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A Triad of Congenital Diaphragmatic Hernia, Meckel’s Diverticulum, and Heterotopic Pancreas

DOI: 10.1155/2014/725945

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Abstract:

Congenital diaphragmatic hernia is a common developmental anomaly encountered by paediatric surgeons. It is known to be associated with extradiaphragmatic malformations, which include cardiac, renal, genital, and chromosomal abnormalities. Herein, we report a newborn born with concurrent congenital diaphragmatic hernia, Meckel’s diverticulum, and heterotopic pancreatic tissue. This is the first case report of such a triad with description of possible mechanisms of the development. 1. Introduction Congenital diaphragmatic hernia (CDH) is relatively a common anomaly that occurs in 2.4 per 10,000 live births [1]. The survival of patients with CDH is dependent on pulmonary hypoplasia and pulmonary vascular abnormality, but association of extradiaphragmatic malformations also plays a vital role in the outcome. The incidence of 1 or more extradiaphragmatic associated anomalies with CDH has been reported as 46% and considered as a major contributory factor in survival of babies born with CDH [2]. Commonly reported extradiaphragmatic associated anomalies with CDH include cardiac (63%), pulmonary sequestration, renal and genital anomalies, neural tube defects, and chromosomal abnormalities [2, 3]. Meckel’s diverticulum (MD), a developmental anomaly of omphalomesenteric duct, is also common in children and the reported incidence is around 2% in the general population. Approximately 4% of patients with MD become symptomatic and require surgical excision [4]. Heterotopic pancreas (HtP), that is, the pancreatic tissue lacking anatomical and vascular continuity with the main body of gland, has an incidence of 2–15% in cadaveric studies and 0.5% in the clinical studies [5]. HtP in pediatric age group is not common and may cause a diagnostic dilemma when it leads to complications [5–7]. The association between CDH and MD [8] and also MD associated with HtP has also been reported [5]. However a concurrent association between CDH, MD, and HtP has not been reported previously and we present first case report of such a triad. We have reviewed the possible molecular embryological mechanism of the development of this triad and have also discussed the management. 2. Case Report A 35-week preterm male neonate was born to a 29-year-old healthy mother. Antenatal ultrasound at the 32-week gestation showed left-sided CDH with no additional anomalies noted on scan. After delivery, the baby was intubated and further evaluation confirmed left CDH and no other associated malformations. On the 3rd day of life, after stabilization, he was taken to operating room for repair of his left

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