Presentation
Severe respiratory distress. Generalized skin laxity and redundancy (cutis laxa).
Patient Data
Nasogastric tube (arrow) coiled in the lower part of the left side of the chest above the left diaphragmatic copula.
Gas-filled gastric shadow (arrows) with a nasogastric tube inside, below the level of diaphragm in the abdomen.
Contrast-filled stomach at the left side of the chest above the diaphragm.
Hiatus hernia containing the contrast-filled stomach.
Part of the rectum and sigmoid colonic loops herniating posteriorly to the left ischiorectal fossa.
Case Discussion
Congenital hiatus hernia is a very rare disease and congenital posterior perineal hernia is even rarer and considered one of the least common types of hernias.
The presence of both congenital hernias in the same patient suggests marked tissue laxity as in this case of cutis laxa (CL) which is a general term for a group of rare disorders named after the characteristic skin phenotype; they are multi-systemic disorders and commonly involve the visceral organs.
Repeated herniation and repositioning of the stomach that was noted in the serial follow up x-ray can cause serious respiratory distress and alarms for rapid surgical correction to avoid serious complications.
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