A rare case of persistent urogenital sinus in an adult woman associated with unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst
Un caso raro de seno urogenital persistente en una mujer adulta asociado a cuerno rudimentario unilateral con agenesia renal ipsilateral y quiste dermoide contralateral
Mohamed Ibrahim Amer
Abstract
Persistent urogenital sinus (PUGS) is an uncommon developmental cloacal anomaly, with Incidence of 0.6 in 10000 female births. Herein we depict the case of a 22-year of age lady who presented with Infertility for 16 months with dyspareunia and was found to have Persistent urogenital sinus associated with other urogenital anomalies as unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst. The patient was successfully treated with the excision of the sinus, the rudimentary horn and the dermoid cyst.
Keywords
Resumen
El seno urogenital persistente (PUGS) es una anomalía cloacal del desarrollo poco común, con una incidencia de 0,6 en 10000 nacimientos de mujeres. Aquí representamos el caso de una señora de 22 años que presentó Infertilidad durante 16 meses con dispareunia y se encontró que tenía seno urogenital persistente asociado con otras anomalías urogenitales como cuerno rudimentario unilateral con agenesia renal ipsilateral y quiste dermoide contralateral. El paciente fue tratado con éxito con la escisión del seno, el cuerno rudimentario y el quiste dermoide.
Palabras clave
References
1. Singh S, Singh P, Singh RJ. Persistent urogenital sinus. J Anat Soc India. 2010;59(2):242-4. doi: 10.1016/S0003-2778(10)80034-6.
2. Bischoff A. The surgical treatment of cloaca. Semin Pediatr Surg. 2016;25(2):102-7. doi: 10.1053/j.sempedsurg.2015.11.009.
3. Thomas DFM. The embryology of persistent cloaca and urogenital sinus malformations. Asian J Androl. 2020;22(2):124-8. doi: 10.4103/aja.aja_72_19.
4. Ganesan A, Smith GH, Broome K, Steinberg A. Congenital adrenal hyperplasia: preliminary observations of the urethra in 9 cases. J Urol. 2002;167(1):275-8; discussion 278-9. doi: 10.1016/s0022-5347(05)65448-1.
5. Marei MM, Fares AE, Abdelsattar AH, Abdullateef KS, Seif H, Hassan MM, et al. Anatomical measurements of the urogenital sinus in virilized female children due to congenital adrenal hyperplasia. J Pediatr Urol. 2016;12(5):282.e1-282.e8. doi: 10.1016/j.jpurol.2016.02.008.
6. Revest JM, Spencer-Dene B, Kerr K, De Moerlooze L, Rosewell I, Dickson C. Fibroblast growth factor receptor 2-IIIb acts upstream of Shh and Fgf4 and is required for limb bud maintenance but not for the induction of Fgf8, Fgf10, Msx1, or Bmp4. Dev Biol. 2001;231(1):47-62. doi: 10.1006/dbio.2000.0144.
7. Ludwikowski BM, González R. The Surgical Correction of Urogenital Sinus in Patients with DSD: 15 Years after Description of Total Urogenital Mobilization in Children. Front Pediatr. 2013;1:41. doi: 10.3389/fped.2013.00041.
8. Sandler TW. Langman’s medical embriology. 11th ed. Baltimore: Wolters Kluwer, Lippincott Williams & Wilkins; 2009.
9. Lottman H, Thomas DF. Disorders of sex development. In: Thomas DF, Duffy PG, Rickwood AM, editors. Essentials of Paediatric Urology. 2nd ed. London:Informa; 2008:189-98.
10. Moore KL, Persaud TVN. The Developing Human: Clinically Oriented Embryology in Genitourinary System. 7th ed. Philadelphia: WB Saunder& Co.; 2004.
11. Amer MI, Ahmed Mel-S, Ali AH. Congenital urethrovaginal fistula with transverse vaginal septum. J Obstet Gynaecol Res. 2016;42(8):1042-5. doi: 10.1111/jog.13022.
Submitted date:
04/29/2021
Reviewed date:
05/31/2021
Accepted date:
06/02/2021
Publication date:
06/02/2021
Facebook
Google+
Twitter
LinkedIn
Mendeley
StumbleUpon
CiteULike
Reddit
Email