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.

Vegan diet in children and adolescents. Recommendations from the French-speaking Pediatric Hepatology, Gastroenterology and Nutrition Group (GFHGNP).

The current craze for vegan diets has an effect on the pediatric population. This type of diet, which does not provide all the micronutrient requirements, exposes children to nutritional deficiencies. These can have serious consequences, especially when this diet is introduced at an early age, a period of significant growth and neurological development. Even if deficiencies have less impact on older children and adolescents, they are not uncommon and consequently should also be prevented. Regular dietary monitoring is essential, vitamin B12 and vitamin D supplementation is always necessary, while iron, calcium, docosahexaenoic acid, and zinc should be supplemented on a case-by-case basis.

Ingestion of neodymium magnet spheres: Three case studies.

We report three pediatric cases of two, five, and 12 neodymium magnet spheres ingested simultaneously. In two cases, endoscopic extraction failed because of the strength of the magnetic attraction and a gastrotomy was performed to remove all magnets. In the third case, the magnets were not accessible endoscopically and were removed by laparotomy. We wish to warn consumer groups and pediatricians about the danger of neodymium magnets.

Chronic intestinal pseudo-obstruction in a child with Treacher Collins syndrome.

BACKGROUND: Treacher Collins syndrome (TCS) mainly presents with severe craniofacial developmental abnormalities characterized by a combination of bilateral downward-slanting palpebral fissures, colobomas of the lower eyelids, hypoplasia of the facial bones, cleft palate, malformation of the external ears, atresia of the external auditory canals, and bilateral conductive hearing loss. It is due to mutations in Treacher Collins syndrome 1 (TCOF1) (5q32-q33.1) and Polymerase RNA 1 polypeptides D and C (POLR1D [13q12.2], and POLR1C [6p21.1]) genes, which are responsible for increased neuroepithelial apoptosis during embryogenesis resulting in the lack of neural crest cells involved in facial bone and cartilage formation. Altered function of the upper digestive tract has been reported, whereas severe dysmotility disorders have never been reported. We describe here the first case of TCS associated with histologically proven chronic intestinal pseudo-obstruction (CIPO) in humans. Case presentatios A 12-year-old boy with TCS due to TCOF1 gene deletion experienced nutritional difficulties and digestive intolerance from birth. CIPO was suspected during childhood because of severe intestinal dysmotility leading to enteral-jejunal nutrition intolerance and dependence on total parenteral nutrition. Diagnosis of CIPO with nervous abnormalities was histologically confirmed on a surgical rectal biopsy that showed enlarged ganglionic myenteric plexus. At the age of 9 years, an isolated colonic stenosis without dilatation responsible for severe abdominal pain and altered quality of life led to digestive derivation contributing to rapid disappearance of chronic abdominal pain. At the age of 12 years, the patient was still dependent on total home parenteral nutrition 7 days a week to maintain regular growth velocity. CONCLUSION: Recently, mice studies have pointed out the role played by TCOF1 in ganglionic cell migration in the foregut, suggesting that the synergistic haploinsufficiency of Tcof1 and Pax3, a transcription factor regulating the RET gene involved in disorders of neural crest cell development, probably results in colonic aganglionosis and may explain the association described here between TCS and CIPO. This case may correspond to this possible mechanism in humans. These findings and our clinical report suggest that CIPO may be assessed as unusual digestive manifestations in TCS with TCOF1 deletion.

[A pseudotumoral lesion revealing Meckel's diverticulum]. / Lésion pseudo-tumorale révélatrice d'un diverticule de Meckel.

INTRODUCTION: Meckel's diverticulum is a common malformation in children, usually asymptomatic, with complications in only 20% of cases. Exceptionally, a tumor can develop in Meckel's diverticulum in children, particularly Burkitt's lymphoma; in adults it can develop into a gastrointestinal stromal tumor, a leiomyosarcoma, or a neuroendocrine tumor such as a carcinoid tumor. The diagnosis of inflammatory pseudotumor following an insidious perforation is rare. OBSERVATION: We report the case of a 14-month-old boy who presented with fever, asthenia, food refusal, and digestive complaints such as vomiting and tender abdomen suggesting appendicitis. Computed tomography showed an ileal mass of 3cm in diameter, which led to the suspicion of Burkitt's lymphoma. Laparoscopy showed Meckel's diverticulum with a mass of 3×2.8×2cm. Histological examination confirmed the diagnosis of Meckel's diverticulum with gastric heterotopia and showed a proliferation of spindle cells in a myxoid background, with an inflammatory infiltrate made of lymphocytes and plasmocytes. Immunostaining ruled out a malignant tumor. The diagnosis of an inflammatory pseudotumor developing on a Meckel diverticulum with gastric heterotopias was made. The outcome was favorable after surgical resection. CONCLUSION: While perforation of a Meckel diverticulum with gastric heterotopia is a common finding, the discovery of an inflammatory pseudotumor following a perforation is rare; the differential diagnosis should include Burkitt's lymphoma.

