Ileal Atresia Research Articles

Hereditary Multiple Intestinal Atresia With a Novel TTC7A Pathogenic Variant: Gastrointestinal Manifestations in Two Cases.

Hereditary multiple intestinal atresia (HMIA) with TTC7A mutation is caused by homozygous or compound heterozygous TTC7A gene mutation. It is characterized by multiple small and large intestinal atresias and/or stenoses. TTC7A mutation is described in some patients with inflammatory bowel disease and mild-severe forms of severe combined immunodeficiency without intestinal atresia or stenosis. We present 2 cases of intestinal atresia and documented TTC7A mutation with a novel variant. Both cases had different clinical and pathological manifestations. The first case is a male infant born at 35 weeks of gestation with failure to pass meconium. Intestinal biopsy reveals apoptotic enteropathy with villous atrophy and increased mucosal eosinophils. The second case is referred at birth for antenatally detected umbilical hernia, polyhydramnios and possible upper intestinal obstruction. The resected specimen reveals ileal atresia with partial villous atrophy, decreased number of lamina propria inflammatory cells and absence of plasma cells. In conclusion, these cases reflect an emerging TTC7A pathogenic variant with different histological manifestations and leads to characterization as immune dysregulation disorder. There is a need to differentiate TTC7A mutation associated ones from cases labeled as very early onset IBD and rule out other hereditary immunodeficiencies.

“Keyboard sign” and “coffee bean sign” in the prenatal diagnosis of ileal atresia: A case report

BACKGROUND Ileal atresia is a congenital abnormality where there is significant stenosis or complete absence of a portion of the ileum. The overall diagnostic accuracy of prenatal ultrasound in detecting jejunal and ileal atresia is low. We report a case of ileal atresia diagnosed prenatally by ultrasound examination with the “keyboard sign” and “coffee bean sign”. CASE SUMMARY We report a case of ileal atresia diagnosed in utero at 31 weeks' of gestation. Prenatal ultrasound examination revealed two rows of intestines arranged in an ‘S’ shape in the middle abdomen. The inner diameters were 1.7 cm and 1.6 cm, respectively. A typical “keyboard sign” was observed. The intestine canal behind the “keyboard sign” showed an irregular strong echo. There was no normal intestinal wall structure, showing a typical “coffee bean sign”. Termination of the pregnancy and autopsy findings confirmed the diagnosis. CONCLUSION The prenatal diagnosis of ileal atresia is difficult. The sonographic features of the “keyboard sign” and “coffee bean sign” are helpful in diagnosing the location of congenital jejunal and ileal atresia.

A rare case of polysplenia syndrome associated with atrioventricular canal defect and type four ileal atresia in a preterm neonate: A case report and literature review

Antenatal diagnosis of heterotaxy syndrome is only the tip of the iceberg. These neonates can have a myriad of multi-system anomalies, each of which requires anticipation and an individualized management plan. The spectrum of heterotaxy syndrome ranges from incidentally detected innocuous anomalies to major life-threatening congenital malformations such as atrioventricular canal defects and bowel atresia. Neonatal sepsis tends to be more fulminant and catastrophic in these neonates with complex anomalies and poor outcomes that necessitates better-informed antenatal counseling regarding the termination of such pregnancies.

Neonatal Intestinal Obstruction: Etiology, Management, and Outcomes in a Tertiary Care Center.

Intestinal obstruction in neonates remains a critical medical emergency in the field of pediatric surgery. Clinical conditions often experience a sudden deterioration in their appearance. Multiple factors contribute to unfavorable clinical outcomes in underdeveloped nations. The study was conducted to identify the etiology, management, and outcomes of neonatal intestinal obstruction at a specialized medical facility. This retrospective study included 168 newborns who had to be operated on in the neonatal intensive care unit between 2021 and 2023 due to intestinal obstruction. The clinical and demographic characteristics of the infants, final diagnosis, surgical complications, and mortality rate were documented. In addition, the relationship between risk factors such as birth weight, gestational age, length of surgery, and postoperative problems was evaluated. The majority of neonatal intestinal obstruction occurred within seven days of birth, with 8-15 days being the second most common. Most babies were born at full term (53.57%) and weighed 2 kg or more (75%). In newborns in our region, duodenal, ileal, jejunal, and colonic atresias were found to be the most common causes of neonatal intestinal obstruction that requires surgery. The study detected 45 postoperative problems, 26.79% of the total. Out of 168 patients, twelve (7.14%) had septicemia, seven (4.17%) had anastomotic leak, seven (4.17%) had aspiration pneumonitis, and two (1.19%) needed re-exploration. Overall mortality was 10.12%, with 17 patients dying. Moreover, 119 patients (70.83%) survived without issues, while 32 (19.05%) survived with complications. Our findings emphasize the significance of promptly diagnosing, intervening, and implementing suitable management approaches to enhance outcomes for newborns with intestinal obstruction. Furthermore, it highlights valuable perspectives for healthcare professionals in enhancing care for this specific group of patients.

