Intestinal Atresias: A Ten-Year Evaluation of Outcomes.

INTRODUCTION: Intestinal atresia is a common cause of neonatal bowel obstruction. Atresias are often associated with other congenital anomalies. The purpose of the study was to evaluate associated anomalies, operative management, and postoperative outcomes of infants with intestinal atresia. METHODS: A review of patients presenting to a single free-standing children's hospital from March 2012 through February 2022 was performed. The variables examined were type of atresia, additional congenital anomalies, type of operative intervention, and postoperative outcomes. Standard statistical methods were utilized. RESULTS: A total of 75 patients with intestinal atresia were identified and several of these patients had multiple atresias. Isolated duodenal atresia patients were the most common (49.3%), followed by jejunal (32%) and ileal (12%). Mixed atresias were rare at 4%, with isolated pyloric and colonic also rare at 1.3% each. Malrotation was associated with 13% of patients and equally associated with duodenal and jejunoileal atresias. A low percentage (3%) of intestinal atresias was seen in conjunction with gastroschisis and concomitant malrotation. A majority of infants with duodenal atresia underwent standard duodenoduodenostomy (19% laparoscopic, 81% open). In infants with jejunoileal atresia, most underwent resection with primary anastomosis. A tapering enteroplasty was performed primarily in 13% of atresias. There were no significant differences noted in time to first feed or length of stay between those with and without tapering enteroplasty. Eleven percent of patients required subsequent intervention for stricture or small bowel obstruction. There was one death in this series. CONCLUSIONS: Consistent with other literature, duodenal atresia was the most common type of intestinal atresia. However, we demonstrated that malrotation was equally associated with duodenal and jejunoileal atresias while prior reports had shown a higher association with duodenal atresia. In our patient population, the use of tapering enteroplasty did not appear to be associated with outcomes. Overall, these infants have a low morbidity and mortality rate with a rare need for reoperation.

CLINICAL FEATURES OF THE COURSE OF HIRSCHSPRING'S DISEASE INCHILDREN OF THE FIRST YEAR.

The purpose of the work - to investigate the peculiarities of the clinical course of Hirschsprung's disease in children of the first year of life and to determine the significance of symptoms in the verification of the disease. From 1980 to 2021, at the pediatric surgery clinic of the National Medical University named after O.O. Bogomolets on the basis of the National Children's Specialized Hospital "OKHMATDYT" and in the pediatric surgery clinic of the Ivano-Frankivsk National Medical University on the basis of the Ivano-Frankivsk Regional Children's Clinical Hospital, 483 children of the first year of life suffering from Hirschsprung's disease were examined and treated. The clinical manifestation and course of aganglionosis varied in length at the time of hospitalization and depended on the time after birth. During the first month of life, 97 (20.08%) patients were hospitalized, of which 39 (8.07%) hadatypical clinical picture due to: colonic atresia in 15 (3.10%), colonic atresia + gastroschisis in 3 (0.62%), ileal atresia in 9 (1.86%), esophageal atresia in 3 (0 .62%), clefts of the hard and soft palate in 9 (1.86%). Depending on the age, there were 280 (57.97%) patients under 6 months, and 203 (42.03%) patients between 6 months and 1 year. The classic typical clinical picture was in 444 (91.93%) patients, which was characterized by the absence of meconium excretion, abdominal distension in 444 (91.93%), delayed physiological weight gain against the background of nutritional insufficiency with the development of hypotrophy in 327 (67.70%) , vomiting of stagnant gastric and intestinal contents in 417 (86.34%). On the other hand, enterocolitis in 315 (65.22%), toxic megacolon in 16 (3.31%), and anemia of various degrees occurred in 241 (49.89%) patients among the complications that arose during the examination of patients with Hirschsprung's disease. According to the results of a comprehensive examination, the following extent of aganglionosis was established: rectal in 100 (20.70%), rectosigmoid in 192 (39.75%), subtotal in 150 (31.06%) and total in 41 (8.49%) patients. Concomitant malformations were found in 98 (20.29%) patients: renal malformations were diagnosed in 7 (1.45%) patients, concomitant heart malformations in 18 (3.73%) patients. Associated intraoperative findings were Meckel's diverticulum in 5 (1.03%) and congenital cyst of the right ovary in 1 (0.21%) patient. The clinical course was affected by concomitant malformations: incomplete bowel rotation in 10 (2.07%) and internal abdominal hernia in 2 (0.42%). The clinical manifestations and course of Hirschsprung's disease primarily depend on the presence of accompanying developmental defects, which may prevail during the examination due to vital disorders. In the clinical course of Hirschsprung's disease, it is necessary to distinguish between typical and atypical forms. Typical clinical symptoms were in 444 (91.93%), and atypical in 39 (8.07%).

