Intestinal Atresia Research Articles

Primary Repair of an Apple Peel Jejunal Atresia and Transanastomotic Feeding in a 1.2 Kilogram Preterm Infant.

Primary Repair of an Apple Peel Jejunal Atresia and Transanastomotic Feeding in a 1.2 Kilogram Preterm Infant.

Percutaneous catheter decompression in a neonate with multiple small bowel atresias

The management of infants with multiple intestinal atresia and complex gastroschisis can represent a challenge to the paediatric surgeon. This case report presents a neonate with multiple jejunal atresias in which a percutaneous foley catheter tube inserted to just beyond the duodenojejunal flexure was used effectively for bowel decompression, instead of the formation of a proximal stoma. By utilising this technique, the well documented complications associated with proximal stomas were avoided, bowel length was preserved and the need for further surgery in the infant was prevented. The infant remained well at six months with no complications at follow-up.

Jejunal web as the cause of bowel obstruction in a toddler: Case report and brief literature review.

Introduction and importanceIntestinal webs which are categorized under type-1 intestinal atresia rarely occur in the jejunum. These webs are occasionally diagnosed late because their central fenestration allows the passage of food.Case presentationWe report a toddler who presented with atypical symptoms of bowel obstruction and non-specific plain radiograph and ultrasound findings. The diagnosis of jejunal obstruction was made possible with contrast-enhanced computed tomography and obstruction was found to be the result of a jejunal web at the surgery.Clinical discussionFew cases of jejunal webs are reported in the literature. The jejunum is the site of only 8% of webs and 33% of jejunal webs are associated with other congenital anomalies and/or prematurity.ConclusionJejunal web needs a high degree of suspicion to be diagnosed and should be kept in mind as a differential diagnosis in the setting of unexplained persistent non-bilious emesis in otherwise normal toddlers.

Intrauterine Intussusception Presenting as Fetal Ascites and Meconium Peritonitis

Intussusception is a well-known cause of intestinal obstruction in children. Its occurrence in fetus as an intrauterine incidence is extremely rare and poses a diagnostic difficulty. Intrauterine intussusception may result in intestinal atresia once the gangrenous segment resorbed. However, a very late occurrence of intussusception just prior to delivery may present as meconium peritonitis. We are reporting a case of premature baby who was born at 35 weeks gestation via emergency caesarean for breech in labour. Routine scan 4 days prior to the delivery showed evidence of fetal ascites. She was born with good Apgar Score and weighed 2.5kg. Subsequently she developed respiratory distress syndrome requiring mechanical ventilation. She passed minimal meconium once after birth then developed progressive abdominal distension and vomiting. Abdominal radiograph on day 4 of life revealed gross pneumoperitoneum and bedside percutaneous drain was inserted to ease the ventilation. Upon exploratory laparotomy, a single ileal perforation was seen 20cm from ileocecal junction with an intussusceptum was seen in the distal bowel. Gross meconium contamination and bowel edema did not favour the option of primary anastomosis, thus stoma was created. Reversal of stoma was performed a month later and she recovered well. Fetus with a complicated intrauterine intussusception may present with fetal ascites and their postnatal clinical and radiological findings need to be carefully assessed for evidence of meconium peritonitis; in which a timely surgical intervention is required to prevent the sequelae of prolonged intraabdominal sepsis in this premature baby.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S18

New sonographic feature (C-sign) to improve the prenatal accuracy of jejunal atresia.

ObjectivesTo describe a new sonographic feature of the C‐sign for prenatal diagnosis of jejunal atresia and evaluate its role in prenatal jejunal atresia, particularly preceding bowel dilatation and polyhydramnios.MethodsThis was a retrospective study from a tertiary maternal hospital. Patients with prenatal sonographic examination and confirmed small bowel atresia postdelivery were included. All sonographic images were reviewed by two senior sonographers. Comparison of sonographic images between prenatal jejunal and ileal atresia using the C‐sign resembles the shape of the entire duodenum and other traditional sonographic features. The control group without bowel atresia was assessed for the presence of the C‐sign.ResultsThe C‐sign and combined bowel dilatation with polyhydramnios were more frequent in jejunal atresia than ileal atresia, but the C‐sign can be used to detect jejunal atresia earlier. The C‐sign can be more likely to diagnose jejunal atresia in persisting bowel dilatation and polyhydramnios. The C‐sign was not reported in any of the control fetuses.ConclusionThe C‐sign is a new sonographic feature that can be used to improve the prenatal accuracy and early detection of jejunal atresia. However, further prospective validation is needed.

