OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly)

Patient history
– An 18 year-old female came to hospital due to amenorrhea with lower abdominal pain.
the patient had menarche at 13 year-old, but since 14 year-old she had no menstruation.

Female genital tract development

1) Ductal development
– Undifferentiated gonads with idential paired genital ducts (Wolffian & Mullerian duct)
– Absence of Y chromosome → Differentiation into ovary
– Estrogenic effect : Mullerian duct development & Wolffian duct degeneration

1-1) Nondevelopment
– Unicornuate uterus
– Rudimentary horn
– Hypoplasia/agenesis of uterus, cervix, or vagina

2) Ductal fusion
– Caudal mullerian duct fusion with intervening septum
→ Forming corpus of uterus, cervix, and upper vagina
– Upper mullerian duct form fallopian tube

2-1) Nonfusion
– Uterine didelphys
– Bicornuate uterus

3) Septal degeneration
– Degeneration of fused margin of mullerian duct
– Canalization of vaginal plate

3-1) Nondegeneration
– Uterine/vaginal septa
  Longitudinal / Transverse septa
– Arcuate uterus

4) Herlyn-Werner-Wunderlich syndrome
OHVIRA syndrome
(= Obstructed hemivagina – ipsilateral renal agenesis)

Uterine didelphys → Complete failure of mullerian duct fusion
+ Unilateral hemivaginal septum → obsturction with hematocolpus
+ Ipsilateral renal agenesis

References)
RadioGraphics 2012; 32:E233–E250
RadioGraphics 2009; 29:1085–1103
AJR 2012; 198:302–310
Pediatr Radiol (2007) 37:657–665

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