Necrotic gangrenous intrathoracic appendix in a marfanoid adult patient: a case report.
BACKGROUND
A diaphragmatic hernia is defined as a defect in part of the diaphragm through which abdominal contents can protrude into the thorax. It may be congenital or acquired. In this case report, we aim to demonstrate a congenital diaphragmatic hernia in an adult marfanoid patient which required emergency treatment


METHODS
A 43 year old woman was admitted with classical appendicitis requiring surgery. She incidentally had Marfan's clinical features with a positive family history for the syndrome. At operation she had grossly abnormal abdominal anatomy. Radiological investigations demonstrated a large right congenital diaphragmatic hernia with an intrathoracic hernial sac containing a perforated gangrenous appendix. The hernial sac was opened surgically and the appendix excised. The patient made a full recovery.


CONCLUSIONS
Diaphragmatic hernias are usually congenital in nature often requiring early corrective surgery for future survival. We have demonstrated the presence of an unusually large diaphragmatic defect, almost a hemidiaphragmatic defect, of unknown direct etiology, but of some possible association with Marfan's syndrome in an adult patient presenting with an acute perforated gangrenous appendix requiring emergency life-saving surgery.
Barakat
Mohannad J
MJ
General Surgery, Weston General Hospital, Weston-super-mare, UK. mbarakat@blueyonder.co.uk
Vickers
Jon H
JH
D000005:Abdomen / Q000002:abnormalities; D000328:Adult; D001064:Appendicitis / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006548:Hernia, Diaphragmatic / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed