Importance of Prenatal Diagnosis of Ileal Atresia in Gestational Diabetes Cases.

BACKGROUND Maldevelopment of the fetal bowel can result in the rare condition of intestinal atresia, which results in congenital bowel obstruction. This report describes a case of prenatal diagnosis of fetal ileal atresia at 22 weeks' gestation. CASE REPORT Here, we present a 24-year old woman who was 22 weeks into her first pregnancy when she underwent routine fetal ultrasound. She was diagnosed with gestational diabetes mellitus. Her body mass index was normal and she had normal weight gain. The ultrasonographic examination performed revealed a hyperechoic bowel and a small dilatation of the bowel. The couple was counselled for possible intestinal atresia and its postnatal implications. At 33 weeks of gestation, polyhydramnios appeared, and the intestinal distension was much more pronounced, with hyperechoic debris in the intestinal lumen (succus-entericus). After birth, surgery was performed and we concluded the patient had type II atresia, which was surgically treated. CONCLUSIONS This report has highlighted the importance of antenatal ultrasound in detecting fetal abnormalities, and has shown that rare conditions such as intestinal atresia can be accurately diagnosed and successfully managed. Surgical correction, if implemented promptly after stabilizing the general condition, can have a relatively good prognosis. Coexisting fetal ileal atresia and gestational diabetes mellitus are rare occurrences, which can make each condition even more difficult to treat.

Laparoscopic repair of duodenal atresia: systematic review and meta-analysis after consistent implementation of the technique in the past decade.

BACKGROUND: Laparoscopic repair of duodenal atresia (LRDA) remains a technically challenging procedure and its benefits ambiguous. To assess the safety and efficacy of LRDA, we performed a systematic review of techniques and material for LRDA and a meta-analysis comparing outcomes with open repair (OR). METHODS: Comprehensive search of EMBASSE, PubMed and Cochrane was performed from 2000 to 2023. Studies comparing LRDA with OR were identified and outcomes extracted included operative time, time to enteral feeds, length of hospitalisation, anastomotic leaks and stricture and total complications. χ2 was used to assess associations between complications and conversions rates of different LRDA approaches (laparoscopic technique, suturing technique). Comprehensive meta-analysis was used for Meta-analysis. RESULTS: Twelve studies were identified and 1731 patients were enrolled in the study (398 [LRDA] and 1325 [OR]). Total rate of complications and conversion for LRDA was 15.58% and 18.84%, respectively. Complication rates were not significantly affected by operative technique and suturing technique. Conversion rates were not significantly affected operative technique; using a combination of interrupted and running suturing was significantly higher than using running or interrupted (χ2 = 7.45, p < 0.05). Anastomotic leaks, strictures and total complications were equivocal between LRDA and OR (OR 1.672, 95% CI 0.796-3.514; OR 2.010, 95% CI 0.758-5.333; OR 1.172, 95% CI 0.195-7.03). Operative time was significantly greater for LRDA (SDM 1.035, 95% CI 0.574-1.495, p < 0.001). Time to initial and full enteral feeds and length of hospitalisation were shorter in the LRDA group (SDM - 0.493, 95% CI - 2.166 to 1.752, p = 0.466; SDM - 0.207, 95% CI - 1.807 to 0.822, p = 0.019; SDM - 0.111, 95% CI - 1.101 to 0.880, p = 0.466, respectively). CONCLUSIONS: LRDA showed equivalent complication rates compared to OR with an additional benefit of quicker establishment of feeds. There was no significant difference in complication and conversion rates between laparoscopic techniques. Despite a longer operative time, LRDA provides a safe minimal access approach for neonates after this consistent implementation of the technique in the past decade.

Efforts to improve outcomes among neonates with complex intestinal atresia: a single-center low-income country experience.

PURPOSE: Intestinal obstruction caused by intestinal atresia is a surgical emergency in newborns. Outcomes for the jejunal ileal atresia (JIA), the most common subtype of atresia in low-income countries (LIC), are poor. We sought to assess the impact of utilizing the Bishop-Koop (BK) approach to JIA in improving outcomes. METHODS: A retrospective cohort study was performed on children with complex JIA (Type 2-4) treated at our national referral hospital from 1/2018 to 12/2022. BK was regularly used starting 1/1/2021, and outcomes between 1/2021 and 12/2022 were compared to those between 1/2018 and 12/2020. Statistical significance was set at p < 0.05. RESULTS: A total of 122 neonates presented with JIA in 1/2018-12/2022, 83 of whom were treated for complex JIA. A significant decrease (p = 0.03) was noted in patient mortality in 2021 and 2022 (n = 33, 45.5% mortality) compared to 2018-2020 (n = 35, 71.4% mortality). This translated to a risk reduction of 0.64 (95% CI 0.41-0.98) with the increased use of BK. CONCLUSION: Increased use of BK anastomoses with early enteral nutrition and decreased use of primary anastomosis improves outcomes for neonates with severe JIA in LIC settings. Implementing this surgical approach in LICs may help address the disparities in outcomes for children with JIA.

