Ileal Atresia Research Articles

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.

Descriptive Study of Pediatric Patients with Ileum Stoma at Inpatient Installation of Dr. Soetomo General Hospital Surabaya

Introduction: Ileostomy is needed as an important part of the management of several congenital disorders of the intestine. One of the indication of ileostomy in children is short bowel syndrome, which is caused by intestinal atresia such as ileal atresia and intussusception. Ileostomy is a surgery procedure which consists of creating holes on abdominal walls to release stool. The objective of this study was to determine the description of pediatric patients who underwent ileostomy in Dr. Soetomo General Hospital Surabaya. Methods: This was a retrospective descriptive study on medical records in the Central Medical Record Department of Pediatrics and General Surgery Dr. Soetomo General Hospital Surabaya from January 2017 to December 2018. Results: There were 26 pediatric patients who underwent ileostomy in Dr. Soetomo General Hospital Surabaya. Samples from male patients consisted of 57.7%, while female patients consisted of 42.3% with the most common age group was neonates with 57.7%, infants 30.8%, and toddlers 11.5%. There were 30.7% patients with 2.0-2.9 kg body weight, 27% with 3.0-3.9 kg body weight, and 42.3% with more than 4 kg body weight. The outcome showed that repair was needed, or the patient was discharged with periodic follow-up after ileostomy. Conclusion: Ileostomy was performed mostly in neonates with average weight within normal body mass index (BMI) and the highest incidence of pediatric patient with ileum stoma was in pediatric patients.

Torsion of proximal pouch in ileal atresia presenting as a large abdominal cyst

Torsion of proximal pouch in ileal atresia presenting as a large abdominal cyst

Meconium peritonitis in trisomy 21 masquerading as hydrops fetalis

Meconium peritonitis (MP) is a rare etiology for nonimmune hydrops fetalis. We report a neonate delivered at 32 weeks' gestation in the context of antenatally diagnosed hydrops fetalis and first-trimester screening at high risk for Trisomy 21. The postnatal abdominal radiograph was striking for gross ascites, the paucity of bowel gas, and widespread calcifications, suggestive of MP and chromosomal analysis confirmed Trisomy 21. Exploratory laparotomy performed on day 3 of life revealed a large pseudocyst, ileal atresia with ileal perforation. MP masquerading as nonimmune hydrops fetalis in Trisomy 21 with ileal atresia is an extremely rare presentation, seldom reported in the literature.

Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation in Children.

Aims:Congenital colonic stenosis (CCS) is an extremely rare cause of low-intestinal obstruction in neonates/child. We report our experience with seven cases of CCS presenting with low-intestinal obstruction and diagnosed intraoperatively and also propose an algorithm for its appropriate treatment for the adequate outcome.Materials and Methods:It was a retrospective study of seven patients of CCS including two neonates (5-days and 15-days old), four infants (age range – 2–11 months), and one 24-month-old child admitted from 2014 to 2019. Information regarding the age of presentation, clinical presentation, physical findings, radiological and laboratory findings, details of surgery, and outcome was retrieved and analyzed.Results:The male-to-female ratio was 5:2. Patients were initially diagnosed as cases of Hirschsprung's disease in five and ileal atresia in two. A final diagnosis of CCS was made during surgery and histopathological examination of resected stenotic segment. The segment involved was ascending colon in three, transverse colon in two, and sigmoid colon and junction of descending and sigmoid colon each in one patient. Resection of stenotic colonic segment and primary end-to-end anastomosis was performed in two, divided stoma after resection of the stenotic segment and secondary anastomosis in three, and proximal loop terminal ileostomy followed by resection of the stenotic colonic segment and ileocolic anastomosis after 10–12 weeks in two.Conclusions:CCS is a rare but possible cause of large-bowel obstruction, in neonatal, infant, and children particularly when associated with a history of chronic constipation since birth. It should be kept in mind as a differential diagnosis while managing a case of neonatal and pediatric intestinal obstruction, particularly low-bowel obstruction along with a history of chronic constipation. Treatment should be individualized for each patient based on clinical status and associated anomalies to give the best results with less morbidity.

Anaesthetic considerations in a neonate with unrepaired D-transposition of great arteries with non-syndromic holoprosencephaly for emergency exploratory laprotomy: A case report

D-Transposition of great arteries (D-TGA) is a cyanotic congenital heart disease (CHD) characterized by parallel circulation. Non syndromic holoprosencephaly is an abnormality of brain development resulting from failure or incomplete division of embryonic forebrain into distinct cerebral hemisphere. Major challenges in patients of D-TGA are hemodynamic instability, cyanotic spell, coagulopathy, acid base and electrolyte imbalance. We report successful management of one day old neonate posted for emergency resection anastomosis for ileal atresia with unrepaired d-TGA and non-syndromic holoprosencephaly.

