atresia, jejunal atresia, esophageal atresia, and need for parenteral nutrition for a minimum of 3 days at RSUD Dr. Soetomo Surabaya between August 2018 and January 2019. These children were divided into two groups, those who received standard intravenous LE (medium-chain triglyceride [MCT]/long-chain triglyceride [LCT]) and those who received intravenous omega-3-enriched LE. Differences in AST, ALT, and TG levels were measured before surgery and 3 days after the administration of parenteral nutrition.RESULTS: Liver enzyme and TG levels in each group did not differ significantly before versus 3 days after surgery. However, TG levels were significantly lower in the omega-3-enriched intravenous LE group (p=0.041) at 3 days after surgery, and statistically significant difference in changes in TG levels was noted at 3 days after surgery between MCT/LCT intravenous LE group and the omega-3-enriched intravenous LE group (p=0.008).CONCLUSION: The intravenous omega-3-enriched LE had a better TG-lowering effect than the MCT/LCT intravenous LE in children undergoing gastrointestinal surgery.]]>
Alanine Transaminase ; Child ; Esophageal Atresia ; Fatty Acids ; Humans ; Intestinal Atresia ; Liver ; Parenteral Nutrition ; Triglycerides

OBJECTIVE: To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA). METHODS: The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively. RESULTS: Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory. CONCLUSIONS For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.
Child ; Humans ; Intestinal Atresia ; complications ; surgery ; therapy ; Intestines ; surgery ; Parenteral Nutrition ; Retrospective Studies ; Short Bowel Syndrome ; complications ; Treatment Outcome

PURPOSE: Santulli enterostomy has been used for various surgical abdominal conditions that require temporary diversion of bowel during a neonatal period. The aim of this study was to report clinical outcomes of Santulli enterostomy and to evaluate its usefulness. METHODS: Between January 2000 and December 2016, 40 neonates who underwent Santulli enterostomy were enrolled; Santulli enterostomies were performed for 25 patients without previous laparotomy (primary Santulli group) and 15 patients with previous laparotomy (secondary Santulli group). RESULTS: Small bowel atresia is the first common indication of Santulli enterostomy (22/40, 55.0%), and luminal discrepancy between proximal and distal bowel was the most common determinant factor of Santulli enterostomy (17/40, 42.5%). The median age at surgery and mean birth weight were 2 days and 2,480 g respectively in the primary group, and 71 days, 2,340 g respectively in the secondary group. Operation time was significantly longer in the secondary group than the primary group (156±48 minutes vs. 224±95 minutes, p=0.019), and there was no difference in the time taken to initiation of oral feeding between the two groups. Santulli enterostomy closure was performed at median 65 days after Santulli enterostomy for primary group and 70 days for secondary group. Six complications (15.0%) were found after Santulli enterostomy, and nine complications (24.3%) after Santulli enterostomy closure (p=0.302). The incidence of complications was significantly higher in secondary group than in primary group (4.5% vs. 53.3%, p=0.001), and the reoperation rate was also significantly higher in the secondary group (4.5% vs. 46.7%, p=0.004). CONCLUSION: Santulli enterostomy could be applied as a temporary enterostomy in neonatal patients with various surgical abdominal diseases. Considering the high complication rate after secondary Santulli enterostomy closure, decision making on the timing of enterostomy closure should be done with caution.
Birth Weight ; Decision Making ; Enterocolitis, Necrotizing ; Enterostomy ; Humans ; Incidence ; Infant, Newborn ; Intestinal Atresia ; Laparotomy ; Methods ; Peritonitis ; Phenobarbital ; Reoperation

PURPOSE: Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors. METHODS: There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed. RESULTS: The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study. CONCLUSION: There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.
Birth Certificates ; Birth Weight ; Cause of Death ; Critical Care ; Ebstein Anomaly ; Esophageal Atresia ; Gestational Age ; Heart Failure ; Humans ; Incidence ; Infant, Newborn ; Intestinal Volvulus ; Length of Stay ; Meconium ; Medical Records ; Mortality ; Parturition ; Premature Birth ; Retrospective Studies ; Sepsis ; Stomach ; Tracheoesophageal Fistula

OBJECTIVETo evaluate the efficacy of bowel plication combined with early enteral nutrition (EEN) in the enhanced recovery after surgery(ERAS) of jejunal atresia (JA) neonates.

