Abstract
This report described the characteristics and management of a rare condition, a segmental dilated small intestine, which has a mimic-liver appearance in giant omphalocele. After the dilated intestine was resected and end-to-end anastomosis was performed, the abdomen could be closed simultaneously without immediate complications. We reviewed the literature on congenital segmental intestinal dilatation. Although the nature of this condition is still unknown, all reports showed the same excellent operative outcome. Finally, awareness of congenital segmental intestinal dilatation in giant omphalocele leads to proper management.
DOI
10.56808/2673-060X.5487
First Page
Case report An eighteen-year-old mother gave birth to a male 39 weeks of gestational age newborn by vaginal delivery. He was diagnosed with omphalocele (Fig 1) without abnormal features and other anomalies. The content of the omphalocele was a pinky appearance like liver, pre-operatively. The primary closure operation seems possible according to his body's large proportion compared to the omphalocele. After adequate resuscitation, he had the surgery on the first day of his life. After dissecting the amniotic membrane away from the abdominal wall, the omphalocele content was the segmental dilated small bowel. The proximal to distal small bowel diameter was 10 to 5 millimeters. The remaining small bowel length was 100 centimeters. The segmental dilated bowel was 10 centimeters distally to the Meckel diverticulum. The intestine was incomplete rotation, the cecum and the appendix were in the right upper quadrant, and the DJ junction was located in the right upper quadrant. A thin band adhesive between the cecum and the dilated bowel caused a narrowed mesentery base. (Fig 2) The dilated segment was resected, end to end anastomosis was done. The segmental dilated bowel contains meconium. The Meckel diverticulum was resected. An appendectomy was performed. The band between the cecum and cyst was divided to widen the mesenteric base. The small bowel was placed in the right-side abdomen, and the large bowel was left in the left-side abdomen. Primary fascial abdominal closure was performed along with umbilicoplasty (Fig 3a). The baby started a feed on the fifth postoperative day and gradually full feed a week later. There was no sign of intestinal dysfunction. He was discharged home two weeks later. His well-being was proper and unevenly well at eight months (Fig 3b) and two years old at the late time. The pathologic result showed a segmental cystically dilatated small bowel, filled with meconium content, measuring 6*5.5*5 centimeters, attached to the middle part of the herniated umbilical cord. (Fig 4) The histologic revealed unremarkable small bowel mucosa and muscularis propria, containing ganglion cells in the myenteric and submucosal plexuses. An appendix is also unremarkable and contains ganglion cells in the myenteric and submucosal plexuses. The heterotopic tissue is not seen in the Meckel diverticulum and dilatated small bowel.
Last Page
Discussion We reported a rare co-incidental finding between a congenital segmental intestinal dilatation and a giant omphalocele. In this case, the omphalocele content appears similar to the liver. Furthermore, the size of this omphalocele is controversial in some points of operative management. After scarification, the delayed abdominal closure may be suitable for liver contenting large omphalocele to avoid the risk of anesthesia in the neonatal period and operative complications, especially the postoperative intra-abdominal hypertension due to excessive pressure from the significant liver. On the contrary, a segmental dilated small bowel must be operatively resected. This abnormal bowel etiology is uncleared. This abnormal bowel etiology is uncleared. The characteristic of this lesion is abruptly changing approximal 3 to 5 times in diameter and then returning to a standard size, and the internal lumen is patent but usually functional obstruction(1–3). The diagnosis criteria for congenital segmental intestinal dilatation are(4)10): (a) limited bowel dilatation with a three- to four-fold increase in size; (b) a sharp transition between the dilated and normal bowel; (c) no intrinsic or extrinsic barrier distal to the dilatation; (d) a clinical picture of intestinal occlusion or sub-occlusion; (e) normality of the neuronal plexus; (f) complete recovery after resection of the affected segment. Congenital segmental