Two cases of type 1 jejunoileal atresia with multiple webs: A case report

Abstract

Introduction and importance

Type 1 jejunoileal atresia with multiple webs is a rare congenital condition that poses significant surgical challenges in neonates. Understanding the unique presentation and management strategies in such cases is crucial for improving patient outcomes.

Case presentation

We report two cases involving full-term neonates presenting with severe abdominal distension and bilious vomiting. Radiologic studies confirmed intestinal obstruction due to multiple jejunoileal webs. The first case was managed with jejunoileal resection and anastomosis, while the second case underwent web excision with jejunoileoplasty. Despite successful surgeries, both patients faced significant postoperative complications, leading to fatal outcomes.

Clinical discussion

These cases illustrate the complexity of managing type 1 jejunoileal atresia with multiple webs, emphasizing the importance of early diagnosis, meticulous surgical intervention, and comprehensive postoperative care. The condition, while rare, requires a tailored approach to optimize surgical outcomes and patient survival.

Conclusion

The cases highlight the critical need for standardized treatment protocols and vigilant postoperative monitoring in managing neonatal intestinal obstructions, particularly in rare conditions such as type 1 jejunoileal atresia with multiple webs. This report contributes to the surgical literature by providing insights into the challenges and potential strategies for managing such complex cases.

Highlights

• •Type 1 Jejunoileal Atresia with multiple webs is a rare congenital anomaly in neonatal surgery.
• •Symptomatic neonates present with severe abdominal distension and bilious vomiting, indicating obstruction.
• •Diagnosis is confirmed through radiologic studies, primarily using abdominal X-rays.
• •Surgical options include jejunoileal resection and anastomosis or web excision with jejunoileoplasty.
• •Postoperative complications are common due to the complexity of the condition, despite successful surgery.

1. Introduction

Jejunoileal atresia is a congenital anomaly causing small bowel obstruction in approximately 1 in 5000 live births. Factors such as prematurity, maternal exposure to specific medications, and familial inheritance heighten the risk. Notably, intrauterine ischemic insult is implicated in its pathogenesis, disrupting mesenteric blood supply and leading to atresia [1,2].

The cases presented in this report are significant due to their rarity and the complexity of surgical management required. While jejunoileal atresia is well-documented in pediatric surgery, the occurrence of multiple webs in Type 1 jejunoileal atresia is exceedingly rare [3,4]. Reporting these cases adds valuable insights to the existing literature, particularly in understanding the surgical challenges and outcomes associated with such anomalies.

The management of jejunoileal atresia, particularly in cases involving multiple webs, requires adherence to established surgical guidelines and best practices. The current standards of care recommend early surgical intervention to prevent complications such as bowel perforation and sepsis [[1], [2], [3], [4]]. The literature on jejunoileal atresia provides various surgical approaches, yet there is limited data on outcomes in cases involving multiple webs, underscoring the importance of this case report.

This work was reported in accordance with the 2023 SCARE criteria [11].

2. Case presentation

2.1. Case one

A 3-day-old male neonate born at 37 weeks of gestation, with a birth weight of 2700 g. The baby was delivered vaginally by a midwife at a regional public hospital and referred to our hospital due to progressive abdominal distension and vomiting.

The patient presented with severe abdominal distension and frequent bilious vomiting since birth. The first passage of stool occurred on the second day of life, which was pale yellowish. There was noticeable abdominal venectation, and the intestinal contour was visible.

Antenatal care was regularly performed by a midwife, with no abnormalities detected on prenatal ultrasound. Upon admission, routine blood tests were within normal limits except for elevated serum bilirubin, particularly the indirect fraction.

On examination, the abdomen was markedly distended with visible dilated veins but no redness. The contour and movement of the intestines were clearly visible through the abdominal wall (Fig. 1). Digital rectal examination revealed pale stool with no forceful gas expulsion.

Fig. 1.

Anterior and right lateral clinical image of the patient's abdomen.

Supine and upright abdominal X-rays revealed signs consistent with jejunoileal atresia without evidence of pneumoperitoneum. Given the clinical and radiological findings, a diagnosis of total intestinal obstruction due to multiple jejunoileal webs was made (Fig. 2).

Fig. 2.

Abdominal x-ray of case one, showed multiple bubble sign.

The patient underwent an exploratory laparotomy, revealing jejunal dilatation 12 cm distal from the Treitz ligament and multiple webs across the ileum. Surgical management included jejunoileal resection and tapering, followed by jejuno-jejunal and ileo-ileal anastomosis, leaving a bowel length of 125 cm (Fig. 3). Postoperatively, the patient experienced significant respiratory distress and hemodynamic instability, requiring high-concentration oxygen and inotropic support. Unfortunately, the patient succumbed on postoperative day two despite resuscitative efforts.

Fig. 3.

Intraoperative findings case one: A) Jejunal dilatation at 12 cm distal from ligament of Treitz (Arrow); B) Longitudinal incision at the obstruction shows a jejunal web; C and D) further findings multiple webs were found (Arrow).

2.2. Case two

A 3-day-old male neonate born at 38 weeks of gestation with a birth weight of 2600 g. The patient was delivered at a regional public hospital and later referred to our hospital due to severe abdominal distension.

