Intestinal Atresia Research Articles

Congenital pouch colon with colonic atresia- An unusual embryological association: A case report

Background: Congenital pouch colon (CPC) is a rare variant of high anorectal malformation. More and more varied associations of CPC with other entities are being added to the literature. Case presentation: A 1-day-old male baby presented to the emergency room with marked abdominal distension and absent anal opening. On exploration, the baby was found to have CPC (Type 2) with colonic atresia. This association has not been reported in English literature. Conclusion: This is a report of a case of type 2 CPC with colonic atresia. To the best of our knowledge, such an association has not been reported so far. The final embryological outcome is dictated both by the topography and timing of vascular insult.

A Comprehensive Review of Intestinal Atresias

A Comprehensive Review of Intestinal Atresias

Gastroschisis with intestinal atresia leads to longer hospitalization and poor feeding outcomes.

Compare in-hospital outcomes in gastroschisis with intestinal atresia versus simple gastroschisis (GS) using a national database. The Children's Hospitals Neonatal Database identified infants with gastroschisis from 2010 to 2016. 2078 patients with gastroschisis were included: 183 (8.8%) with co-existing intestinal atresia, 1713 (82.4%) with simple gastroschisis, the remainder with complex gastroschisis without atresia. Length of hospitalization was longer for those with atresia, and yielded higher rates of mortality, medical NEC, and intestinal perforation. They began enteral feedings later, were less likely to initiate feeds orally, and reached full feedings later. They were less likely to be receiving any maternal breast milk or breastfeeding at discharge and more likely than simple gastroschisis to be discharged with a feeding tube. A large multicenter cohort showed gastroschisis with atresia results in worse outcomes and complications, including necrotizing enterocolitis, feeding delays, and enteral feeding tube dependence.

Late-Onset Presentation of the Jejunal Web: A Case Report and Review of the Literature

Introduction: The congenital intestinal web is a rare cause of small bowel obstruction classified as type I intestinal atresia. The second portion of the duodenum is the most prevalent site for these webs, whereas the jejunal web is rare. Case presentation: In this study, we present a very uncommon late presentation of the jejunal web in an 18-month-old girl complaining of chronic recurrent vomiting, constipation, and failure to thrive (FTT). After thorough investigations, the diagnosis was confirmed by the small bowel follow-through. The surgical procedure resulted in a significant improvement in patient’s quality of life, given the subsequent increase in her weight and height. Conclusions: Vomiting that occurs at any age is pathologic and requires careful history taking thorough clinical examination and evaluation, especially in patients treated with proton pump inhibitors such as omeprazole for a long time with a diagnosis of gastroesophageal reflux disease (GERD) without any response. In these patients, gastrointestinal abnormalities, including complete or incomplete obstructions such as malrotation and web, should be made in the differential diagnosis.

Death of Paediatric Surgical Patients in NICU of CMH, Dhaka

Introduction: The purpose of this study was to explore clinical characteristics and primary surgical diagnosis associated with in-hospital death in pediatric surgical patients admitted to the neonatal intensive care unit (NICU) of Combined Miilitary Hospital (CMH), Dhaka. Aim: To explore the clinical factors associated with in-hospital death in paediatric surgical patients admitted to the NICU over a period of 4 years in CMH Dhaka. Methods: This retrospective study includes all patients admitted to NICU of CMH Dhaka for paediatric surgical diseases between July 2013 and December 2017. Data analyzed to asses factors associated with in-hospital death. Results: A total of 72 cases were included and 61(84.7%) underwent surgery. Fifteen patients (20.8%) died while hospitalized in the NICU. The 5 most common surgical diagnoses were Anorectal Malformation, Intestinal Atresia/ Stenosis, Hirschsprungs disease, Intestinal perforation and meconium related obstruction. Esophageal atresia, necrotizing entero-colitis cases had the highest mortality rate. Conclusion: This study describes EA, NEC, Low birth weight (LBW), prematurity and caesarean delivery associated with significant number of deaths of surgical patients in NICU. Novel approaches for these conditions are required to improve the survival. JAFMC Bangladesh. Vol 15, No 1 (June) 2020: 26-28

Multiple Small Bowel Atresia: Resection or Conservation?