intestinal dilatation can be an isolated lesion with a challenging diagnosis and mandatory differentiation from other common causes of intestinal obstruction, such as intestinal atresia(5). Hirschsprung's disease(5) or gut duplication(4) Segmental intestinal dilation with omphalocele has been mentioned. Although mostly, the entry dilated bowel was tapped in the omphalocele sac(1,5,6). However, in some cases, the dilated bowel was in the abdomen(4,7). Irving and Lister(1)proposed that the involved dilated segment in the omphalocele sac could result from entrapment from the umbilical ring or this lesion itself causing omphalocele. This possibility explained the etiology in other isolated lesions caused by temporally intestinal obstruction at an early stage of intestinal development (before intestinal involution). Due to the location of segmental bowel dilatation with omphalocele primarily found in the terminal ileum, there was some evidence of a congenital band adhered to the dilated bowel (4,6). Some authors proposed that congenital segmental intestinal dilatation is a variation of Meckel's diverticulum (4,6,8). Our finding has not supported this theory, even though we found the band adhered between the cecum and the dilated bowel. Still, we also found Meckel's diverticulum 10 centimeters proximal to the segmental dilatation. In addition to omphalocele, other associated anomalies have been reported, such as esophageal atresia(5), neural tube defect(1), congenital heart disease(3), duplication cyst(2), Hirschsprung disease(5), intestinal atresia (3,9) and malrotation(8,9) The histopathological finding of the segmental intestinal dilatation also showed a normal mucosal and muscular layer(5). The heterotopic tissues such as cartilage(1), pancreatic(8), or gastric tissue(5) were rarely found in the dilated bowel(1). All the reported cases have no evidence of aganglionosis and neuronal intestinal dysplasia(1–8). The congenital segmental intestinal dilatation has been found in many locations of the alimentary tract, occasionally ileum(1,5) and colon(3), but rarely in the duodenum and other parts of the intestine(3). The clinical presentation depended on the intestinal location. Moreover, the onset of the clinical relies on the length of the involved segment. Dysmotility bowel mainly causes clinical intestinal obstruction during the neonatal period (1–3). Retro-sigmoid lesions have to different from Hirschsprung's disease. Terminal ileal lesion led to miss diagnosis as total colonic aganglionosis or intestinal atresia (5). Some are un-eventually incidental findings in laparotomy (5). One patient presented with clinical midgut volvulus (8). The recommended operative management for congenital segmental intestinal dilatation is completely resected the involved bowel and restorative intestinal continuity(1–9). In the case of congenital segmental intestinal obstruction with omphalocele, complete resection with end-to-end anastomosis at the same time as the closure of the abdomen is acceptable with minor complications(4,6). A small omphalocele patient suffered from a partial intestinal obstruction recurrence due to remaining segmental intestinal dilatation(7). If the complete resection is impossible, the tapering procedure results in proper bowel function has been reported (3). A patient abdominal distention could be temporality improved after irrigation with gastrografin enema. However, surgical intervention was finally required(1). Besides congenital segmental intestinal dilatation and omphalocele, the malrotation in omphalocele is a challenge. Intestinal malrotation and non-rotation have been found to be associated with omphalocele and gastroschisis. The recommendations of management are varied. In this case, the content in omphalocele has only congenital segmental intestinal dilatation. The rest of the intestine is grossly normal. After resection of the dilatation bowel, the final position of the cecum and DJ was in the same position causing a narrowing mesenteric base compatible with intestinal malrotation. So, we decided to perform the modified Ladd's procedure and appendectomy.
Recommended Citation
Liukitithara, Sirima
(2024)
"The giant omphalocele contenting a surprised congenital segmental intestinal dilatation, a case report and review literature.,"
Chulalongkorn Medical Journal: Vol. 69:
Iss.
1, Article 8.
DOI: https://doi.org/10.56808/2673-060X.5487
Available at:
https://digital.car.chula.ac.th/clmjournal/vol69/iss1/8
