Lessons from a large nationwide cohort of 350 children with ovarian mature teratoma: A study in favor of ovarian-sparing surgery.

BACKGROUND: Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%-20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian-sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed. DESIGN: This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved. RESULTS: Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not. CONCLUSION: This study is a plea in favor of OSS as the first-choice treatment of OMT when possible. Close follow-up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors.

Opioid effect on the autonomic nervous system in a fetal sheep model.

PURPOSE: Opioid use during labour can interfere with cardiotocography patterns. Heart rate variability indirectly reflects a fluctuation in the autonomic nervous system and can be monitored through time and spectral analyses. This experimental study aimed to evaluate the impact of nalbuphine administration on the gasometric, cardiovascular, and autonomic nervous system responses in fetal sheep. METHODS: This was an experimental study on chronically instrumented sheep fetuses (surgery at 128 ± 2 days of gestational age, term = 145 days). The model was based on a maternal intravenous bolus injection of nalbuphine, a semisynthetic opioid used as an analgesic during delivery. Fetal gasometric parameters (pH, pO2, pCO2, and lactates), hemodynamic parameters (fetal heart rate and mean arterial pressure), and autonomic nervous system tone (short-term and long-term variation, low-frequency domain, high-frequency domain, and fetal stress index) were recorded. Data obtained at 30-60 min after nalbuphine injection were compared to those recorded at baseline. RESULTS: Eleven experiments were performed. Fetal heart rate, mean arterial pressure, and activities at low and high frequencies were stable after injection. Short-term variation decreased at T30 min (P = 0.02), and long-term variation decreased at T60 min (P = 0.02). Fetal stress index gradually increased and reached significance at T60 min (P = 0.02). Fetal gasometric parameters and lactate levels remained stable. CONCLUSION: Maternal nalbuphine use during labour may lead to fetal heart changes that are caused by the effect of opioid on the autonomic nervous system; these fluctuations do not reflect acidosis.

Prostaglandin E1 in infants with congenital diaphragmatic hernia (CDH) and life-threatening pulmonary hypertension.

BACKGROUND: Suprasystemic pulmonary hypertension (PH) is highly predictive of pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH). OBJECTIVES: To report the effects and tolerability of Prostaglandin E1 (PGE1) in newborns with severe CDH and life-threatening PH. METHODS: Newborn infants with isolated CDH and life-threatening PH defined by an acute worsening of the cardiorespiratory function, and bidirectional or exclusive right-to-left shunting across the ductus arteriosus (DA) with an acceleration of the blood flow >1.5 m.s-1 assessed by Doppler echocardiography. Serial measurements of cardiorespiratory variables have been recorded before and after PGE1. RESULTS: 18 infants (out of 102 in the cohort) were included in the study (gestational age: 39 ±â€¯2 weeks). The median FiO2, and preductal and postductal SpO2 were 80% [50; 100], 91% [88; 95] and 86% [82; 91], respectively, before treatment. FiO2 decreased to 35% [30-40] (p = 0.001) at H6. Maximal blood flow velocities in the DA decreased after starting PGE1 from 2.2 m.s-1 [1.5-2.5] to 1 m.s-1 [0.55-1.2] (p < 0.001). CONCLUSIONS: PGE1 treatment improved oxygenation and circulatory function in newborn infants with severe CDH and life-threatening PH. Our data provide evidence that restrictive DA may result in suprasystemic pulmonary hypertension in CDH infants, and that PGE1 may improve cardiorespiratory failure through reopening of the DA. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Unusual case of anorexia.

We report the case of a 15-year-old female patient suffering from progressive anorexia, weight loss and recurrent abdominal pain, initially diagnosed as anorexia nervosa. She eventually presented with severe malnutrition and acute bowel obstruction, revealing a mass of the transverse colon. A well-differentiated Lieberkühn adenocarcinoma was established by histology. The patient underwent transverse and right colectomy and was treated with adjuvant chemotherapy. Colorectal cancer (CRC) is predominantly a disease of older adults and is extremely rare in children and adolescents. Seldom suspected, it is more likely to be diagnosed at an advanced stage, with unfavourable tumour histology and poor outcome. Young patients diagnosed with CRC should receive genetic counselling regardless of their family history or tumour type. This reports' take-home message is that recurrent and persistent digestive symptoms in the young should alert physicians and lead to further investigations.