[Duodenal duplication revealed by acute pancreatitis]. / Duplication duodénale révélée par une pancréatite aiguë.

INTRODUCTION: Duodenal duplications are rare congenital malformations whose revealing signs are highly variable and nonspecific. OBSERVATION: We report the case of a female infant who presented with neonatal acute pancreatitis complicated by recurrent ascites, profound hypoalbuminemia responsible for pleural and pericardial effusions, revealing a duodenal duplication cyst. The unusual and original clinical presentation as well as the difficulty detecting the duplication radiologically delayed the diagnosis. A prolonged medical treatment with octreotide, albumin infusions, and exclusive parenteral nutrition led to an almost total disappearance of the ascites before surgery. The outcome was favorable after surgical removal of the duplication with 1 year of follow-up. CONCLUSION: The diagnosis of duodenal duplication can be difficult and it may be necessary to repeat the ultrasound examinations. Surgical resection is delicate, especially when there is an abundant pancreatic ascites. Therefore, an adequate prolonged medical treatment to reduce this ascites is recommended before the surgery.

[Eosinophilic gastroenteritis and colitis in pediatric patients: Increasingly frequent diseases]. / Les gastro-entéro-colites à éosinophiles chez l'enfant : des maladies de plus en plus fréquentes.

Eosinophilic gastrointestinal disorders are a heterogeneous group of disorders characterized by no specific digestive symptoms associated with dense eosinophilic inflammation of the gastrointestinal tract in the absence of known causes for such tissue eosinophilia. Among these diseases, eosinophilic gastroenteritis (EGE) and colitis (EC) are less common than esophagitis, but their incidence and prevalence have been increasing over the past decade due in part to increased disease recognition. The exact pathophysiology is not clear: EGE and EC are immune-mediated diseases implicating adaptive T-helper cell type 2 immunity. According to the site of eosinophilic infiltration, there is a wide spectrum of digestive symptoms ranging from food refusal, nausea, vomiting, abdominal pain, weight loss, gastrointestinal bleeding (anemia), protein loosing enteropathy, ascites, bowel obstruction or perforation for EGE and diarrhea ± bleeding for CE. Endoscopic lesions are not specific: friability, erythematous mucosa with superficial erosions, or ulceration is often observed. Histologically, markedly increased numbers of mucosal eosinophils are seen in biopsy specimens. However, no standards for the diagnosis of EGE or CE exist and few findings support the diagnosis: intraepithelial eosinophils, eosinophil crypt abscesses, and eosinophils in muscularis mucosa and/or submucosa. Other organs are not involved. The other causes of tissue eosinophilia (infections, inflammatory bowel diseases) should be excluded. Food allergy appears to play a central role in driving inflammation in EGE and CE, as evidenced by symptomatic improvement with initiation of food exclusion or elemental diets. Dietary treatment should be the first therapeutic option in children. If the elimination diet fails, corticosteroids are currently the best characterized treatment but appropriate duration is unknown and relapses are frequent. In severe forms, immunomodulators or biologic agents (anti-IL5, anti-IgE, or anti-TNFa) can potentially play a role in the treatment of EGE and CE.

[Severe nutritional deficiencies in young infants with inappropriate plant milk consumption]. / Conséquences nutritionnelles de l'utilisation de boissons végétales inadaptées chez les nourrissons de moins d'un an.

Over the past few years, we have observed increasing consumption of inappropriate plant milks as an alternative to infant milk formula. Some families believe that foods labeled as natural are the most healthy and an appropriate nutritional choice. However, their composition does not respect European recommendations. They are always hypocaloric and protein, vitamin, and mineral concentrations are inadequate. The aim of this study was to report severe nutritional complications after inappropriate plant milk consumption. Between 2008 and 2011, we studied severe nutritional deficiencies caused by consumption of plant milks bought in health food stores or online shops. Infants were identified in our centers and examined through medical history, physical examination, and laboratory testing. Nine cases of infants aged from 4 to 14 months were observed. In all cases, these milks were used as an alternative to milk formulas for supposed cow's milk allergy. At diagnosis, four patients were aged 6 months or less. They had received plant milk exclusively for 1-3 months. The beverages consumed were rice, soya, almond and sweet chestnut milks. In three cases, infants presented severe protein-calorie malnutrition with substantial hypoalbuminemia (<20 g/L) and diffuse edema. In the other cases, the nutritional disorders were revealed by a refractory status epilepticus related to severe hypocalcemia (one case), growth arrest of both height and weight secondary to insufficient caloric intake (five cases), and severe cutaneous involvement (one case). Five children had severe iron deficiency anemia (<70 g/L), three children had a very low 25-hydroxy vitamin D level (nutritional rickets), and two had severe hyponatremia (<130 mmoL/L). Milk alternative beverages expose infants to severe nutritional deficiencies. Serious complications can occur. Early, exclusive, and extended use is riskier. These diseases are preventable, and parental education should be provided. Statutory measures forbidding their use in young infants should be organized to slow down the progress of this social trend.

[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.