OBSTRUCTED CONGENITAL HERNIA OF UMBILICAL CORD IN A NIGERIAN NEWBORN AND ITS DIAGNOSTIC CONUNDRUM

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A.A Ishola¹, K.I Egbuchulem², D.I Olulana^2,3

1. Division of General Surgery, Department of Surgery, University College Hospital, Ibadan.
2. Division of Paediatric Surgery, Department of Surgery, University College Hospital, Ibadan.
3. Department of Surgery, University of Ibadan, Ibadan.

Abstract

Background: Congenital Hernia of umbilical cord (CHUC) is a rare presentation. The failure of return of intestinal loops following physiological herniation has been postulated as the embryological basis of this condition. This is usually mistakenly termed as omphalocele minor. We aim to highlight this rare finding, the clinical features, diagnostic dilemma, and management of CHUC.

Case Presentation: He is a 4-day old male with obstructed CHUC. He had an oedematous umbilical stump, with circumferential normal abdominal wall skin folds in the form of a collar wrapping round the proximal aspect of the protrusion. The umbilical defect measured about 4 cm in diameter. Subsequently, surgical reduction and repair of defect was done, with resolution of symptoms.

Conclusion: CHUC though a rare entity and easily missed, accurate diagnosis and management offer good prognosis. Also, recognition will prevent iatrogenic bowel injury from careless clamping of the cord with associated morbidity and mortality. It also helps to prevent mis categorization and prevent unwarranted anxiety with misdiagnosis.

Keyword: Congenital hernia, Physiologic herniation, Umbilical defect, Bowel injury

Introduction

Congenital hernia of the umbilical cord (CHUC), also known as congenital hernia of the cord or umbilical cord hernia, is a rare anomaly which is often misdiagnosed as omphalocele minor and an underreported entity.^1–3 It is the rarest form of the anterior abdominal wall defects and results from failure of return of the physiologically herniated bowel loops. These bowel loops ideally should return into the abdominal cavity around 10 – 12 weeks of gestation. However, persistence of a patent umbilical ring predisposes to herniation of bowel into the umbilical
cord. This is unlike omphalocele which results from failure of fusion of the four body wall folds and progression of the lateral body wall folds to the midline.^4,5 CHUC usually is not associated with any chromosomal or other organ anomalies unlike in omphalocele that could be syndromic.^4,5 It could be confused with cord hematoma, cord cyst, urachal cyst, giant cord or cord teratoma during antenatal scans and postpartum.^1,4 However, it is characterised by aproximal cord swelling, covered by a circumferentialstrip of normal skin which always enwraps the umbilical ring and a variable length of the proximal part of the cord.⁴

A number of complications can be associated with CHUC which include intestinal malrotation and obstruction.^4–7 Also, trauma to the intestines from accidental clamping of the cord over a herniating bowel loop due to uncharacteristic appearance or lack of awareness of CHUC among health care workers.^1,2 It is also thought that misdiagnosis could also cause unwarranted anxiety among parents. We report a case of obstructed congenital hernia of umbilical cord in a new-born male, highlighting its characteristic presentation thus creating awareness to prevent misdiagnosis and untoward complications of iatrogenic bowel injury.