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

ABSTRACT


INTRODUCTION
Several prenatal intestinal events may necessitate surgical intervention shortly after birth; among them are prenatal volvulus, meconium ileus (MI), or intestinal ischemia.These events can result in perforation with meconium peritonitis and in some cases, the bowel necrosis causes jejunal or ileal atresia (IA).Additionally, in-utero intussusception has similar consequences, albeit relatively uncommon (less than 2% of jejunal or ileal atresias) [1].

CASE REPORT
A male newborn, at 36+1 weeks of gestation, was transferred to our NICU from a rural hospital following a Cesarean section due to suspected hydrops.The fetus had developed ascites and gained about 1 kg within the prior week.After birth, the abdomen was enormously distended, however, other features of hydrops were absent.A pigtail catheter was placed in the left lower quadrant draining more than 100 ml of clear yellow liquid.An abdominal radiograph showed only a few air-filled bowel loops (Fig. 1).Due to suspicion of a surgical cause, the patient was urgently planned for laparotomy.Upon opening the peritoneum, more fluid gushed out and the bowel exhibited typical coatings for meconium peritonitis.A type IIIa IA was discovered 66 cm after Treitz's ligament; the distal part measured 23 cm up to the ileocecal valve.The colon and the remaining abdomen appeared normal apart from the meconium peritonitis.The discrepancy difference between the proximal and distal bowel at the atresia was approximately 3:1.The configuration of the ileal atresia was remarkable: while the proximal end was completely closed, the distal part was invaginated (Fig. 2); upon enterotomy, the invaginated part continued into a dry green tissue (Fig. 3).The distal ileum contained hard fragments of meconium reminiscent of meconium ileus.These intraoperative findings in conjunction with the prenatal ascites may fit the diagnosis of an intrauterine intussusception (IUI) with the subsequent necrosis of the affected bowel and perforation, or a complicated meconium ileus with additional intrauterine intussusception.A short segment of the ileum was resected on both ends and an ileostomy was created; a primary anastomosis was avoided due to the meconium peritonitis and edema.The postoperative period was uneventful.After a contrast study of the distal limb, chyme re-feeding was started and well-tolerated.Cystic fibrosis was suspected due to the hard meconium plugs but could be excluded by the sweat test.The stoma was reversed at three months, without any further complications.

DISCUSSION
Fetal ascites may represent the first and only detected sign of an IUI, however, it is not specific [2].Unlike other cases, there was no significant bowel dilatation noted both prenatally on ultrasound and also not on the abdominal radiograph after birth in our patient.This suggests that the event likely occurred later in pregnancy.However, the exact etiology and order of the separate pathologies in this case may not be ascertained.Perforation and meconium peritonitis are typical complications of meconium ileus, with the IUI potentially being a concurrent condition.Conversely, apart from the IUI, we did not identify any sites of (occult) perforation.
While there are reports and series documenting intrauterine intussusception (IUI), many cases remain undetected during pregnancy and for several hours or even days after birth until symptoms of bowel obstruction manifest [3].The most frequently reported subsequent types of ileal atresia are type II and IIIa [4].Similar cases involving IUI, ileal atresia (IA), and ascites/peritonitis are summarized in Table 1.Despite several reports of IUI and IA, only a few were accompanied by perforation and/or peritonitis.Specifically, there are only five cases documented with prenatal ascites, of which three had transient ascites and two, like our case, had accumulating ascites [2,5].No cases involving both IUI and meconium ileus (MI) were found.Furthermore, some reports of neonatal intussusception in the presence of a meconium plug have been published, indicating a potential relationship between intraluminal meconium obstruction and the development of intussusception.
IUI is generally considered a rare cause of intestinal atresia, while vascular accidents or a volvulus being more common.The diagnosis of IUI and its sequelae is usually only made at the time of laparotomy [6], although prenatal ultrasound findings like fetal ascites, pseudocysts, calcifications, or dilated bowel loops indicate an intestinal obstruction that may require surgery postnatally.Therefore, parents with these prenatal findings should be counseled to deliver at a center with available pediatric surgery services [7].
This case underscores the association between fetal ascites, intrauterine intussusception (IUI), and meconium peritonitis, while also emphasizing the favorable prognosis similar to that observed in cases of intestinal atresia with prompt management.photographs/material (if any used), from the legal guardian of the patient with an understanding that every effort will be made to conceal the identity of the patient, however it cannot be guaranteed.
Author Contributions: Author(s) declared to fulfil authorship criteria as devised by ICMJE and approved the final version.

Figure 1 :
Figure 1: Abdominal radiograph with air-filled bowel loops and ascites despite drainage with pigtail catheter (*)

Table 1 :
Similar cases with IUI and IA.Abbreviations: GA = gestational age, FT = full term, PT = preterm, NPU = no prenatal ultrasound