Neonatal Intestinal Obstruction Research Articles

004. Confirming The Diagnosis of Hirschsprung Associated Enterocolitis Based on The Hirschsprung Associated Enterocolitis Guidelines: A Literature Review

Background: Hirschsprung's disease (HD) is one of the most common causes of intestinal obstruction in neonates. One of the most frequently encountered complications, and the leading cause of morbidity and mortality in HD patients, is Hirschsprung-associated enterocolitis (HAEC). In 2017, Gosain et al. developed a guideline to aid in the diagnosis of HAEC, but there are limited studies discussing this guideline and diagnosing HAEC remains unclear. The aim of this study is to establish the diagnosis of HAEC in patients using the HAEC guideline. Methods: The studies were selected from online databases such as Scopus, EBSCOhost, PubMed, ScienceDirect, and Medline. Searches were conducted using Boolean OR and AND. Literature selection was based on abstracts, titles, and keywords. The literature selected were based on predetermined inclusion and exclusion criteria. Results: A total of 1,610 studies were identified from the literature search conducted on five databases using appropriate keywords. The articles were then screened using the PRISMA flow diagram, resulting in the selection of three articles that met the inclusion and exclusion criteria. These three selected studies included two retrospective cohort studies and one literature review and expert consensus study for the diagnosis of HAEC. Conclusion: The HAEC guideline by Gosain et al. can be used to clinically establish HAEC; however, it has not been tested in the population and remains an expert consensus, so its validity as a diagnostic guideline cannot yet be compared to previous scoring methods that have been analyzed in the HAEC patient population.

Postoperative bowel function in children operated for Hirschsprung's disease in a low-income setting: Institution-based cross-sectional study.

Hirschsprung's disease is a common cause of lower intestinal obstruction in newborns. It has variable postoperative outcomes affecting quality of life. The study was aimed at assessing postoperative bowel function in children with Hirschsprung's disease. It was conducted on 120 children operated for Hirschsprung's disease. A structured questionnaire for bowel function score was used and analyzed using relevant statistical tests. Of the 120 children in the study, 97 (80.8%) were male with 49 (40.8%) diagnosed during neonatal age and others by 2years of age. Ninety-three (77.5%) of them had the classic type. Diversion colostomy was done in 104 (86.6%), and two-staged endorectal pullthrough was performed in 62 (72.5%) of cases with a 16% rate of retained aganglionosis. Postoperative continence was excellent in 46 (57%) and good in 26 (32%) with an incontinence rate of 11%. None of the outcome predictor showed significant influence. Optimal postoperative bowel function was obtained in the majority of patients with two-stage procedures, and the overall outcome of bowel function in children was not influenced by age, gender, level of aganglionosis, and type of procedure. Longer follow-up periods are required for definitive information.

Treating neonatal intestinal obstruction with ostomy in continuity: a single-center comparative study.

There is an array of surgical modalities available to treat neonatal intestinal obstruction, but there is no consensus on the optimal method. The study aims to evaluate the therapeutic efficacy of ostomy in continuity (OIC), providing a reference for surgeons to determine the appropriate surgical approach. The study involved a retrospective analysis of the clinical data of 46 neonates with intestinal obstruction hospitalized between June 2019 and February 2024. The types of intestinal injuries included in the study comprised atresia, necrotizing enterocolitis (NEC), meconium ileus and volvulus. Based on their surgical procedures, patients were divided into the OIC group and the control group. General information, as well as perioperative and postoperative complications, were compared between the two groups. There were 18 patients underwent OIC, and 28 patients underwent double-barrel or single-barrel enterostomies. There were no statistically significant differences between patients in the two groups in terms of general information, duration of the ostomy surgery (P=0.66), bleeding volume (P=0.25), length of post-ostomy hospital stay (P=0.08), and time to first defecation after surgery (P=0.23). Compared to the control group, neonates in the OIC group had a shorter duration of parenteral nutrition (P=0.02), a shorter interval between stoma creation and closure surgeries (P=0.02), a shorter duration of stoma closure surgery (P<0.001), and fewer postoperative complications (P<0.001). The weight-for-age Z-score before the stoma closure surgery was better in the OIC group than the control group (P=0.01). In this study, we found that OIC, as a treatment for neonatal intestinal obstruction, was effective in maintaining intestinal continuity, improving the nutritional status of neonates, and shortening the interval between the stoma creation and closure surgeries.

