Congenital hernia of umbilical cord with patent vitellointestinal duct along with evisceration of gut—a rare presentation and unusual management

Abstract

Background Congenital hernia of umbilical cord is a rare congenital anomaly. Due to its presentation as herniation of small bowel or other viscera into the umbilical cord, it is often misdiagnosed as omphalocele minor. Case presentation We are reporting a rare case of congenital hernia of umbilical cord which presented with rupture of hernia sac and evisceration of small bowel loops with reverse ileal intussusception via a patent vitellointestinal duct. It was managed by first repositioning the gut with stoma at umbilical site as a temporizing measure followed by definitive surgery in the form of resection of patent vitellointestinal duct and end to end ileal anastomosis. Conclusion Congenital hernia of umbilical cord with patent vitellointestinal duct is a rare abdominal wall defect which can present beyond neonatal period with evisceration of gut. A high index of suspicion should be kept by the first attending clinician to avoid a delay in management.

Distinct presentations and management of hernia of the umbilical cord: 15 years’ experience in a tertiary hospital

“…In our experience and that of others, HUC can be accompanied with additional anomalies such as congenital short gut, PVID, and intestinal, ileal, and colonic atresia as a result of intrauterine vascular accidents such as intussusception and volvulus [3]. In seldom cases, fetal demise is reported due to the rupture of the umbilical cord that led to in utero bowel evisceration and umbilical vessel rupture [16].…”

“…The umbilicus is a place for a number of embryopathies, including the urachus, blood vessels, midgut herniation, anterior abdominal wall abnormalities, and congenital cysts. During embryological development, the anterior abdominal wall is composed of caudal, cephalic, and two lateral folds; failure of four folds to meet in midline results in the formation of an umbilical defect [1][2][3]. Abdominal wall abnormalities encompass a wide range of congenital malformations that range from fatal limbbody wall syndrome to benign hernia of the umbilical cord (HUC) [4].…”

“…Umbilical abnormality can be diagnosed with certainty after delivery, but antenatal ultrasonography (USG), amniocentesis, karyotyping, and amniotic fluid alphafetoprotein (AFP) can be helpful in determining these abnormalities as HUC, omphalocele, and cord teratoma [4]. The complications accompanying HUC include intestinal malrotation, obstruction of the bowel, atresia, and trauma to the bowel caused by accidental umbilical cord clamping [3].…”

Congenital Hernia of the Umbilical Cord: A Retrospective Case Study

“…6,9 A thin strip of normal skin always enwraps the umbilical ring and proximal part of the cord in the case of CHUC. 10 CHUC with patent omphalomesenteric (OMD) anomaly was reported by Raicevic et al in their case report on neonates. 2 In their study, they described the intraoperative identification of patent OMD in CHUC.…”