortalities are significantly higher in late preterm than in term infants. In addition, late preterm infants carry an increased risk of long-term morbidities, such as neurodevelopmental delay, cerebral palsy, chronic respiratory or metabolic diseases. This review highlights the evidence that late preterm infants are high risk newborns and require adapted follow-up.

Les enfants nés entre 34 semaines d'aménorrhée et 36 semaines 6 jours sont dans la période de prématurité tardive. Ils sont également appelés «late-preterm¼. Ces enfants représentent près de 75 % des naissances prématurées dans les pays industrialisés. Cette prématurité tardive est en augmentation croissante sur les dernières décennies. Alors qu'initialement les études se concentraient essentiellement sur la mortalité et les morbidités liées à la grande prématurité, la population des prématurés tardifs a fait l'objet d'une attention accrue ces 15 dernières années. Il est ainsi démontré que ces enfants présentent un risque accru de complications respiratoires, d'infections, de problèmes d'alimentation, d'hypothermie et d'hypoglycémie. La mortalité néonatale, infantile et jusqu'à l'âge adulte des late-preterm est significativement plus élevée que chez les nouveau-nés à terme. De plus, les morbidités à long terme, tels que le retard neurodéveloppemental, l'infirmité motrice cérébrale, les pathologies respiratoires chroniques ou métaboliques, sont significativement plus élevées. A travers cette revue de la littérature, nous revoyons ces risques, qui contribuent à faire des «late preterm¼ une population fragile et nécessitant un suivi adapté.

A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon.

A vascular mass localized in the face and the neck was displayed by ultrasonography in a 38-week-old male fetus. At birth, the mass was bulky and purplish. The newborn breathed spontaneously but with severe desaturation. During laryngoscopy, we observed an obstruction of the larynx with a left-shift caused by the hemorrhagic mass. Blood analysis revealed anemia, severe thrombocytopenia, and coagulation disorders. The diagnosis of kaposiform hemangioendothelioma (KHE) complicated by a Kasabach-Merritt phenomenon (KMP) was put forward and treatment with propranolol, corticoids, and vincristine was initiated. Platelets were transfused daily for 8 days but did not resolve the thrombocytopenia. At day 8, we added sirolimus to the treatment and noted a rapid response with the normalization of the platelet count within 1 week and a significant regression of the mass. In this paper, we review the clinical and biological features of hemangioendothelioma associated with KMP and discuss its current and future treatment. Sirolimus seems to be very promising.

[Alcohol, pregnancy and breast-feeding]. / Alcool, grossesse et allaitement.

Perinatal studies over the last ten years report that 3 % of pregnant women have alcohol consumption. The malformative and neurological consequences of prenatal exposure to alcohol have been known for more than 50 years. Research has shown that the effects of alcohol during pregnancy and breast-feeding go far beyond visible abnormalities at birth. Neurological consequences, from minor psychomotor retardation to severe behavioural disorders, make all the severity of this intoxication and justify strict instructions to refrain from taking alcohol during pregnancy and breast-feeding.

Les enquêtes périnatales réalisées au cours des dix dernières années rapportent que 3 % des femmes enceintes consomment de l'alcool. Les conséquences malformatives et neurologiques de l'exposition prénatale à l'alcool sont pourtant connues depuis plus de 50 ans et l'avancée des recherches a montré que les effets de l'alcool durant la grossesse et l'allaitement vont bien au-delà d'anomalies visibles à la naissance. Les conséquences neurologiques, du retard mineur aux troubles comportementaux sévères, illustrent toute la gravité de cette intoxication. Ils justifient une consigne stricte d'abstention de consommation d'alcool durant la grossesse et l'allaitement.

[Newborn infants' first outpatient visit]. / Première consultation ambulatoire du nouveau-né.

The focus on outpatient follow-up of newborn infants increases as the duration of hospital stay after birth decreases. The first outpatient visit addresses the adequacy of the home transition. Appropriate feedings are checked. Sudden infant death syndrome prevention and security advices are reminded. Realisation of both neonatal dried blood screen and hearing test is confirmed, as well as planning of specific follow-up appointments. The physical exam will focus on red flags for diseases or malformations with a delayed presentation.

Le suivi ambulatoire du nouveau-né devient important en raison de la réduction progressive des séjours en maternité. Lors de cette première consultation, il convient de s'assurer du bon déroulement du retour à domicile. L'alimentation est vérifiée. Les conseils de sécurité et ceux de prévention de la mort subite du nourrisson sont rappelés. La réalisation des dépistages sanguin et auditif est contrôlée, de même que la programmation des suivis spécifiques potentiellement nécessaires. L'examen clinique recherchera les signes d'alerte des pathologies d'apparition secondaire, comme l'ictère, ou les malformations congénitales à expression tardive.