Intestinal atresia: histopathologist view

Background: Intestinal atresia forms one of a common cause for intestinal obstruction in neonates. There is a debate about its pathogenesis and many theories have been suggested. Studies regarding its clinical and histomorphological features are less in Indian literature. The present study aimed to determine the clinical and histomorphological features of cases of intestinal atresia.Methods: Thirty-nine cases of intestinal atresia were studied both retrospectively (twenty-six) and prospectively (thirteen) over a period of two years. Their clinical and histomorphological features were studied.Results: Intestinal obstruction was most common clinical diagnosis. Type II atresia was most common. Ileal atresia was highest in number. Associated congenital anomalies noted were situs inversus with splenunculi, patent vitelo-intestinal duct, duplication cyst, Meckel’s diverticulum, ileocecal web, duodenal web and omphalocele. Histological features such as inspissated meconium, calcification, ulceration, fibrosis, thick-walled vessels, edema were noted.Conclusions: Findings such as mucosal edema, congestion, ulceration, submucosal edema, thick-walled blood vessels, fibrosis, hemorrhage, transmural ischemia, calcification, suggest that an intra vascular accident may be responsible for origin of the atresia.

A female neonate with absent anal opening and dug perineum: Think high

Background: A female neonate with anorectal malformation (ARM) may have one, two, or three openings in the perineum. One opening represents cloaca while three openings usually suggest low ARM. Females with two openings in the perineum may be ARM without any fistula, rectovaginal fistula, or absent vagina. The association of ARM with multiple intestinal atresias and malrotation is rare. Case Presentation: We present here a case of a female neonate with two perineal openings, whose perineum was explored by a general surgeon without any radiological investigation and was later found to have a high ARM, multiple jejunal and ileal atresia with malrotation. Conclusion: ARM associated with small bowel atresia and malrotation are rarely described. Such cases require management in Pediatric surgery settings.

Recycling of Stoma Losses: A Good Practice for Neonates with High Output Stomas—Our Experience and Comparison with Literature

Introduction: The recycling of proximal stoma losses is known as a good practice to manage patients with high output stoma. In our center, we introduced this practice about 10 years ago. We revised our series before and after recycling protocol and we reviewed the literature. Methods: This is a retrospective study. We included all neonates who underwent ileostomy between 2000 and 2019. They were divided into two groups, Group A and Group B, before and after the introduction of recycling of stoma losses. Demographic data were included. Outcomes evaluated to demonstrate the efficacy of stools recycling were: weight at the time of anastomosis and at discharge, length of total parenteral nutrition, and time (expressed in days) between anastomosis and passage of the stools. Data were analyzed using Student’s t-test. P values <.05 were considered significant. Results: A total of 85 patients (29 females, 56 males) were included—Group A: 20 (24%) (13 males/7 females); Group B: 65 (76%) (43 males/22 females); Group A: 12/enterocolitis, 5/meconium-related ileum (MRI), 3/single intestinal perforation; Group B: 45 enterocolitis, 10/MRI, 8/single intestinal perforation, 1/an ileal atresia, 1/meconial peritonitis. Outcome about weight at anastomosis and discharge and about time of canalization were significant. Conclusion: We believe that the recycling of stoma losses is a safe and effective practice in neonates with ileostomy. Our technique of recycling is simple and practical. The benefit/risk rate is certain, in our opinion, in favor of the benefits as showed from our data.

Gastrointestinal perforations in newborns with high mortality: A series of 18 cases

Gastrointestinal perforations (GIP) in newborns are serious neonatal conditions that have significant morbidity and mortality, especially in resource constrained setups. This study was done to describe our experience of GIP in neonates and identify the factors leading to high mortality in our setup. We collected 18 cases with GIP in neonates. The average age was 5.7 days (range, 1-7 days). The sex ratio was 0.64. The average time to treatment was 3.3 days (range, 1-10 days). The etiology of GIP was perforated necrotizing enterocolitis (13 patients), spontaneous gastric perforation (2 patients), perforated ileal atresia (1 patient), ileal perforation complicating a strangulated inguinoscrotal hernia (1 patient), and peritonitis after colostomy (1 patient). Mortality was 77.8% (n=14), among which 9 newborns (64.3%) died preoperatively. Prematurity, management delay, and lack of a neonatal intensive care unit were the main poor prognostic factors. Mortality from GIP is still high in our context due to several factors, especially prematurity, management delay, and lack of a neonatal intensive care unit.