Ileal Atresia in Down Syndrome, A Rare Finding

A 37-week-old baby boy was born by cesarean section to a 40-yearold G7P6006 mother who tested positive for 21+ by noninvasive prenatal testing. At delivery, the baby had features of Down Syndrome, including low-set ears, bilateral epicanthal folds, a flat nasal bridge, a protruding tongue, a Simian crease on the right hand, an umbilical hernia, and excess skin on the nape of the neck. He presented with Apgar’s of 7 and 8. He was admitted to the NICU requiring CPAP due to respiratory distress, kept NPO, and started on IV fluids. When feeds were started at the end of the first day of life, he was noted to have episodes of bilious emesis, with abdominal distention, and failure to pass meconium till 36 hours of life.

A remarkable case of intrauterine intussusception, ileal atresia, and complicated meconium ileus: A case report

Background: Intrauterine intussusception, although very rare (<2%), is a potential cause of intestinal atresia. It usually manifests as bowel obstruction during the first hours or days of life. Case Presentation: We report a case with fetal ascites prompting early Cesarean section, with subsequent percutaneous abdominal drainage and laparotomy. Intraoperative findings showed meconium peritonitis and type IIIa ileal atresia with intussusception of the distal part, with the necrosed intussusceptum likely causing perforation and ileal atresia; in addition, the distal ileum demonstrated signs of meconium ileus. After ileostomy creation, the postoperative course was uneventful. Cystic fibrosis was excluded via a sweat test. Conclusion: Prompt diagnosis and management of neonatal intestinal obstruction secondary to the unusual combination of fetal ascites, intussusception, complicated meconium ileus, and ileal atresia results in a favorable outcome.

Neonatal Human Enteroids Take Up Human Milk Extracellular Vesicles That Survive Neonatal Human Digestion

Background: Human milk (HM) is the ideal infant nutrition and reduces infant death and disease. HM contains thousands of bioactive components, including extracellular vesicles (HMEVs). HMEVs are lipid bilayer-encased nanoparticles released from mammary epithelial cells that carry biological cargo to the infant. Pre-clinical models suggest that HMEVs may enhance intestinal function and limit inflammation; however, it is unknown if HMEVs or their cargo survive neonatal human digestion. This limits the ability to leverage HMEV cargo as additives to infant nutrition or as therapeutics. This study aims to develop a neonatal enteroid model and EV isolation pipeline to study HMEV-intestinal epithelial cell (IEC) interactions with the ultimate goal of therapy development. Hypothesis: HMEVs survive in vivo neonatal digestion to be taken up by infant IECs. Methods: All studies were conducted under approved IRB protocols. Neonatal intestinal contents (digesta) were collected after gastric feeding from post-pyloric tubes. HMEVs were isolated from raw and pasteurized human milk and digesta (n=3 each) by density-gradient ultracentrifugation following two-step skimming, acid precipitation of caseins, and multi-step filtration. EVs were validated by electron microscopy, nanoparticle tracking analysis (NTA), and western blotting. HMEV sub-populations were examined by super-resolution microscopy. HMEV uptake was tested using apical-out neonatal human enteroids isolated from a 2-month-old patient undergoing surgery for ileal atresia and validated by qPCR for proliferation and differentiation markers (mean ± SEM). Enteroid uptake was assessed with CMTPX dye-labeled-HMEVs, dynamin inhibitor, and confocal microscopy. Results: Our HMEV isolation pipeline reliably isolates HMEVs exhibiting classic EV morphology and enriched in extracellular vesicle markers CD81 and CD9, but depleted of b-casein and lactalbumin from HM and digesta. NTA data indicates that only 17.8 ± 2.9% (p>0.0001) of input HMEVs survive to reach the neonatal intestine. Human mammary gland-derived protein BTN1A1 is present in digesta EVs. Neonatal apical-out human enteroids represent a less proliferative and more differentiated epithelium, e.g. down-regulated KI67 (0.05 ± 0.03-fold relative to basal-out, p<0.0001, n=4-6) and elevated CHGA (14.22 ± 9.30-fold relative to basal-out, p=0.0072, n=4-6) as measured by qRT-PCR and normalized to housekeeping gene RPLP0. Neonatal enteroids take up digested HMEVs largely via dynamin-mediated endocytosis, as measured by CMTPX dye intensity normalized to enteroid area using Fiji (2.05 x 105 ± 2.95 x 104 vs 5.38 x 104 ± 6.08 x 103, p<0.0001, n=62-115 enteroids, HMEV-treatment alone and HMEVs + dynamin inhibitor, respectively).Conclusion: Our data suggest that HMEVs survive to the neonatal intestine and can be absorbed by neonatal enteroids largely via dynamin-mediated endocytosis. These data are an important first step to leveraging HMEVs and their cargo to treat neonatal intestinal inflammation. Funding: NIH K01DK129401, NIH R01HD097367, USDA NIFA, Collins Medical Trust, Medical Research Foundation, OHSU Exploratory Research Seed Grant. This is the full abstract presented at the American Physiology Summit 2024 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Extremely low birth weight (ELBW) neonates for emergency surgery- A challenge for the Anesthesiologist: A case series

