Epispadia

Absence of any visible bladder can reveal either cloa-cal or bladder exstrophy. In both malformations, which may present with omphalocele (Grignon and Dubois 1999), there is an open defect of the abdominal or perineal wall. Widening of the distance between pubic bone echoes can be shown by prenatal sonography. This finding (Fig. 6.1) can also be shown in epispadias, the third and less severe malformation in this spectrum. 

Epispadia is part of the heterogeneous exstrophy-epispadia complex. It may occur in males and females. Widening of the pubic symphysis is usually associated. Continence is variable in those patients, so imaging and urodynamic studies in these patients should be directed towards this handicap and the detection of associated anomalies. Duplication of the bulbous urethra is extremely rare. It may be complete or blind-ended, ventral or dorsal (Barbagli et al. 1996). Finally, megalourethra (Stephens and Fortune 1993) is an enlargement of the pendulous urethra with no evidence of distal obstruction. It... 

Epispadias 177 Male Epispadias 177 Female Epispadias 177 Classical Bladder Exstrophy 178 Urogenital Features 178 Musculoskeletal Features 178 Variants 178... 

Surgical Repair 181 Initial Bladder Closure 181 Epispadias Repair 181 Bladder Neck Reconstruction 182... 

The exstrophy-epispadias complex represents a spectrum of malformations ranging from epispadias to cloacal exstrophy (Wood 1990). The most common entities of this complex and their frequencies are listed in Table 9.1 (Duckett and Cladamone 1985). 

Table 9.1. Types and frequencies of epispadias-exstrophy complex...

Epispadias occurs in 1 in 117,000 of the population, with a 5 1 male predominance. The incidence of classical bladder exstrophy is reported to he between 1 in 10,000 and 1 in 50,000 births (Duffy 1996). There is a 3 1 male predominance. Variants of bladder exstrophy tend to occur more often in females than in males (Duckett and Cladamone 1985). 

The risk of occurrence is 400-500 times higher if one parent suffers from bladder exstrophy (Ben-Chaim et al. 1996 Duffy 1996). There is evidence that a slightly increased risk for bladder exstrophy or epispadias exists in children whose mothers are less than 20 years old (Ben-Chaim et al. 1996). In addition, a possible concordance of intrauterine exposure to diazepam and occurrence of omphalocele-exstrophy-imperforate anus-spina bifida (OEIS) complex has been published (Lizscano-Gil et al. 1995). Cloacal exstrophy has an incidence of about 1 200,000 births. For this malformation no sex predominance is reported (Duckett and Cladamone 1985). 

The embryological defect producing the epispadias-exstrophy complex occurs about the 8th week of gestation. Immunohistochemical differenc es between exstrophied and normal bladders exist. 

Three different types can be differentiated bala-nitic, penile, and penopubic (or complete) epispadias. In balanitic and penile epispadias, continence is present and musculoskeletal deformities of bladder exstrophy are mild or absent. On the other hand, in penopubic or complete epispadias, the whole length of the penis is involved, and these patients are always incontinent to a certain degree. Usually the penis is short and stubby. In addition, this subtype reveals the musculoskeletal deformities of bladder exstrophy, but to a minor degree (Currarino et al. 1993). 

In 90% of all patients with epispadias, vesicoureteral reflux (VUR) will be present. This is due to an abnormal trigone and laterally displaced ureterovesical junctions (Duckett and Cladamone 1985) (Fig. 9.3)... 

Fig. 9.3. Spot film of VCU in a patient with epispadias bilateral VUR is present as well as a hook deformity of the ureterovesical junction. The urethra is short and irregular...

All subtypes of the epispadias-exstrophy complex exhibit a split symphysis to a certain degree. Incontinent epispadias is more common than continent epispadias. In addition to classic bladder exstrophy, covered variants exist. Cloacal exstrophy represents the severe part of the spectrum, comprising urogenital, intestinal, and musculoskeletal malformations. 

The overall goal of treatment in patients with the epispadias-exstrophy complex is the well, dry, fertile, and happy (both cosmetically and functionally) adult (Kelly 1998). 

Currarino G, Wood B, MajdM (1993) The genitourinary tract and retroperitoneum-epispadias-exstrophy complex. In Silverman F, Kuhn J (eds) Caffey s pediatric X-ray diagnosis. An integrated imaging approach. Mosby, St. Louis, pp 1298-1301... 

Kelly CH (1998) Extrophy and epispadias Kelly s method of repair. In O Neill JA (ed) Pediatric surgery, 5th edn. Mosby, St Louis, pp 1732-1759 Lizscano-Gil LA, Garcia-Cruz D, Sanchez-Corona J (1995) Omphalocele-exstrophy-imperforate anus-spina bifida complex in a male prenatally exposed to diazepam. Arch Med Res 26 95-96... 

Perren P, Erey P (1998) The exstrophy-epispadias complex in the duplicated lower urinary tract. J Urol 159 1681-1683 Riccabona M (2007) (Paediatric) magnetic resonance urography just fancy images or a new important diagnostic tool Curr Opin Urol 17 48-55 Sahoo SP, Gangopadhyay AN, Sinha CK et al (1997) Covered exstrophy a rare variant of classical bladder exstrophy. Scand J Urol Nephrol 31 103-106 Smith NM, Chambers HM, Purness ME et al (1992) The OEIS complex (omphalocele-exstrophy-imperforate... 

Cloacal extrophy, bladder extrophy, and epispadias are developmental abnormalities of ranging severity, as a result of disruption of the formation and opposition of the pelvic bones, the cavitation of the pelvic organs, and the partitioning of the pelvic cavity (see also Chap. 9). Urinary tract diversion using an ileal conduit to treat this and other bladder abnormalities... 

Dedicated chapters are devoted to specific problems of the newborn and infant, such as imaging and interpretation of upper urinary tract dilatation, postnatal imaging of fetal uropathies, associated urinary problems with imperforate anus, epispadias... 

---