How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review.

INTRODUCTION: Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. METHODS: All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. RESULTS: Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1-2) resections-anastomoses (RA) and 6 (1-10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15-25 cm). Median time to full oral feed was 46 days (14-626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2-8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. CONCLUSION: Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.

[Oxidative stress after preterm birth: origins, biomarkers, and possible therapeutic approaches]. / Stress oxydant chez l'enfant prématuré : causes, biomarqueurs et possibilités thérapeutiques.

The survival of preterm babies has increased over the last few decades. However, disorders associated with preterm birth, known as oxygen radical diseases of neonatology, such as retinopathy, bronchopulmonary dysplasia, periventricular leukomalacia, and necrotizing enterocolitis are severe complications related to oxidative stress, which can be defined by an imbalance between oxidative reactive species production and antioxidant defenses. Oxidative stress causes lipid, protein, and DNA damage. Preterm infants have decreased antioxidant defenses in response to oxidative challenges, because the physiologic increase of antioxidant capacity occurs at the end of gestation in preparation for the transition to extrauterine life. Therefore, preterm infants are more sensitive to neonatal oxidative stress, notably when supplemental oxygen is being delivered. Furthermore, despite recent advances in the management of neonatal respiratory distress syndrome, controversies persist concerning the oxygenation saturation targets that should be used in caring for preterm babies. Identification of adequate biomarkers of oxidative stress in preterm infants such as 8-iso-prostaglandin F2α, and adduction of malondialdehyde to hemoglobin is important to promote specific therapeutic approaches. At present, no therapeutic strategy has been validated as prevention or treatment against oxidative stress. Breastfeeding should be considered as the main measure to improve the antioxidant status of preterm infants. In the last few years, melatonin has emerged as a protective molecule against oxidative stress, with antioxidant and free-radical scavenger roles, in experimental and preliminary human studies, giving hope that it can be used in preterm infants in the near future.

[Risk factors for developing pneumothorax in full-term neonates with respiratory distress]. / Détresse respiratoire du nouveau-né à terme : quels facteurs de risque de développer un pneumothorax ?

OBJECTIVES: To describe respiratory distress (RD) in full-term neonates hospitalized in the NICU and to determine risk factors in this population for pneumothorax. STUDY DESIGN: Retrospective inclusion for 4 years of full-term neonates hospitalized for RD before the 2nd day of life. Neonates were separated into Group I (RD with no pneumothorax) and Group II (RD with pneumothorax). Data collected from maternal and newborn medical records were obstetrical, perinatal, and postnatal. P<0.05 was set as the significance level. RESULTS: Ninety-six neonates were included. In this population, 64 (66.7%) were male, 45 (46.9%) were born by cesarean section, and 30 (31.3%) by elective cesarean section. Neonatal outcome was 4.6 days of hospital stay, 47.4% odds of mechanical ventilation, and 17.7% of persistent pulmonary hypertension of the neonate (PPHN). A central catheter was needed in 25% of the patients and amine treatment in 3.1%. The number of neonates born by cesarean section was lower as term increased. Those born by cesarean section were more likely to develop PPHN (26.7 vs 9.8%; P=0.03), and those born without labor were more likely to require oxygen (83.3 vs 63.6%; P=0.05). When comparing Group I and Group II (32 neonates), absence of labor (RR 1.5) and birth outside of a level III maternity unit (RR 1.6) were risk factors for pneumothorax. These results were confirmed in multivariate analysis. In Group II, birth before 39 weeks was a risk factor for bilateral pneumothorax (P=0.01). The median length of hospitalization was significantly longer in Group II than in Group I (5.8 days vs 4 days, P=0.03). CONCLUSIONS: RD at term exposes the infant to high morbidity and pneumothorax, especially if born outside of a level III maternity unit and absence of labor.

[Prenatal intestinal volvulus: A life-threatening event with good long-term outcome]. / Volvulus prénatal du grêle : risque vital immédiat mais bon pronostic à long terme.

BACKGROUND AND AIMS: To describe the outcome of neonates with prenatal intestinal volvulus. PATIENTS AND METHODS: All neonates with prenatal intestinal volvulus managed in our institution between May 2004 and December 2010 were retrospectively studied. All neonates with prenatal or neonatal diagnosis of prenatal intestinal volvulus were included. We analyzed age at diagnosis, fetal ultrasound (US) scan and magnetic resonance imaging (MRI) findings, clinical signs at birth, surgical findings, management, and postoperative outcome. RESULTS: Ten neonates with prenatal intestinal volvulus were identified. Prenatal US scans or MRI demonstrated evidence of meconium peritonitis in one fetus and bowel dilatation in 2 others. The mean gestational age at birth was 36 weeks (range, 31-38 weeks) and the mean birth weight was 2811g (range, 2050-3700g). One premature neonate developed respiratory distress and required ventilatory support at birth. In 7 neonates, clinical examination showed distended abdomen and emesis, whereas plain abdominal radiographs showed intestinal obstruction. All neonates underwent surgery and all had normal intestinal rotation, except one with total intestinal volvulus secondary to malrotation. Other causes of volvulus were suspected in 4 neonates: mesenteric defect (n=1), intestinal atresia (n=2) and narrow mesentery (n=1). Detorsion of total volvulus, ileostomy, or intestinal resection with primary anastomosis was performed in 2, 5, and 3 neonates, respectively. One patient with total intestinal volvulus secondary to malrotation died, whereas all other neonates survived. In one patient, the postoperative course was complicated by intestinal dysmotility of the distal small bowel requiring a secondary jejunoileostomy. Stoma closure was subsequently performed at 1 year of age with good outcome. One patient developed angiocholitis treated successfully with antibiotics. Median time to initiate enteral feeds was 7 days (range, 4-16 days) and all patients were subsequently weaned from parenteral nutrition. Median duration of parenteral nutrition was 29 days (range, 6-667 days). None of the patients had cystic fibrosis. Unlike postnatal volvulus, most prenatal volvulus occurs without malrotation. Although prenatal volvulus is a life-threatening condition, our results suggest that good long-term outcome can be achieved in most cases.

