Single kidney eliciting a search for associated genital tract anomaly

Nephrol Dial Transplant 2004 19: 731–732DOI: 10.1093/ndt/gfg612 Case Report Single kidney eliciting a search for associated genital tract anomaly Tzung-Hai Yen1, Ping-Chin Lai1, Chiu-Chi

Nephrol Dial Transplant (2004) 19: 731–732

DOI: 10.1093/ndt/gfg612

Case Report

Single kidney eliciting a search for associated

genital tract anomaly

Tzung-Hai Yen1, Ping-Chin Lai1, Chiu-Ching Huang1and Yu Chen2

1

Department of Nephrology and2Department of Urology, Chang Gung Memorial Hospital, Taipei, Taiwan

Introduction

obstructed hemivagina and ipsilateral renal agenesis

is rare [1] Generally, the condition is not detected until

puberty, after menstrual blood accumulates in the

obstructed side The accumulation of menstrual blood

then distends the vagina, uterus and fallopian tubes

and can even spill into the pelvic cavity The clinical

presentation varies considerably depending on the

extent of the obstruction of the unilateral hemivagina

and on the location of the opening The most common

clinical presentation is dysmenorrhoea associated

with a pelvic mass resulting from a completely

hemi-obstructed vagina Other presentations include pelvic

pain, hypermenorrhoea, menometrorrhagia,

intermit-tent vaginal spotting, malodorous vaginal discharge

and urinary symptoms The variable clinical

presenta-tion makes diagnosis difficult Early and accurate

diagnosis and management is important for preventing

future fertility problems

Case

A 23-year-old female had enjoyed good health

previously At a routine examination she was found

to have a solitary kidney and was referred to us for

further investigation She had suffered from

dysme-norrhoea for several years Additionally, the patient

reported intermittent, foul, mucopurulent vaginal

discharge and polymenorrhoea with unpredictable

staining between menstrual cycles Physical examina-tion was unremarkable Laboratory examinaexamina-tions and urinalysis were normal Renal ultrasound revealed a solitary right kidney of normal renal size and echogenicity Magnetic resonance imaging and urog-raphy confirmed a solitary right kidney Additionally, two uterine corpuses and two uterine cervixes were seen, compatible with double uterus (Figure 1) Gynaecological examination under anaesthesia found

an opening in the partially obstructed small vaginal pouch The final diagnosis was a double uterus with an incompletely obstructed left hemivagina and left renal agenesis

Discussion Embryologically, this complex anomaly probably results from three different developmental failures Duplication of the female reproductive tract results from lack of fusion of the paired mullerian ducts appearing at the sixth week of embryonic development [1] The lack of a caudal opening on one side of the duplicated vagina results from the failure of formation

of the uterovaginal canal, mesodermal proliferation or the vaginal canalization [2] The reason for unilateral occurrence of the anomaly is unclear Finally, because urinary and genital systems are derived from a common embryonic mesoderm understanding a simul-taneous defect is easy With agenesis of one kidney in females, the incidence of associated genital anomalies has been estimated to be up to 50% [3] The precise aetiology and pathogenesis of the syndrome and its embryologic origin remain unknown Conservative surgical treatment, that is excision of the obstructing vaginal septum and marsupialization of the blind hemivagina, is considered the most appropriate treatment [4] This procedure enables evacuation of the sequestered material and preserves reproductive function

Correspondence and offprint requests to: Tzung-Hai Yen, MD,

Department of Nephrology, Chang Gung Memorial Hospital,

199, Tung Hwa North Road, Taipei 105, Taiwan Email: m19570@

adm.cgmh.org.tw

Nephrol Dial Transplant Vol 19 No 3 ß ERA–EDTA 2004; all rights reserved

Conflict of interest statement None declared.

References

1 Golan A, Caspi E Congenital anomalies of the mullerian duct.

Contemp Obstet Gynecol1992; 37: 39–55

2 Wenof M, Reyniak V, Novendstern, Castadot MJ Transverse

vagina septum Obstet Gynecol 1979; 54: 60–64

3 Thompson DP, Lynn HB Genital anomalies associated with

solitary kidney Mayo Clin Proc 1966; 41: 538–548

4 Candiani GB, Fedele L, Candiani M Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and

long-term follow-up Obstet Gynecol 1997; 90: 26–32

Received for publication: 21.8.03 Accepted in revised form: 14.10.03

Fig 1 Magnetic resonance imaging and urography revealed non-visualization of the left kidney in the presumed left renal fossa location.

Non-visualization of the left kidney also was found in the abdomen and pelvic cavity Additionally, there was visualization of two uterine

corpuses and two uterine cervixes.