Clinical and radiological findings for early diagnosis of Herlyn–Werner–Wunderlich syndrome in pediatric age: experience of a single center

Abstract

Objective: The authors present their experience in the management of pediatric patients with Herlyn–Werner–Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation.Methods: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices).Results: Six patients were treated in the study period. The mean age was 9 years (2 months–15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free.Conclusions: Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.