J Korean Soc Radiol.  2010 Apr;62(4):383-388. 10.3348/jksr.2010.62.4.383.

Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up

• Park NH
• Park HJ
• Park CS
• Park SI

• KMID: 1460074
• DOI: http://doi.org/10.3348/jksr.2010.62.4.383

Abstract

Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. Therefore, a follow-up study is very important for confirming the uterine malformation in girls with renal agenesis. We report a patient with renal agenesis and microscopic hematuria, who showed symptoms before menarche. A follow-up study eventually revealed uterine didelphys with a hemivaginal obstruction. A biopsy proved that the microscopic hematuria was caused by thin glomerular basement membrane disease of the contralateral kidney.

MeSH Terms

Abnormalities, Multiple
Biopsy
Congenital Abnormalities
Female
Follow-Up Studies
Glomerular Basement Membrane
Hematuria
Humans
Kidney
Kidney Diseases
Magnetic Resonance Imaging
Menarche
Tomography, X-Ray Computed
Uterus
Congenital Abnormalities
Kidney
Kidney Diseases

Figure

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  Fig. 1 Initial ultrasonography of a 9-year-old female on April 2004 demonstrating the absence of the right kidney in the renal fossa (A) and hypertrophic contralateral kidney (10.6 cm in length) (B). Pelvic ultrasonography revealed a hypoplastic uterus (cursors in C), and associated Mullerian duct anomaly was suspected.

• 

  Fig. 2 Contrast enhanced CT at the age of 11 shows right renal agenesis (A) and a newly developed small cystic lesion in the right sided pelvic cavity (arrow in B).

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  Fig. 3 Follow-up enhanced CT scan at the age of 12 after menarche shows an enlarged cystic lesion (arrow in A, B) in the right pelvic cavity, as compared with a previous examination, suggestive of hematocolpos. Ultrasonography (C) also reveals the hematocolpos. Subsequent MR shows a blind hemivagina with hematocolpos (D, E).

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  Fig. 4 Pathology of the contralateral kidney shows normal appearing but enlarged glomeruli with preserved tubulointerstitium (×200) on the methenamine silver stain (A). The electron microscopy section (B) revealed a thinned glomerular membrane (174-260 nm in thickness range) and no electron dense deposit (×2,500).

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