Loss-of-function variants in PPP1R12A: From isolated sex reversal to holoprosencephaly spectrum and urogenital malformations

In two independent ongoing next-generation sequencing projects for individuals with holoprosencephaly and individuals with disorders of sex development, and through international research collaboration, we identified twelve individuals with de novo loss-of-function (LoF) variants in protein phosphatase 1, regulatory subunit 12a (PPP1R12A), an important developmental gene involved in cell migration, adhesion, and morphogenesis. This gene has not been previously reported in association with human disease, and it has intolerance to LoF as illustrated by a very low observed-to-expected ratio of LoF variants in gnomAD. Of the twelve individuals, midline brain malformations were found in five, urogenital anomalies in nine, and a combination of both phenotypes in two. Other congenital anomalies identified included omphalocele, jejunal, and ileal atresia with aberrant mesenteric blood supply, and syndactyly. Six individuals had stop gain variants, five had a deletion or duplication resulting in a frameshift, and one had a canonical splice acceptor site loss. Murine and human in situ hybridization and immunostaining revealed PPP1R12A expression in the prosencephalic neural folds and protein localization in the lower urinary tract at critical periods for forebrain division and urogenital development. Based on these clinical and molecular findings, we propose the association of PPP1R12A pathogenic variants with a congenital malformations syndrome affecting the embryogenesis of the brain and genitourinary systems and including disorders of sex development.

Prevalence of Tracheoesophageal Anomaly Cases among Neonates Undergoing Surgery in a Tertiary Care Children’s Hospital.

Tracheo-oesophageal anomaly is the abnormal communication between trachea and oesophagus. The most common type of tracheo-oesophageal anomaly is oesophageal atreasia with distal tracheo oesophageal fistula. Tracheo-oesophageal anomaly is a common neonatal problem requiring an urgent surgery. Tracheo-oesophageal anomaly can be diagnosed as an isolated malformation or as part of polymalformative syndrome with possible vertebrae anomalies also known as Vacterl syndrome. The aim of the study is to find out the prevalence of tracheo-oesophageal anomaly cases among neonates undergoing surgery in a tertiary care childrens hospital in Nepal. This is a descriptive cross sectional study conducted in a tertiary care children’s hospital in Nepal taking into account the medical records from period of 01 Jan, 2018 to Dec 31, 2019. Study population included the infants undergoing operative procedure in the operation theatre of Kanti Children’s Hospital. The calculated sample size was 306. Data was collected by retrospective chart review technique and listed in performa. Consecutive sampling technique was used. Thus collected data was entered in SPSS version 20 and necessary calculations were done. The prevalence of tracheo-oesophageal anomalies was found out to be 30 (9.8%) at 95% confidence interval. The most common problem, with which infants were brought to Kanti Children’s Hospital, that required urgent neonatal surgical intervention was anorectal malformation constituting 94 (30.72%) of the surgeries followed by intestinal obstruction 76 (24.84%) which included duodenal atresia, jujunal atresia and ileal atresia. Tracheo-oesophageal anomaly constitutes a major portion of neonatal disease condition requiring surgery in Nepal.

Patterns and Outcome of Neonatal Intestinal Obstruction in Kanti Children’s Hospital

Introduction: Neonatal intestinal obstruction is the most common neonatal surgical emergency. More than 75% of neonatal surgeries are done for the correction of this problem. The outcome of this obstruction mainly depends upon the causes of obstruction, clinical condition of the patient, associated anomalies, expertise and centre where the patient is dealt. This study was undertaken to study the clinical features and outcome of neonatal intestinal obstruction presented in our centre.
 Methods: The medical records of all neonates admitted in the surgical NICU were selected and cases with the diagnosis of neonatal intestinal obstruction and managed surgically during two years period from September 1, 2015 to September 1, 2017 were retrospectively reviewed and analysed in regard to age at presentation, sex, weight, gestation, postoperative diagnosis and their outcome.
 Results: Out of 235 admitted neonates, 205 cases were treated surgically for intestinal obstruction. There were a total of 154 (75.2%) males and 51 (24.8%) females. Mean age at presentation was 4.5 days and average weight was 2.25 kg. Among them, anorectal malformation (ARM) 88 (42.92%), intestinal atresia 59 (28.78%), Hirschsprung’s disease 38 (18.53%), malrotation of gut 10 (4.87%) and meconium ileus 10 (4.87%) were the etiology. Among intestinal atresia, duodenal atresia 27 (45.76%) was the commonest one followed by ileal atresia 24 (40.67%), six jejunal atresia (10.16%) and two colonic atresia (3.38%). Overall, 160 neonates survived (78.04%). Among the survival, Hirschsprung’s disease was 34 (89.47%), ARM 78 (88.63%), seven malrotation of gut (70%), six meconium ileus (60%) and intestinal atresia 35 (59.32%).
 Conclusion: Neonatal intestinal obstruction is the most common neonatal surgical emergency in Kanti Children’s Hospital. Anorectal malformation was the commonest etiology followed by intestinal atresia and Hirschsprung’s disease. Overall survival rate was 78%. Cases with Hirschsprung’s disease and anorectal malformation had about 90% survival rate. Intestinal atresia was the major cause of mortality occupying 53.35% of total mortality of 21.95%. Mortality is mainly due to delayed diagnosis, referral and intervention which lead to higher complications of cases.

