Objective To study the relationship between polymorphisms of RET gene and the incidence of Hirschsprung's disease, investigate the haplotypes of RET gene in patients with Hirschsprung's disease, and analyze the characteristics of single nucleotide polymorphisms of RET gene in Zhejiang Han population. Methods Peripheral blood samples were collected from 123 patients with Hirschsprung's disease and 194 healthy children from 2005 to 2007 at Children's Hospital of Zhejiang University. G enomic DNA was extracted from the peripheral blood, and the genotypes were analyzed using polymerase chain reaction and direct sequencing. The frequencies of haplotypes were estimated using the PHASE software. The frequencies of RET alleles of the 194 healthy children were compared with those of other races. The correlation between RET gene and Hirschsprung's disease was analyzed using the chi-square test, and it was expressed in the form of odds ratio and 95% confidence interval.Results Increased risk of Hirschsprung's disease was observed in homozygous genotypes of the RET alleles -5AA, - 1CC, c135AA and c2307GG when compared with other genotypes ( x2 =57. 775, 20.469, 57. 040,38. 869, P ＜ 0. 05 ). Increased risk of Hirschsprung's disease was also observed in RET alleles -5A, - 1 C,c135A, c2307G when compared with other alleles ( x2 = 85.114, 53.117, 77. 005, 70. 161, P ＜0.05). There was no relationship between the frequencies of the alleles and the types of Hirschsprung's disease ( x2= 0.048,0.265, 0. 395, 0.027, P ＞ 0.05 ). The percentage of patients with haplotype ACAG was 75.2%, which was significantly higher than that of 38.7% in healthy children ( x2 = 62. 776, P ＜ 0.05 ). The frequencies of Hirschsprung's disease-associated alleles of RET - 5A, c135A and c2307G in Chinese were significantly higher than thosein the Caucasians or Yorubas (P＜0.05). Conclusions RET -5G＞A, -1A ＞C, c135G＞A and c2307T ＞ G are associated with Hirschsprung's disease and haplotype ACAG is the core one in Zhejiang Han population. There is no relationship between the frequency of allele and the types of Hirschsprung's disease. The frequencies of alleles of RET -5A, c135A and c2307G in Chinese are significantly higher than those in the Caucasians or Yorubas.

Objective To evaluate one-stage procedure for the treatment of neonatal intermediate and high imperforated anus. Methods Clinical data of 21 neonates with intermediate or high imperforated anus undergoing one-stage surgical procedure were summarized. Results Postoperative complication included mucous overlap and soiling in 1 case, and occasional soiling in 2 cases. No postoperative constipation developed in any case. Conclusions Neonatal one-stage procedure avoids short comings often seen in staged procedures and needs no colostomy, promoting hyperplasia and prolongs sphincter during early stage, preventing secondary megacolon.

OBJECTIVETo analyze complications after laparoscopic Ladd operation for intestinal malrotation, related causes and possible solutions.

METHODSClinical data of 81 neonates who underwent laparoscopic Ladd operations for intestinal malrotation in the Children's Hospital, Zhejiang University School of Medicine between January 2015 and January 2018 were reviewed. The abdominal complications and findings during operation and reoperation were analyzed.

RESULTSOperations were successfully completed in all patients, and there was no patient converted to open surgery. The annular pancreas in 6 cases and duodenal diaphragm in 4 cases were confirmed during the operation. The recurrent volvulus developed in 3 patients (3.7%), of whom 2 cases were confirmed to have midgut necrosis during open surgery 1 week and 3 months after laparoscopic Ladd operation, and both finally died; 1 case was corrected by second laparoscopic operation. Cecal perforation occurred in 1 patient (1.2%), which was caused by intensive high frequency coagulation of the appendiceal stump. One patient (1.2%) developed chylous ascites and improved after conservative treatment. Adhesive small bowel obstruction was observed in 3 cases (3.7%), and all relieved after conservative treatment.

CONCLUSIONSLaparoscopic Ladd operation for intestinal malrotation in neonates was effective, and the incidence of abdominal complications may be minimized by experienced skills and strict perioperative management.

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

OBJECTIVETo evaluate the efficacy and safety of transumbilical single-site laparoscopic surgery for congenital duodenal obstruction (CDO) in neonates.

METHODSA retrospective analysis of clinical data of 15 patients with CDO undergoing transumbilical single-site laparoscopic treatment during November 2017 and January 2018 (single-site group), and 20 patients with CDO undergoing conventional three-hole laparoscopic treatment during August 2017 and October 2017 (three-hole group) was performed. All patients were from the Children's Hospital, Zhejiang University School of Medicine. The operation time, time of initial feeding, time of adequate feeding, length of hospital stay after operation and postoperative complications were compared between two groups.

RESULTSThe operations were completed in all patients. No patient converted to laparotomy, and no massive hemorrhage was observed during operation. The operation time of single-site group was (90±10) min for patients with duodenal diamond-shaped anastomosis and (81±15) min for patients with Ladd operation, while those of three-hole group were (85±9) min and (72±11) min, respectively. Postoperative initial feeding time of single-site group was (5.0±1.0) d, and that of the three-hole group was (4.8±0.8) d. The adequate feeding time was (9.0±1.2) d in the single-site group, and (9.3±0.8) d in the three-hole group. The length of hospital stay after operation was (11.2±2.5) d in the single-site group, and (11.5±2.8) d in the three-hole group. There was no significant difference in operation time, postoperative initial feeding time, adequate feeding time and length of hospital stay after operation between two groups (all >0.05).

CONCLUSIONSTransumbilical single-site laparoscopic surgery for CDO in neonates is safe and effective, and the postoperative abdominal scar is more hidden.

OBJECTIVE: To analyze clinical manifestations, diagnosis and treatment of annular pancreas in neonates. METHODS: Clinical data of 114 neonates with annular pancreas admitted in the Children's Hospital of Zhejiang University from January 2009 to December 2018 were reviewed. The demographic parameters (gestational age, birth weight), clinical manifestations, onset time, results of antenatal examination, associated anomalies, radiological findings, operations, postoperative complications were analyzed. RESULTS: One hundred and two cases were examined by abdominal echography, in which 68 cases showed duodenal obstruction, 4 cases showed annular pancreas. Plain abdomen X-ray examination performed in 113 cases before operation, 76 cases presented double-bubble sign, 12 cases presented single-bubble sign and 5 cases had high-position intestinal obstruction. Upper gastrointestinal radiography (UGI) was performed in 103 cases, which suggested duodenal obstruction in 102 cases. Operations were performed in all cases, of which 69 cases were operated under laparoscopy including 1 case converted to open laparotomy. The mean fasting time after surgery was (7.8±2.7) d, and the mean length of hospital stay was (16.9±10.1) d. Five patients had postoperative complications. The incidence of postoperative complications in antenatal abnormal group was lower than that in the antenatal non-abnormal group (<0.05); the average fasting time in laparoendscopic surgery group was shorter than that in traditional laparotomy group (<0.05). CONCLUSIONS Neonates with recurrent vomiting early after birth should be highly suspected to have annular pancreas. The fetal chromosome examination should be performed with abnormal antenatal screening. Surgery is the only effective way to diagnose and treat annular pancreas, and laparoscopic surgery could be the first choice for experienced doctors.
Duodenal Obstruction ; diagnostic imaging ; surgery ; Humans ; Infant, Newborn ; Laparoscopy ; Pancreas ; abnormalities ; diagnostic imaging ; pathology ; surgery ; Pancreatic Diseases ; diagnostic imaging ; pathology ; surgery ; Retrospective Studies