Non-immune fetal hydrops in late pregnancу: antenatal ultrasound monitoring and pregnancy outcomes in a series of 14 cases

Background. Fetal hydrops is the accumulation of extracellular fluid in two or more fetal cavities, often in combination with subcutaneous edema. An isolated accumulation of fluid only in the abdominal, pleural, or pericardial cavities is described as ascites, pleural effusion (hydrothorax), pericardial effusion (hydropericardium). Features of the pathogenesis of non-immune hydrops fetalis (NIHF) are the follow: high hydrophilicity of fetal tissues, obstruction of the lymphatic vessels, impaired lymph return, congestive heart failure, obstruction of venous return, changes in fetal venous pressure. All these factors lead to the release of fluid from cells and tissues into the «third» spaces – the abdominal, thoracic, pericardial cavities, as well as the subcutaneous space. The oncotic pressure of fetal plasma proteins is not of great importance in the formation of the fetal circulating blood volume. Currently, all the links in the pathogenesis of hydrops fetalis syndrome with various etiological factors are not fully known. The prevalence of NIHF is unknown because it is difficult to collect relevant data; many cases of the disease are not diagnosed until intrauterine fetal death or may spontaneously resolve during the prenatal period. Currently, up to 90% of all cases of fetal dropsy are attributed to NIHF. Purpose – acquaintance of the medical community with the diagnosis and treatment of non-immune hydrops as well as the analysis of clinical features, ultrasound monitoring and perinatal outcomes of 14 cases of non-immune fetal hydrops onset in a second half of pregnancy. Materials and methods. The material for the study were publications and results of clinical trials found in the databases Scopus, Web of Science Core Collection and PubMed for the period 2009–2020 and the analytic report of the own series of 14 cases of non-immune hydrops fetalis (NIHF) of various origins. During the period of 2005–2020 under the supervision in the Ultrasound Department of Kharkiv regional hospital with regional perinatal center there were 14 pregnant women with NIHF diagnosed in a second half of pregnancy. Clinical features are described, ultrasound images and Doppler monitoring are given, perinatal / postnatal results are studied. Results and discussion. The analysis of clinical features, ultrasound monitoring and pregnancy outcomes of 14 cases of non-immune fetal hydrops developed in the second half of pregnancy in presented series was carried out. 6/14 fetuses had structural anatomical defects (lung sequestration, СDH, myasthenia gravis, megacystis and hydronephrosis, epidermolysis bullosa, meconium peritonitis, intestinal atresia). Mortality rate (including perinatal and infant losses) was as high as 9/14 cases (64.2%): 3 of antenatal, 4 of neonatal, 2 of infant death). Surgical treatment was performed on 2 newborns. 5 newborns had apparently a favorable clinical postnatal outcome. In 2 cases, spontaneous resolution with complete regression of hydrops was observed (parvovirus-B19 and idiopathic NIHF). Complete recovery of fetus (spontaneous regression of hydrops without any deterioration and pathological consequences) was observed in 1 case. Conclusions. Antenatal ultrasound monitoring of fetus with NIHF is based on the assessment of PSV CMA, ductus venous, umbilical vein, atrioventricular flow. According to the results of the study, it was revealed that the cardiovascular profile of the fetus with NIHF is disturbed earlier, and the placental profile and arterial Doppler-later. Normal umbilical artery Doppler do not exclude the possibility of an adverse outcome, including intrauterine fetal demise. Extended Doppler monitoring is essential at NIHF. All neonates with NIHF in an antenatal anamnesis require postnatal follow-up.

