Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report

Stefania Cappello,Francesco Cucinelli,Luisa Casadei,Eleonora Piccolo,Maria Giovanna Salerno,Emilio Piccione

doi:10.1186/s12884-018-2133-2

Stefania Cappello, Francesco Cucinelli + Show 4 more

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https://doi.org/10.1186/s12884-018-2133-2

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Abstract

BackgroundHerlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature.Case presentationA 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. She suffered from threats of abortion in the first trimester, recurrent urinary tract infections during all pregnancy. At 33 weeks + 5 days of gestational age, she was hospitalized for premature rupture of the membranes and uterine contractions and a caesarean section was performed because of breech presentation. Postpartum period was complicated by a pelvic abscess resolved with parental antibiotic therapies.ConclusionsOur literature review shows an unusual aspect in our case: HWWS is not classically associated with skeletal anomalies. Moreover, the most frequent urogenital side affected is the right, not left side as in this woman. Preterm spontaneous rupture of membranes and fetal abnormal presentation represent frequent complications and probably post-caesarean infections are related to pregnancies in the context of this syndrome.

Highlights

Herlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis
Herlyn –Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female genital tract, characterised by the triad of uterus didelphys, blind hemivagina, and ipsilateral renal agenesis [1, 2]. It is even called OHVIRA syndrome and it is a result of the arrest of the midline fusion of the Müllerian ducts
We presented a case of an unusual variant of HWWS associated with ectrodactyly [8] of the right foot, a combination never described [9], and the difficult course of her first preterm pregnancy with a particular postpartum complication

Summary

Conclusions

HWWS is an unusual congenital anomaly with clinical significance and different options for surgical management. An early correct diagnosis and treatment are the goal to relieve symptoms and prevent complications to preserve sexual and reproductive abilities. The pregnancies of these women are at an increased risk for unfavorable obstetric outcomes and can be characterized more frequently by complications that must be managed promptly by an accurate and regular maternal and fetal follow-up

Background

Discussion

Findings

Funding Not applicable