Advertisement

Genitoplasty for Congenital Adrenal Hyperplasia: Anatomy and Technical Review

  • R. H. Whitaker
Part of the Progress in Pediatric Surgery book series (PEDIATRIC, volume 23)

Summary

There is variable virilisation in female pseudohermaphrodites with congenital adrenal hyperplasia, but they always have normal ovaries, uterus and upper part of the vagina. The various anatomical stages of virilisation are outlined and a historical review is given. For psychological and practical reasons it is generally accepted that the operation should be performed when the patient is between 6 and 18 months of age. The aim is to reduce the size of the clitoris and to expose the vagina so that it opens onto the perineum. Operative procedures are described. The child is reviewed at intervals to determine the size and shape of the vagina and clitoris. Adjustments can be made around the time of puberty but are rarely necessary.

Keywords

Congenital Adrenal Hyperplasia Skin Flap Urogenital Sinus Labium Minora Bull Johns Hopkins Hosp 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Zusammenfassung

Weibliche Pseudohermaphroditen mit adrenogenitalem Syndrome zeigen eine Virilisation verschiedenen Ausmaßes, jedoch besitzen sie immer normale Ovarien, Uterus und obere Vagina. Die Anatomie der einzelnen Virilisationsgrade wird dargelegt und ein historischer Überblick gegeben. Aus psychologischen und praktischen Gründen soll die Operation anerkanntermaßen zwischen dem 6. und 18. Lebensmonat durchgeführt werden. Das Ziel der Operation ist eine Reduktion der Clitorisgröße und eine Darstellung der Vagina, so daß diese in den Damm mündet. Die Operationstechniken werden beschrieben. Das Kind wird regelmäßig nachuntersucht, um die Größe und Form von Vagina und Clitoris zu verfolgen. Korrekturen können um die Pubertätszeit vorgenommen werden, sind aber selten erforderlich.

Résumé

Le pseudohermaphrodisme féminin avec hyperplasie surrénale congénitale entraîne une virilisation plus ou moins prononcée mais les ovaires, l’utérus et la partie supérieure du vagin sont normaux. Les auteurs décrivent les caractéristiques anatomiques des différents degrés de virilisation et donnet un aperçu de l’évolu tion. Pour des raisons pratiques autant que psychologiques, cette intervention est généralement pratiquée entre 6 et 18 mois. Elle visera à réduire la taille du clitoris et à modifier le vagin de façon à ce qu’il débouche dans le périnée. Les techniques opératoires sont décrites. On suivra l’enfant et on pratiquera des examens réguliers pour vérifier taille et forme du vagin et du clitoris. Il est possible, le cas échéant, de procéder à des corrections à la puberté mais cela s’avère rarement nécessaire.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Allen TD (1976) Reconstruction of the female with ambiguous genitalia. In: Kelalis PP, King LR (eds) Clinical pediatric urology. Saunders, Philadelphia, pp 1023–1028Google Scholar
  2. Fortunoff S, Lattimer JK, Edson M (1964) Vaginoplasty technique for female hermaphrodites. Surg Gynecol Obstet 118: 545–548PubMedGoogle Scholar
  3. Glassberg KI, Laungani G (1981) Reduction clitoroplasty. Urology 17: 604–605PubMedCrossRefGoogle Scholar
  4. Goodwin WE (1969) Anomalies of the genitourinary tract. In: de la Camp HB, Linder F, Trede M (eds) American College of Surgeons and Deutsche Gesellschaft für Chirurgie (Joint meeting, Munich, 1968 ). Springer, Berlin Heidelberg New York, p 256Google Scholar
  5. Grant DB, Johnston JH (1982) Congenital adrenal hyperplasia and female pseudohermaphroditism. In: Wilhams DI, Johnston JH (eds) Paediatric urology, 2nd edn. Butterworth, London, pp 525–536Google Scholar
  6. Hendren WH, Crawford JD (1969) Adrenogenital syndrome: the anatomy of the anomaly and its repair; some new concepts. J Pediatr Surg 4: 49–58PubMedCrossRefGoogle Scholar
  7. Jones HW, Scott WW (1971) Construction of the feminine external genitalia. In: Jones HW, Scott WW (eds) Hermaphroditism, genital anomalies and related endocrine disorders, 2nd edn. Wilhams and Wilkins, Baltimore, pp 335–348Google Scholar
  8. Kogan SJ, Smey P, Levitt SB (1983) Subtunical total reduction clitoroplasty: a safe modification of existing techniques. J Urol 130: 746–748PubMedGoogle Scholar
  9. Lattimer JK (1961) Relocation and recession of the enlarged clitoris with preservation of the glans: an alternative to amputation. J Urol 86: 113–116PubMedGoogle Scholar
  10. Money J, Hampson JG, Hampson JL (1955) Hermaphroditism: recommendations concerning assignment of sex, change of sex and psychologic management. Bull Johns Hopkins Hosp 97: 284–300PubMedGoogle Scholar
  11. Newns GH, Williams DI (1968) Abnormalities of sexual development. In: Williams DI (ed) Paediatric urology. Butterworths, London, pp 486–520Google Scholar
  12. Randolph JG, Hung W (1970) Reduction clitoroplasty in females with hypertrophied clitoris. J Pediatr Surg 5: 224–231PubMedCrossRefGoogle Scholar
  13. Spence HM, Allen TD (1973) Genital reconstruction in the female with the adrenogenital syndrome. Br J Urol 45: 126–130PubMedCrossRefGoogle Scholar
  14. Whitaker RH (1981) Reconstruction of the genitalia in the adrenogenital syndrome: In: Westenfelder M, Whitaker RH (eds) Malformations of the external genitalia. Monographs in paediatrics. Karger, Basel, pp 86–88Google Scholar
  15. Young HH (1937) Genital abnormalities, hermaphroditism and related adrenal diseases. Williams and Wilkins, BaltimoreGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • R. H. Whitaker
    • 1
  1. 1.Department of UrologyAddenbrooke’s HospitalCambridgeUK

Personalised recommendations