Female Epispadia: SUI and POP

Abstract

Background Female epispadia is a rare congenital urogenital system syndrome, which is characterized by a bifid clitoris, depressed mons pubis, the labia is separated and variable urethral defect. The dorsal wall of the urethra is defective and via cystoscopy it is seen as short and wide . The syndrome is presented with primary urinary incontinence and abnormal anatomical features . Urinary incontinence level in female patient depends from level of the defect and imperative surgical treatment. Incidence of epispadia without extrophy is 1/117000 in males and 1/480000 in females. This malformation can be diagnosed in the fetus during pregnancy. In female epispadia often vagina and internal genitalia is normal, but can be associated with sexual dysfunction . Method We describe here a 34 years old patient who was born with epispadia – extrophia of urethra to abdominal wall, without pubic bone. At the age of 8 she underwent an operation . During the operation the neck of the bladder was formed as well as urethra, which opens in vulva, place of clitoris. When she was 8 years old, her bladder capacity was 30 ml, in teenage years – 90 ml. The patient also reported history of recurrent urinary tract infections. At 34 years old the patient took medical advice in out patient department of Kaunas university hospital with a complaint of stress urinary incontinence : when going, coughing, sneezing, doing exercises, having sex and at rest of time. Also it was the sexual intercourse problems with orgasm. Gynecological examination: absence of pubic bone, vulva is abnormal: absence of labium major and clitoris. Urethra opens into the place of clitoris. Front and back walls of vagina are moving down (POP-Q II-III stage prolapsed). Results Surgical treatment- TOT (tension obturator tape). Anterior and posterior colporrhaphy and perineoplastic was performed. There were no complications during and after surgery. On the 2 day after operation patent was released from the hospital. 2 months later patient visited outpatient department. She could hold urine well during intercourse, when resting, walking, but there is urine leakage when coughing and during physical exercises. Patient is satisfied with sexual life, she manages to achieve orgasm in about 90% of all intercourses. The patient estimates the treatment effectiveness about 60 %. Conclusion Cosmetic appearance is very important for the patients with epispadia, because abnormal external genitalia restricts sexually active female patients from sexual intercourse nearly 30% . This congenital anomaly can present serious psychological problems and social disruption.