Multiple small bowel atresia is a relatively uncommon cause of intestinal obstruction in neonates. About one-third of the neonatal intestinal obstruction is caused by intestinal atresia. As suggested by Louw and Barnard, the vascular accident in utero is the principal reason, resulting in bowel atresia.1 The condition presents soon after birth, with bilious vomiting and abdominal distension, requiring emergency surgical intervention. A subtype of small bowel atresia (type IV), which presents with involvement of multiple segments, is unique due to the difficulties in management, accompanied by a variety of postoperative morbidities, which are highlighted in this case. The choice lies between resection of all the atretic segments with resultant single anastomosis and conservation with anastomoses of multiple small bowel segments involving meticulous repair by fine sutures, taking care to maintain luminal patency. The dilemma of whether to conserve the atretic segments of bowel, which is a time consuming procedure but preserves the bowel length or to proceed with excision of the atretic bowel segments, accepting the likelihood of short bowel, in order to provide a quick, safe and stable single anastomosis in a timely manner, is a valid challenge. The background of limited resource setting also need to be taken into consideration. Key Words: Atresia, Neonates, Bowel segments, Anastomosis.

Intussusception and Jejunal Atresia Caused by an Ectopic Pancreas in a Newborn

Intussusception and Jejunal Atresia Caused by an Ectopic Pancreas in a Newborn

Management and Outcomes of Malformations Associated with Intestinal Malrotations in Newborns: About 5 Cases

Bowel malrotation is an abnormality in the rotation of the primary intestinal loop during embryonic life. It is a severe abnormality exposing the patient to fatal complications. We report here a series of five patients presenting digestive malrotation with various malformative associations. The first three cases are multiple intestinal atresia on incomplete common mesentery. They underwent an intestinal anastomosis: end-lateral duodeno-ileal for one and end-to-end jejuno-jejunal for the other two with Ladd procedure. In the latter two cases, the omphalocele was the associated malformation; complications such as rupture of the omphalocele with intestinal perforation for one and occlusion by strangulation at the level of the neck for the other were recorded. Ileal resection removing the perforation with end-to-end ileo-ileal anastomosis was performed for the first, while reduction of strangulation at the origin of the occlusion was sufficient for the second. The LADD procedure was the complementary gesture in all cases. The outcome was favorable in 3 cases; two of the newborns who had jejunal atresia died, one on day 5 in a picture of acute respiratory distress, the other on day 6 in a picture of disseminated intravascular coagulation.

Ultrasonographic Imaging of the Fetal Duodenal Tract.

To investigate whether fetal duodenal tract sections can be visualized in the prenatal ultrasonographic examination. This study was designed in cross-section. Healthy singleton pregnant women who applied to the perinatology outpatient clinic for second-level ultrasound scanning between September 2020 and February 2021 were included in the study. Demographic information of the participants was obtained and an ultrasound scan was performed. The fetal duodenal tract was evaluated in three sections, including the pylorus. The fetal duodenal tract was differentiated from adjacent organs by its anatomical location, hyperechoic nature, and presence of fluid in the lumen. A total of 278 eligible participants between 18 and 22 weeks of gestation were evaluated. While the fetal pylorus was closed in 76.6% of the participants, it was open in 23.4%. Duodenum pars superior, pars descendens, and pars inferior imaging rates were 99.3%, 98.2%, and 95.7%, respectively. It was possible to distinguish these parts from neighboring organs by 99.6%, 100%, and 100%, respectively. While the first, second, and third parts of the duodenum were observed as solid in 42.0%, 58.2%, and 52.2%, respectively, 57.9%, 41.7%, and 47.7% had fluid in the lumen. The fetal duodenal tract can be viewed with prenatal ultrasonography in pregnant women who are not in a dorsoanterior position. This may make an additional contribution to the diagnosis of duodenal obstructions, which is the most common cause of intestinal atresia in prenatal screening.