The neonate presented with significant abdominal distension and bilious vomiting within the first 24 h of life. The first passage of meconium was delayed until 36 h after birth. There was a tense and hyper-tympanic abdomen with visible intestinal contours.

Antenatal care was consistent, with no abnormalities detected on prenatal ultrasound. Laboratory investigations revealed leukocytosis and elevated bilirubin levels.

The patient's abdomen was notably distended with prominent intestinal contours (Fig. 4). Digital rectal examination found no stool or blood and no forceful gas expulsion.

Fig. 4.

Anterior and left lateral clinical image of the patient's abdomen.

Supine and upright abdominal X-rays confirmed jejunal atresia without pneumoperitoneum. This finding was consistent with Type 1 jejunal atresia with multiple webs (Fig. 5).

Fig. 5.

Abdominal x-ray of case two, showed multiple bubble sign with multiple air fluid level.

An emergency laparotomy was performed, revealing jejunal dilatation 15 cm distal from the Treitz ligament, with four webs identified in the ileum. Surgical intervention involved web excision and jejunoileoplasty (Fig. 6). The patient was managed in the NICU postoperatively but developed septic shock, leading to fatality on postoperative day 21 despite intensive care.

Fig. 6.

Intraoperative findings case two: A and B) Multiple web was found (Arrow); C) Web excisions was performed; D) Intestine passage were clear after web excisions.

3. Discussion

In these two cases of Type 1 jejunoileal atresia with multiple webs, the surgical management strategies were tailored to address the unique challenges posed by this rare congenital anomaly. Despite early diagnosis and prompt surgical intervention, both cases resulted in fatal outcomes, underscoring the complexity of managing such conditions in neonates. The findings highlight the critical need for careful surgical planning and vigilant postoperative care.

Type 1 jejunoileal atresia with multiple webs is a rare and challenging condition, with limited cases reported in the literature. Previous studies have documented various surgical approaches, some literature suggests that vertical umbilical incisions provide better exteriorization of the small bowel [2]. Other options such as the laparoscopic approaches may be challenging due to the dilated small intestine and limited working space [1]. Previous case reports involving multiple jejunoileal webs and type 1 jejunoileal atresia have similarly utilized laparotomy, leading to jejunostomy [3,4].

A cross-sectional study of jejunoileal atresia patients treated at a tertiary center from 2001 to 2019 reported that 70 % of patients underwent primary anastomosis [5]. Resection and anastomosis were the most common procedures in another study of 60 cases, but with a lower survival rate compared to tapering enteroplasty with trans-anastomotic tubes [6]. Our approach was consistent with these findings, highlighting the need for careful inspection of the bowel to identify any additional abnormalities and prevent postoperative complications.

In our cases, tapering enteroplasty was utilized to achieve better outcomes. This approach aligns with the findings from a tertiary pediatric surgery center in Western India, which reported better survival rates with tapering enteroplasty compared to simple resection and anastomosis [7].

When considering anastomosis versus enterostomy, the literature suggests that primary anastomosis is preferred to minimize complications associated with enterostomy [8]. Our cases did not warrant enterostomy as there was no evidence of intestinal perforation or meconium peritonitis. Advanced techniques, such as the Jejunal Turnover and Bowel Plication (JTBP) and Kimura technique, have shown promising results in altering the diameter of the proximal bowel and promoting improved postoperative intestinal function [9,10].

Despite literature indicating a 90 % survival rate for jejunoileal atresia, both of our cases resulted in mortality. This underscores the significant challenges in managing this condition, particularly when complicated by multiple webs. Factors such as concomitant infections and severe postoperative complications played a critical role in the adverse outcomes observed.

Future research should focus on developing standardized treatment protocols and exploring advanced surgical techniques to improve outcomes in patients with this rare condition. The integration of multidisciplinary care, including neonatology, pediatric surgery, and intensive care, is essential for optimizing patient outcomes.

The study's strength lies in its detailed case presentation and management of a rare condition. However, the small sample size and retrospective design are limitations that affect the generalizability of the findings.

4. Conclusion

The two cases presented in this report highlight the significant challenges in managing Type 1 jejunoileal atresia with multiple webs. Early recognition, meticulous surgical planning, and vigilant postoperative care are critical for optimizing outcomes. Future research should focus on developing standardized treatment protocols and exploring advanced surgical techniques to improve outcomes in patients with this rare condition.

Consent

Consent to publish the case report was obtained from the parents of the patient. This report does not contain any personal information that could lead to the identification of the patient.

Ethical approval

Ethical approval was not required as this study involves case reports. Consent from patients and their families was obtained for publication. The Ethical Committee at Dr. Hasan Sadikin General Hospital and Dr. Mohammad Hoesin General Hospital has confirmed that ethical approval is not required for case series reports.

Funding

No external funding was received for this study.

CRediT authorship contribution statement

Rizki Diposarosa: Study conception, critical revision.

Emiliana Lia: Analysis and interpretation of data, drafting of the manuscript.

Samuel Amosilo Santoso Kesek: Data acquisition, drafting of the manuscript.

Windi Astriana: Analysis and interpretation of data, drafting of the manuscript.

Guarantor

Rizi Diposarosa.

Research registration number

None.

Declaration of competing interest

All authors have no conflicts of interest to declare.