Abstract
Exstrophy-epispadias complex encompasses a spectrum of pathologies ranging from isolated epispadias to classical bladder exstrophy to cloacal exstrophy as the most severe and the rarest variant, are quite uncommon and constitute about 8% of all the cases. The treating surgeon should be aware of all the variants, their diagnosis and modalities of treatment for the same. We present two such unique variants and successful surgical management of the same.
Highlights
The Exstrophy-epispadias Complex (EEC) is a group of rare congenital anomalies with a spectrum of genitourinary, musculoskeletal and gastrointestinal abnormalities
Bladder exstrophy-epispadias complex is a spectrum of rare genitourinary abnormalities with musculoskeletal defects and sometimes gastrointestinal abnormalities
Osteotomies are not required as diastasis is not wide
Summary
The Exstrophy-epispadias Complex (EEC) is a group of rare congenital anomalies with a spectrum of genitourinary, musculoskeletal and gastrointestinal abnormalities. We report 2 rare variants of EEC including superior vesical fissure and covered exstrophy bladder, their clinical presentations, the diagnostic challenges and the management strategies of these unique cases. On examination there was abdominal swelling which bulged on straining with single opening in the midline with dribbling of urine. Local examination revealed a poorly formed urethral opening, a separate vaginal opening, poorly formed labia majora without pubic diastasis. A 1 year-old female was admitted with complaints of continuous dribbling of urine through perineal opening since birth and intermittent bulge in the lower abdomen on crying/straining. Intra-operative findings revealed a covered bladder exstrophy with divarication of recti and pubic diastasis. Patient underwent a formal exstrophy repair with pubic ramotomy and vesical calculus removal at the age of 2 years.
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