Congenital band causing small bowel obstruction in a newborn

Intestinal atresia refers to a complete obstruction of the bowel lumen. Anomalous congenital bands are rarely found as causes of intestinal obstruction in children. Most congenital bands occur in the small intestine. This report presents 1-day-old newborn girl with intestinal atresia due to a congenital band. We report a case of a full-term female newborn who presented with symptoms and signs of intestinal obstruction. She was in good condition with bilious residue in the gastric tube, abdominal distension and compressible wall. Anus was in normal position. Introduction of a rectal tube found no obstacle and brought no meconium. X-ray examinations have shown digestive obstruction. Surgical intervention was performed. There was a congenital band identified between a dilated ileal loop and the mesentery causing compression and ileal atresia. Surgical procedures consisted of section of congenital band and resection of dilated aperistaltic small bowel, cecum and appendix. We then proceeded to end-to-end ileocolic anastomosis. The patient recovered without complications. Congenital bands are a rare cause of intestinal atresia in neonate. This entity is very uncommon and difficult to identify preoperatively. Surgery is necessary and diagnosis of intestinal atresia due to congenital band is then confirmed.

Intrauterine midgut volvulus as a rare cause of intestinal obstruction: a case report

BackgroundIntrauterine midgut volvulus is a very rare, life-threatening condition, and prenatal diagnosis is difficult. In this article, we present a case of midgut volvulus followed by a pre-diagnosis of antenatal jejunal atresia.Case presentationA 1-day-old Turkish male baby, who was followed with a diagnosis of antenatal jejunal atresia, with a birth weight of 3600 g, delivered by cesarean section at 38 weeks of gestation from a 19-year-old mother in her fourth pregnancy, was taken to the newborn intensive care unit. The patient underwent surgery on the postnatal first day with a preliminary diagnosis of jejunal atresia. It was observed that the small intestine was rotated two full cycles from the mesenteric root. Bowel blood circulation was good. Volvulus was untwisted. There was malrotation. Ladd's procedure was performed. The baby was discharged on the seventh postoperative day with full oral feeding. The patient is still in the first postoperative year and follow-up has been uneventful.ConclusionIntrauterine midgut volvulus has been associated with high mortality in the literature. Differential diagnosis of midgut volvulus in patients with antenatal intestinal obstruction, close prenatal follow-up, appropriate delivery and timing of surgical intervention may significantly reduce morbidity and mortality.

Rare case report of anorectal malformation and intestinal atresia.

IntroductionIntestinal malformations, such as intestinal atresia, malrotation, and anorectal malformation, are rare events, but concurrent intestinal atresia and anorectal malformation are rare in combination. These anomalies have similar in utero and perinatal symptoms, which can make the diagnosis of both defects prenatally challenging.Presentation of caseWe present a case of a male infant with a prenatal diagnosis of truncus arteriosus who on a 32-week routine prenatal ultrasound was suspected to have an intestinal malformation. On day of life one, the patient was taken to the operating room and found to have both type IIIa ileal atresia and anorectal malformation with normal bowel in between. A complete work up for vertebral defects, anorectal malformation, cardiac septal defects, esophageal atresia, renal anomalies, and radial limb defects (VACTERL) anomalies did not reveal additional anomalies.DiscussionIn this case, prenatal information supported intestinal obstruction, and the rare combination of both intestinal atresia and anorectal malformation proved surprising and interesting.ConclusionWe suggest providers be aware of the potential of multiple alimentary tract malformations to improve operative preparation and reduce the morbidity or mortality risk from repeat procedures when possible.