METHODSBetween January 2005 and January 2014, 58 neonates with JA underwent surgical treatment in Children's Hospital of Nanjing Medical University. Their clinical data, including operation procedures, ages, birth weight, concomitant diseases, age at surgery, hospital stay, total parenteral nutrition (TPN), postoperative intestinal function recovery (the time to the first oral feeding and the time to oral feeding volume reaching 150 ml·kg·d), complications and reoperation, were retrospectively analyzed.

RESULTSAccording to the surgical procedures, the 58 neonates were divided into three groups: control group(18 cases, undergoing atretic segments resection and primary anastomosis), bowel plication group(19 cases, undergoing bowel plication after atretic segments resection and primary anastomosis) and bowel plication combined with EEN group (21 cases, undergoing bowel plication combined with EEN). No significant differences of ages, birth weight, age at operation, and concomitant diseases were found among 3 groups (all P>0.05). The time of hospital stay, the time to the first oral feeding, the time to oral feeding volume reaching 150 ml·kg·d, and the time of TPN in bowel plication group were significantly shorter than those of control group [(19.3±4.4) d vs. (22.7±3.1) d, t=2.696, P=0.011; (9.8±3.3) d vs. (12.5±3.0) d, t=2.630, P=0.013; (18.5±4.1) d vs. (21.5±2.5) d, t=2.726, P=0.011; (13.1±2.9) d vs. (15.0±2.3) d, t=2.219, P=0.033]. However, above parameters of bowel plication combined with EEN group were significantly shorter than those of bowel plication group [(15.3±3.5) d vs. (19.3±4.4), t=4.120, P=0.003; (7.7±2.2) d vs. (9.8±3.3) d, t=2.428, P=0.020; (14.8±2.5) d vs. (18.5±4.1) d, t=3.752, P=0.001; (9.5±3.0) vs. (13.1±2.9) d, t=4.370, P=0.000].

CONCLUSIONThe bowel plication combined with EEN contributes to the early use of intestinal function, shorten the time to the first oral feeding, and reduces the use of TPN, which can improve the recovery of jejunal atresia neonates.

Anastomosis, Surgical ; Comparative Effectiveness Research ; Defecation ; Digestive System Surgical Procedures ; methods ; Enteral Nutrition ; methods ; Humans ; Infant, Newborn ; Intestinal Atresia ; rehabilitation ; surgery ; Jejunum ; abnormalities ; surgery ; Length of Stay ; Parenteral Nutrition, Total ; Postoperative Period ; Retrospective Studies

Umbilical cord ulceration is a rare condition presenting with sudden fetal bradycardia due to fetal hemorrhage and in most cases leading to intrauterine death. A strong association with intestinal atresia has been reported. Most cases present after 30 weeks of gestation, with preterm labor or rupture of membranes followed by sudden fetal bradycardia. We report two such cases of umbilical cord ulceration and review the available literature. One of the cases interestingly presented at 26 weeks, much earlier than what is reported in the world literature. In view of high perinatal mortality and morbidity, awareness of this condition is mandatory for timely and appropriate management to improve the fetal outcome.
Bradycardia ; Female ; Hemorrhage ; Intestinal Atresia ; Membranes ; Obstetric Labor, Premature ; Perinatal Mortality ; Pregnancy ; Rupture ; Ulcer* ; Umbilical Cord*

Umbilical cord ulceration is a rare condition presenting with sudden fetal bradycardia due to fetal hemorrhage and in most cases leading to intrauterine death. A strong association with intestinal atresia has been reported. Most cases present after 30 weeks of gestation, with preterm labor or rupture of membranes followed by sudden fetal bradycardia. We report two such cases of umbilical cord ulceration and review the available literature. One of the cases interestingly presented at 26 weeks, much earlier than what is reported in the world literature. In view of high perinatal mortality and morbidity, awareness of this condition is mandatory for timely and appropriate management to improve the fetal outcome.
Bradycardia ; Female ; Hemorrhage ; Intestinal Atresia ; Membranes ; Obstetric Labor, Premature ; Perinatal Mortality ; Pregnancy ; Rupture ; Ulcer* ; Umbilical Cord*

OBJECTIVETo explore the feasibility and safety of Bishop-Koop stoma procedure in the treatment of neonates with refractory congenital intestinal atresia.