Rigid catheters reduced duration of less invasive surfactant therapy procedures in manikins.

AIM: Different catheters can be used for less invasive surfactant therapy (LIST): feeding tubes inserted with or without Magill forceps, different angiocatheters and centre specific devices, such as umbilical catheters affixed to a stylet. This study compared the effectiveness of LIST devices and endotracheal tubes (ETT). METHODS: Video recordings of 20 neonatologists simulating different LIST techniques on two manikin heads were analysed. Procedural effectiveness was evaluated by the duration of procedures and failure rates. Ease of use was scored. RESULTS: The median procedure time for the Neonatal Intubation Trainer was significantly longer with feeding tubes without Magill forceps. For the more difficult ALS Baby Trainer, successful procedures lasted a median of 24 (17-32) seconds with ETT, 24 (15-36) seconds with stylet-guided catheters and 34 (27-46) seconds and 37 (29-42) seconds with 13-cm and 30-cm angiocatheters, respectively. Both methods using feeding tubes were statistically slower than ETT intubation, lasting 32 (25-44) seconds and 39 (27-95) seconds with or without Magill forceps. Failure rates (7-20%) were no different between the LIST methods. Techniques using feeding tubes were rated as more difficult. CONCLUSION: Only rigid or stylet-guided catheters required tracheal catheterisation times similar to those of endotracheal intubation and neonatologists found them easier.

[EARLY ENTEROVIRUS NEONATAL INFECTION: WHEN SHOULD WE THINK ABOUT IT?]. / INFECTION NÉONATALE PRÉCOCE À ENTÉROVIRUS : QUAND FAUT-IL Y PENSER? Cas cliniques et revue de la literature.

Enterovirus (EV) may cause a broad spectrum of clinical syndromes and even cause a sepsis-like picture. Although they are responsible for high morbidity and mortality rates, viral testing does not appear in the algorithms for the evaluation of neonatal infections. During the month of June 2013, we identified 3 cases of EV meningitis in our unit of neonatology. All three infants had fever during the first week of life and their clinical examination revealed an irritability. The EV infection was detected by Real-Time Polymerase Chain Reaction (RT-PCR) EV on the cerebrospinal fluid (CSF). Two of the patients also had a positive RT-PCR EV in the blood. The 3 newborns were discharged from the hospital after a few days with no adverse outcome. Our clinical observations and the literature review suggest that EV infections in neonates ought to be identified as soon as possible by an early RT-PCR EV on the blood, and on the CSF if a lumbar puncture is indicated. This could help reduce the administration of antibiotics and the length of hospital stay.

[Gonococcal ophthalmia neonatorum: Clinical illustration, prevention, and future prospects]. / Conjonctivite néonatale à Neisseria Gonorrhoeae : illustration clinique, prophylaxie et perspectives d'avenir.

A 6-day-old infant presented with a bilateral suppurative ocular discharge with a conjunctival erythema. Polymerase chain reaction was performed on the pus and showed the presence of Neisseria gonorrhoeae DNA. Therapy with intravenous cefotaxime was initiated and completed with local application of tobramycin. This infection was associated with a small unilateral corneal lesion, with rapid resolution. This case provides the opportunity to focus on newborn suppurative conjunctivitis and its treatment. The different prophylaxes available (silver nitrate, povidone-iodine, local antibiotics, etc.) and their respective advantages and disadvantages are reviewed. There is no clear consensus on the most effective solution. Additionally, universal prophylaxis is challenged in several countries, where it is no longer recommended.

[How I explore and treat a neonatal renal vein thrombosis: a case report]. / Comment j'explore et traite une thrombose veineuse renale néonatale: àpropos d'un cas.

Neonatal renal vein thrombosis is a rare condition. The present case is rather unfrequent and particularly educative since it shows the complete diagnostic triad including hematuria, flank mass and thrombocytopenia. The diagnosis relies on the demonstration, by Doppler ultrasound, of an obstructed renal venous bed. The investigation is completed by a platelet count and the determination of the prothrombin time, of the activated partial thromboplastin time as well as of the concentration of fibrinogen. The screening also includes the search for a possible etiology, such as a deficiency in coagulation proteins, the presence of antiphospholipid antibodies or of a genetic mutation of one of the coagulation factors. Since there exist no evidence based guidelines for the management of the disease, we will discuss the diagnosis and treatment in relation with the published literature.

