CASE REPORTS Congenital Diaphragmatic hernia presenting in adult life: A study of two cases

Congenital Diaphragmatic hernia (CDH) usually presents in the newborn and rarely present as undetected cases in adult life. Defects in the diaphragm can cause abdominal organs to herniate into the thoracic cavity resulting in respiratory distress or strangulation of abdominal organs. Reported here are 3 cases of congenital CDH presenting in adult life. A 59-year-old male presenting with epigastric pain, bilious vomiting and constipation demonstrated an air-fluid level in the anterior mediastinum on chest radiograph. Surgery revealed an anterior Morgagni defect with a loop of terminal ileum herniated and strangulated into the pericardial sac. A15-year-old girl presenting with abdominal pain and pyrexia for 2 days, on CT chest and abdomen revealed right lobe of liver and transverse colon in the right hemithorax with the pericardium pushed to the opposite side. Surgery revealed a large right sided Bochdelaks type hernia. A strangulated gangrenous transverse colon was removed from within the thoracic cavity. In these cases the body had adapted by maintaining total lung volume by compensatory hyperplasia of the contralateral lung allowing the patients to survive with no symptoms for a long duration of time. Even though rare, the possibility of CDH presenting as acute abdomen is important to remember.


Introduction
Congenital diaphragmatic herniae (CDH) are a relatively rare group of disorders occurring in 1-5: 10000 births 1 . CDH is usually diagnosed soon after birth or during childhood mostly because its association with lung hypoplasia which leading to dyspnoea early in life.
However cases have been reported where patients have lived with such defects without any complications to adult life 2,3 . Here we report the clinical findings and outcomes in two such cases of CHD which survived to adult life undiagnosed.
The diaphragm develops from four main structures: septum transversum, pleuroperitoneal membrane, dorsal Bochdalek hernia occurs in approximately 85% of cases. Left-sided hernias allow herniation of both the small and large bowel and intra-abdominal organs into the thoracic cavity. In right-sided hernias which account for 13% of cases, the liver and parts of the colon herniate into the thoracic cavity.  A 15 year old girl presented to surgical casualty with severe abdominal pain and difficulty in breathing that had developed rapidly over the preceding 8 Hrs. She had been having on-off abdominal pain related to meals over the past several years. Previously she had been investigated for eventration of the right hermidiaphragm in a specialist thoracic unit conservatively. On admission she was ill febrile tachycardic and tahypnoic. Right sided air entry was reduced and percussion note dull.
The abdomen was soft and non-tender. Chest X ray PA revealed a large loop of large bowl in the right hemithorax and a diffuse opacity in the right lower zone (Figure 2). CT scan confirmed the presence of bowel in the right hemithorax and the lower zone opacity was found to be the right hemi liver (Figure 3).
Following resuscitation, emergency laparotomy was performed. A modified rooftop incision was employed to gain access to the upper abdomen. A Bochdalek type posterior defect was found in the right hemidiaphragm. Through this defect the right hemi liver had hermiated into the right hemithorax along with the entire transverse colon, which was gangrenous. The right hemiliver was adapted to fit the cavity of the right hemithorax indicating that this anatomy may have been present for a long time. The diaphragmatic defect was enlarged. The right hemi-liver was delivered into the abdomen after division of its anchoring ligaments whilst safeguarding the vascular architecture. The  In the second case which was a Bochdalek type hernia where the liver and the small bowel had herniated into the thorax, the body had adapted by maintaining total lung volume by compensatory hyperplasia of the contralateral lung. Bochdalek hernia occurs due to incomplete fusion of the pleuroperitoneal membrane posterolaterally.
This type of hernia in invariably diagnosed at birth or infancy due to respiratory symptoms.
Since the left side of the pleuroperitoneal membrane closes last left sided defects are commoner. However in the case described here the defect was right sided and was diagnosed at the age of 15 years. It was fascinating to find the liver molded to the shape of the thoracic cavity. It appeared that the herniation of bowel loops had occurred as an acute event giving rise to symptoms.
Even though rare, the possibility of CDH presenting as acute abdomen in later life is important to remember.