Exstrophy bladder

OR = 4.2 Cl = 1.5,12), terminal transverse limb reduction (OR = 12 Cl = 3.5, 41), and limb constriction ring or skin scars (OR = 40 Cl = 11, 153). There were 13 different defects not previously described in the misoprostol-exposed cases, but only holoprosencephaly and bladder exstrophy significantly exceeded the expected number. 

Fig. 16.13. Complications after urethral surgery. Axial image obtained after retrograde distention of the urethra with saline solution shows a hair-bearing ectatic neourethra curved arrows) in a man with bladder exstrophy and urethral duplication corrected during childhood using a scrotal skin flap...

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. 

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

Pseudoexstrophy of the Bladder 178 Covered Exstrophy of the Bladder 180 Superior Vesical Fissure 180 Inferior Vesical Fissure 180 Duplicate Exstrophy 180 Bladder Exstrophy with Normal Umbilicus and Normal Infraumbilical Wall 180 OEIS Complex 180 Cloacal Exstrophy (Vesicointestinal Fissure) 180 Urogenital and Intestinal Features 180 Musculoskeletal Features 180... 

Historically, bladder exstrophy was first mentioned in 2000 BC. The earliest description was given in 1597 by Scheuke and Grafenberg, a complete one about 150 years later by Mowat in 1747. The term exstrophy was coined by Chaussier in 1780 (Kelly 1998). 

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). 

Bladder exstrophy is more common in boys. Variants of bladder exstrophy are more common in girls. There is no sex predominance in cloacal exstrophy. 

The urinary bladder can be visualized on prenatal ultrasound scans after 14-weeks of gestation (Ben-Chaim et al. 1996). Therefore, in the absence of the urinary bladder on prenatal ultrasound scans or if any of the other above-mentioned symptoms are present, the diagnosis of bladder exstrophy should be raised. 

Bladder exstrophy is an underdiagnosed condition on prenatal ultrasound scans. Absence of the urinary bladder is th e hallmark of bladder exstrophy on those scans. 

Bladder exstrophy is diagnosed prenatally in only 13% of cases according to Ben-Chaim et al. (1996). In a retrospective study on 43 prenatal ultrasound... 

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 patients with bladder exstrophy, associated anomalies of the upper urinary tract are infrequent (Currarino et al. 1993). After surgical correction VUR will be present in almost all cases of bladder exstrophy (Ben-Chaim et al. 1996). Figure 9.5 depicts the features of bladder exstrophy in a newborn boy. 

This malformation is a rare variant of classical bladder exstrophy. It is characterized by the pres-... 

Fig. 9.4. Schematic urogenital findings in a male patient suffering from bladder exstrophy...

The musculoskeletal features of classic bladder exstrophy can be observed as well as a minimal bladder eventration below an abnormally low set umbilicus (Borwankar et al. 1998 Sahoo et al. 1997). 

To date only a few cases have been reported (Johnson et al. 1995). Bladder exstrophy is limited to the bladder neck, whereas the penis and urethra are normal. Split symphysis is present too. 

Bladder Exstrophy with Normal Umbilicus and Normal Infraumbilical Wall... 

Bladder exstrophy can be part of the OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects). The OEIS complex is rare and affects only 1 in 200,000 to 1 in 400,000 pregnancies (Smith et al. 1992). 

The same skeletal features as in classic bladder exstrophy can be observed. Myelomeningoceles are present in 50% of these patients. Spinal abnormalities are common. 

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. 

Management of bladder exstrophy aims to result in a well, dry, fertile, and happy (both cosmetically and functionally) adult. Therapy of bladder exstrophy consists of staged surgical repair. Factors for successful outcome of bladder closure have been identified. 

Various imaging modalities are involved during the tortuous course of patients suffering from the epi-spadias-exstrophy complex (Wood 1990). In newborns with bladder exstrophy chest films are taken as a part of the preoperative work-up within the... 

Ahmed S, Abu Daia J (1998) Exstrophic abdominal wall defect without bladder exstrophy (pseudo-exstrophy). Br J Urol 81 762-763... 

Ben-Chaim J, Gearhart JP (1996) Current management of bladder exstrophy. Techniques Urol 2 22-33... 

Ben-Chaim J, Docimo S, Jeffs RD et al (1996) Bladder exstrophy from childhood into adult life. J R Soc Med 89 39-46... 

Borwankar SS, Kasat LS, Naregal A et al (1998) Covered exstrophy a rare variant. Pediatr Surg Int 14 129-130 Cadeddu JA, Benson JE, Silver R1 et al (1997) Spinal abnormalities in classic bladder exstrophy. Br J Urol 79 975-978... 

Canning DA (1996) Bladder exstrophy the case for primary bladder reconstruction. Urol 48 831-834 Connolly JA, Peppas DS, Jeffs RD et al (1995) Prevalence and repair of inguinal hernias in children with bladder exstrophy. J Urol 154 1900-1901 Crankscon SJ, Ahmed S (1997) Female bladder exstrophy. Int Urogynecol J 8 98-104... 

Diamond DA, Bauer SB, Dinlenc C et al (1999) Normal uro-dynamics in patients with bladder exstrophy are they achievable J Urol 162 841-845 Duckett JW, Cladamone AA (1985) Bladder and urachus. In Kelalis PP, King LR, Befinan AB (eds) Clinical pediatric urology. Saunders, Philadelphia, pp 726-742 Duffy PC (1996) Bladder exstrophy. Semin Pediatr Surg 5 129-132... 

Lottmann HB, Melin Y, Cendron M et al (1997) Bladder exstrophy evaluation of factors leading to continence with spontaneous voiding after staged reconstruction. JUrol 158 1041-1044... 

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... 

Sripathi V, Sen S, Ahmed S (1997) Normal umbilicus and infra-umbilical abdominal wall in bladder exstrophy. Br J Urol 80 672... 

Yazici M, SOzubir S, Kilicoglu G (1998) Three-dimensional anatomy of the pelvis in bladder exstrophy description of bone pathology by using three-dimensional computed tomography and its clinical relevance. J Pediatr Orthop 18 132-135... 

Gargallo PC, Borer JG, Retik AB et al (2005) Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patients before and after complete primary repair of bladder exstrophy. J Urol 174 1559-1566, discussion 1566... 

Latex precaution neuro tube defect, bladder exstrophy... 

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