A Sex Cord-stromal Tumor, Specifically a Fibroma, Arising From the Uterine Corpus: A Case Report.

A 12-yr-old girl presented with lordosis and an intraperitoneal mass that revealed a tumor attached to the uterine fundus. The fallopian tubes and ovaries were spared. The mass was completely excised, and a patch of the uterine fundus and the proximal one third of the fallopian tubes were resected. The lesion was composed of bland spindle cells that were positive for sex cord-stromal markers, with particularly strong staining for inhibin and CD56, as well as patchy staining for calretinin, WT1, and steroidogenic factor 1. Thus, the patient was diagnosed with a sex cord-stromal tumor, specifically a fibroma, arising from the uterine corpus. The pathogenesis of this tumor is unclear. An ovarian origin in the context of adherence or a tumor arising from sex cord-stromal ectopic tissues cannot be excluded, but seem unlikely. The tumor might appear as a particular form of uterine tumor resembling an ovarian sex cord tumor. However, this tumor would differ from the presently described classical form of uterine tumor resembling an ovarian sex cord tumor owing to a pure stromal differentiation instead of a pure sex cord differentiation. Finally, because of the low risk for recurrence, long-term follow-up was prescribed for the patient.

Maternal PUFA ω-3 Supplementation Prevents Neonatal Lung Injuries Induced by Hyperoxia in Newborn Rats.

Bronchopulmonary dysplasia (BPD) is one of the most common complications of prematurity, occurring in 30% of very low birth weight infants. The benefits of dietary intake of polyunsaturated fatty acids ω-3 (PUFA ω-3) during pregnancy or the perinatal period have been reported. The aim of this study was to assess the effects of maternal PUFA ω-3 supplementation on lung injuries in newborn rats exposed to prolonged hyperoxia. Pregnant female Wistar rats (n = 14) were fed a control diet (n = 2), a PUFA ω-6 diet (n = 6), or a PUFA ω-3 diet (n = 6), starting with the 14th gestation day. At Day 1, female and newborn rats (10 per female) were exposed to hyperoxia (O2, n = 70) or to the ambient air (Air, n = 70). Six groups of newborns rats were obtained: PUFA ω-6/O2 (n = 30), PUFA ω-6/air (n = 30), PUFA ω-3/O2 (n = 30), PUFA ω-3/air (n = 30), control/O2 (n = 10), and control/air (n = 10). After 10 days, lungs were removed for analysis of alveolarization and pulmonary vascular development. Survival rate was 100%. Hyperoxia reduced alveolarization and increased pulmonary vascular wall thickness in both control (n = 20) and PUFA ω-6 groups (n = 60). Maternal PUFA ω-3 supplementation prevented the decrease in alveolarization caused by hyperoxia (n = 30) compared to PUFA ω-6/O2 (n = 30) or to the control/O2 (n = 10), but did not significantly increase the thickness of the lung vascular wall. Therefore, maternal PUFA ω-3 supplementation may protect newborn rats from lung injuries induced by hyperoxia. In clinical settings, maternal PUFA ω-3 supplementation during pregnancy and during lactation may prevent BPD development after premature birth.

Pathophysiology of persistent pulmonary hypertension of the newborn: impact of the perinatal environment.