Primary anastomosis is the preferred surgical approach for proximal intestinal atresia: a retrospective 20-year analysis.

PURPOSE: We aimed to compare the outcomes of primary anastomosis (PA) and enterostomy as treatments for intestinal atresia in neonates to identify the factors influencing the choice of modality. METHODS: We conducted a retrospective single-centre analysis of all neonates with intestinal atresia between 2000 and 2020 and measured the clinical outcomes. We performed logistic regression to identify factors that influenced the choice of surgical approach. RESULTS: Of 62 intestinal atresia neonates, 71% received PA. There were no significant differences in gestation, gender, age at operation, birth weight, or body weight at operation between the PA and enterostomy groups. PA reoperation was not required for 78% of patients, and the PA group had shorter hospital stays. Complications, operative time, duration on parenteral nutrition, time to full enteral feeding were comparable in both groups. Upon multivariate regression analysis, surgeons favoured PA in proximal atresia [Odds ratio (OR) 38.5, 95% Confidence Interval (CI) 2.558-579] while enterostomy in smaller body size [OR 2.75, CI 0.538-14.02] and lower Apgar score [OR 1.1, CI 0.07-17.8]. Subgroup analysis in these patient groups demonstrated comparable outcomes with both surgical approaches. CONCLUSION: Both surgical approaches achieved comparable outcomes, but PA was associated with short hospital stays and the avoidance of stoma-related complications, and reoperation was generally not required. This surgical approach was suitable for patients with proximal atresia, but enterostomy remained a sensible choice for patients with smaller body sizes and lower Apgar scores.

[Delayed complication of surgical treatment of duodenal atresia in neonatal period]. / Pozdnee oslozhnenie khirurgicheskogo lecheniya atrezii dvenadtsatiperstnoi kishki v neonatal'nom periode.

We report a 48-year-old woman who underwent surgery in early neonatal period for duodenal atresia and developed subsequent diseases of the upper gastrointestinal tract. Symptoms of gastric outlet obstruction, gastrointestinal bleeding and malnutrition developed over the past 5 years. Inflammatory and cicatricial lesions of gastrojejunostomy formed for congenital duodenal obstruction following annular pancreas required reconstructive surgery.

Predictors of Survival: A Retrospective Review of Gastroschisis and Intestinal Atresia in Rwanda.

INTRODUCTION: Neonatal surgical diseases are prime examples of the global disparity in surgical access and outcomes, with survival for conditions like gastroschisis reaching above 95% in high-income settings but usually fatal in low-income settings. This study aims to examine outcomes and predictors of mortality in patients with two specific neonatal surgical conditions that often require early transfer and prolonged inpatient care (gastroschisis and intestinal atresia) at Rwanda's main pediatric referral hospital. METHODS: A single-institution retrospective chart review of neonates with gastroschisis and intestinal atresia was conducted between January 2016 and June 2019. Abstracted data included demographics, referral history, admission interventions, operative details, in-hospital complications, nutrition patterns, length of stay, and mortality. Daily logs were created to evaluate feeding status, infection status, and antibiotic usage. Descriptive and univariate analysis was conducted, with the primary outcome being survival to hospital discharge. RESULTS: A total of 112 patients met inclusion criteria (82% gastroschisis [n = 92] and 18% intestinal atresia [n = 20]). Median age at arrival was 0 d (GS) [IQR 0-1 d] and 8.5 d (IA) [IQR 4-10 d] (P < 0.0001). Survival to discharge was 22.8% (GS) (n = 21) and 60% (IA) (n = 12) with a mean length of stay of 28.3 d (GS) and 18.4 d (IA). The median number of days to initiation of oral feeds was 8.5 d [IQR 7-11] for gastroschisis survivors. CONCLUSIONS: Neonatal surgical conditions that require early transfer and prolonged nutritional intervention are challenging in low-resource settings, but through treatment by a comprehensive pediatric surgical service, improving survival is possible.