Fistula between a patent omphalomesenteric duct and a ruptured omphalocele sac in a neonate

This case report serves to document a rare case of a neonate who presented to the NICU with a patent omphalomesenteric duct (OMD) connected to the top of a ruptured omphalocele sac. Initial evaluation of the neonate in-utero led to the diagnosis of gastroschisis due to the presence of free-floating intestines on ultrasound. However, a ruptured omphalocele sac was discovered grossly upon delivery. Additionally, intestines were found in the midline of the abdomen, a common characteristic of omphaloceles. Therefore, the diagnosis was modified from gastroschisis to ruptured omphalocele. Further gross examination revealed a patent omphalomesenteric duct forming a fistula to the top of the ruptured omphalocele sac. After the appropriate workup and necessary procedures, the neonate had only one bowel movement over 2.5 weeks and was intolerant of food, raising suspicion for an intestinal atresia. Evaluation revealed a type 1 intestinal atresia distal to the patent OMD and omphalocele sac. The atresia was initially masked by bowel inflammation in the neonate at birth. Resection of the atretic bowel led to appropriate bowel movements and increased food intake. The neonate was eventually discharged in good health. • A patent omphalomesenteric duct has been observed to form a fistula with the top of an omphalocele sac in a neonate in-utero. • Ruptured omphalocele should be on the differential when in-utero ultrasound reveals free-floating intestines. • In neonates, intestinal atresia can initially be masked by inflammatory states from other complications.

Laparoscopic diagnosis and treatment of high jejunal atresia and stenosis in neonates

Laparoscopic diagnosis and treatment of high jejunal atresia and stenosis in neonates

Biallelic PI4KA variants cause neurological, intestinal and immunological disease

Phosphatidylinositol 4-kinase IIIα (PI4KIIIα/PI4KA/OMIM:600286) is a lipid kinase generating phosphatidylinositol 4-phosphate (PI4P), a membrane phospholipid with critical roles in the physiology of multiple cell types. PI4KIIIα’s role in PI4P generation requires its assembly into a heterotetrameric complex with EFR3, TTC7 and FAM126. Sequence alterations in two of these molecular partners, TTC7 (encoded by TTC7A or TCC7B) and FAM126, have been associated with a heterogeneous group of either neurological (FAM126A) or intestinal and immunological (TTC7A) conditions.Here we show that biallelic PI4KA sequence alterations in humans are associated with neurological disease, in particular hypomyelinating leukodystrophy. In addition, affected individuals may present with inflammatory bowel disease, multiple intestinal atresia and combined immunodeficiency. Our cellular, biochemical and structural modelling studies indicate that PI4KA-associated phenotypical outcomes probably stem from impairment of PI4KIIIα-TTC7-FAM126's organ-specific functions, due to defective catalytic activity or altered intra-complex functional interactions.Together, these data define PI4KA gene alteration as a cause of a variable phenotypical spectrum and provide fundamental new insight into the combinatorial biology of the PI4KIIIα-FAM126-TTC7-EFR3 molecular complex.

A Comprehensive Review of Intestinal Atresias

Introduction: Intestinal atresia is an uncommon but challenging disease that requires a high level of suspicion for timely diagnosis and expeditious treatment. Its incidence ranges anywhere from 3.4 per 10,000 to 1 per 66,000 live births. Herein we present a comprehensive review of the various presentations of Intestinal Atresia and provide an algorithm for its evaluation. Methods: A review of modern English Language in Index databases (PubMed, SCOPUS, EMBASE, MEDLINE, etc) in the English language was preformed. The etiology, pathophysiology, and management of the intestinal atresia at different levels of the intestinal tract were studied. Results: Atresia is often diagnosed soon after birth and requires a multidisciplinary approach for optimal outcomes. Duodenal atresia is associated with many congenital anomalies and requires a multisystem workup once diagnosed. Jejuno-ilealatresia is the most common intestinal atresia and has fewer associated congenital defects than duodenal atresia. Colonic atresia is the rarest of the intestinal atresias but often has the best outcome as long as it is promptly diagnosed and treatment is not delayed.

Clinical Features of Congenital Heart Disease Accompanied by Congenital Intestinal Atresia

Clinical Features of Congenital Heart Disease Accompanied by Congenital Intestinal Atresia

Editorial Comment to Hirano et al. “Clinical Features of Congenital Heart Disease Accompanied by Congenital Intestinal Atresia”

Editorial Comment to Hirano et al. “Clinical Features of Congenital Heart Disease Accompanied by Congenital Intestinal Atresia”

WITHDRAWN: Immunohistochemical study of inflammatory responses in septa arising from type I intestinal atresia.

Ahead of Print article withdrawn by publisher. This article has been withdrawn at the request of the author(s) and/or editor. The publisher apologizes for any inconvenience this may cause.