Neonatal intestinal obstruction secondary to Meckel's diverticulum with associated band and ileal stricture: A case report

Background: Symptomatic Meckel’s diverticulum is exceptionally rare in neonates, often presenting as intestinal obstruction or perforation. The differential diagnosis may encompass more prevalent conditions like intestinal atresia, malrotation, or Hirschsprung's disease. Case Presentation: We present a case of a 21-day-old female who presented with intestinal obstruction. Exploratory laparotomy revealed Meckel's diverticulum with a mesodiverticular band causing closed-loop obstruction along with three passable strictures in the ileum. Surgical resection of the affected bowel and the creation of an ileostomy were performed due to bowel ischemia and hemodynamic instability. Conclusion: Early diagnosis and prompt surgical intervention are pivotal in managing neonatal Meckel's diverticulum, reducing risks of bowel necrosis and complications.

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.

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

Background: Intrauterine intussusception, although very rare (&lt;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.

Unveiling neonatal intestinal obstruction due to total colonic atresia: A case report

Background: Total colonic atresia is an uncommon congenital anomaly associated with neonatal intestinal obstruction, often necessitating prompt diagnosis and surgical intervention to prevent life-threatening complications. Understanding the etiology and optimal management strategies is crucial for ensuring favorable outcomes. Case Presentation: We present a case of a full-term male neonate with total colonic atresia, who presented with failure to pass meconium, abdominal distension, and vomiting. Diagnostic imaging confirmed intestinal obstruction, leading to surgical exploration revealing an atretic colon extending from the Ileocolic Junction to the Rectosigmoid Junction. An ileostomy was performed, followed by meticulous postoperative care, resulting in a successful resolution of symptoms and discharge from the hospital. Conclusion: Timely recognition and staged surgical management are paramount in addressing total colonic atresia, emphasizing the importance of early intervention for optimal patient outcomes. Long-term follow-up and comprehensive care are essential to ensure the overall well-being of affected newborns.

Ultrasound imaging of bowel obstruction in neonates.

Bowel obstruction (BO) in children has a wide differential diagnosis, ranging from non-urgent conditions to surgical emergencies. Abdominal radiographs are most often used as the first imaging modality for the evaluation of obstruction. However, for some indications, ultrasound can be the primary imaging modality. Therefore, it is incumbent on radiologists to recognize the types of bowel obstruction that can be recognized with US. Key sonographic features of BO include differential dilation of bowel loops, bowel wall thickening, and free fluid. "Do Not Miss" findings that indicate need for emergent treatment include volvulus, pneumoperitoneum, and/or signs of ischemia (bowel wall thinning and/or absent perfusion). The aim of this pictorial essay is to provide guidance on the sonographic technique and findings that enable identification of BO on US. Examples of neonatal BO on US, including common and less frequently encountered etiologies, are illustrated in this pictorial essay.

Secondary Carnitine Deficiency in Neonates and Infants Requiring Surgery for Intestinal Obstructions-An Underestimated and Undermanaged Problem.

This study aims to elucidate the relationship between intestinal obstruction and carnitine deficiency in neonates and infants. We retrospectively reviewed medical records of 330 neonates and infants, younger than six months, who underwent surgery for intestinal obstruction at our institute from January 2009 to April 2022. The analysis focused on clinical symptoms, related signs, complications, and etiology of the intestinal obstruction. Tandem mass spectrometry (MS/MS) or urine organic acid analysis was conducted for 47 patients, revealing carnitine deficiency in 16 patients. The incidence of carnitine deficiency was 34.0% in the suspicious group and 4.8% overall, significantly higher than in the general population in Taiwan. Notably, patients with carnitine deficiency experienced prolonged ileus, with a mean fasting duration of 41.7 days (range 7.8-65.5 days), compared to 10.8 days (range 8.2-13.4 days) in patients without carnitine deficiency. Carnitine replacement therapy was administered to twelve patients at dosages ranging from 32 to 90 mg/kg/day. One patient exhibited a drug allergy with skin rashes. Our findings suggest that carnitine deficiency should be considered in cases of neonatal and infant intestinal obstruction. Replacement therapy is straightforward and can be prognostically beneficial for some patients. Therefore, we recommend generalizing MS/MS and urine organic acid analysis, particularly for patients with prolonged ileus.