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.

Correlation analysis of feeding intolerance and defecation after primary anastomosis for neonatal intestinal atresia.

PURPOSE: To investigate the correlation between postoperative feeding intolerance and defecation, with a view to carrying out prognostic assessment and timely intervention for the recovery of postoperative gastrointestinal function. METHODS: The 114 neonates with congenital intestinal atresia who underwent primary anastomosis admitted to Shenzhen Children's Hospital from January 2014 to December 2022 were studied, and the patients' basic information, intraoperative conditions, postoperative feeding and defecation, and hospitalization time were retrospectively analyzed. RESULTS: The risk factors for feeding intolerance after primary anastomosis for intestinal atresia are the gestational days, the time of the first postoperative defecations, the number of defecations on the previous day and the average number of defecations before feeding. CONCLUSION: The incidence of postoperative feeding intolerance is higher in preterm infants, and pediatricians can decide the timing of breastfeeding on the basis of the patients' defecation. The focus on accurate defecation may be more meaningful in determining and predicting postoperative feeding intolerance in the infants.

A rare occurrence of multiple intestinal atresias, with successful one-procedure resection and primary anastomosis.

SUMMARY: Intestinal atresia is a common cause of neonatal bowel obstruction. Many theories exist relating to intestinal atresia, though the best explanation is related to vascular events. Duodenal atresia is thought to be due to a developmental anomaly of the intestine. A rare combination of concomitant Type III duodenal atresia, Type III B jejunal atresia, and Type I ileal atresia is presented. The differing pathogenesis of these atresias makes the condition exceptionally rare. This patient was successfully treated, via explorative laparotomy, with resection of the atretic segments and two primary anastomoses, without the need for enterostomies or stents.

Type IIIb jejunal atresia treated surgically with two end-to-end anastomoses.

A term newborn presented after birth with abdominal distension and vomiting in a developing country in Asia. Ultrasonography suggested intestinal obstruction and abdominal X-ray showed a 'double-bubble' sign suggestive of intestinal atresia. The newborn was diagnosed with early-onset neonatal sepsis secondary to intestinal obstruction and transferred to the neonatal intensive care unit. Surgery was performed and type IIIb jejunal atresia was found. Type IIIa/b jejunal atresia is associated with high incidence of poor outcome in lower socioeconomic countries. The affected intestinal segment and a distal serosal tear were resected and two end-to-end anastomoses formed. Oral feeding commenced on day 13 postoperatively. The patient was discharged on day 20 with adequate nutritional status. At follow-up, the patient continued to thrive. An operation involving resection of two different segments of bowel repaired with two end-to-end anastomoses in a neonate had a successful outcome for the patient.

Prenatal and postnatal peculiarities and consequences of intestinal loop patency disorder with expansive giant cystic abdominal mass in a preterm newborn.

AIM: To analyse the findings in a newborn (36 gestational weeks, birth weight: 4,030 grams, birth length: 48 cm, Apgar score 7/8/8 points) with prenatal suspicion of intestinal obstruction at the duodenum/jejunum level. The patient required urgent surgery on the first day of life. RESULTS: Examination of the abdominal cavity confirmed the presence of a cystic mass with a volume of approximately 800 ml, which was located at the site of jejunal atresia. As part of the surgical solution, the cystic formation and the atretic segment of the intestine were resected with subsequent end-to-end jejuno-jejunal anastomosis and Bishop‒Koop ileostomy. The histological examination of 3 samples collected confirmed the presence of mucous membrane and smooth muscle. CONCLUSION: The cyst anatomically communicated with the aboral section of the jejunum, but the lumen of the jejunum was functionally obturated by solid whitish masses. The histological examination confirmed the diagnostic features of a cyst of intestinal origin. The ileum and colon were patent throughout, but of smaller diameter, so a Bishop-Koop relieving anastomosis was indicated. The condition of the child at the age of 9 months was stabilised and surgical closure of the stoma was carried out (Tab. 1, Fig. 8, Ref. 21). Text in PDF www.elis.sk Keywords: newborn, jejunal atresia, intestinal cyst.

Duodenal Atresia Repair: A Single-Center Comparative Study.