Intrauterine intestinal volvulus prompting emergency delivery and surgery in a 32wk fetus

Abstract Fetal intestinal volvulus is a medical emergency with potentially fatal consequences thus requires a high index of suspicion and prompt intervention. Echogenic dilated bowels, Whirlpool/Coffee bean signs, polyhydramnios, fetal anemia, fetal ascites with aperistalsis, nonreaasuring CTG are few important diagnostic signs. Mostly it has been described without associated malrotation,as segmental volvulus due to malfixation of gut with or without associated anaomalies such as meconium ileus, CDH, abdominal wall defects. We describe for the first time a case of 1.9kg female fetus detected to have intestinal volvulus associated with multiple ileal atresias and mesenteric defect. The fetus had classical Whirlpool sign, polyhydramnios and anemia, was delivered by emergency Ceasarian Section, post natally detected to have tense abdomen, confirmed Whirlpool sign on post natal USG, underwent emergency laparotomy, resection of necrotic ileal loops & I-C junction and Jejuno-Colic anastomosis. Child adapted to elemental feeding with 50cm of Jejunum without Ileo-cecal valve without shortbowel syndrome. Importance of multidisciplinary perinatal care, possible intrauterine vascular accident in pathogenesis of atresia and fetal volvulus has been emphasized in the case report.

Appearance of fetal intestinal obstruction on fetal MRI.

To retrospectively analyze the imaging findings of fetal intestinal obstruction diagnosed by MRI and compare with postnatal surgery findings. MRI data of 3346 pregnant women were retrospectively analyzed; we found 47 cases of suspected fetal small intestinal obstruction. Twenty-nine underwent postnatal surgery. We identified one case of jejunal obstruction secondary to perforation, five annular pancreas, 10 duodenal stenoses, four jejunal stenoses, five jejunal atresias, two ileal atresias, four intestinal volvulus, and four intestinal malrotations. We further found four cases of duodenal stenosis with intestinal malrotation (two cases also showed volvulus). On fetal MRI, annular pancreas and duodenal obstruction manifested as a "double bubble." Jejunal stenosis appeared as a "triple bubble." Jejunal and ileal atresia mainly manifested as proximal dilatation with high signal on T1WI. Intestinal volvulus showed a sausage-like intestinal distortion and mixed signals on T1WI and DWI sequences. Intestinal malrotations were characterized as abnormal duodenal morphology. We missed two malrotations; one jejunal obstruction was misdiagnosed. Prenatal MRI can accurately determine the degree of intestinal obstruction and help qualitatively diagnose its possible etiology according to changes in MRI signals in multiple sequences, providing guidance for prenatal counseling.

In-utero segmental volvulus around vitellointestinal duct remnants leading to ileal atresia and stenosis: A case series

Background: The etiology of intestinal atresia is multifactorial with vascular accidents being the most common event in its development. In-utero volvulus is a well-known type of vascular accident leading to intestinal atresia. Segmental volvulus around vitellointestinal duct remnants is rarely described vascular accident leading to ileal atresia. Case Series: We report two cases of segmental ileal volvulus around vitellointestinal duct remnants in neonates leading to ileal atresia in one case and intestinal stenosis in the other one. Both neonates presented with signs of neonatal intestinal obstruction. X-ray abdomen performed in both neonates showed air-fluid levels. Both neonates were operated with suspicion of intestinal atresia. At surgery, a segment of the small bowel was found twisted around vitellointestinal duct remnants in both cases. The first case also had intestinal atresia whereas the second one had intestinal stenosis secondary to the segmental volvulus. Conclusion: Segmental volvulus around vitellointestinal duct remnants can also cause ileal atresia or stenosis.

