Intraoperative Endoscopic Ultrasound Guided Surgical Treatment of Herlyn-Werner-Wunderlich Syndrome. Case Report and a Systematic Literature Review

Abstract

Aim: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal-ureteral agenesis, due to an embryogenesis defect. Clinically, HWW results in hematometrocolpos on the obstructed hemivagina side, which produces a mass effect with pain. The diagnosis is difficult because this syndrome is infrequent, and its clinical presentation is variable. Early detection and treatment are important to prevent further complications that could affect the reproductive performance. Ultrasound (US) is the first step when a Mullerian anomaly is suspected but MRI has a higher accuracy to manage surgery. Surgical treatment consists in vaginal septum excision. This review provides information about HWW syndrome and about its diagnosis and treatment. The main objective of this report is to illustrate the clinical presentation, the ultrasound features of a rare syndrome and the use of a laparoscopic ultrasound guidance to allow a fertility sparing treatment without complications. Material and Methods: A search of PubMed Database identified articles published from the inception to February 2019. Results: 186 articles were identified: 125 articles were excluded for any reason. Overall, 63 articles were incorporated for further assessment. Conclusions: The case reported was treated successfully by minimally invasive surgical drainage procedure supported by full time intraoperative ultrasound guidance. Only few researches described transvaginal and transabdominal probe use during surgery; this is the first case in the literature in which a laparoscopic ultrasound probe was used to guide surgeons in the excision of the vaginal septum to prevent complications and to preserve future fertility.