BACKGROUND: The use of laparoscopy in the repair of duodenal atresia has been increasing. However, there is no consensus regarding which surgical approach has better outcomes. We aimed to compare the different surgical approaches and types of anastomoses for duodenal atresia repair. METHODS: Patients who underwent duodenal atresia repair at a single pediatric center were identified between January 2006 and June 2022. Those with concomitant gastrointestinal anomalies or who required other simultaneous operations were excluded. The primary outcome was rate of complications, defined as rate of leak, stricture, and re-operation by surgical approach and technique of anastomosis. RESULTS: A total of 78 patients were included. The majority were female (51.3%, n = 40), with a median age of 4 days (IQR 3.0,8.0) and a median weight of 2.7 kg (IQR 2.2,3.3) at repair. The re-operation rate was 7.7% (n = 6), of which two were anastomotic leaks, and four were anastomotic strictures. The leak rate was 5.6% (n = 1/18) for the open handsewn and 4.8% (n = 1/21) for the laparoscopic handsewn technique. The stricture rate was 12.5% (n = 1/8) for the laparoscopic-assisted handsewn, 9.1% (n = 2/22) for the laparoscopic U-clip, 4.8% (n = 1/21) for the laparoscopic handsewn, and none with laparoscopic stapled and laparoscopic converted to open handsewn techniques. No differences were found in complication rate when controlling for surgical approach. CONCLUSION: The method of surgical approach did not affect the outcomes or complications in the repair of duodenal atresia.

[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.

Type II Congenital Pyloric Atresia with Desquamative Enteropathy Diagnosed Postoperatively: A Case Report.

Congenital pyloric atresia (CPA) is a rare condition that presents as gastric outlet obstruction in the first few weeks of life. Isolated CPA typically carries a good prognosis but when associated with other conditions such as multiple intestinal atresia or epidermolysis bullosa (EB), the outcomes are generally poor. This report describes a four-day-old infant who presented with nonbilious emesis and weight loss in whom an upper gastrointestinal contrast study revealed gastric outlet obstruction determined to be consistent with pyloric atresia. The patient underwent operative repair via Heineke-Mikulicz pyloroplasty. Postoperatively, the patient continued to have severe diarrhea and was found to have desquamative enteropathy though had no skin findings consistent with EB. This report emphasizes consideration of CPA as a differential diagnosis for neonates presenting with nonbilious emesis and demonstrates the association between CPA and desquamative enteropathy without EB.

The profile and outcome of small bowel atresia at Universitas Academic Hospital.

BACKGROUND: In patients with intestinal atresia, associated congenital anomalies and prematurity are the main predictors of mortality in high-income countries (HIC). In low-and middle-income countries (LMIC), late referrals and lack of resources contribute to mortality. This study aims to describe the outcome of small bowel atresia at a tertiary institution in a LMIC. METHODS: A retrospective descriptive and analytical study was done on all neonates admitted with small bowel atresia from 1 January 2010 to 31 October 2016. Birth demographics, status on arrival, atresia type, surgical interventions and clinical course were analysed in relation to outcomes including mortality. RESULTS: The study included 92 patients. Thirty-three patients had duodenal atresia and 59 jejunal atresia. Median gestation was 34 weeks, with average birth weight 2.2 kg. Seven were diagnosed antenatally and 13 (14.1%) were born in a tertiary institution. A cardiac congenital anomaly was present in 43.5%. Late referrals comprised 72.8%. Sixty per cent were dehydrated and 55.4% were septic on arrival. The median age at surgery was 7.5 days after being admitted, for a median of 2 days. The median age reaching full feeds was 18 days, and 28.6% had a feeding intolerance. The median number of septic episodes were two and the median length of stay 28 days. Of the 92 patients, 8.7% were palliated and 10.9% died. Mortality was strongly associated with prematurity and sepsis with hospital stay. CONCLUSION: Despite late referrals, our mortality was comparable to HICs. We overcame this burden due to access to resources, sophisticated equipment and a low surgical complication rate. The length of stay could be decreased with a feeding protocol and discharge goals.

Does Preterm Status Hinder the Timely Diagnosis of Intestinal Atresia?

Intestinal atresia is one of the most common causes of bowel obstruction in newborns. Unfortunately, this diagnosis is often missed or delayed in extremely preterm infants because of complications of prematurity including feeding intolerance and necrotizing enterocolitis. Here we report 2 cases of jejunoileal atresia in extremely preterm infants who were diagnosed beyond 30 days of age. Case 1 had jejunoileal atresia type IIIa, whereas case 2 had type IV jejunoileal atresia complicated by short bowel syndrome. Ideally, intestinal atresia should be diagnosed as early as possible in a patient's hospital course to attain full enteral feedings earlier and avoid prolonged hospitalization. Planned surgical intervention prior to the development of sepsis or bowel perforation is associated with better outcomes. Ultimately, this report serves as a reminder to physicians that intestinal atresia remains an important differential for infants, including preterm infants, with feeding intolerance in the first days to weeks of age.

Delayed presentation of duodenal atresia.