[Antenatal treatment of congenital diaphragmatic hernia: an update]. / Traitement anténatal de la hernie de coupole diaphragmatique: le point en 2008.

Despite advances in neonatal care, the associated mortality in isolated congenital diaphragmatic hernia remains in the order of 30%. Death is mainly due to consecutive pulmonary hypoplasia and severe associated pulmonary hypertension. This statement led to the implement of fetal therapy in order to improve fetal lung development. The first phase of fetal surgery consisted in open repair with one-stage surgical correction of the anatomic defect. It was followed by tracheal occlusion technique based on the decrease egress of lung fluid in order to improve lung growth. Initial approach of tracheal occlusion used clips on the trachea. It is now performed with intra-tracheal inflatable balloon. The challenge of such prenatal treatment is to establish accurate prenatal prognosis factors in order to offer this therapeutic in the subgroup of patients in whom outcome of postnatal treatment remains dismal. Current factors used for prenatal prognosis evaluation are thoracic liver position and lung-to-head ratio (LHR). The balloon is inserted at 26 to 28 weeks and removed at 34 weeks. First results are encouraging. However, many patients developed premature prelabour rupture of the membrane and paediatric data are sparse. Only few patients were currently treated and long term evaluation is needed. A powered multicentric study is needed to determine the value of fetal tracheal occlusion in the management of fetuses with isolated severe congenital diaphragmatic hernia.

Prenatal diagnosis of dumbbell neuroblastoma.

A neuroblastoma that develops in the sympathetic nodes can infiltrate the intervertebral foramina and invade the spinal canal, leading to spinal cord and nerve root compression and neurological impairment. Dumbbell neuroblastomas are now considered to be unresectable tumors and preoperative chemotherapy is recommended. We report the prenatal diagnosis of a dumbbell neuroblastoma successfully managed through premature delivery followed by immediate chemotherapy. We suggest that delivering prematurely in such cases is only of benefit if chemotherapy can be administered under favorable conditions. Chemotherapy should proceed immediately after delivery in order to reduce the size of the tumoral mass and its effects on the spine.

Gorlin syndrome presenting as prenatal chylothorax in a girl.

Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant condition with an estimated prevalence of 1:57 000. GS is associated with congenital malformations and predisposition to neoplasms. The main features observed in patients with GS are basal cell carcinomas, odontogenic keratocysts, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar epidermal cysts, facial dysmorphism, and cerebral falx calcification. More than 100 other clinical manifestations have also been described in the literature including ovarian fibroma, enlarged cerebral ventricles, and lymphatic as well as chylous mesenteric cysts. The Patched (PTCH) gene is responsible for GS when mutated. Here, we report on a prenatal diagnosis of GS in a girl with a chylothorax, a previously unreported feature in GS. We discuss the clinical features observed in this family and we comment on the molecular studies that allowed us to describe a previously unreported Patched gene mutation.

Delayed surgery in pericardial teratoma with neonatal hydrops.

Timing of neonatal surgery in cases of pericardial teratoma with hydrops is not standardised. We report two cases of hydropic premature newborns with pericardial teratoma in which surgery was delayed until respiratory and haemodynamic stabilisation. Mature teratoma was removed on day 3. The newborns were weaned from the ventilator on postoperative day 5 and 10, respectively. Both infants were doing well at 18 months, suggesting delayed surgery may be feasible and effective.

Pericardial teratoma complicated by hydrops: successful fetal therapy by thoracoamniotic shunting.

Pericardial teratoma is a potentially curable lesion that may become life threatening when it induces mediastinal compression and fetal hydrops. So far, cases with fetal hydrops have been managed by elective delivery or pericardial needle decompression. We report a case in which pericardial teratoma resulted in fetal hydrops. Following transpleural needling of the fetal pericardium at 29 weeks and 6 days, pericardial effusion decreased but hydrops persisted, while major unilateral pleural effusion appeared. A thoracoamniotic shunt was placed at 30 weeks and 5 days. Hydrops resolved, although incompletely. The baby was delivered at 32 weeks and was operated upon on day 3. This observation suggests that fetal hydrops associated with pericardial teratoma may improve following thoracoamniotic shunting. Fetal therapy may limit the risks of respiratory distress arising from the combined effect of airways compression and lung immaturity.