Background: Extremely Low Birth Weight (ELBW) newborns, defined as those with a birth weight of 1000 grams or less, present challenging anesthetic scenarios. Emergency surgeries in these infants elevate their vulnerability to various complications. Case Presentation: We present a case series involving 5 ELBW neonates who underwent emergency surgery. Three of them were at a post-conceptual age (PCA) of ≥ 33 weeks and underwent procedures for congenital birth defects, including esophageal atresia, ileal atresia, and gastroschisis. The remaining two neonates, with a PCA of 29-30 weeks, underwent surgery for necrotizing enterocolitis (NEC). Conclusion: The compromised physiology resulting from distinct disease processes and the underdeveloped systems of ELBW preterm neonates necessitate focused care and strategic anesthesia. This approach is crucial to mitigate morbidity and mortality risks in such vulnerable patients.

Peculiarities of the Clinical Picture of Hirschsprung’s Disease in Children of the First Year of Life Taking Into Account the Extent of Aganglionosis

The aim 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.
 Research materials and methods. Since 1980 up to 2021, at the pediatric surgery clinic of the National Medical University named after O.O. Bohomolets 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, there were examined and treated 483 children of the first year of life suffering from Hirschsprung’s disease.
 Results of the study. 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 them 39 (8.07%) patients had an atypical 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%), cleft of the hard and soft palate in 9 (1.86%) patients. 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%) patients, delayed physiological weight gain against the background of nutritional insufficiency with the development of hypotrophy in 327 (67.70%) patients, vomiting of stagnant gastric and intestinal contents in 417 (86.34%) patients. On the other hand, there occurred enterocolitis in 315 (65.22%) patients, toxic megacolon in 16 (3.31%) patients, and anemia of various degrees 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 determined: rectal form in 100 (20.70%) patients, rectosigmoid form – 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%) patients, and congenital cyst of the right ovary in 1 (0.21%) patient. The clinical course was affected by the concomitant malformations: incomplete bowel rotation in 10 (2.07%) patients and internal abdominal hernia in 2 (0.42%) patients.
 Conclusions. Clinical manifestations and course of HD 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%) patients.

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

PurposeIntestinal 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.MethodsA 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.ResultsA 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.ConclusionIncreased 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.

Analysis of the clinical diagnosis and treatment of fetal meconium peritonitis

Background The purpose of this study was to improve diagnostic and therapeutic standards by examining the clinical features, treatment, and prognosis of fetal meconium peritonitis (FMP), as well as the diagnostic efficacy of ultrasound for FMP. Methods The clinical data of 41 infants and pregnant women diagnosed with meconium peritonitis (MP) and treated at the Fujian Maternal and Child Health Hospital from January 2013 to January 2020 were analyzed retrospectively. Clinical data, imaging data, complications, treatment strategies, pregnancy outcomes, neonatal prognoses, and follow-up outcomes were all analyzed. Results The MP prenatal diagnosis rate was 56.1% (23/41), the neonatal surgery rate was 53.7% (22/41), and the survival rate was 85.4% (35/41). Intraperitoneal calcification (23 pregnant women, 56.1%), intestinal dilatation (13 pregnant women, 31.7%), peritoneal effusion (22 pregnant women, 53.7%), intraperitoneal pseudocyst (7 pregnant women, 17.1%), and polyhydramnios were diagnosed via prenatal ultrasound (18 pregnant women, 43.9%). Twenty-two pregnant women were assigned to the surgical treatment (operation) group, while 18 were assigned to the conservative treatment group. In the operation group, there were 9 cases of ileal atresia (40.9%), 7 cases of jejunal atresia (31.8%), 2 cases of atresia at the jejunum-ileum junction (9.1%), 2 cases of ileal perforation (9.1%), 1 case of ileal necrosis (4.5%), and 1 case of adhesive obstruction (4.5%). There was no statistically significant difference (p > .05) in the occurrence of various prenatal ultrasound findings by etiology. Conclusion Multiple prenatal ultrasound markers have been identified for MP. To improve the efficacy of newborn treatment for FMP and reduce neonatal mortality, dynamic monitoring of ultrasound image alterations and strengthened integrated perinatal management are necessary.