Laparoscopic Versus Open Surgical Repair of Duodenal Atresia: A NSQIP-Pediatric Analysis.

INTRODUCTION: Laparoscopic repair of duodenal atresia in neonates has gained popularity among some pediatric surgeons. Single-center studies suggest comparable short-term outcomes to open surgery. The purpose of this study was to utilize a large, multi-institutional pediatric dataset to examine 30-day post-operative outcomes by operative approach for newborns who underwent duodenal atresia repairs. METHODS: We identified neonates aged ≤1 wk in the 2016-2018 National Surgical Quality Improvement Program-Pediatric -database that underwent a laparoscopic or open repair for duodenal atresia. Preoperative characteristics were compared between operative approaches. Postoperative complications, operative time, postoperative length of stay (LOS), and supplemental nutrition at discharge were assessed using multivariate regressions. RESULTS: There were 267 neonates who met inclusion criteria. There were 233 (87%) infants who underwent open repairs and 34 (13%) who underwent laparoscopic repairs. Ten (29%) children who had laparoscopy were converted to open. After adjusting for confounding, laparoscopy was associated with an increase in operative time by 65 min (95% confidence interval 45-87 min, P < 0.001) but a five-day shorter LOS (95% confidence interval -9 to -2, P = 0.006) when compared to laparotomy. There were no significant differences in postoperative complications or supplemental nutrition at discharge. CONCLUSIONS: Our findings suggest that laparoscopic repairs of duodenal atresia are associated with shorter postoperative LOS but longer operative times when compared to open repairs. Although the conversion rate to laparotomy remained relatively high, the laparoscopic approach was associated with comparable 30-day postoperative outcomes.

The role of preserved bowel and mesentery fixation in apple-peel intestinal atresia.

OBJECTIVE: This study explored the feasibility of mesoplasty with end-to-side anastomosis in the treatment of different apple-peel mesenteric defects with high jejunal atresia. METHODS: A retrospective analysis was performed on 42 premature infants admitted to the hospital between 2014 and 2021. Prenatal ultrasound scans revealed bowel dilatation. The patients experienced vomiting after birth and produced white or no meconium. Plain radiography showed double or triple bubble signs and the patients underwent emergency laparotomy. High jejunal atresia with different apple-peel atresia appearance was discovered intraoperatively, involving mobilization of the ileocecal region. Patients received end-to-side anastomosis between the enlarged blind pouch and atretic bowel, as well as mesoplasty. A jejunal feeding tube was placed trans-nasally. Patients were discharged after achieving full enteral feeding. We also reviewed the literature on the subject. RESULTS: Three patients died and 39 survived. The discharged patients were followed up for 12 months, and none showed post-operative complications such as intestinal obstruction, malnutrition, or chronic diarrhea. All surviving patients reached the expected height and weight for children of the same age. CONCLUSION: For cases of high jejunal atresia with apple-peel intestinal atresia, mesoplasty may be a good option to avoid postoperative volvulus.

Intestinal Atresias.

Intestinal atresia is a form of congenital bowel obstruction that requires operative repair in the early neonatal period. Duodenal atresia and jejunoileal (JI) atresia are appropriately seen as distinct entities. Both can be suspected with fetal imaging, which can assist with prenatal counseling of families. Duodenal atresia is more commonly associated with comorbidities, whereas JI atresia is more often an isolated finding. Surgical repair is essential and is typically well tolerated. Although it may take time to achieve intestinal function postoperatively, these infants are usually able to tolerate full feeds after resolution of the ileus. Excellent short- and long-term outcomes for isolated duodenal atresia and JI atresia are expected.

Long-term outcomes of various pediatric short bowel syndrome in China.

