Recurrent rectoneovaginal fistula caused by an incidental squamous cell carcinoma of the neovagina in Mayer-Rokitansky-Küster-Hauser syndrome.

Objective: Mayer-Rokitansky-Küster-Hauser syndrome is a congenital malformation characterized by an absence of the vagina associated with a variable abnormality of the uterus and the urinary tract but functional ovaries. Surgical correction requires the creation of a neovaginal canal by the performance of a neovaginoplasty and an accurate long-term application of an artificial phallus phantom to avoid secondary shrinkage of the canal. Due to the chronic alteration of the posterior neovaginal wall, ulcers and consecutive fistulae may occur. We report the clinical course of a female who required surgical intervention for a rectoneovaginal fistula and developed a recurrence of the fistula due to one of the extremely rare squamous cell carcinomas of the neovaginal epithelium in order to show potential diagnostic and therapeutic features.

Method: The systematic report of a case is presented.

Result: Almost 13 years following the initial construction of a neovagina the patient developed a single-tract rectoneovaginal fistula. After surgical repair she represented with a recurrence due to a vast squamous cell carcinoma of the former operation site. Tumor en bloc resection was performed and currently (follow-up: 4 months) she has no signs of new tumor progression.

Conclusion: Creation of a neovagina is the standard procedure for treating vaginal atresia or aplasia. Because of the long clinical course postoperatively, complications may occur. This report of a case of a malignant transformation in neovaginal epithelium shows the potential risk of malignancy and underlines the necessity of a close follow-up.