Introduction
Meconium plug syndrome typically presents as failure to pass meconium within the first 24 to 48 hours of life. Antenatally, it may manifest as progressive dilatation of large bowel loops but is ultimately a diagnosis of exclusion [
1]. In contrast, prenatal diagnosis of jejunal atresia remains challenging. It is suggested by findings such as multiple dilated bowel loops (diameter >7 mm), increased mural thickness (>3 mm), hyperperistalsis, and polyhydramnios [
2]. Intestinal atresias account for approximately 50% of neonatal intestinal obstruction cases, with around 20% associated with chromosomal anomalies [
2]. The proposed etiology involves an in-utero ischemic insult leading to bowel necrosis and subsequent resorption of the affected segment. Prompt diagnosis and management are essential, as undetected cases can deteriorate rapidly after birth. Prenatal identification plays a vital role in guiding postnatal care. We report a rare case of jejunal atresia associated with an unusually long meconium plug, initially suspected antenatally as an anorectal malformation or large bowel obstruction.
Case Report
A 28-year-old unbooked primigravida, married for seven years and pregnant following fertility treatment, was referred at 31 weeks of gestation with severe preeclampsia and anemia. Fetal ultrasound at the referring centre suggested large bowel obstruction or imperforate anus. A repeat scan at our institute confirmed these findings along with fetal growth restriction (FGR) and oligohydramnios. The mother was managed conservatively with antihypertensives and magnesium sulfate until 36 weeks. Due to worsening Doppler parameters, an elective lower segment caesarean section (LSCS) was performed.
A female neonate weighing 1.96 kg was delivered. She cried after bag-and-mask ventilation and was admitted to the NICU for evaluation and management of suspected congenital anomalies. She was kept nil per oral (NBM) and started on intravenous fluids and antibiotics. Clinical examination revealed no external anomalies except for abdominal distension and visible bowel loops in the upper abdomen. An infant feeding tube could not be advanced beyond 5 cm from the anal verge. Imaging including abdominal X-ray and ultrasound showed multiple dilated bowel loops (maximum diameter 2.8 cm) in the right iliac fossa with sluggish peristalsis and normal vascularity. A pediatric surgery consult was obtained.
Per-anal warm saline and Mucomix washes, as advised, expelled a 16 cm long meconium plug [Fig.1,2]. Screening for cystic fibrosis using whole genome sequencing was negative. Despite this, barium enema and repeat ultrasound continued to suggest features of small bowel obstruction. On day 4 of life, the baby underwent exploratory laparotomy under general anesthesia for persistent abdominal distension and failure to pass stool. Intraoperatively, a Type I jejunal atresia was identified 30 cm distal to the duodenojejunal (DJ) junction. The proximal jejunum was grossly dilated, while the distal segment was collapsed. A 20 cm segment, including the atretic portion, was resected, and an end-to-end jejunojejunal anastomosis was performed.
Postoperatively, the neonate required inotropic support, blood product transfusions, and escalation to broad-spectrum antibiotics. Despite intensive care, the baby developed septic shock and disseminated intravascular coagulation and succumbed on day 7 of life.
Discussion
Meconium plug syndrome was first described by Clatworthy in 1956 as a cause of functional colonic obstruction in neonates due to inspissated meconium [
1]. It is relatively common, occurring in approximately 1 in 500 live births [
2]. While usually benign and self-limiting, it can mimic more serious conditions such as Hirschsprung disease or distal intestinal atresia. In a case series of 25 neonates with meconium plug syndrome, 24% were found to have mutations associated with cystic fibrosis [
3]. However, in our case, whole genome sequencing ruled out any CFTR gene mutations, excluding cystic fibrosis as an underlying cause. Jejunal atresia, often grouped under jejunoileal atresia (JIA), occurs in approximately 1 in 12,000 live births [
4]. It is believed to result from a late intrauterine ischemic insult causing necrosis and resorption of the affected bowel segment [
5]. JIA is associated with other anomalies such as cardiac defects, gastroschisis, and cystic fibrosis. Antenatal diagnosis of intestinal atresia remains challenging, with detection rates ranging from 25% to 90% depending on gestational age, site of obstruction, degree of bowel dilatation, presence of polyhydramnios, and operator expertise [
6].
In our case, antenatal imaging suggested a large bowel obstruction, possibly due to imperforate anus or meconium plug syndrome. The presence of oligohydramnios, however, was unusual in isolated meconium plug syndrome and should have raised suspicion of a proximal obstruction. Postnatal evaluation confirmed a Type I jejunal atresia located 30 cm distal to the duodenojejunal (DJ) junction, with an unusually long meconium plug contributing to the diagnostic confusion. While jejunal atresias are associated with cystic fibrosis in up to 20% of cases [7], this was not observed in our patient. The definitive management of intestinal atresia involves resection of the atretic segment and primary anastomosis. Although the surgical procedure was successful, our neonate succumbed to postoperative sepsis, possibly exacerbated by low birth weight and borderline prematurity. This highlights the critical importance of accurate prenatal diagnosis, which allows for timely referral, appropriate parental counselling, and planned delivery at a tertiary centre with neonatal intensive care and pediatric surgical support.
Conclusion
This case emphasizes the diagnostic complexity of jejunal atresia coexisting with an unusually long meconium plug, especially in the absence of cystic fibrosis. The misinterpretation of prenatal findings as large bowel obstruction emphasizes the need for a high index of suspicion and multidisciplinary evaluation in suspected fetal intestinal obstruction.
Contributors: Conception and acquisition of the work done by AZ. Drafting and substantively revising done by PV and NB. PV will act as a study guarantor. All authors approved the final version of this manuscript and are responsible for all aspects of this study.
Funding: None; Competing interests: None stated.
Acknowledgments: We acknowledge Department of Radiology, Paediatrics and Paediatric surgery, GMCH Nagpur for their support.
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