CHARGE syndrome in a neonate with esophageal atresia / 中华围产医学杂志

This article reported a case of neonatal CHARGE syndrome complicated by congenital esophageal atresia. A prenatal ultrasound examination at 30 weeks of gestation revealed polyhydramnios and a small magenblase of the fetus, then fetal MRI suggested congenital esophageal atresia. The infant was born with severe asphyxia at 37 +5 gestational weeks by cesarean section due to placental abruption with a birth weight of 2 310 g. Gastric tube could not be placed after resuscitation. Congenital esophageal atresia complicated by tracheoesophageal fistula was diagnosed by esophageal imaging. Bilateral choanal atresia was detected by electronic nasopharyngoscopy and MRI. Moreover, skull defect, suspected meningocele were also observed. CHARGE syndrome was confirmed by whole exome sequencing, revealing a frameshift deletion of c.2155delA (p.Thr719GlnfsTer9) in the CHD7 gene. The infant died after withdrawing treatment.

Clinical experience of home enteral nutrition in newborn surgical patients / 中华临床营养杂志

Objective:To explore the complications and nutritional outcomes of Home Enteral Nutritional (HEN) in newborn surgical patients.Method:The medical records of neonates with HEN after surgery between 2017 and 2020 were retrospectively reviewed and complications of HEN and the nutritional status before and after HEN were analyzed.Results:A total of 66 neonates were included. The average gestational age at delivery and birth weight were (35.7 ± 3.0) weeks and (2426 ± 709) g, respectively. Diagnoses were mainly congenital esophageal atresia and intestinal diseases, such as intestinal atresia, intestinal torsion and necrotizing enterocolitis. The median age at HEN initiation was 92 (50, 112) days and HEN duration was 64 (41,95) days. HEN was conducted with tube feeding, with 14 patients (21.2%) through gastrostomy, 52 (78.8%) through nasal feeding tube, 20 (30.3%) through intermittent bolus infusion and 46 (69.7%) through continuous infusion. As for the formulas, 19 patients (28.8%) were given whole protein formula, 33 (50%) extensively hydrolyzed formula and 14 (21.2%) free amino acid-based formula. During the follow-up, 10 patients (71.4%) in gastrostomy group experienced 18 cases of catheter-related complications, including accidental removal (6 patients, 42.8%), catheter displacement (4 patients, 18.6%) and excessive granulation tissue at the gastrostomy site (4 patients, 18.6%). In nasal tube feeding group, 14 patients (26.9%) experienced 21 cases of catheter-related complications, including accidental tube removal (19 cases in 12 patients, 23.1%) and tube breakage (2 patients, 3.8%). Both the weight for age Z score and the height for age Z score were improved after HEN.Conclusions:HEN can help to improve the nutrition status in postoperative neonates. Management of catheter-related complications is challenging and warrants team work to improve the outcome of HEN.

Complications and nutritional status following different timings of post-enterostomy stoma reversal in necrotizing enterocolitis / 中国新生儿科杂志

Objective:To study the incidences of postoperative complications and nutritional status following different timings of stoma reversal in necrotizing enterocolitis (NEC).Method:From January 2017 to December 2019, NEC patients receiving enterostomy surgery and later stoma reversal in our hospital were retrospectively analyzed. They were assigned into three groups according to the timing of stoma reversal: early group (reversal within 8 weeks of stoma formation), middle group (reversal at 8~12 weeks from stoma formation) and late group (reversal after 12 weeks from stoma formation). Weight-for-age-Z-score (WAZ) was used to evaluate nutritional status.Result:A total of 56 infants were enrolled, including 6 cases in the early group, 11 cases in the middle group, and 39 cases in the late group. The gestational age and birth weight were (33.4±3.4) weeks and (1 894±640) g, respectively. The median age of stoma formation and the interval between stoma formation and reversal were 16.5 (8.0, 28.2) days and 94.0 (76.5, 126.5) days. No significant differences existed on gestational age, birth weight, age of stoma formation and complications of stoma reversal among the three groups ( P>0.05). The incidence of growth retardation (WAZ<-2) was 14.3% at stoma formation, and significantly increased to 62.5% at stoma reversal ( P<0.05). The WAZ at stoma reversal in early, middle and late groups were (-3.2±1.9), (-3.0±1.6) and (-2.3±1.5), without significant differences( P>0.05). The WAZ gradually increased to (-0.7±1.2), (-0.1±2.0) and (-0.1±0.8) at 42~48 weeks after reversal, respectively. Conclusion:The timing of stoma reversal may not influence the complications of reversal. Growth retardation are common in NEC infants with stoma formation and stoma reversal may improve the nutritional status of the infants. Early reversal of stoma is suggested to improve the nutritional status of patients with poor weight gain after stoma formation.

