Author's response to reviews Leiomyomatosis peritonealis disseminata in association with multiple congenital malformations: a Title: new feature of Currarino syndrome? Authors:

Background Leiomyomatosis peritonealis disseminate (LPD), is a rare disease in which multiple smooth muscles or smooth muscle-like nodules develop in a subperitoneal location in any part of the abdominal cavity. No reports of multiple congenital malformations associated with LPD have been found in English literature. Case presentation We report such a case of a 27 yr-old patient referred to our Gynaecology Unit for pelvic pain, amenorrhea, stress incontinence, chronic constipation and recurrent intestinal and urinary infections. Multiple congenital malformations had been previously diagnosed mostly of whom had required surgical treatment in the first years of her life: ectopic right ureteral orifice, megadolicouretere, ectopic vulvar anal orifice and sacral-coccygeal agenesis. An ultrasound performed in our Department showed a complex 20 cm-size mass originating from the right pelvis that reached the right hypochondrium and the epigastrium. The patient underwent laparotomy. A great irregular complex mass was found in the abdominal cavity and was progressively resected. The histological diagnosis was of LPD. Conclusions The case we report has the peculiarity of the coexistence of this rare condition with multiple congenital malformations reminding Currarino’s syndrome. The Currarino’s syndrome belongs to the group of persistent neuroenteric malformations and consists of anorectal malformation, sacral bone deformity and presacral mass. Our report suggests that if two components of the triad are identified, the possibility of the third one (presacral tumor) should be always considered. Pelvic ultrasound, CT and MR should be performed to quickly recognize and opportunely treat the tumor.