| Summary: | We present a healthy 8-year-old female with no past medical or surgical history who presents with perianal drainage. Exam noted a posterior perianal sinus tract with associated fullness on digital exam. Cross-sectional imaging demonstrated a peri-rectal cystic structure concerning for a rectal duplication cyst. On surgical exploration, the sinus tract and cystic mass did not communicate with each other, nor did they communicate with the rectum. Pathologic evaluation of the sinus tract demonstrated the presence of both urothelium and columnar epithelium, suggesting a cloacal remnant. The adjacent cystic structure was noted to be an epidermoid inclusion cyst. There is a wide variety of cloacal defects that have been reported, ranging in severity from inconsequential to severe. The relative scarcity of such defects, as well as the variability between patients and inconsistency seen in the literature, has resulted in limited standardization of diagnosis and treatment of these malformations. Our patient's presentation is most similar to congenital prepubic sinuses of cloacal origin. However, our patient had a unique presentation based on the anatomic location of her defect as compared to defects previously described, as well as the presence of the concomitant epidermoid cyst.
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