Megalourethra and urethrorectal fistula: a rare presentation and a challenging reconstruction

Macedo, António; Ottoni, Sérgio Leite; Parizi, João Luiz Gomes; Martins, Gustavo Marconi Caetano; Garrone, Gilmar; Cruz, Marcela Leal da

doi:10.1590/s1677-5538.ibju.2015.0676

Cited by 3 publications

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“…Urethral tapering and tube urethroplasty is the standard operative method for scaphoid variety. While in fusiform variety, urethral reconstruction is much more complex and individualized depending on intraoperative and endoscopic findings, condition of phallus and presence of associated multiple systemic anomalies [2].…”

Section: Introductionmentioning

confidence: 99%

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Congenital megalourethra (CMU) is a dilatation of penile urethra without any distal obstruction. It occurs due to one or both corporal defect. About 80-100% cases of CMU are accompanied by multiple systems malformations which make the treatment more challenging. Cysto-urethrogram is necessary for radiological diagnosis and surgical planning. Depending on the type of the CMU and associated congenital anomalies, reduction urethroplasty or major reconstructive surgery is fashioned. We report a case of scaphoid variety of CMU in a 2 year child who was managed by reduction urethroplasty with additional dartos double breasting to prevent postoperative urethrocutaneous fistula (UCF).

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Section: Introductionmentioning

confidence: 99%

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2020

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“…Macedo et al ( 1 ) present a video that elegantly demonstrates a one-stage repair for a child with a urethrorectal fistula along with a megalourethra. The anterior sagital trans-ano-rectal approach (ASTRA) was first described by Di Benedetto and Di Benedetto for use in clitoro-vaginoplasty ( 2 ).…”

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Editorial Comment: Megalourethra and urethrorectal fistula: a rare presentation and a challenging reconstruction

Swana

2017

Int. braz j urol.

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Scaphoid megalourethra associated with congenital urethrocutaneous fistula: a case report of a rare association

Chrun,

Egger,

Toda

et al. 2023

Ann Pediatr Surg

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Background Congenital megalourethra is a rare male genital malformation, with less than 100 cases reported in literature. Urethrocutaneous fistula, in turn, has only 65 cases described, which what makes the association between these two anomalies an even rarer condition. Case presentation The patient was male and born at 37 weeks of gestational age. At birth, the testicles were impalpable, and he had a penile malformation compatible with scaphoid megalourethra associated with congenital urethrocutaneous fistula, with urine output exclusively through a small orifice in the ventral region of the penis. When the child completed 7 months and 22 days, underwent the first surgery, laparoscopically. The first Fowler-Stephens was performed, and the gonadal vessels were ligated, with reconstruction of the penis. Nine months later, the second Fowler-Stephens procedure was performed, with bilateral orchidopexy and penoplasty to improve penis aesthetics. The urethra region was not approached. The postoperative period of the second surgery progressed uneventfully. Nowadays, the patient presents adequate urinary stream. Conclusion The scaphoid megalourethra associated with congenital urethrocutaneous fistula is an extremely rare pathology, with very few cases reported in the literature to date. The obstetric USG is important to increasing the chances of early diagnosis and treatment.

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Megalourethra and urethrorectal fistula: a rare presentation and a challenging reconstruction

Cited by 3 publications

References 4 publications

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Editorial Comment: Megalourethra and urethrorectal fistula: a rare presentation and a challenging reconstruction

Scaphoid megalourethra associated with congenital urethrocutaneous fistula: a case report of a rare association

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