Colonic and type IV jejunal atresias with apple-peel bowel segment as the content of right inguinal hernia: A case report

Background: Colonic atresia is an uncommon entity. It may be associated with multiple anomalies. Case Presentation: We present a case, which was initially suspected to be jejunal atresia, but was found to have colonic atresia as well intra-operatively. The associated jejunal atresia with apple-peel configuration was present as the content of inguinal hernia. Conclusion: This case report highlights the rarity of the condition and the postoperative issues in the management of such patients.

Problems with diagnostics in newborn having the combined pathology of gastric membrane and intestinal atresia

Introduction. Congenital malformations of the gastrointestinal tract (GIT) are one of the most common causes of intestinal obstruction and require urgent surgical care in the neonatal period. In the overall structure of GIT, gastrointestinal defects amount up to 25%. Duodenal atresia accounts for 1 case per 7,000–10,000 newborns; jejunal and ileac atresia - 1 case per 1,000–3,000 newborns. Often, the diagnosis is made antenatal. In most cases, diagnostics of intestinal atresia is not difficult, and treatment is carried out in a timely manner. However, in rare cases, intestinal atresia is combined with other congenital malformations in GIT, for example, stomach or duodenum webs. In such a case, if during the atresia surgical correction, a gastric membrane was not suspected, then in the postoperative period there may be problems with treatment requiring additional diagnostics. As a result, web surgical correction is postponed, and the child’s general state can worsen.Material and methods. The authors discuss two cases and their treatment in newborn children who had membranes in their stomach and duodenum combined with the intestinal atresia.Results. The authors also discuss problems which they faced in the diagnostics and treatment. They give their recommendations how to improve diagnostics in children with combined congenital malformations in their gastro-intestinal tract.Conclusion. The full range of diagnostic measures before surgery and a thorough revision of abdominal organs during surgery are key issues in children with gastric and duodenal abnormalities. Undiagnosed and undetected malformations during the first surgical intervention may require a repeated surgical intervention which can worsen the child’s general state.

The colon membrane as a cause of large bowel obstruction in a child of 11-month old: a case report

Introduction. Colon atresia is a rare form of congenital bowel obstruction. In scientific literature, there are isolated publications devoted to colon atresia, and only some authors discuss the membranous form of atresia.Material and methods. The article describes a rare clinical case of an incomplete colon membrane in an infant. At the age of 11 months, the child developed symptoms of acute intestinal obstruction. Irrigography was performed; Hirschsprung’s disease was suspected. At the first stage, the child was taken a floor-by-floor biopsy of the colon, and an ileostomy was put. Visually, Hirschsprung’s disease seemed to be present; however, histological examination turned aside this diagnosis. Given this, the colon membrane was suspected. Partial resection of the altered part of the sigmoid colon was performed with good clinical effect. Histological examination revealed an incomplete serous-muscular membrane of the colon.Conclusion. Colon atresia is a rare disease that presents a serious problem for diagnosis and treatment. Preoperative morphological verification of the diagnosis is important, even in case when Hirschsprung’s disease seems obvious. Preoperative diagnostics is important because surgical tactics may be considerably different in colon local lesions.

Multiple intestinal anastomosis in newborns and infants

Introduction. The present trial systematizes data, taken from one surgical center as an example, on treating patients with intestinal atresia and necrotizing enterocolitis with multiple intestinal anastomoses.Material and methods. The trial is a retrospective review on the treatment of 13 newborn infants who since 2010 have been put multiple intestinal anastomoses; the treatment was approved by the Hospital Ethics Committee. The average gestational age of patients was 31.2 weeks. The average age at the time of surgery – 7,9 days. Average weight - 2007 grams. The average number of anastomoses was 3.7 (range: 2-7). The average length of remained small intestine after the second surgery was 67.4 cm (range: 12-120 cm). No other surgical procedures, including gastrostomy or enterostomy, were performed. In all cases, surgical intervention ended with hermetic suturing of the abdominal cavity. Among them, there were 6 patients with the multifocal form of necrotizing enterocolitis; 6 patients had type IV atresia of the small intestine; 1 patient had the Ladd’s syndrome. Connection of intestinal segments was made by constructing several “end-to-end” anastomoses, double-row precision seam with PDS II 7/0 suture.Results. In the postoperative period, complications associated with anastomosis construction, such as leakage and narrowing, were not recorded. Transit function of the gastrointestinal tract restored on day 4, on average, after the surgery (range: 2-6 days). There were no early lethal outcomes within the first 28 days after the surgery which were associated with the surgery. 2 patients with short bowel syndrome (remained small intestine was 12 and 25 cm) and multivisceral disorders died on day 72 and 64 after the surgery. Survived patients were transferred to full enteral feeding in 56 days, in average, after the second surgery (range - 15-120 days).Conclusion. In our study, we have demonstrated potentials of a new surgical approach: one-stage formation of multiple intestinal anastomoses in case of multiple intestinal atresias as well as in case of multifocal forms of necrotizing enterocolitis. Maintaining the bowel length with multiple bowel anastomoses is very important factor for better survival of patients with the short bowel syndrome.