METHODSClinical and follow-up data of 25 neonates with refractory congenital intestinal atresia undergoing Bishop-Koop stoma procedure in our center from January 2011 to December 2014 were retrospectively analyzed. Of 25 neonates, 13 (52%) were male, 12(48%) were female, the birth weight was 1600-3800 g (mean 2920 g), the age of admission was 10 hours to 20 days, and the age of operation was 1-58 d (mean 7 d). Diameter ratio of proximal atresia intestine to distal atresia intestine was all greater than 4. Eleven cases(44%) were high jejunal atresia, 3 cases(12%) type III( b, 7 cases(28%) type IIII(, 14 cases(56%) were identified as complex meconium peritonitis, and 3 cases (12%) received reoperation.

RESULTSAll the cases completed their Bishop-Koop stoma operations successfully with median operative time of 3 (1.2-4.5) hours and median intra-operative blood loss of 3.5(1-18) ml. The postoperative complication rate was 20%(5/25), including 3 cases of cholestasis, 1 case of ileus, and 1 case of neonatal necrotizing enterocolitis with septicemia who died 6 days after operation resulting in the mortality of 4%. Besides, 1 case gave up treatment because of economic reason. For the rest 23 neonates, the median first feeding time was 11 days and mean time was 11(5 to 20) days; the median time of postoperative total parenteral nutrition (TPN) was 15 days and mean time was 21 (5 to 68) days; the median hospital stay was 33 days and mean hospital stay was 25(12 to 81) days, respectively. Two-stage stoma closure operations were performed in all the 23 cases afterwards and no postoperative associated complications were found. When discharge after Bishop-Koop stoma operations, Z score of body weight was normal in 3 cases(13.0%) and lower than normal in 20 cases(87.0%), while in hospitalization for stoma closure, Z score of body weight was normal in 19 cases(82.6%) and lower than normal in 4 cases (17.4%). Of 23 cases, serum albumin level was normal in 9 cases(39.1%) before operation, in 3 cases (13.0%) when discharge and in 22 cases(95.7%) in hospitalization for stoma closure.

CONCLUSIONBishop-Koop stoma procedure is safe and feasible in the treatment of neonates with refractory congenital intestinal atresia, and can obviously improve the nutritional status.

Female ; Humans ; Ileus ; Infant, Newborn ; Intestinal Atresia ; surgery ; Length of Stay ; Male ; Parenteral Nutrition, Total ; Postoperative Complications ; Reoperation ; Retrospective Studies ; Surgical Stomas

OBJECTIVETo investigate the safety and efficacy of endoscopic duodenal valvectomy in the treatment of congenital duodenal membranous stenosis.

METHODSClinical data of two children with congenital duodenal membranous stenosis undergoing endoscopic duodenal valvectomy in our institute within October 2014 were analyzed retrospectively. This procedure was performed with Microknife XL and CRE balloon catheter through porous channel in the 9 mm flexible endoscope.

RESULTSThe first case was a 2-year-old boy who received two endoscopic operations including duodenal diaphragm resection and duodenal dilatation because of incision retraction. The second case was a 19-month-old gril who received once endoscopic duodenal valvectomy. Duodenal obstruction of these two children was relieved after operation. Postoperative x-rays showed no perforation. They could play in the floor 6 hours after operation without any complains, drink water 12 hours, take liquid diets 2 days and half solid food 3 days after operation. During follow-up a month after operation, the body weight gained was 1.5 and 1.0 kg respectively, and the dietary components was significantly improved.

CONCLUSIONEndoscopic duodenal valvectomy is feasible and effective in the treatment of congenital duodenal membranous stenosis.

Child, Preschool ; Constriction, Pathologic ; Duodenal Diseases ; Duodenoscopy ; Female ; Humans ; Infant ; Intestinal Atresia ; Intestine, Small ; abnormalities ; Male ; Retrospective Studies

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion (c.313DeltaTATC; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.
B-Lymphocytes ; Humans ; Immune System ; Intestinal Atresia* ; Liver Diseases ; Male ; Meconium ; Parturition ; Peritonitis ; Prognosis ; Severe Combined Immunodeficiency* ; T-Lymphocytes ; Writing