NEW CLASSIFICATION OF HYPOSPADIAS IN GIRLS ACCORDING TO MODERN CONCEPTS OF DISORDERS OF SEXUAL DEVELOPMENT AND SURGICAL MANAGEMENT STRATEGY


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Abstract

Objective: To optimize the classification of hypospadias in girls based on a review of the literature and experience with this pathology. Materials and methods: The study included follow-up data from 114 female hypospadias patients aged from 6 months to 42 years. All patients were diagnosed with different variants of disorders of sexual development (DSD) including salt-wasting congenital adrenal hyperplasia (CAH) (n=79), virile form of CAH (n=17), idiopathic virilization (n=3), mixed dysgenesis of gonads (n=2), partial dysgenesis of gonads (n=3), and hypospadias due to urethral malformation (n=10). All patients with urogenital sinus underwent surgery. Urethral transposition was performed in three patients with vestibular and vestibulovaginal forms of hypospadias complicated by recurrent urinary tract infection. Two girls with total hypospadias underwent surgery for upper urinary tract malformations and urinary incontinence. Results: Based on the observations presented, we proposed a new classification of hypospadias in girls: 1. Hypospadias due to urethral malformations a) Vestibular; b) Vestibulovaginal; c) Total. 2. Hypospadias due to virilization of the external genitalia a) Urogenital sinus 1) Mild form; 2) Medium form; 3) Severe form. b) Low-vaginal ectopia of the external urethral opening c) High-vaginal ectopia of the external urethral opening. Conclusion: The new classification will help to determine the rational surgical management strategy for hypospadias in girls and facilitate the interaction of physicians of different specialties.

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About the authors

Alexander V. ANIKIEV

National Medical Research Center for Endocrinology, Ministry of Health of Russia

Email: anikieal70@gmail.com
PhD, Surgeon at the Pediatric Surgery Department anikieal70@gmail.com

Alexey B. OKULOV

Russian Medical Academy of Continuous Professional

Email: okulov20@yandex.ru
Dr. Med. Sci., Professor at the Pediatric Surgery Department Moscow, Russia

Elena A. VOLODKO

Russian Medical Academy of Continuous Professional

Email: okulov20@yandex.ru
Dr. Med. Sci., Professor at the Pediatric Surgery Department Moscow, Russia

Dmitriy N. BROVIN

National Medical Research Center for Endocrinology, Ministry of Health of Russia

Email: brovin-dn@yandex.ru
PhD, Head of the Pediatric Surgeon Department Moscow, Russia

Elena N. ANDREEVA

National Medical Research Center for Endocrinology, Ministry of Health of Russia; A.I. Yevdokimov Moscow State University of Medicine and Dentistry, Ministry of Health of Russia

Email: endogin@mail.ru
Dr. Med. Sci., Professor at the Reproductive Medicine and Surgery Department Moscow, Russia

Natalya G. MOKRYSHEVA

National Medical Research Center for Endocrinology, Ministry of Health of Russia

Email: nm70@mail.ru
Corresponding Member of the RAS, Dr. Med. Sci., Professor, Director Moscow, Russia

References

  1. Деревянко И.М., Рыжков Т.И., Елисеева В.В. Влагалищная эктопия уретры и мочеполовой синус у женщин. Ставрополь; 2004. 195c.
  2. Соловьев А.Е. Диагностика и лечение гипоспадии у девочек. Урология и нефрология. 1993; 6: 11-3.
  3. Мираков К.К., Володько Е.А., Окулов А.Б., Годлевский Д.Н., Аникиев А.В., Поварнин О.Я. Лапароскопия в диагностике и лечении нарушений формирования пола у детей. Medicus. 2016; 2: 122-4.
  4. Lee P.A., Houk C.P., Ahmed S.F., Hughes I.A. Consensus statement on management of intersex disorders.International Consensus Conference on Intersex. Pediatrics. 2006; 118(2): e488-500. https://dx.doi.org/10.1542/peds.2006-0738.
  5. Комяков Б.К., Тарасов В.А. Способ хирургического лечения гипермобильности и влагалищной эктопии уретры. Патент на изобретение RU 2686948 C1, Бюллетень Роспатента № 13, 06.05.2019.
  6. Halleran D.R., Thompson B., Fuchs M., Vilanova-Sanchez A., Rentea R.M., Bates D.G. et al. Urethral length in female infants and its relevance in the repair of cloaca. J. Pediatr. Surg. 2019; 54(2): 303-6. https://dx.doi.org/10.1016/j.jpedsurg.2018.10.094.
  7. Marei M.M., Fares A.E., Abdelsattar A.H., Abdullateef K.S., Seif H., Hassan M.M. 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. https://dx.doi.org/10.1016/j.jpurol.2016.02.008.
  8. Адамян Л.В., Курило Л.Ф., Макиян З.Н., Поддубный И.В., Окулов А.Б., Глыбина Т.М., Глыбина Т.М., Файзуллин А.К. Тактика реконструктивнопластических операций у пациенток с нарушениями формирования пола (disorders of sex development), отнесенных к женскому полу. Андрология и генитальная хирургия. 2010; 3: 56-62.
  9. Аникиев А.В., Володько Е.А., Бровин Д.Н., Калинченко Н.Ю., Туманян Г.Т., Окулов А.Б. Дифференцированный подход к лечению гипоспадии у девочек. Вопросы гинекологии, акушерства и перинатологии. 2019; 18(4): 5-11. https://dx.doi.org/10.20953/1726-1678-2019-4-5-11.
  10. Knight H.M., Philip N.J., Mouriquand P.D. Female hypospadias: a case report. J. Pediat. Surg.1995; 30(12): 1738-40.
  11. Nabhan Z.M., Eugster E.A. Upper-tract genitourinary malformations in girls with congenital adrenal hyperplasia. Pediatrics. 2007; 120(2): e304-7. https://dx.doi.org/10.1542/peds.2006-2993.
  12. Рудин Ю.Э., Марухненко Д.В., Гарманова Т.Н. Гипоспадия у женщин и девочек: обзор литературы и клинический случай. Экспериментальная и клиническая урология. 2015; 4: 110-4.

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