Abstract

Congenital diaphragmatic hernias (CDHs) occur mainly in two locations: the foramen of Morgagni and the more common type involving the foramen of Bochdalek. Hiatal hernia and paraesophageal hernia have also been described as other forms of CDH. Pulmonary hypertension and pulmonary hypoplasia have been recognized as the two most important factors in the pathophysiology of congenital diaphragmatic hernia. Advances in surgical management include delayed surgical approach that enables preoperative stabilization, introduction of fetal intervention due to improved prenatal diagnosis, the introduction of minimal invasive surgery, in addition to the standard open repair, and the use of improved prosthetic devices for closure.

Highlights

  • The estimated incidence of congenital diaphragmatic hernia is 1 in 2000–5000 live births [1].The aetiology of congenital diaphragmatic hernia is unknown, 2% of cases have been noted to be familial and another 15% of patients have associated chromosomal abnormalities [2].Pulmonary hypoplasia is an important defect in congenital diaphragmatic hernia [2], and the severity of this pathology is largely dependent on the degree of pulmonary hypoplasia, pulmonary hypertension, and associated malformations.There have been recent advances in the medical management and postnatal care, all targeting the primary pathophysiological mechanism, and in the surgical management with the introduction of fetal interventions, laparoscopic repair, and lung transplantation

  • We aim to update clinicians in the recent advances in the diagnosis and surgical management of patients who present with Bochdalek hernia and Morgagni hernia

  • Bochdalek popularized the concept of herniation due to failed closure of the primitive communication between the pleura and the peritoneal

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Summary

Introduction

The estimated incidence of congenital diaphragmatic hernia is 1 in 2000–5000 live births [1].The aetiology of congenital diaphragmatic hernia is unknown, 2% of cases have been noted to be familial and another 15% of patients have associated chromosomal abnormalities [2].Pulmonary hypoplasia is an important defect in congenital diaphragmatic hernia [2], and the severity of this pathology is largely dependent on the degree of pulmonary hypoplasia, pulmonary hypertension, and associated malformations.There have been recent advances in the medical management and postnatal care, all targeting the primary pathophysiological mechanism, and in the surgical management with the introduction of fetal interventions, laparoscopic repair, and lung transplantation. The aetiology of congenital diaphragmatic hernia is unknown, 2% of cases have been noted to be familial and another 15% of patients have associated chromosomal abnormalities [2]. Pulmonary hypoplasia is an important defect in congenital diaphragmatic hernia [2], and the severity of this pathology is largely dependent on the degree of pulmonary hypoplasia, pulmonary hypertension, and associated malformations. The treatment focus has changed from emergency surgery at birth or presentation to surgical closure of defect after stabilization of the patient. Despite all these advances in neonatal care and surgical management, congenital diaphragmatic hernia (CDH) remains a condition with a significantly high mortality rate [3]

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