The main cause of pulmonary hypertension in newborn babies results from the failure of the pulmonary circulation to dilate at birth, termed 'persistent pulmonary hypertension of the newborn' (PPHN). This syndrome is characterized by sustained elevation of pulmonary vascular resistance, causing extrapulmonary right-to-left shunting of blood across the ductus arteriosus and foramen ovale and severe hypoxaemia. It can also lead to life-threatening circulatory failure. There are many controversial and unresolved issues regarding the pathophysiology of PPHN, and these are discussed. PPHN is generally associated with factors such as congenital diaphragmatic hernia, birth asphyxia, sepsis, meconium aspiration and respiratory distress syndrome. However, the perinatal environment-exposure to nicotine and certain medications, maternal obesity and diabetes, epigenetics, painful stimuli and birth by Caesarean section-may also affect the maladaptation of the lung circulation at birth. In infants with PPHN, it is important to optimize circulatory function. Suggested management strategies for PPHN include: avoidance of environmental factors that worsen PPHN (e.g. noxious stimuli, lung overdistension); adequate lung recruitment and alveolar ventilation; inhaled nitric oxide (or sildenafil, if inhaled nitric oxide is not available); haemodynamic assessment; appropriate fluid and cardiovascular resuscitation and inotropic and vasoactive agents.

Tracheal occlusion alters pulmonary circulation in the fetal lamb with normally developing lungs.

BACKGROUND: Tracheal occlusion (TO) promotes fetal lung growth through an increase in intraluminal pressure. Although evidence suggests that fetal TO (FETO) decreases the occurrence of pulmonary hypertension in severe congenital diaphragmatic hernia, controversies on its effect on the pulmonary circulation remain. Therefore, we investigated the effects of FETO on the lung hemodynamics in a chronically catheterized fetal lamb model. METHODS: Fifteen pregnant ewes were operated on between 125 and 128 days of gestation (term: 145 days). Catheters and ultrasonic flow transducer were placed through a left thoracotomy in the lamb fetus to determine aortic, pulmonary and left atrial pressures, and left pulmonary artery blood flow. A balloon was positioned between the carina and vocal cords under fetoscopic control. The animals were assigned to either control (n=6) or FETO (n=9) groups. TO was performed by inflating the balloon. We studied the acute effects of temporary (2-h) and prolonged (4-day) TO on basal pulmonary vascular tone and on the pulmonary vascular reactivity to acetylcholine and to increased fetal oxygen tension. RESULTS: We found that left pulmonary blood flow (LPA) increased and pulmonary vascular resistance (PVR) decreased by 20% during brief TO (p<0.05). After balloon deflation, LPA blood flow further increased by 40%, and PVR decreased by 50% compared to baseline values (p<0.05). In contrast, no change in LPA blood flow or PVR was observed during prolonged TO. Moreover, the vasodilator responses to acetylcholine and to increased fetal PaO2 were blunted during TO. CONCLUSIONS: These data indicate that antenatal tracheal occlusion promotes active pulmonary vasodilation, which is partly blunted by the mechanical effects of elevation of the intraluminal pressure.

[Diagnosis of acute abdominal pain in infants]. / Douleurs abdominales du nourrisson, démarche diagnostique.

In front of infant and toddler presenting with unexplained cries, unusual behavior, and tachycardia, pain should be recognized, and signs and symptoms of shock and intestinal occlusion should be sought without any delay. Meningitis, pyelonephritis, and pneumonia must be taken into consideration in a young child with fever and irritability. In the presence of any paroxystic pain with refusal of feeding, one should consider acute intestinal occlusion, volvulus due to intestinal malrotation if associated with signs of shock, and volvulus related to postsurgical adhesions if history of abdominal surgery. Abdominal ultrasonography is the exam of choice in these cases. Examination of inguino-scrotal region is essential in order to rule-out inguinal hernia, ovarian hernia, and testicular torsion. Infant colic and peptic esophagitis are common causes of recurrent pain.

[Duodenal atresia and choledochal cyst: a rare combination]. / Atrésie duodénale et kyste du cholédoque: une association rare.

Congenital duodenal atresia is usually associated with various congenital anomalies. The embryological events in the development of the biliary tract and duodenum are linked. Although an association between duodenal atresia and biliary anomalies is predictable, it is rarely observed. We describe the case of a 27 year-old woman, operated on for duodenal atresia in her childhood, who presented cholangiolitis. Morphologic investigation allowed the diagnosis of choledochal cyst with an intracystic stone developed in the pancreatic head. A pancreatico-duodenectomy was performed because of the importance and the location of dilatation and stone with biliary obstruction. To our knowledge, the association between a congenital duodenal atresia and a choledochal cyst revealed at adult age had never been described in the literature.