Appendiceal torsion in Ehlers-Danlos syndrome: A case report of a rare phenomenon in a rare disease

Abstract

Highlights Appendiceal torsion, a rare phenomenon, may be seen in Ehlers-Danlos syndrome. Appendiceal torsion is clinically indistinguishable from acute appendicitis. Ehlers-Danlos syndrome contributes to surgical problems due to fragility of tissue.

Uncommon cause of acute appendiceal torsion mucocele

“…Appendicular torsion is most often diagnosed intraoperatively; some preoperative radiologic imaging findings could suggest torsion’s presence, particularly secondary torsion 2–6. A missed torsion diagnosis preoperatively is usually not catastrophic because the differentials often include acute appendicitis or ovarian torsion, of which, for exclusion, they require a diagnostic laparoscopy 2–6. Possible causes of appendicular torsion and a brief description of presentation patterns are summarised in table 1.…”

“…In all cases, minimal handling of the appendix proper is highly recommended 2–6. This is important so as not to rupture the appendicular mucocele accidentally.…”

“…The degree of torsion is usually between 180 0 and 1080 0 , usually directed anticlockwise 2. The torsion causes luminal obstruction, compromising the blood supply and leading to strangulation and infarction, presenting clinically as an acute abdomen 3…”

“…Meanwhile, secondary torsion of the appendix involves a primary lesion such as a faecolith, lipoma or appendicular mucocele. Appendiceal mucocele is further subclassified into non-neoplastic variants (mucosal hyperplasia and mucosal retention cyst) and neoplastic variants (mucosal adenoma, low-grade mucinous neoplasm and mucinous cystadenocarcinoma) 2–6…”