Diagnosis and management of epispadias
Section snippets
Diagnosis and initial management
Boys are usually diagnosed at birth because of the characteristic appearance of a split dorsal urethra with dorsal chordee and a ventral foreskin hood (Figure 1). The phallus is short because of 50% deficiency of corporal length.8 Glandular epispadias can be occult until the foreskin retracts during attempted circumcision or natural retraction of the foreskin occurs. Penopubic forms also exhibit a diastasis of the pubic bones. Female epispadias can be difficult to identify at birth and often
Surgical reconstruction
The goals of epispadias repair are the same regardless of the technique used or time point chosen. In female epispadias, surgery focuses on attaining continence with surgical reconstruction of the outer genitalia often at the time point of bladder neck surgery. In boys, bladder neck surgery is not always necessary, and phallic reconstruction is often performed as a separate procedure earlier in life. Penile reconstruction aims to correct the dorsal chordee while achieving the most corporal
Modified CR repair
Cantwell11 initially introduced a technique based on complete mobilization of the urethral plate that was then tubularized and transplanted ventrally between the corpora. Ransley et al12 and others13 later presented their modification of the Cantwell repair. The technique was based on dissection of the urethral plate from the corpora, with separation of the 2 corporal bodies, with the exception of the distal part of the corpora and hemiglans, which are left intact to ensure a better blood
MB repair
The complete penile disassembly follows many of the same principals. It begins along the ventral aspect to separate the urethral wedge from the corpora cavernosa. The lateral NVB are left intact. In sharp contrast with the CR repair, the penis is disassembled into 3 components, the right and left corporal bodies with their hemiglans attached and the urethral wedge. Corporal bodies and the urethra with the attached corpus spongiosum are dissected proximally, carefully leaving their individual
Discussion
Pediatric urology encompasses a variety of very complex procedures requiring advanced specialty training. Some conditions are so rare that, even within our subspecialty, only a few centers will gather enough patients and experience to assure continuous successful outcomes. The importance to refer patients with rare conditions to an experienced specialist cannot be over emphasized because initial failure usually will lead to a worse outcome. Although failure can occur even in the most
Conclusions
The repair of epispadias remains a challenging operation even in expert hands. The incidence is rare and repair should stay in specialized hands to provide the patient the best possible outcome. Different techniques and modifications to these repairs have been developed by various centers. Each group has their unique experience and feels passionate about their approach. Controversies in surgical approach are common in major birth defects and reflect the intense research and dedication that
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Cited by (13)
Unusual morphology of isolated male epispadia: A rare case report
2024, Urology Case ReportsDevelopmental Abnormalities of the Genitourinary System
2023, Avery's Diseases of the NewbornLong-term outcomes in primary male epispadias
2020, Journal of Pediatric UrologyCitation Excerpt :To date, there is no uniform agreement on the best surgical management, and thus each center performs surgery in line with their expertise and experience. Currently, two major reconstruction principles are used in specialist pediatric urology centers, the MCR repair, and the Mitchell-Bagli complete disassembly technique [2,12,13]. In 1989 Ransley et al. described a modification to the to the Cantwell repair that resulted in fewer complications and better cosmesis [14].
Developmental Abnormalities of the Genitourinary System
2018, Avery's Diseases of the Newborn: Tenth EditionDevelopmental Abnormalities of the Genitourinary System
2017, Avery's Diseases of the Newborn, Tenth EditionThe fate of the complete female epispadias and exstrophy bladder - Is there a difference?
2013, Journal of UrologyCitation Excerpt :A possible explanation for the larger BC in CBE cases is identification of the defect at a younger age with earlier urethroplasty. Epispadias repair is associated with an increase in BC.2 However, if earlier creation of outlet resistance is responsible, one would expect better BC in patients with CFE who are identified and treated within year 1 of life.