High incidence of EDNRB gene mutation in seven southern Chinese familial cases with Hirschsprung's disease.

Hirschsprung's disease (HSCR) is the leading cause of neonatal functional intestinal obstruction, which has been identified in many familial cases. HSCR, a multifactorial disorder of enteric nervous system (ENS) development, is associated with at least 24 genes and seven chromosomal loci, with RET and EDNRB as its major genes. We present a genetic investigation of familial HSCR to clarify the genotype-phenotype relationship. We performed whole exome sequencing (WES) on Illumina HiSeq X Ten platform to investigate genetic backgrounds of core family members, and identified the possibly harmful mutation genes. Mutation carriers and pedigree relatives were validated by Sanger sequencing for evaluating the gene penetrance. Four familial cases showed potential disease-relative variants in EDNRB and RET gene, accounting for all detection rate of 57.1%. Three familial cases exhibited strong pathogenic variants as frameshift or missense mutations in EDNRB gene. A novel c.367delinsTT mutation of EDNRB was identified in one family member. The other two EDNRB mutations, c.553G>A in family 2 and c.877delinsTT in family 5, have been reported in previous literatures. The penetrance of EDNRB variants was 33-50% according mutation carries. In family 6, the RET c.1858T>C (C620R) point mutation has previously been reported to cause HSCR, with 28.5% penetrance. We identified a novel EDNRB (deleted C and inserted TT) mutation in this study using WES. Heterozygote variations in EDNRB gene were significantly enriched in three families and RET mutations were identified in one family. EDNRB variants showed an overall higher incidence and penetrance than RET in southern Chinese families cases.

Congenital Absence of Ileum, Caecum and Appendix in a Preterm Neonate: A Rare Case Report

There are numerous birth defects that can affect the gastrointestinal tract, starting from the oesophagus or stomach to the small and large intestines. Intestinal atresias are one of the most common causes of intestinal obstruction in newborns. The most common location is the jejunum, followed by the duodenum and the colon. Congenital absence of the ileum, caecum, and appendix is an extremely rare condition. Here, an exceptional instance of congenital absence involving the distal jejunum, ileum, caecum, and appendix in a male child is presented, who presented with chief complaints of multiple episodes of bilious vomiting after initiating the first feed and non passage of meconium. Upon initial examination, the patient was suspected to have an intestinal obstruction and subsequently underwent an exploratory laparotomy. However, intraoperative findings revealed a grossly dilated proximal jejunal loop, and the next segment of the intestine was the narrow ascending colon. In due course, the baby recovered well and was discharged. Currently, there is no documented occurrence of a comparable case involving a viable neonate in the existing body of scholarly literature. Surgeons need to understand positional variations and congenital anomalies, as well as the procedures needed to identify these abnormalities during surgery.

Neonatal Intestinal Obstruction in Acute Renal Failure in Premature Infant: A Case Report

Neonatal Intestinal Obstruction in Acute Renal Failure in Premature Infant: A Case Report

Hirschsprung disease and intestinal malrotation: A rare association with unique perioperative considerations

BackgroundMultiple etiologies exist for neonatal intestinal obstruction with similar presenting symptoms. While single etiology is the most common cause, concomitant surgical diagnoses exist requiring unique peri‑operative considerations. We present a retrospective review of 5 patients with a rare association of Hirschsprung disease (HD) and intestinal malrotation. MethodsAfter IRB approval, patients were identified utilizing Data Direct and Electronic Medical Records ID with pathologically confirmed HD and intestinal malrotation from a single institution and retrospective review was performed. ResultsFive patients were identified with the concomitant malrotation and HD diagnoses over a 15-year period. Intestinal malrotation was confirmed by intra-operative findings. HD was confirmed in all patients by rectal biopsy. Feeding intolerance was the most common chief complaint. A contrast enema was the initial diagnostic test of choice in 3 of 5 of patients. All patients underwent a Ladd procedure, including appendectomy, during the index admission. Two of 5 patients underwent surgical management for HD during the index admission, while surgery was delayed in the remaining 3. ConclusionsAssociation between HD and malrotation causing intestinal obstruction has been previously described, albeit rare. The two diagnoses can present similarly in neonatal patients; although, the underlying pathophysiology, work up and surgical management is quite different. Traditionally, a Ladd procedure is performed utilizing an open approach and includes an appendectomy. However, patients undergoing a Ladd procedure with high suspicion of HD pre-operatively pose unique considerations. We present data of 5 patients to highlight unique considerations regarding diagnostic work up and operative planning.

