Rare diaphragmatic hernia.
Incidence of 1-3%.
Accounts for 3% of congenital diaphragmatic hernias.
A defect in the diaphragm in the retroxiphoid sternocostal hiatus through which omentum, colon, stomach or other viscera can herniate.
Results from the congenital failure of the diaphragm’s septum tranversum to fuse with costal arches.
Most often present later in life as the diaphragm attenuates.
Defect usually located on the right side at the level of the seventh rib on either side of the xiphoid where epigastric vessels pass.
Left-sided hernia ref2242ed to associated a Larrey hernia.
Differential diagnosis includes, pleuropericardial cyst, mesothelioma of the pleura, pericardial fat pad, thymoma, tumor or cyst of the diaphragm.
Diagnosis of such a hernia requires surgical repair.
Surgery can be performed conventionally associated an open approach through the thorax or via laparoscopic approach.
May be associated with congenital abnormalities including Down’s syndrome, Noonan syndrome, Prader-Willi syndrome, Turner syndrome and pentalogy of Cantrell.
As patients age the diaphragm attenuates and increased abdominal pressure from pregnancy, obesity and trauma leads to presentation.
Patients may be asymptomatic on presentation or have cardiopulmonary symptoms from pressure on intrathoracic organs or pulmonary infections.
Gastrointestinal symptoms may be present as a result of visceral herniation.
Is a direct type hernia and is composed of a sac with omentum, colon, stomach, liver or other gastrointestinal contents.