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Exstrophy

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Exstrophy of the bladder is a rare congenital anomaly with complete ventral defect of the urogenital sinus and the overlying skeletal system (Gambhir et al, 2008). Incidence of this anomaly is reported to be 3.3 per 100,000 births. Male to female ratio is about 1.5:1 (Anon, 1987).

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Signs and Symptoms

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The lower central abdomen is occupied by the inner surface of the posterior wall of the bladder, whose mucosal edges are fused with the skin. Urine spurts onto the abdominal wall from the ureteral orifices.

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The rami of the pubic bones are widely separated. The pelvic ring thus lacks rigidity, the femurs are rotated externally, and the child “waddles like a duck.”

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The rectus muscles are widely separated from each other caudally. A hernia, made up of the exstrophic bladder and surrounding skin, is present.

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Epispadias is almost always associated. Undescended testicles may be seen, and the anus and vagina located anteriorly. Urinary tract infection and hydronephrosis are common.

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Diagnosis

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Prenatal diagnosis is difficult (Austin et al, 1998; Emanuel et al, 1995). Obvious anomaly of exposed bladder mucosa makes diagnosis easy at birth. X-ray studies will reveal separation of pubic bones.

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Treatment

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Historical treatment includes staged repair of this anomaly (Stec et al, 2011). The first stage is to close the abdominal wall, the bladder, and the posterior urethra. Orthopedic procedure of sacral osteotomy in order to close the abdominal wall may be necessary (Meldrum et al, 2003; Suson et al, 2011; Vining et al, 2011). Mollard (1980) recommends the following steps for satisfactory repair of bladder exstrophy (Mollard, 1980): (1) bladder closure with sacral osteotomy in order to close the pelvic ring at the pubic symphysis (Cervellione, 2011), plus lengthening of the penis; (2) antiureteral reflux procedure and bladder neck reconstruction; and (3) repair of the epispadiac penis. Treatment needs to be initiated prior to the fibrosis of the bladder mucosa in order to repair this anomaly completely (Oesterling and Jeffs, 1987). When the bladder is small, fibrotic, and inelastic, functional closure becomes inadvisable, and urinary diversion with cystectomy is the treatment of choice. Modern approach is to perform primary repair of this anomaly completely (Mitchell, 2005; Mourtzinos and Borer, 2004). Recent studies show better outcomes with primary repair (Grady et al, 1999; Kiddoo et al, 2004; Lowentritt et al, 2005).

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Prognosis

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Common complications after surgical repair include incontinence (Gargollo et al, 2011; Light and Scott, 1983; Perlmutter et al, 1991; Toguri et al, 1978), infertility, vesicoureteral reflux, and urinary tract infection (Ebert et al, 2008; Gargollo and Borer, 2007). ...

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