Colonic atresia in a newborn: a rare case report

Colonic atresia is a rare cause of intestinal obstruction, and the transverse colon is the rarest site of colonic atresia. Our case was an index case of colonic atresia to be first operated on and effectively managed by the collaboration of paediatricians and surgeons in the Bundelkhand region of Uttar Pradesh. A 5-day-old male baby presented with complaints of abdominal distension, bilious vomiting, and failure to pass meconium and was diagnosed as a case of colonic atresia. The present case and the pertinent literature were discussed with an emphasis on surgical management along with medical post-op care. Colonic atresia must be considered an important cause of distal intestinal obstruction in neonates, and other causes of congenital small left colon should be ruled out.

A rare cause of neonatal feeding intolerance: congenital segmental intestinal dilatation—a case report

BackgroundCongenital segmental intestinal dilatation (CSID) is a rare cause of neonatal bowel obstruction. Patients can present in the early newborn period with feeding intolerance and abdominal distension. Diagnosis is challenging due to its rarity and non-specific clinical and radiological findings in a well neonate. Surgical excision remains the mainstay treatment for this disease.Case presentationWe report a case of a newborn male with congenital segmental ileal dilatation, who presented with abdominal distension and feeding intolerance. He was born preterm at 32 weeks and was treated for presumed sepsis due to a premature rupture of the membrane. The patient developed persistent abdominal distension upon initiation of feedings with large amount of gastric aspirates. Otherwise, there was no delay in the passage of meconium. Serial abdominal radiographs showed persistent focal bowel dilatation on the right side. He was initially diagnosed with small bowel atresia. Ultrasound abdomen and lower gastrointestinal contrasted study showed focal ileal dilatation with no evidence of pneumoperitoneum. Intraoperative findings revealed a segmental ileal dilatation with no evidence of mechanical obstruction. The abnormal dilated bowel was excised and sent for histopathological examination. He recovered well after surgery.ConclusionDiagnosis of CSID can be challenging due to its clinical polymorphism and non-specific radiological findings. CSID should be suspected in neonates with feeding intolerance and focal intestinal dilatation without clinical features of sepsis, peritonitis, or mechanical obstruction. Surgical excision is curative with a good outcome.

Imaging Features of Neonatal Bowel Obstruction.

Radiologic evaluation of neonatal bowel obstruction is challenging owing to the overlapping clinical features and imaging appearances of the most common differential diagnoses. The key to providing an appropriate differential diagnosis comes from a combination of the patient's gestational age, clinical features, and imaging findings. While assessment of radiographs can confirm bowel obstruction and indicate whether it is likely proximal or distal, additional findings at upper or lower gastrointestinal contrast study together with use of US are important in providing an appropriate differential diagnosis. The authors provide an in-depth assessment of the appearances of the most common differential diagnoses of proximal and distal neonatal bowel obstruction at abdominal radiography and upper and lower gastrointestinal contrast studies. These are divided into imaging patterns and their associated differential diagnoses on the basis of abdominal radiographic findings. These findings include esophageal atresia variants including the "single bubble," "double bubble," and "triple bubble" and distal bowel obstruction involving the small and large bowel. Entities discussed include esophageal atresia, hypertrophic pyloric stenosis, pyloric atresia, duodenal atresia, duodenal web, malrotation with midgut volvulus, jejunal atresia, ileal atresia, meconium ileus, segmental volvulus, internal hernia, colonic atresia, Hirschsprung disease, and functional immaturity of the large bowel. The authors include the advantages of abdominal US in this algorithm, particularly for hypertrophic pyloric stenosis, duodenal web, malrotation with midgut volvulus, and segmental volvulus. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Modified circumumbilical approach for duodenal atresia repair : A scarless surgery