PURPOSE: The goal of this study was to analyze long-term outcome of various pediatric short bowel syndrome (SBS) at an intestinal rehabilitation center in China. METHODS: One hundred and fifty-seven children with SBS were enrolled in this study from October 1988 to July 2019. Their long-term follow-up outcome was analyzed according to the age of disease onset, parenteral nutrition (PN) duration, and anatomic types of short bowel, respectively. The clinical characteristics, which included demographics, the length of residual small bowel, PN duration, PN dependence, SBS-related complications such as IF-related liver disease (IFALD), catheter-related bloodstream infection (CRBI), and mortality were compared among the groups. RESULTS: The main etiology for SBS were intestinal atresia, NEC, and volvulus. Five of 157 patients did not wean off PN. The incidence of IFALD and CRBI was 24.2 and 22.3%, respectively. Sixteen cases died because of infection and liver failure and eight patients lost to follow-up. The survival rate of the 157 patients was 84.7%. PN duration was longer in the infants and children group (284 ± 457 d vs. 110 ± 64 d, P = 0.021; R = 0.264, P = 0.001) and more patients did not wean off PN than in the neonates group (11.6% vs. 0, P = 0.001; R = 0.295, P < 0.001). Patients with PN with a duration of longer than 90 days had more CRBIs (30.6%, P = 0.025; R = 0.236, P = 0.003). Additionally, the rate of CRBI was higher in patients with stoma (30.0%, P = 0.032). There was no difference in mortality among the groups. In five PN dependence patients, none was SBS onset in neonates. CONCLUSION: Pediatric patients with SBS could achieve favorable long-term survival and enteral autonomy. Different standards of SBS classification such as the age of disease onset, PN duration, and anatomic types of short bowel did not impact the overall mortality of pediatric SBS. Prolonged PN duration positively correlated with the age of disease onset and the incidence of CRBI. Patients with the complete continuity of intestinal tract suffered less from CRBI.

The risk factors for cholestasis in patients with duodenal atresia in a single institutional cohort.

PURPOSE: The etiology of cholestasis in neonates is associated with several factors including gastrointestinal disease and surgery. We aimed to identify the potential risk factors for perioperative cholestasis in patients with duodenal atresia and determine specific cutoff values for the risk factors. METHODS: This retrospective cohort study included 76 neonates diagnosed with duodenal atresia surgically treated during the neonatal period at our institution between January 1990 and March 2017. The neonates were categorized into two groups: those with and without cholestasis. Univariate and multivariate analyses were performed to identify the possible risk factors for cholestasis. RESULTS: Among the 76 neonates with duodenal atresia, 21 (27%) developed cholestasis. The duration of total parenteral nutrition was identified as a risk factor in univariate analysis; however, it was not an independent risk factor for cholestasis. Gestational age and highest C-reactive protein (CRP) values were independent risk factors, with adjusted odds ratios of 0.53 and 1.25, respectively. To predict the occurrence of cholestasis, the cutoff value for gestational age was 35.0 weeks, and highest CRP value was 2.4 mg/dL. CONCLUSIONS: The occurrence of cholestasis in patients with duodenal atresia was associated with preterm delivery and severity of the inflammatory response during the perioperative period.

Is Tapering Enteroplasty an Alternative to Resection of Dilated Bowel in Small Intestinal Atresia?

BACKGROUND: Intestinal atresia is a congenital defect resulting in intestinal discontinuity and can be associated with significant morbidity related to intestinal failure. The bowel proximal to the atresia is often significantly dilated and dysfunctional. The treatment approaches of this dilated bowel include resection with primary anastomosis versus tapering enteroplasty with preservation of bowel length. The purpose of this study was to compare these two approaches in regard to bowel function as characterized by the time to full enteral feeding. METHODS: A retrospective review was performed of intestinal atresia repair performed at a tertiary referral pediatric hospital from 2007 to 2017. Length of stay, time to full enteral feeds, and complications were assessed in patients who underwent repair with tapering enteroplasty (n = 8) and compared with those who underwent resection and anastomosis (n = 39). RESULTS: The median age at surgery, gender distribution, weeks gestational age (WGA), location of the atresia, and comorbidities were similar between the two groups. Overall, there was no statistically significant difference in length of stay and time to full enteral feeds between groups. Three of eight (38%) patients in the tapered group and five of 39 patients (13%; P = 0.12) in the nontapered group underwent further surgical exploration because of bowel dysmotility. Factors associated with longer length of hospital stay were abdominal reoperation and WGA, and factors associated with longer time to full enteral feeds were WGA, abdominal reoperation, and gastroschisis. CONCLUSIONS: Tapering enteroplasty at initial operation for intestinal atresias preserves bowel length and has statistically equivalent outcomes to resection and anastomosis in regard to the length of stay and time to full enteral feeds.