Challenges in managing Neonatal Ileal Atresia in limited-resource settings: a case report

Challenges in managing Neonatal Ileal Atresia in limited-resource settings: a case report

Fetal MRI Revealing a Rare Jejuno-Ileal Atresia: Apple Peel Intestinal Atresia

Apple peel intestinal atresia is a rare congenital malformation. Prenatal diagnosis can be done with obstetric ultrasound and confirmed by Fetal magnetic resonance imaging. We report a case of jejuno- ileal atresia (JIA) diagnosed during obstetric ultrasound showed small bowel dilation associated with hydramnios. Neonatal abdominal x-ray of the newborn confirmed the signs of intestinal occlusion. The aim of our work is to make a focus on this uncommon neonatal condition as well as the benefits of the antenatal diagnosis for adequate and timely management.

Congenital mesenteric defect with distal ileal atresia and segmental volvulus in a neonate: A case report

Intestinal volvulus is a rare condition that can be life-threatening. It often occurs after birth and requires emergent surgery. Most cases of volvulus in infants and fetuses are linked to intestinal malrotation. However, Intestinal volvulus may occur even if the midgut is in normal position, due to mesenteric defects. We present the case of a A 2-day-old, female phenotype neonate who presented with recurrent episodes of bilious vomiting. Clinical and radiological presentation was consistent with distal intestinal atresia. In Surgical exploration, we found a segmental volvulus of the terminal ileum complicating a congenital mesenteric defect and associated with a distal ileal atresia. An ileo-caecal resection was performed as well as a resection of the dilated distal bowel. Then an ileo-colic end-to-end anastomosis was done followed by closure of the mesenteric defect. Postoperative course was uneventful. Mesenteric defects can lead to segmental intestinal volvulus even when the midgut is normally rotated. Treatment should be tailored according to the anatomy and bowel status of the patient.

Intestinal volvulus in utero causing torsion of dilated bowel with ileal atresia: a case report

BackgroundIn utero intestinal volvulus with intestinal atresia is a rare and life-threatening condition that can cause torsion of the dilated bowel. The management and outcomes of this disease remain unclear.Case presentationA 19-year-old woman noticed a decrease in fetal motion at 35 weeks. Fetal ultrasound showed dilated fetal bowel and the whirlpool sign. The patient was referred to our hospital for an emergency cesarean section. The neonate’s abdomen was dark and severely distended, and a laparotomy was performed. Necrotic ileum and cord-type intestinal atresia (Type II) were observed in the dilated terminal ileum. The necrotic ileum was resected, and a second-look surgery was performed the following day. Then, we anastomosed the remaining intestine, and the total intestine length was 52 cm. There were no surgical complications, and the patient was discharged without requiring total parenteral nutrition or fluid infusion. The patient’s height and weight were within the − 2 standard deviation range of the growth curve at 5 months.ConclusionsEmergency and appropriate management of intestinal volvulus in utero causing torsion of the dilated bowel resulted in good outcomes in a patient with intestinal atresia. Perinatal physicians should be aware of this emergency condition and plan their treatment approach accordingly.

Ileal atresia with intraluminal Meckel's diverticulum

Ileal atresia with intraluminal Meckel's diverticulum

Retrospective Analysis of Neonatal Surgery at Tottori University over the Past Ten Years.

In recent years, the number of neonatal surgeries has been on the rise despite the decline in the number of births, and we examined the actual trends and problems at Tottori University Hospital located in the Sanin region. Medical records were retrospectively searched for patients who underwent major surgery during the neonatal period (within 30 days of age) at the Tottori University Hospital over the past 10 years (Jan. 2011 to Dec. 2020). Sixty-five cases were included. Early birth infants (< 37 gestational weeks) comprised 15 cases (23%) and low birth weight (< 2500 g) infants involved 27 cases (42%). In the latter half (2016-2020), early birth and low birth weight infants were significantly less than in the first half (2011-2015). The common diseases were anorectal malformation (14 cases), esophageal atresia (10), duodenal atresia (10), and diaphragmatic hernia (9). Prenatal diagnosis was obtained in 26 cases (40%), with high diagnostic rate obtained in duodenal atresia (100%), abdominal wall defect (100%), ileal atresia (75%), meconium peritonitis (67%), and diaphragmatic hernia (67%). Fifty-five cases (85%) were operated on within 7 days of age. Other major malformations were associated in 23 cases (35%). There were 6 deaths (9%), of which 3 cases were low birth weight infants with gastrointestinal perforation, 2 cases with severe chromosomal abnormalities (esophageal atresia, omphalocele), and 1 case with diaphragmatic hernia with severe pulmonary hypertension. Home medical care has been required with gastrostomy tube in 2 cases. Neonatal surgery at Tottori University has been well performed as required with acceptable results along with the progression of other perinatal care. However, further investigation for improvements in premature delivery or organ hypoplasia may be required.