Background: Duodenal atresia is a common congenital anomaly causing neonatal intestinal obstruction. The definitive treatment is to restore the intestinal continuity. Laparotomy via upper transverse abdominal incision has been described by many authors as a standard approach for duodenal atresia repair. Here we report a case of newborn underwent duodenal atresia repair via modified circumumbilical approach with satisfactory postoperative outcome. Case presentation: This is a case of premature baby boy, born at 32-week gestation with a birth weight of 1.5kg. Antenatally he was detected to have double bubble sign which was confirmed upon postnatal abdominal radiograph, suggestive of duodenal atresia. He was noted to have a fleshy lump at the umbilicus presumed to be a patent vitello-intestinal duct from the referring hospital. Subsequently he developed umbilical swelling suspicion of umbilical hernia with incarceration and scheduled for emergency umbilical exploration. A circumumbilical incision was made and found to have an umbilical defect with omental herniation, instead of a patent vitello-intestinal duct. A proximal midline incision was made as an extension to the circumumbilical wound enabling a duodenal kocherization and subsequently had duodeno-jejujonostomy anastomosis for the duodenal atresia repair. Postoperative recovery was uneventful with appealing cosmesis. Conclusion: A modified circumumbilical incision is an attractive alternative approach for a duodenal atresia repair. Apart from its cosmetic advantage, the approach provides adequate exposure for similar outcome with transverse abdominal incision.

Choledochal cyst presenting with neonatal intestinal obstruction: A case report

Choledochal cyst presenting with neonatal intestinal obstruction: A case report

Congenital internal hernia in the neonate: Case series with review of potential comorbidities and imaging findings

Internal hernias are a rare but serious complication that typically present in older children and adults and can be congenital or iatrogenic in origin. They are often difficult to diagnose due to their similar radiological and clinical presentation to more common abdominal pathologies. We describe three cases of congenital internal hernias presenting in preterm infants followed by a literature review of congenital internal hernias presenting shortly after birth. When presenting in the neonatal period, congenital internal hernias have been exclusively reported as transmesenteric type. The exact etiology of transmesenteric congenital internal hernias is unclear, but may be a result of vascular compromise to the mesentery creating a small defect in which bowel may become entrapped. Certain genetic factors may also play a role in increasing risk for congenital internal hernias as they can be comorbid with other pathologies. Delayed diagnosis postpones surgical treatment, increasing risk of hernia incarceration, strangulation, irreversible bowel ischemia, and long-term risks inherent to bowel resection. Multimodal imaging plays a key role in the evaluation of these infants by excluding many causes of neonatal intestinal obstruction, however, a definitive prospective radiologic diagnosis has not been reported to date. Radiographic findings and comorbidities associated with these cases can be used along with future reported cases to potentially aid in timely diagnosis.

Ultrasonic Diagnosis of Intestinal Obstruction in Neonates-Original Article

Intestinal obstruction in neonates is a common problem that requires prompt diagnosis and treatment, and ultrasound could be a potential tool for it. The purpose of this study was to investigate the accuracy of ultrasonography in diagnosing and identifying the cause of intestinal obstruction in neonates, the corresponding ultrasonic manifestations, as well as to utilize the diagnostic method. We conducted a retrospective study of all neonatal intestinal obstruction in our institute between 2009 and 2022. The accuracy of ultrasonography in the diagnosis of intestinal obstruction and the identification of its etiology was compared with the results of operation as the gold standard. The accuracy of the ultrasonic diagnosis of intestinal obstruction was 91%, and the accuracy of the ultrasonic etiological diagnosis of intestinal obstruction was 84%. The main ultrasound findings for the neonatal intestinal obstruction were dilation and high tension of the proximal bowel and collapse of the distal intestinal. Other major manifestations were the presence of corresponding diseases causing intestinal obstruction at the junction of the dilated and collapsed bowel. Ultrasound has the advantages of being a flexible multi-section dynamic evaluation and a valuable tool to diagnose and identify the cause of intestinal obstruction in neonates.