Modification of the association between diabetes and birth defects by obesity, National Birth Defects Prevention Study, 1997-2011.

Maternal pregestational diabetes and obesity are risk factors for birth defects. Diabetes and obesity often occur together; it is unclear whether their co-occurrence compounds birth defect risk. We analyzed 1997-2011 data on 29,671 cases and 10,963 controls from the National Birth Defects Prevention Study, a multisite case-control study. Mothers self-reported height, pregestational weight, and diabetes (pregestational and gestational; analyzed separately). We created four exposure groups: no obesity or diabetes (referent), obesity only, diabetes only, and both obesity and diabetes. We estimated odds ratios (ORs) using logistic regression and the relative excess risk due to interaction (RERI). Among mothers with pregestational obesity without diabetes, modest associations (OR range: 1.1-1.5) were observed for neural tube defects, small intestinal atresia, anorectal atresia, renal agenesis/hypoplasia, omphalocele, and several congenital heart defects. Pregestational diabetes, regardless of obesity, was strongly associated with most birth defects (OR range: 2.0-75.9). Gestational diabetes and obesity had a stronger association than for obesity alone and the RERI (in parentheses) suggested additive interaction for hydrocephaly (1.2; 95% confidence interval [CI]: -0.1, 2.5), tetralogy of Fallot (0.9; 95% CI: -0.01, 1.8), atrioventricular septal defect (1.1; 95% CI: -0.1, 2.3), hypoplastic left heart syndrome (1.1; 95% CI: -0.2, 2.4), and atrial septal defect secundum or not otherwise specified (1.0; 95% CI: 0.3, 1.6; only statistically significant RERI). Our results do not support a synergistic relationship between obesity and diabetes for most birth defects examined. However, there are opportunities for prevention by reducing obesity and improving glycemic control among women with pregestational diabetes before conception.

Neonatal intestinal obstruction: Type I - Septal atresia of distal small intestine

Congenital Intestinal atresia is common cause of neonatal intestinal obstructions. Small intestinal atresia accounts for majority of cases than colonic atresia. In small intestinal atresia Type I is rare form. The plausible etiology is mainly from intra-uterine mesenteric vascular accident and the extent depends on the time and the site of development of obstruction. A 2-day-old baby with history of maternal antenatal scan suspecting of Hirschsprung’s disease presented with clinical signs of lower intestinal obstruction. Barium enema showed features of distal small intestine obstruction for which baby underwent surgery with resection of obstructed segment with end to end anatomises was done. Type I small intestinal atresia was confirmed on histological examination. Thus intestinal obstruction is an emergency encountered in newborn infants requiring early accurate diagnosis by radio imaging modalities and histopathological evaluation. Keywords: Intestinal obstruction, Small intestine atresia, Histopathology.

Gut lumen formation defect can cause intestinal atresia: evidence from histological studies of human embryos and intestinal atresia septum

Intestinal atresia (IA), a common cause of neonatal intestinal obstruction, is a developmental defect, which disrupts the luminal continuity of the intestine. Here, we investigated (i) the process of lumen formation in human embryos; and (ii) how a defective lumen formation led to IA. We performed histological and histochemical study on 6-10 gestation week human embryos and on IA septal regions. To investigate the topology of embryonic intestine development, we conducted 3D reconstruction. We showed that a 6-7th gestation week embryonic gut has no lumen, but filled with mesenchyme cells and vacuoles of a monolayer of epithelial cells. A narrow gut lumen was formed by gestation week-9, the gut was filled with numerous vacuoles of different sizes, some vacuoles were merging with the developing embryonic gut wall. At gestation week-10, a prominent lumen was developed, only few vacuoles were present and were merging with the intestine wall. At IA septal regions, vacuoles were located in the submucous layer, covered by a single layer of epithelium without glandular structure, and surrounded with fibrous tissue. The mucosal epithelium was developed with lamina propria and basement membrane, but the submucosa and the longitudinal smooth muscle layers were not properly developed. Hence, the vacuoles in IA septum could represent a remnant of vacuoles of embryonic gut. In conclusion, the fusion of vacuoles with the developing intestine wall associates with the disappearance of vacuoles and gut lumen formation in human embryos, and perturbation of these developmental events could lead to IA.