A study on the prognosis of different surgical procedures for severe jejunoileal atresia / 中国新生儿科杂志

Objective To compare the prognosis of different surgical procedures and to find the relatively safe and effective treatment for severe jejunoileal atresia(sJA).Method From January 2007 to June 2018,children with sJA receiving different surgical procedures in our hospital were retrospectively reviewed.Their clinical data were analyzed,including the survival rate,complication rate,unplanned re-operation rate and postoperative nutritional status.Result A total of 130 patients were enrolled in this study.According to the different types of surgical procedures,the patients were assigned into primary anastomosis group (58 cases,44.6％),Mikulicz double barrel ileostomy group (17 cases,13.1％) and Bishop-Koop anastomosis group (55 cases,42.3％).The overall mortality rate was 6.2％ (8/130).No significant differences existed in mortality rates among the three groups (P＞0.05).The incidences of gastrointestinal complications in primary anastomosis group (70.6％,12/17) and Mikulicz group (70.6％,12/17) were both higher Bishop-Koop group (34.5％,19/55),the differences were statistically significant (P＜0.05).The unplanned re-operation rates were 34.5％ (20/58) in the primary anastomosis group and 17.6％ (3/17) in the Mikulicz group,both higher than the Bishop-Koop group (3.6％,2/55),the differences were also statistically significant (P＜0.05).Multivariate analysis showed that the risk of complications in the primary anastomosis group (OR=3.434,95％CI 1.392～8.471) and Mikulicz group (OR=5.933,95％CI 1.467～23.991) were higher than the Bishop-Koop group.The risk of unplanned re-operation in the primary anastomosis group was 12.422 times as the Bishop-Koop group (95％CI 2.535～60.877).No significant differences existed between the Mikulicz group and the Bishop-Koop group in the risk of unplanned re-operation (P＞0.05).The weight for age (Z-score) in the Bishop-Koop group (-1.4,95％CI-2.0～-0.8) at the stoma closure time was better than the Mikulicz group (-3.2,95％CI-4.4～-2.0),the difference was statistically significant (P＜0.01).Conclusion Bishop-Koop anastomosis has lower complication rate and lower unplanned re-operation rate in the treatment of sJA.The nutritional status of children who received Bishop-Koop anastomosis is better than Mikulicz double barrel ileostomy at the stoma closure time.Bishop-Koop anastomosis is relatively safe and effective for sJA patients.

Nutritional outcomes and risk factors of neonatal enterostomy / 中国新生儿科杂志

Objective To study the nutritional status and risk factors in neonates receiving enterostomy.Method From January 2015 to July 2017,patients who had enterostomy during neonatal period and had the stoma closed in our hospital were retrospectively studied.Z score (weight-for-age) was used to evaluate their nutritional status.The patients were divided into two groups according to the nutritional status when the stoma closed:the malnutrition group and the normal nutrition group.The differences in gender,premature birth,low birth weight,primary disease,ostomy methods,length of proximal small intestine and high output diarrhea through stoma were compared between the two groups.Multivariate Logistic regression analysis was used to determine the risk factors of malnutrition at the closure of the stoma.Result A total of 75 infants with various primary diseases were included.Among them,23 patients were diagnosed with neonatal necrotizing enterocolitis,27 patients jejunoileal atresia,5 patients meconium ileus,15 patients meconium peritonitis and 5 patients Hirschsprung's disease.The median age of enterostomy was 3 (2,8) days,and the median hospital stay after enterostomy was 26 (20,40) days.The median age of stoma closure was 6.0 (5.0,8.5) months.The median Z score at discharge and stoma closure were -1.6 (-2.9,-0.9) and-1.5 (-2.6,-0.5) respectively.No statistically significant differences existed between the two groups (P ＞ 0.05).28 infants (37.3％) were malnutrition when the stoma was closed.The incidence of low birth weight and high output diarrhea through stoma in malnutrition group were significantly higher than the normal nutrition group (35.7％ vs.10.6％,32.1％ vs.10.6％,P ＜0.05).No significant differences in gender,premature birth rate,primary disease,ostomy method and length of proximal intestine between the two groups (P ＞ 0.05).Multivariate Logistic regression analysis indicated that low birth weight,high output diarrhea and jejunoileal atresia were risk factors for malnutrition.Conclusion Malnutrition is common in neonates after enterostomy,and the risk factors for malnutrition are low birth weight,high output diarrhea through stoma and the primary disease jejunoileal atresia.