Rare Adult Presentation of Currarino Syndrome–A Case Report
摘要
Currarino syndrome is a rare congenital disorder characterized by the triad of anorectal malformation, sacrococcygeal osseous defect, and a presacral mass. Adult presentation is extremely uncommon, with an estimated incidence of approximately one in 100,000 individuals. Such cases often pose significant diagnostic and surgical challenges.
Case PresentationA 37 year-old female, P1L1, presented with lower abdominal pain for 4–5 years. Her last childbirth was 12 years ago by LSCS at a private hospital, but no records were available. Examination revealed a 24 week-sized, hard abdominopelvic mass with restricted mobility. No external anal sphincter was present and anal tone was absent. However, the patient remained continent. The anal opening was displaced leftward with cranial orientation. There was a double vagina separated by a 2–4 mm septum: The anterior vagina contained the cervix, while the posterior vagina opened into the rectum/anal canal ~ 5 cm above the fourchette. Imaging revealed a heterogeneous presacral mass (15 × 15 cm) abutting a septate uterus, left crossed fused ectopic kidneys, and a partial sacrococcygeal osseous defect. Intraoperatively, a large right-sided abdominopelvic mass was encountered whose lower pole was not accessible. Complete removal of mass was tried intracapsular but was not possible due to adhesions with the bowel wall. Approximately 200 ml of thick, putty-like material was evacuated and the cyst wall biopsy was sent for histopathology which was suggestive of an epidermoid cyst.
DiscussionThis case fulfilled the diagnostic traid of Currarino syndrome-anorectal malformation, partial sacrococcygeal osseous defect, and presacral mass.
ConclusionCurrarino syndrome may rarely present in adulthood. High index of suspicion, detailed imaging, and multidisciplinary approach are key to diagnosis and management.