A Case of Idiopathic Diffuse Pneumoperitoneum Presented with Neonatal Abdominal Color Change

Background: Necrotizing enterocolitis is the leading cause of intestinal perforation and pneumoperitoneum in neonates. In addition, neonatal pneumoperitoneum includes several conditions requiring surgery, such as gastric and duodenal perforation, intestinal atresia, volvulus, meconium ileus, and Hirschsprungג��s disease. A type of pneumoperitoneum in which intra-abdominal free air is detected through direct X-ray; however, no cause is found clinically and radiologically during the surgery is named ג��benign pneumoperitoneumג��. Case report: This case report presents a 5-day-old male newborn who is presented with complaints of abdominal color change and vomiting and is diagnosed with Idiopathic Benign Pneumoperitoneum (IBP) during the operation. Neonatal pneumoperitoneum usually occurs as a result of perforation and requires emergency surgery. Rare cases of IBP should also be kept in mind. Unnecessary surgery can be prevented by establishing a differential diagnosis with clinical, laboratory, and imaging methods.  Conclusion: This case demonstrated that laparotomy is not a true routine in neonates with IBP if a timely diagnosis is established.

Особливості клінічних проявів атрезії тонкої кишки в перші хвилини життя новонароджених дітей

Clinical signs of small bowel atresia, which are widely described in the literature, usually develop after the first day of life. Studies on the clinical manifestations of intestinal atresia in newborns in the first minutes of their lives are still lacking. Purpose — to identify clinical signs of different types of intestinal atresia which can be detected by physical examination of the newborn, immediately after birth. Materials and methods. A retrospective study of 77 newborns with congenital small intestinal obstruction (SIO) who underwent surgical management at the Neonatal Surgery Center for Congenital Malformations and their Rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after acad. E.M. Lukyanova of the NAMS of Ukraine» was conducted. The clinical manifestations of SIO in patients with duodenal (n=44), jejunal (n=14) and ileal (n=19) atresia were studied and a statistical analysis was performed. Results. There was no significant difference in the volume of gastric stasis, abdominal bloating and meconium discharge in patients with different types of duodenal obstruction (P>0.05). It was found that patients with duodenal and jejunal obstruction, immediately after birth, have a significantly higher volume of gastric stasis (38.0±23.9 ml and 42.3±20.0 ml, respectively) compared with patients who had ileal atresia (14.7±12.8 ml), (P<0.05). There were no significant differences in the incidence of abdominal bloating and meconium discharge (P>0.05). Conclusions. Duodenal and jejunal atresia are associated with excessive gastrostasis, but meconium discharge may be normal. Abdominal bloating always occurs in duodenal atresia and may be absent in jejunal and ileal atresia. In patients with ileal atresia, there may be no signs of intestinal obstruction immediately after birth. Level of evidence. Level III. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of an participating institution. The informed consent of the child's parents was obtained from the studies. No conflict of interest was declared by the authors. Key words: atresia, small intestine, clinical manifestations, newborns.

Benchmarking utilization, length of stay, and complications following minimally invasive repair of major congenital anomalies.

