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Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation
Author(s) -
Morino Masaaki,
Hoshino Masaya,
Musha Ikuma
Publication year - 2013
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12088
Subject(s) - medicine , uterus didelphys , intestinal malrotation , hypodontia , renal agenesis , agenesis , abdomen , concomitant , situs inversus , surgery , radiology , kidney , uterus , orthodontics
The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called H erlyn‐ W erner‐ W underlich syndrome ( HWWS ) or obstructed hemivagina and ipsilateral renal anomaly ( OHVIRA ) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non‐rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high‐riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS . The patient underwent a preventative L add's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.