The reported incidence of congenital hernia of umbilical cord (CHUC) is low [1 in 5000] as compared to postnatal umbilical hernia, partly because most of these are misdiagnosed as omphalocele minors . Embryologically, during early fetal life, a greater portion of the intestine lies in the proximal part of the umbilical cord which is called as extracelomic cavity. Normally, the intestines withdraw into the abdominal cavity at about 10‑12 weeks gestation, the umbilical ring closes and the extracelomic cavity is thereby obliterated. In rare cases, the umbilical ring does not close and in these situations variable portions of the intestines remain in the extracelomic cavity, which persists as congenital hernia into the umbilical cord. So, the CHUC is a separate entity which is distinct from other anterior abdominal wall anomalies such as gastroschisis and omphalocele . The characteristic features of CHUC are as follows: an intact umbilical ring, absent abdominal wall deficiency, a cuff of skin from one half to one inch wide, which extends from the abdominal wall onto the neck of the sac which comprises of an outer layer of amnion and inner peritoneum and its contents varying from loops of the intestine to any movable intraperitoneal organ and even liver and gall bladder as contents have been reported [7, 8].
The associated anomalies in hernia of the cord are PVID; congenital short gut; and intestinal atresia, ileal, and colonic atresias probably as a consequence of intrauterine vascular accidents such as volvulus, intussusception . In rare instances, even fetal demise has been reported owing to rupture of umbilical cord that led to in utero bowel evisceration and rupture of umbilical vessels . The present case presented with evisceration of gut via patent vitellointestinal duct and with reverse intussusception even in post neonatal period. This case has been described as a case of CHUC because of history of swelling over umbilicus and a small umbilical defect; however, it is difficult to rule out the omphalocele minor. By reporting this case, the authors aim to highlight the aspect of management of such a presentation. There are reports where the umbilical cord hernia has been overlooked at the time of birth and even clamped resulting in ischemia and injury of intestines . Even meconium discharge from patent vitellointestinal duct in case of CHUC has also been reported as in the index case . Gasparella et al. reported a case of an intestinal laceration due to clamping of an unrecognized herniation of umbilical cord in a newborn . Although in the index case, cord clamping had not resulted in injury of intestine but callous umbilical cord clamping have been observed in literature .