Infants with newborn congenital anomalies are increasingly undergoing minimally invasive surgical (MIS) repair. Currently available data on outcomes are limited. This study provides national estimates for length of stay and 30-day complications following MIS for congenital anomalies. Using the ACS-NSQIP Pediatric (2013-2018), a retrospective analysis of MIS for congenital anomalies was performed. MIS repairs for the following diagnoses were included: pyloric stenosis (PS), congenital lung lesion (LL), mediastinal mass (MM), congenital malrotation (CM), anorectal malformation (ARM), Hirschsprung's disease (HD), congenital diaphragmatic hernia (CDH), tracheoesophageal fistula (TEF), biliary atresia/choledochal cyst (HB), and intestinal atresia (IA). Postoperative LOS (pLOS) and complication rates were examined using multivariable analysis for risk factors after categorizing surgeries by complexity of care related to congenital anomaly: Simple (PS), Complex Group 1 (LL, MM, CM, and ARM), and Complex Group 2 (HD, CDH, TEF, HB, and IA). Across 10 anomalies, 8,326 repairs were performed using an MIS approach. Procedure-specific median postoperative LOS (75th-percentile, 90th-percentile) for PS was 1day (1, 3); LL was 3 (4, 7); MM was 2 (3, 5); CM was 4 (7, 14); ARM was 3 (5, 8); HD was 5 (8, 12); CDH was 8 (18, 31); HB was 5 (8, 12); TEF was 20 (31, 53); and IA was 17 (25, 40). The overall surgical complication rates (95% CI) were: PS, 5.1% (4.7%-5.6%); LL, 14.2% (12.3-16.4); MM, 8.4% (6.4-11.0); CM, 14.6% (11.9-17.9); ARM, 12.0% (7.1-19.5); HD, 22.1% (19.5-25.0); CDH, 21.1% (17.1-25.6); HB, 20.6% (13.7-29.7); TEF, 36% (27.5-45.5); and IA, 28.6% (19.3-40.1). Risk factors for increased pLOS and complications varied by procedure category and included patient-level and admission characteristics. This study provides national benchmarks and risk factors for expected postoperative LOS and 30-day complications following MIS for congenital anomalies.

Pattern and outcome of neonatal gastrointestinal surgical emergencies: A prospective analysis.

Objectives: This study is aimed at determing the pattern and treatment outcome of neonatal gastrointestinal surgical emergencies. Study Design: Prospective Descriptive study. Setting: DHQ Teaching Hospital Sahiwal. Period: January 2018 to December 2019. Material & Methods: All neonates (<28 days old) who underwent surgery for acute gastrointestinal emergency during the study period were included. Newborns more than 28 days and diseases such as esophageal atresia, esophageal atresia with trachea-esophageal fistula, diaphragmatic hernia, omphalocele, gastroschisis and infantile hypertophic pyloric stenosis were not included in the study. Results: A total of 104 cases of neonatal gastrointestinal surgical emergency were included in the study. The most common cause was anorectal malformation 37(35.57%), followed by intestinal atresia 26(25.0%), Hirschsprung disease 13(12.50%), necrotizing enterocolitis 9(8.65%), intestinal malrotation 8(7.69%), meconium ileus 7(6.73%) and others 4(3.84%). Males were 59(56.73%), females 45(43.26%) and male to female ratio 1.3:1. Mean weight was 2.62kg, ranging from 1.5 to 4.4kg. Post-operative complications were septicemia 26(25.0%), wound infection 17(16.34%), respiratory problems 19(18.26%). Mortality rate in necrotizing enterocolitis was 55.55%, meconium ileus 42.85% and intestinal atresia 38.46%. Mortality rate in premature neonates was 48.0% and mature 12.65%. Overall mortality rate was 21.15%. Conclusions: Anorectal malformation is the commonest cause of neonatal gastrointestinal surgical emergency. Necrotizing enterocolitis, intestinal atresia and meconium ileus are the neonatal surgical diseases with high mortality rate. Surgical outcome depends on the complexity of the disease. Sepsis, late presentation, prematurity and low birth weight are significant contributory factors for high morbidity and mortality.

How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review

IntroductionMultiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. MethodsAll cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. ResultsSeven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1–2) resections–anastomoses (RA) and 6 (1–10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15–25 cm). Median time to full oral feed was 46 days (14–626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2–8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. ConclusionBowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.