[A rare case of placental chorioangioma associated with neonatal disseminated hemangiomatosis]. / Association rare d'un choriangiome et d'une hémangiomatose néonatale diffuse.

Placental chorioangioma is a benign vascular tumor. Lesions larger than 4 cm may cause fetal and maternal complications. Its association with disseminated neonatal hemangiomatosis is rarely described. We report a case of a large chorioangioma associated with an hydrops foetalis and disseminated neonatal hemangiomatosis. The relationship between placental chorioangioma and hemangioma is briefly discussed.

[Infantile pyknocytosis: a rare form of neonatal hemolytic anemia. 5 case-studies]. / La pyknocytose infantile : une anémie néonatale mal connue à propos de 5cas.

UNLABELLED: Infantile pyknocytosis (IP) is a rare hematological entity of newborns. It is a form of hemolytic anemia with unusual red cell morphology: the red blood cells are distorted, irregular, and small with many projections. Spontaneous resolution usually occurs by 4-6months of age. OBSERVATION: We describe the clinical features and biological parameters of 5 cases of IP. The first symptoms were always early jaundice, which required phototherapy. Anemia was severe in all babies and red blood cell transfusion was needed. CONCLUSION: IP is a rare cause of neonatal anemia whose diagnosis is based on a careful peripheral blood smear examination. In our study, anemia was severe and required red blood cell transfusion. Ethnic specificity and familial occurrence are reported in our experience.

[Update in the management of persistent pulmonary hypertension of the newborn]. / Actualités thérapeutiques dans l'hypertension artérielle pulmonaire persistante du nouveau-né.

Persistent pulmonary hypertension of the newborn is a severe disease leading to persistent and refractory hypoxemia with bad outcomes. The introduction of inhaled nitric oxide therapy significantly improved short and long term prognosis of those infants. More recently, sildenafil also appeared promising, but regimen and indications still need to be delineated.

[Hypotension in the very preterm infant]. / Hypotension du grand prématuré.

One out of four very preterm infants will present with circulatory maladaptation during the first two days of life, with an increased risk of early complications and long term sequelae. Appreciation of those transitional difficulties cannot be limited to blood pressure. Assesment of blood pressure itself must be done in relation with gestational age and birth weight adapted norms. The effects of therapies for low systemic blood flow on blood pressure, organs and cerebral circulations are better understood, but none of them has assessed for mortality or neurodevelopmental outcomes.

Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias.

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-adactylia or hemifacial microsomia syndromes. We report a young girl with bony syngnathia associated with multiple defects (severe microcephaly, coloboma, vertebral segmentation defects), growth and mental delay. This patient is very similar to the patient described by Dobrow in 1983 and confirms the existence of this extremely rare disorder.

[The role of continent perineal colostomy in surgical emergencies of the distal bowel]. / Il ruolo della colostomia perineale continente nelle emergenze chirurgiche del grosso intestino.

Diverting colostomy is commonly required in surgical emergencies of the distal colon, especially if the patient is in poor condition. This paper shows that the colostomy could be unnecessary in most cases. In the last 10 years, 6 patients (4 with high-risk or postoperative complicated Hirschsprung disease, 1 with intestinal neuronal dysplasia and 1 with iatrogenic rectal stenosis obstruction) have been submitted to primary continent perineal colostomy-modified Duhamel's procedure. The modified technique consists of the exteriorization of the normal colon or ileum by a retrorectal and trans-anal way, while the excision of redundant tissue and rectal spur section are postponed at least for 10 days. In personal experience this peculiar surgical approach allows to avoid not only the complications due to the fashioning of a contraindicated primary anastomosis, but even those due to stomy performance and its closure, promoting good anatomo-functional results in all patients. The advantages of modified Duhamel's operation compared to other procedures (Swenson-Pellerin or Soave pull-through) depend on the rapidity of execution and on the quality of short and long term outcome.

Day surgery: the value of monitored anesthesia care and intraoperative monitoring.

Recently an increasing percentage of patients is receiving care and surgical procedures in Day Surgery (DS), undergoing local or loco-regional anesthesia techniques, to which many patients appear to be rather reluctant and show fear and anxiety. The anesthesiologist can resolve this problem administering i.v. hypnotic and analgesic drugs and adjusting their level to patient's needs and type of surgery. They increase the patient's compliance to DS care and contribute to its diffusion. Nowadays, commonly used drugs permit quick changes of anesthesia depth with a fast and safe recovery but these require an appropriate monitoring in order to prevent and rapidly detect the onset of complications. In this study we analyze the peculiarity of Monitored Anesthesia Care and the appropriate intraoperative monitoring especially regarding the use of pulse oximetry.