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

Carmine Noviello, Mercedes Romano, Fabiano Nino, A Martino, Giovanni Cobellis
Gynecological endocrinology : the official journal of the International Society of Gynecological Endocrinology · 2017 · vol. 34(1) , pp. 56–58 · doi:10.1080/09513590.2017.1332178 · PMID:28562147 · W2619696681
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This study analyzed clinical and radiological findings in six pediatric Herlyn-Werner-Wunderlich syndrome patients, reporting good outcomes after surgical treatment and recommending imaging for early diagnosis.

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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.

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MeSH descriptors

Abnormalities, Multiple Congenital Abnormalities Kidney Kidney Diseases Uterus Vagina Abnormalities, Multiple Adolescent Child Child, Preschool Congenital Abnormalities Early Diagnosis Female Humans Infant Kidney Kidney Diseases Kidney Diseases Magnetic Resonance Imaging Syndrome

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