Jejuno-ileal atresia: its characteristics and peculiarities concerning apple peel atresia, focused on its treatment and outcomes as experienced in one of the leading South African academic centres.

PURPOSE: Jejuno-ileal atresia remains the most common form of intestinal obstruction in the neonatal and infantile age group and has an incidence of 1:300 to 1:1500. Apple peel atresia (APA) is the rarest of the five described types. The morbidity and mortality of patients with APA managed at our institution are high, and we review our experience with this paper. We compared our outcomes with other developed and developing countries. In addition, we looked at factors that affect outcome and how we can change them. METHODS: The study was a retrospective review of all patients treated with APA at IALCH between January 2002 and December 2010 and includes 34 patients. RESULTS: The results revealed a mortality in excess of 70%. There were poor antenatal screening, a high rate of prematurity and often delays in transfer to our institution. Relaparotomy and sepsis rates were high. CONCLUSION: This review represents a significant number of patients with APA from a single institution. Although survival rates have improved significantly over the years, APA remains a life-threatening malformation and results in significant morbidity and mortality in our setting.

Obstructive jaundice caused by ulcerative duodenal stenosis: A case report.

A peptic ulcer is a rare cause of distal common bile duct stricture, Obstructive jaundice as a complication of ulcerative duodenal stenosis is quite difficult to differentiate from malignant disease, especially in those in which esophagogastroduodenoscopy examination does not reveal an ulcer. In this case report, a 61-year-old male suffered from right upper quadrant pain, chills and fever caused by duodenal and distal common bile duct stenosis originating from ulcer and was treated surgically.

Gastrointestinal disorders in Down syndrome.

Down syndrome is the most common human chromosomal disorder. Among clinical findings, one constant concern is the high prevalence of gastrointestinal system alterations. The aim of this study was to determine the prevalence of gastrointestinal disorders at a Down syndrome outpatient clinic during a 10-year follow-up period. Data from medical files were retrospectively reviewed from 1,207 patients. Gastrointestinal changes occurred in 612 (50.7%). The most prevalent disorder was chronic intestinal constipation. Intestinal parasite occurred in 22% (mainly giardiasis), gastroesophageal reflux disease in 14%, digestive tract malformations occurred in 5%: 13 cases of duodenal atresia, 8 of imperforate anus, 4 annular pancreases, 2 congenital megacolon, 2 esophageal atresias, 2 esophageal compression by anomalous subclavian and 1 case of duodenal membrane. We had 38/1,207 (3.1%) patients with difficulty in sucking and only three with dysphagia that resolved before the second year of life. Peptic ulcer disease, celiac disease, and biliary lithiasis were less prevalent with 3% each. Awareness of the high prevalence of gastrointestinal disorders promotes outstanding clinical follow-up as well as adequate development and greater quality of life for patients with Down syndrome and their families.

Feeding Outcomes in Neonates With Trisomy 21 and Duodenal Atresia.

BACKGROUND: Duodenal atresia (DA), a common cause of congenital duodenal obstruction, is associated with trisomy 21. The postoperative feeding issues are not well described in this population. We hypothesize that the combination of DA and trisomy 21 is associated with the need for postoperative enteral feeding access. METHODS: A retrospective cohort of patients between 2010 and 2017 with the diagnosis of DA or duodenal stenosis was identified. Relevant prenatal and postnatal clinical data were abstracted. Univariate analyses were performed. RESULTS: Forty-three patients were identified. Nineteen patients (44%) were diagnosed with trisomy 21. Eight patients (25% with trisomy 21) had gastrostomy placed at the time of DA repair. In the remaining patients (n = 35), 40% ultimately had a gastrostomy button placed. The indications for placement included poor oral skills (n = 8), aspiration (n = 5), and failure to thrive (n = 1). All these patients had trisomy 21, resulting in 82.4% of trisomy 21 patients having a gastrostomy. There was a significant association between trisomy 21 and placement of a gastrostomy button both during index admission (P = 0.003) and lifetime (P < 0.001). All trisomy 21 patients with congenital heart disease (n = 9) had a gastrostomy placed versus only five of eight trisomy 21 patients (62.5%) without structural heart disease (P = 0.006). CONCLUSIONS: Our data suggest that a correlation exists between trisomy 21, structural congenital heart anomalies, DA, and the eventual need for gastrostomy. These data should inform operative planning for this patient population.

