A case of congenital diaphragmatic hernia with malrotation of gut, delayed presentation in adult

Pitchika Sm,Chowdary Dbp,Mallipudi Bvp

doi:10.17727/jmsr.2021/9-35

Abstract

Congenital diaphragmatic hernia (CDH), which mainly occurs in the new born or in childhood with severe respiratory distress and is associated with high mortality, is rarely found in adult. Pulmonary hypoplasia and pulmonary hypertension contribute to the high mortality and morbidity due to CDH. These patients adjust their lifestyle to manage symptoms associated with frank herniation of the large bowel, omentum, and other viscera inside the diaphragmatic hernia. We report a 25-year-old male who presented with shortness of breath on exertion diagnosed as having CDH. The disease was detected using chest X-ray (CXR) and was confirmed on contrast enhanced CT scan. A defect was noted in the posterolateral right diaphragm with omentum, small bowel, caecum, appendix, and colon herniating through it with malrotation (incomplete rotation) of gut. CT scan and CXR were the two useful methods in diagnosis of CDH in this patient, but sometimes becomes challenging because of rare incidence and types prior to surgery. For better outcome of CDH patients long term follow up and more investigations are needed. In some cases, where conventional CT scan and ultrasonography fails to diagnose a case of CDH & malrotation of gut, diagnostic laparoscopy plays a key role in facing such situations. Keywords: diaphragmatic hernia; malrotation of gut

Highlights

Congenital diaphragmatic hernia (CDH) occurs due to incomplete muscularization of the diaphragm
Incidence of CDH is 1:2500 to 1:3000 live births, and increase of mortality when pulmonary hypoplasia and pulmonary hypertension are associated with CDH [1]
Due to the complexity of congenital diaphragmatic hernia, the factors involved in the development of Bochdalek hernia are unknown; at present it is thought to be due to the lack of closure of pleural and peritoneal cavity due to disruption of molecular signalling during organogenesis around 9th to 10th weeks of gestation [7–10]

Summary

Introduction

Congenital diaphragmatic hernia (CDH) occurs due to incomplete muscularization of the diaphragm. In 15–20% of cases with CDH, the hernia occurs on the right side and in 80–85% of subjects on the left side. We have reported an adult patient with left congenital diaphragmatic hernia, presenting with respiratory symptoms. We reported an adult patient with incomplete rotation of gut presenting incidentally at the time of repair of CDH. Case presentation 25-year-old male patient presented with shortness of breath on exertion for 4 months To confirm the diagnosis and have precise knowledge of the herniating viscera, axial cross-sectional chest CT scan was done with intravenous and oral contrast This confirmed CDH in the left hemi diaphragm with omentum, small bowel, and colon herniating into thoracic cavity (Figures 1, 2). Chest X-ray was normal (Figure 8) on sixteenth postoperative day

Findings

Discussion

Conclusion