De Castro falloplasty of the with penile agenesis

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Abstract

Introduction. Penile agenesis (aphallia) is an extremely rare congenital disorder. At present, about 100 cases of the anomaly have been described around the world. The incidence of this congenital anomaly is estimated to be one case per 20–30 million births. One of the most commonly used reconstructive procedures today is the technique proposed by Roberto De Castro in 2007. In this article, we present a clinical case of aphallia in a boy who underwent phalloplasty.

Materials and methods. A newborn boy presented with aphallia, combined with abnormal development of both kidneys. At birth, the child had difficult urination through the fistula at the edge of the anus. The meatotomy and urethral dilation were performed on the fifth day of the infant`s life. On day 28 of the child`s life, surgical intervention was performed to remove the left non-functioning kidney and a right cutaneous ureterostomy because of the recurrence of febrile urinary tract infection. At 13 months, the tissue expander was installed above the pubic bone. At 16 months, the child underwent the De Castro phalloplasty technique while in the supine position.

Results. The child was examined 3, 6, and 12 months after the surgical treatment. Phalloplasty has had good outcomes as evaluated by parents and surgeons. At present, the boy is under the supervision of urologists, who plan to perform further surgical treatment of the urinary system.

Conclusion. Phalloplasty, in the case presented, had a good cosmetic result. In our opinion, it proved to be the correct initial stage of treatment for aphallia, an extremely rare genital malformation.

About the authors

Ilia M. Kagantsov

Pitirim Sorokin Syktyvkar State University; Republican Children’s Clinical Hospital

Author for correspondence.
Email: ilkagan@rambler.ru
ORCID iD: 0000-0002-3957-1615

Dr. Sci. (Med.), Professor at the Department of Surgical Diseases; Head of Department of Urology

Russian Federation, Syktyvkar

Vitalii I. Dubrov

2nd Children’s Hospital

Email: dubroff2000@mail.ru
ORCID iD: 0000-0002-3705-1288
SPIN-code: 5833-4928

Cand. Sci. (Med.), Head of the Department of Urology

Belarus, Minsk

Roberto De Castro

Centro di Chirurgia Hospital Petrucciani, Lecce, Italy

Email: decastro1610@gmail.com
ORCID iD: 0000-0001-7602-5560

Professor

Italy, Lecce

References

  1. Kane AD, Ngom G, Ndour O, Alumeti DM. Aphallia: A case report and literature review. Afr J Paediatr Surg. 2011;8(3):324-325. DOI: https://doi.org/10.4103/0189-6725.91675.
  2. Joshi A, Gross J, Thomalla JV. Congenital aphallia: review of pathogenesis and current treatment guidelines. Urology. 2015;86(2):384-387. DOI: https://doi.org/10.1016/j.urology.2015.04.031.
  3. Evans JA, Erdile LB, Greenberg CR, Chudley AE. Agenesis of the penis: Patterns of associated malformations. Am J Med Genet. 1999;84(1):47-55.
  4. Hendren WH. The genetic male with absent penis and urethrorectal communication: experience with 5 patients. J Urol. 1997;157(4):1469-1474. DOI: https://doi.org/10.1016/s0022-5347(01)65026-2.
  5. Oliveira DEG, da Cruz ML, Luquori R, et al. Neophalloplasty in boys with aphallia: a systematic review. J Pediatr Urol. 2016;12(1):19-24. DOI: https://doi.org/10.1016/j.jpurol.2015.10.003.
  6. De Castro R, Merlini E, Rigamonti W, Macedo A Jr. Phalloplasty and urethroplasty in children with penile agenesis: preliminary report. J Urol. 2007;177(3):1112-1117. DOI: https://doi.org/10.1016/j.juro.2006.10.095.
  7. Jack S Elder. In: Walsh PC, Retik AB, Vaughan ED Jr, Wein AJ, editors. Campbell’s Urology. 8th ed. Saunders: Elsevier Science; 2002. P. 2343–2345.
  8. Skoog SJ, Belman AB Aphallia: its classification and management. J Urol. 1989;141(3):589-592. DOI: https://doi.org/10.1016/s0022-5347(17)40903-7.
  9. Mirshemirani A, Khaleghnejad A, Pourang H, et al. Penile agenesis: report on 8 cases and review of literature. Iran J Pediatr. 2009;19(2):173-179.
  10. Raveenthiran V. Controversies of sex re-assignment in genetic males with congenital inadequacy of the penis. Indian J Pediatr. 2017;84(9):700-708. DOI: https://doi.org/10.1007/s12098-017-2412-3.
  11. Perovic SV, Djinovic R, Bumbasirevic M, et al. Total phalloplasty using a musculocutaneous latissimus dorsi flap. BJU Int. 2007;100(4):899-905; discussion 905. DOI: https://doi.org/10.1111/j.1464-410x.2007.07084.x.
  12. Djordjevic ML, Bumbasirevic MZ, Vukovic PM, et al. Musculocutaneous latissimus dorsi free transfer flap for total phalloplasty in children. J Pediatr Urol. 2006;2(4):333-339. DOI: https://doi.org/10.1016/j.jpurol.2006.05.003.
  13. Terrier J-É, Courtois F, Ruffion A, Journel NM. Surgical outcomes and patients’ satisfaction with suprapubic phalloplasty. J Sex Med. 2014;11(1):288-298. DOI: https://doi.org/10.1111/jsm.12297.
  14. Massanyi EZ, Gupta A, Goel S, et al. Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy. J Urol. 2013;190(4 Suppl.):1577-1582. DOI: https://doi.org/10.1016/j.juro.2012.12.050.
  15. Gouvea J, Garrone G, da Cruz ML, et al. Penile prosthesis implantation in a patient with congenital aphallia treated using the De Castro technique 10 years previously. Is it feasible? J Pediatr Urol. 2015;11(5):287-288. DOI: https://doi.org/10.1016/j.jpurol.2015.05.022.

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2. Fig. 1. A newborn boy with aphallia: а — the penis is absent; the scrotum is developed correctly with pronounced skin folds; b — there is a skin crest 0,5 cm wide on the perineum between the anus and the scrotum along the midline

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3. Fig. 2. Voiding cystourethrography VCUG: a — in the direct projection, the bladder is irregularly shaped, enlarged, the contour is uneven, the diverticulum of the urachus is detected; b — in the oblique-lateral projection, the urethra is short, the proximal part is not expanded, vesicoureteral reflux is detected into the sharply expanded ureter of the left kidney; c — excretory urogram — the function of the left kidney is not identified, pronounced ureterohydronephrosis is on the right

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4. Fig. 3. Phalloplasty surgical technique (continued): a — patient appearance 3 months after tissue expansion; b — marking of the skin flap prior to phalloplasty; c — incisions; d — flap mobilization with subcutaneous fat

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5. Fig. 4. Phalloplasty surgical technique (continued): a — the initial stage of the formation of the fascial-muscle flap from the lower section of the rectus abdominis muscle; b — the length of the flap corresponds to the height of the skin flap from which the neophallus will be formed

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6. Fig. 5. Phalloplasty surgical technique (continued): a — a skin flap is folded into a cylinder with matching Z-shaped edges on the dorsal surface; b — a free skin flap is sutured into the de-epithelialized area of the “coronal sulcus” of the neophallus; c — the navel is formed in a new place when suturing the wounds of the anterior abdominal wall

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7. Fig. 6. The appearance of the patient after phalloplasty: a — is a top view; b — view from below; 12 months after phalloplasty: c — view from below; d — top view

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