Bladder Exstrophy
Classical bladder eaxstrophy and epispadias comprise two of the malformations known as BEEC (bladder exstrophy-epispadias complex). The cause of these conditions is unknown, but although rare, there is a clear significant genetic component.
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Alan Dickson
22.1 Introduction Bladder exstrophy is a congenital abnormality of the urinary tract and is the most common of the conditions within the bladder exstrophy-epispadias complex (BEEC), the other two being epispadias and cloacal exstrophy. It is a rare but very serious anomaly which causes significant urological, sexual and psychological problems. The embryology of bladder exstrophy remains unclear and various theories have been suggested. The evidence is that it starts developing early in embryonic life. Marshall and Muecke [1] have postulated an overdevelopment of the lower part of the cloacal membrane, which obstructs the medial migration of mesenchymal tissue, thereby preventing normal growth of the lower abdominal wall. More recently, Manner et al [2] developed this theory more, demonstrating in chick embryos a mechanical obstruction of the medial migration of the lateral layers of the abdominal wall. Whilst most births with exstrophy are sporadic, there are multiple reports [3] of familial cases, clearly showing that there is a genetic basis to the condition. The incidence of bladder exstrophy is about 1 in 40,000 live births [4] and is three times more common in males than girls.
22.2 Anatomy of Bladder Exstrophy The features of classic bladder exstrophy are shown and listed in Figs. 22.1 and 22.2. There is a lower anterior abdominal wall defect through which the open bladder herniates. In the male, the epispadias aspect of the abnormality includes the A. Dickson, BSc, MBChB, FRCS(Ed), FRCS(Eng) Consultant Paediatric Urologist, Royal Manchester Childrens Hospital, Stony Littleton, Bath, UK e-mail: [email protected] © Springer International Publishing AG, part of Springer Nature 2018 G. Mosiello et al. (eds.), Clinical Urodynamics in Childhood and Adolescence, Urodynamics, Neurourology and Pelvic Floor Dysfunctions, https://doi.org/10.1007/978-3-319-42193-3_22
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A. Dickson The low umbilicus
The exposed small bladder plate The exposed bladder neck The epispadias Pubis diastasis
Fig. 22.1 Male bladder exstrophy (Top). Male exstrophy, 6 weeks after primary repair (Bottom)
open dorsal penis and the open bladder neck. In the female, the bladder, bladder neck and urethra are similarly open and the clitoris is bifid. Associated congenital anomalies are uncommon but include renal anomalies, cardiovascular anomalies, spinal anomalies, anorectal malformations and undescended testes.
22.3 Presentation of Bladder Exstrophy The features of bladder exstrophy on antenatal scanning are well-defined and some babies are therefore diagnosed antenatally. These features include, in particular, inability to visualise urine in the foetal bladder but also a low-set umbilical cord, a pubic diastasis, a short wide penis and a bulging bladder plate [5]. Indeed, the features are so well-defined that it is surprising that only 25% are detected on maternal ultrasound [5].
22 Bladder Exstrophy
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The low umbilicus
The open exposed small bladder plate
The ureteric orifices
The expos
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