Five Hundred Patients With Gut Malrotation: Thirty Years of Experience With the Introduction of a New Surgical Procedure.

Objectives:Define clinical spectrum and long-term outcomes of gut malrotation. With new insights, an innovative procedure was introduced and predictive models were established.Methods:Over 30-years, 500 patients were managed at 2 institutions. Of these, 274 (55%) were children at time of diagnosis. At referral, 204 (41%) patients suffered midgut-loss and the remaining 296 (59%) had intact gut with a wide range of digestive symptoms. With midgut-loss, 189 (93%) patients underwent surgery with gut transplantation in 174 (92%) including 16 of 31 (16%) who had autologous gut reconstruction. Ladd's procedure was documented in 192 (38%) patients with recurrent or de novo volvulus in 41 (21%). For 80 patients with disabling gastrointestinal symptoms, gut malrotation correction (GMC) surgery “Kareem's procedure” was offered with completion of the 270° embryonic counterclockwise-rotation, reversal of vascular-inversion, and fixation of mesenteric-attachments. Concomitant colonic dysmotility was observed in 25 (31%) patients.Results:The cumulative risk of midgut-loss increased with volvulus, prematurity, gastroschisis, and intestinal atresia whereas reduced with Ladd's and increasing age. Transplant cumulative survival was 63% at 10-years and 54% at 20-years with best outcome among infants and liver-containing allografts. Autologous gut reconstruction achieved 78% and GMC had 100% 10-year survival. Ladd's was associated with 21% recurrent/de novo volvulus and worsening (P > 0.05) of the preoperative National Institute of Health patient-reported outcomes measurement information system gastrointestinal symptom scales. GMC significantly (P ≤ 0.001) improved all of the symptomatology domains with no technical complications or development of volvulus. GMC improved quality of life with restored nutritional autonomy (P < 0.0001) and daily activities (P < 0.0001).Conclusions:Gut malrotation is a clinicopathologic syndrome affecting all ages. The introduced herein definitive correction procedure is safe, effective, and easy to perform. Accordingly, the current standard of care practice should be redefined in this orphan population.

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

SummaryBackgroundCongenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality.MethodsWe did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis.FindingsWe included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in low-income countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality.InterpretationUnacceptable differences in mortality exist for gastrointestinal congenital anomalies between low-income, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030.FundingWellcome Trust.

Sutureless closure for the management of gastroschisis.

Gastroschisis is a common congenital anomaly in which the midgut fails to return to the abdominal cavity resulting in exposed intestines, which are not covered by a membrane in a neonate. The incidence of gastroschisis has been increasing worldwide resulting in an evolving medical and surgical management. Gastroschisis can be either simple or complicated. Complicated gastroschisis occurs when gastroschisis is associated with gastrointestinal conditions such as intestinal atresia, volvulus, stenosis or perforation. In this instance, the mortality and morbidity of patients significantly increases. Initial management of gastroschisis requires a multi-modal, interdisciplinary approach in order to successfully care for a neonate. Patients should be managed in a neonatal intensive care unit under the care of intensivists, respiratory therapists and pediatric surgeons. Temperature regulation, hydration and protection of the bowel are of the utmost priorities. Surgical management of gastroschisis focuses on reduction of the bowel and closure of the abdominal wall defect. Initially, the defect was closed primarily with suture, however, more recently, a sutureless closure has become prevalent. This, in conjunction with use of a silo, has led to a shift from the operating room and general anesthesia to the bedside. This article aims to discuss the presentation, diagnosis and management of gastroschisis.