Spontaneous Sigmoid Colon Perforation and Ruptured Subserosal ("Zebra" Pattern) Small-Bowel Hematomas in Type IV Ehlers-Danlos Syndrome: A Case Report and a Short Review.

ABSTRACT

Background and Objectives: Spontaneous colonic perforations (SCPs) in teenagers and young adults are extremely rare. Common underlying conditions, such as colonic tumors and diverticulitis, are absent at that age. The vascular type of Ehlers-Danlos Syndrome (vEDS) is one cause of SCP. Methods: A 23-year-old male presented with an acute abdomen. The abdominal CT showed pneumoperitoneum with a large amount of fluid in the pelvis and abdomen, indicating hollow viscus rupture. At the level of the sigmoid colon, a defect in the intestinal wall and gas bubbles were seen. Results: Exploratory laparotomy confirmed sigmoid colon perforation without underlying pathology. Loop sigmoid colostomy was performed. Revisional surgery was undertaken due to clinical deterioration and intra-abdominal free fluid with small-bowel distension and air-liquid levels on abdominal CT 6 days later. Ileal subserosal hematomas were found, and many had ruptured, leaving a "zebra" pattern with lines of residual hematomas on the borders of subserosal hematomas. Genetic analysis confirmed vEDS. Conclusions: SCP in young adults or teenagers, in the absence of colonic disease, with clinical manifestations of connective tissue disorders should trigger genetic investigations for vEDS. SCP with a known vEDS could be treated with total colectomy to prevent further SCPs in the remaining colon. If segmental resections are performed, further SCP should be immediately excluded with any significant abdominal pain.