[Therapeutic experience of type â ¢-b congenital intestinal atresia].

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.

Retrospective study of umbilical cord ulceration related to congenital intestinal atresia: A single-center report.

AIM: Umbilical cord ulceration (UCU) is a disease in which an ulcer forms in the umbilical cord in the pregnant uterus and is accompanied by hemorrhaging from the same site. UCU occurs in fetuses with congenital upper-intestinal atresia (CUIA); however, its onset mechanism remains unclear. Here, we report our investigation of cases of UCU in our hospital. METHODS: Among the 9825 deliveries performed between 2007 and 2016 at this hospital, 20 fetuses were diagnosed with CUIA, 4 (20%) of which had UCU. There was no difference in the backgrounds of the fetuses with UCU (UCU group: 4 fetuses) and those without (non-UCU group: 16 fetuses). RESULTS: There was no intergroup difference in gestational age at delivery. Four cases in the UCU group had maternal age 35 weeks (26-39), weeks of delivery 35 weeks (35-36) and weight 2178.5 g (1600-2640); three out of four fetuses were female; and the location of gastrointestinal obstruction was in the duodenum in one case and in the jejunum in three cases. Death occurred in three of four fetuses in the UCU group versus none in the non-UCU group. CONCLUSION: We performed a retrospective statistical investigation on the risk of UCU onset in cases from this hospital; however, we could not identify any prognostic factors for its onset. We investigated a total of 27 past reported UCU cases and the 4 cases in this study. Mean gestational age at onset was 33.3 ± 2.7 for all 27 cases. Various methods for the early discovery of UCU have been reported in the past; however, there is currently no gold standard. Based on this report and a review of past papers, for CUIA, it is desirable to perform in-hospital management from gestational week 30 onward and decide proper delivery timing on a case-by-case basis.

Laparoscope-assisted intestinal lengthening using an anterior flap in jejunal atresia.

BACKGROUND: Jejunal atresia with short bowel syndrome is an unusual type of jejunoileal atresia. They present with jejunal atresia near the ligament of Treitz and a foreshortened small bowel. In this paper, we report our preliminary experience to emphasize the advantages and feasibility of enteroplasty for intestinal lengthening and primary anastomosis with an anterior flap in jejunal atresia with short bowel syndrome in neonates. METHODS: Between January 2014 and December 2014, four neonates with jejunal atresia and short bowel syndrome were submitted to this procedure in our hospital. Enteroplasty for intestinal lengthening procedures was accomplished in all the neonates by laparoscopic-assisted procedure. The procedure was manually performed after exteriorization of the atretic bowel via the slightly enlarged umbilical port site incision. RESULTS: The mean operative time was 80 min (range 65-110 min). Blood loss was minimal. There was no mortality or surgical complication so far. The median follow-up duration was 14.5 months (range 9-20 months). In all the cases, the autonomy for oral/enteric feeding was obtained within 1 month after surgery. One neonate was readmitted because of associated cholestasis 1 month after the operation, and was cured by conservative therapy. CONCLUSIONS: Enteroplasty for intestinal lengthening and primary anastomosis with an anterior flap is a safe and feasible technique that could allow increased tolerance to oral/enteric feeding, thereby improves their chances for quality survival.

Tetratricopeptide repeat domain 7A (TTC7A) mutation in a newborn with multiple intestinal atresia and combined immunodeficiency.

In the past year, two centers reported autosomal recessive mutations in tetratricopeptide repeat domain 7A (TTC7A) gene in patients with multiple intestinal atresia and immunodeficiency. Here, we present clinical progress of an infant with multiple intestinal atresia and combined immunodeficiency who carries novel compound heterozygote mutations in TTC7A gene.