Association of biliary atresia with jejuno-ileal atresia: Case report

Jejuno‐ileal atresia (JIA) is a congenital anomaly characterized clinically by bilious vomiting and abdominal distension. The incidence of JIA is approximately between 1 in 5000 live births in different parts of the world. It occurs equally in males and females, and about one in three of affected infants is premature. It has been associated with various congenital anomalies but the association of JIA with biliary atresia is extremely rare. We herein present a case of ileal atresia associated with biliary atresia. The patient was a boy who was born at 42 weeks of gestation with APGAR score 9/10 and no reported abnormality during antenatal follow-up. He was operated initially for intestinal atresia then was operated for kasai PE. He tolerated both procedure well. We support developmental etiology of Biliary Atresia rather than congenital etiology. Kasai portoenterostomy is bridge for liver transplant. Patients with BA should have better prognosis when operated early.

The Outcome of Different Surgical Conditions in Neonates at A Tertiary Care Hospital: A Cross-Sectional Study

Aim: To determine the outcome of different surgical conditions in neonates at a tertiary care hospital Study design: A cross-sectional study Place and Duration: Department of Pediatric Surgery Chandka Medical College Children’s hospital Larkana from Nov 2020 to Nov 2021 Methodology: All the neonates with surgical conditions were admitted through emergency and OPD. The variables were age, gender, diagnosis, surgery performed, postoperative course and complications if any. Patients soon after birth to 28 days of age with neonatal surgical conditions were enrolled in the study. The diagnosis was established either with pre-operative, intra-operative or post-operatively with relevant clinical, radiological, biochemical and histopathological findings described in standard textbooks of paediatric and neonatal surgery Results: A total of 208 patients were managed. The age range was 1 hour to 28 days. Male was 159 (76.4%) and 49 (23.6%) were female. The commonest conditions presented were anorectal malformations followed by necrotizing enterocolitis. Other conditions include Hirschsprung’s Disease, Omphalocele, Gastroschisis, Duodenal and Jejuno ileal Atresia, Myelomeningocele, Infantile Hypertrophic Pyloric Stenosis, Obstructed Inguinal Hernias, sacrococcygeal teratoma and skin and soft tissue infections. Frequently performed operation was the creation of stoma (Colostomy/Ileostomy). 180 (86.53%) neonates were discharged and 24 (11.53%) neonates expired while only 4 (1.92%) neonates were referred to other centers. Conclusion: Neonatal surgical conditions carry high mortality and morbidity even at centers with all facilities available. Our results at the resource-limited centre are comparable to the centres having state of art facilities. Keywords: Neonatal surgery, Anorectal Malformations, Abdominal wall defects, Necrotizing Enterocolitis (NEC)

Diagnostic challenge of concomitant small bowel atresia and Hirschsprung's disease

The incidence of ileal atresia ranges from 1/5000 to 1/12000 births. The incidence of Hirschsprung's disease is 1/5000. The combination of Hirschsprung disease and ileal atresia during infancy seems to be a rare event and the exact incidence of the association of the two malformations remains unknown. Several embryo-pathological theories have been offered to explain the correlation of these two malformations. Some of them state that atresia would be a consequence of aganglionnosis and some others the contrary. The urgency of an early detection of this associations is related to her increased morbidity during management, especially if part of the phenotype remains unidentified. To date, less than 25 case of ileal atresia associated with Hirschsprung's disease are reported in Literature. We present two cases managed in our department initially for ileal atresia. Diagnosis of total colonic Hirschsprung's disease associated was placed later, after the appearance of an intestinal occlusion at the time of the restoration of digestive continuity. The first case was derived three times, and the second twice before becoming digestive self-sufficient. The dysfunction of the anastomosis after restoration of continuity is a dangerous complication which underlines the interest of the early diagnosis of the association ileal atresia and Hirschsprung's disease. It is necessary to have the rectal biopsy early, especially when the patient continues to exhibit poor bowel function after corrective surgery.