Staged repair of colonic atresia with non-contiguous perforation of the proximal colon

Abstract We present the case of a neonatal patient with colonic atresia who was transferred to our hospital for perforated peritonitis. In this case report, we describe the location of perforation and the treatment provided for the preservation of the ileocecum. A 2-day-old boy presented to our hospital in a state of shock, with continuous bilious vomiting and abdominal tension. His abdominal X-ray showed free and intestinal air; he was diagnosed with ileal atresia with perforation. The operative findings showed the perforation to be on the ascending colon just next to cecum and the atresia to be on the transverse colon. We were unable to complete a primary anastomosis. To preserve the ileocecum, an ileostomy was performed on the ileum 10 cm away from the ileal end. The perforation was repaired, and the colostomy was performed on the anal side of the atresic colon. Since 22 days of age, stool had been injected into the colostomy several times per day. The ileostomy was closed, and the atresic colon was anastomosed at the age of 77 days. This case suggests that the perforation site is not always near the site of atresia. When the perforation is near the ileocecum, performing an ileostomy can preserve the ileocecum from resection.

Fourth Ventricular Outflow Obstruction in an Infant with Ileal Atresia and Laryngomalacia: Endoscopic Management

AbstractFourth ventricle outflow obstruction (FVOO) is a rare cause of obstructive hydrocephalus. In this study, we described a case of idiopathic FVOO with ileal atresia and laryngomalacia which was managed with endoscopic third ventriculostomy (ETV) and re-endoscopy. We also described the techniques of fenestration of Liliequist membrane and partial removal of arachnoid membrane over dorsum sella (DS) to prevent closure of fenestration and recurrence of hydrocephalus. The patient was a 4-month-old infant presented with progressively increasing head size, feeding difficulty, respiratory distress, and tense fontanel. The infant had a history of laparotomy for ileal atresia. CT scan showed panventriculomegaly due to FVOO. ETV with fenestration of Liliequist membrane was done on emergency basis. After operation, the patient improved clinically and radiologically. Four weeks later, the patient returned with recurrent hydrocephalus. Endoscopic reoperation showed closure of fenestration in arachnoid membrane (Lilieqiest membrane). Endoscopic refenestration with partial excision of arachnoid on DS was done. The patient again recovered radiologically and clinically till last follow-up. In idiopathic FVOO, ETV with wide fenestration of Liliequist membrane, preferably with partial removal of arachnoid on DS, may be very useful in treating hydrocephalus (HCP) and preventing recurrent HCP even in infants.

Role of Bishop–Koop Procedure for Jejunal and Proximal Ileal Atresia

Background: This study was undertaken to review the Bishop–Koop procedure as a treatment option with a grossly dilated proximal segment in jejunal and proximal ileal atresia.Materials and Methods: This was a retrospective cohort study conducted from January 2012 to June 2018 in the Department of Pediatric Surgery at King George's Medical University, Lucknow, India. The outcome, complication rate, and the follow-up study for postoperative adverse outcomes were assessed.Results: Thirty-two neonates underwent Bishop–Koop procedure. The mean age at presentation was 4.37  2.3 days. The male (n=22) to female (n=10) ratio was 2.2:1. Sixteeen had jejunal (type II-9, type III- 7), and 16 (type II-6, type III-10) had proximal ileal atresia. The mean duration of the hospital stay was 13.03  5.7 days. Oral feeds were initiated by the 7th postoperative day. In our study, the complication rate was 31.25% (n=10) and mortality rate was 37.5% (n=12).Conclusions: Bishop–Koop procedure appears to be a technically efficient method in desperate cases of jejunoileal atresia with a grossly dilated proximal segment, although more extensive studies may be needed to compare Bishop–Koop procedure and other operation techniques.

Jejunoileal atresias associated with colonic atresia

Abstract Colonic atresia (CA) is one of the rarest causes of neonatal intestinal obstruction. The combination of jejunoileal atresia (JIA) and CA is particularly rare. To the best of our knowledge, only 21 cases of JIA associated with CA have been described in the English-language literature. We herein present a case involving a 34-week preterm female infant with bilious vomiting by the first day of life. Laparotomy revealed jejunal gap atresia and ileal gap atresia. In addition, colonic gap atresia was found at the hepatic flexure with a wide mesenteric defect. The JIA and CA appeared to have been caused by a thromboembolic event in the superior mesenteric artery. We also review the 21 previously published cases and discuss the etiologies of the association between JIA and CA.