Background: The most common type of duodenal atresia (DA) (Type I), also known as duodenal web or membrane can present later in infancy or early childhood if the membrane or web is fenestrated. We describe six patients with delayed presentation of DA. Materials and Methods: Retrospective review of hospital records of six patients with delayed presentation of DA due to fenestrated web managed in Paediatric Surgery Department at a tertiary care institute over a period of 2 years (January 2019 to December 2020) was done. The data of these patients were analysed on the basis of age at presentation, clinical presentation, associated anomalies, radiological findings, intra-operative findings, management and postoperative course. Results: The median age at presentation was 6.5 months (range: 1 month to 10 years). There were four males and two females. The most common presentation was emesis seen in all six patients. Two patients had Down syndrome. Associated congenital anomalies were cardiac in one patient, anterior ectopic anus in one patient and malrotation of midgut in one patient. Upper gastrointestinal contrast suggested incomplete duodenal obstruction in all patients. At laparotomy, fenestrated duodenal membrane was observed in all patients - preampullary in three patients and postampullary in three patients. Lateral duodenotomy, web excision and transverse closure was done in all six patients. The postoperative period was uneventful in all patients and mean duration of hospital stay was 9 days. Conclusion: Fenestrated duodenal webs present a diagnostic challenge to the paediatric surgeons because of delayed and variable clinical presentation. A modification of the present classification of DA has been proposed in this study which would help in better reporting of epidemiology and aid in early diagnosis of this congenital anomaly.

BISHOP-KOOP OSTOMY REVISITED: A "TEST-DRIVE" INTESTINAL DIVERSION FOR CHILDREN WITH SUSPECTED BOWEL DYSMOTILITY.

BACKGROUND: Bishop-Koop ileostomy has been widely used in pediatric patients with the intention of including as much bowel as possible in the intestinal transit early in the management of children with meconium ileus and intestinal atresia. In recent years, we have been using it as an alternative to test the distal bowel function before closure of a previously constructed ostomy in selected children with questionable distal bowel motility. AIMS: The aim of this study was to present our experience with this alternative use of the Bishop-Koop ostomy. METHODS: This is a cross-sectional retrospective review of hospital records, combined with a comprehensive literature review. RESULTS: Seven children were included: five had suspected aganglionosis, one had gastroschisis complicated with ileal atresia, and one had a colonic stricture secondary to necrotizing enterocolitis. In this short series of patients, motility of the distal bowel was correctly assessed in six patients and partially correctly assessed in one patient. One patient did not pass stools per anus after the Bishop-Koop, and he was later confirmed to have Hirschsprung disease. Four patients resumed normal evacuation pattern after closure of the Bishop-Koop. One patient had a Bishop-Koop colostomy because of recurrent enterocolitis after a transanal pull-through. Although he evacuated normally while having the colostomy, the diarrhea recurred after the ostomy was closed. An additional patient, with a severe behavioral problem, did not evacuate per anus after her colostomy was transformed in a Bishop-Koop-type ostomy, despite the apparent presence of normal ganglia in the bowel wall. CONCLUSIONS: Data from the present series allow us to affirm that Bishop-Koop-type ostomy is a safe and efficient procedure that can be used to assess distal bowel function before a definitive transit reconstruction, in children with uncertain motility issues.

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.

Perioperative and Long-Term Outcome in Patients Treated for Jejunoileal Atresia.

OBJECTIVES: Jejunoileal atresia is a common cause of neonatal intestinal obstruction. Results of long-term outcome are very limited. The aim of this study was to describe perioperative and postoperative outcome, and to evaluate long-term gastrointestinal quality of life (QoL) in patients treated for jejunoileal atresia. METHODS: We conducted a combined retrospective and cross-sectional observational study of patients treated for jejunoileal atresia during 2001-2019. Perioperative data were registered, and complications were classified according to the Clavien-Dindo classification. To evaluate long-term clinical status and QoL, the PedsQL Gastrointestinal Symptoms Scales questionnaire and a self-designed questionnaire were used. Approval from the Data Protection Office was obtained. RESULTS: Seventy patients were included in the retrospective part of the study. Of these, 70% got a primary anastomosis. Concomitant anomalies were registered in 46%. Median length of stay was 28 (5-140) days. Early (<28 days) complication rate was 66%. Early abdominal surgical reinterventions were performed in 11%, most frequently due to anastomotic leak. Late (>28 days) abdominal surgical reinterventions were performed in 21%, and most commonly for bowel obstruction. Overall mortality rate was 4%. Thirty-two patients with median 8 (2-19) years returned the questionnaires. Overall gastrointestinal QoL was good. However, concomitant gastrointestinal anomalies were associated with impaired outcome. Embarrassment of the scar was reported in 38%. Three (9%) patients used medication (laxatives, proton pump inhibitor) for gastrointestinal symptoms. CONCLUSIONS: Jejunoileal atresia is associated with significant morbidity during initial treatment. Despite this, the majority of the patients have excellent long-term outcomes.