Abstract
The ideal timing and nature of surgical reconstruction in individuals with Disorders
of Sex Development (DSD) is highly controversial. Despite the increasing number of
publications on this topic, evidence-based recommendations still cannot be made. However
it is generally accepted that optimal care for DSD requires an experienced multidisciplinary
team. This means that surgical decisions are now made within the context of a multidisciplinary
team and all members of the team – and not just specialist surgeons – may be called
upon to discuss choices for surgery with patients and parents. To do this well, every
clinician in the team should have an understanding of the range of techniques available
for genital surgery, the risks and benefits of procedures and the controversies surrounding
timing of surgery.
The aim of this paper is to give an overview of the variety of surgical procedures
in current use and in what situation a particular technique would be indicated. The
short-term risks and benefits are described and where available long-term outcome
data is discussed. To date, discussions surrounding genital surgery have been led
primarily by surgeons. Some non-surgical clinicians have expressed unease about decision
making in genital surgery but have felt ill equipped to comment on an area with which
they are unfamiliar. This review gives a detailed explanation of current surgical
practice offered in a specialized center for DSD and such information should facilitate
a more balanced discussion.
Keywords
To read this article in full you will need to make a payment
Subscribe to Journal of Pediatric Urology
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Psychosexual outcome in women affected by congenital adrenal hyperplasia due to 21-hydroxylase deficiency.J Urol. 2004 Jun; 171: 2497-2501
- Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia.J Clin Endocrinol Metab. 2008 Feb; 93: 380-386
- Sexual function and attitudes toward surgery after feminizing genitoplasty.J Urol. 2011 Mar 28; 185 (American Urological Association Education and Research, Inc): 1900-1904
- Disclosing disorders of sex development and opening the doors.Sex Dev. 2010 Sep; 4: 304-309
- Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood.Lancet. 2001 Jul 14; 358: 124-125
- Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study.BJU Int. 2004 Jan; 93: 135-138
- The long term outcome of feminizing genital surgery for congenital adrenal hyperplasia: anatomical, functional and cosmetic outcomes, psychosexual development, and satisfaction in adult female patients.J Pediatr Adolesc Gynecol. 2003 Oct; 16: 289-296
- Sexual function and surgical outcome in women with congenital adrenal hyperplasia due to CYP21A2 deficiency: clinical perspective and the patients' perception.J Clin Endocrinol Metab. 2010 Aug; 95: 3633-3640
- Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an endocrine society clinical practice guideline.J Clin Endocrinol Metab. 2010; : 4133-4160
- Clitoroplasty in intersex: a new technique.Br J Urol. 1981 Aug; 53: 371-373
- Subtunical total reduction clitoroplasty: a safe modification of existing techniques.J Urol. 1983 Oct; 130: 746-748
- Self-assessment of genital anatomy, sexual sensitivity and function in women: implications for genitoplasty.BJU Int. 2004 Sep; 94: 589-594
- Corporeal sparing dismembered clitoroplasty: an alternative technique for feminizing genitoplasty.J Urol. 2007 Oct; 178 ([Discussion 1801]): 1796-1800
- Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia.J Urol. 2008 Feb; 179: 634-638
- Consensus statement on management of intersex disorders.Pediatrics. 2006 Aug 1; 118: e488-e500
- Clitoroplasty and vaginoplasty in adolescents and adults with disorders of sex development (DSD): lessons learned.Can Urol Assoc J. 2012 Jun; 6: S48-S49
- Vaginoplasty technique for female pseudohermaphrodites.Surg Gynecol Obstet. 1964 Mar; 118: 545-548
- Passerini-glazel feminizing genitoplasty: modifications in 17 years of experience with 82 cases.Eur Urol. 2007 Dec; 52: 1638-1644
- Total urogenital mobilization–an easier way to repair cloacas.J Pediatr Surg. 1997 Feb; 32 ([Discussion 267–8]): 263-267
- Total urogenital sinus mobilization: expanded applications.BJU Int. 1999 May; 83: 820-822
- Partial urogenital mobilization: a limited proximal dissection.J Pediatr Urol. 2006 Aug; 2: 351-356
- Total and partial urogenital mobilization: focus on urinary continence.J Urol. 2012 Apr; 187: 1422-1426
- Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia.J Urol. 2006 Nov; 176: 2199-2204
- Correction of congenital abnormalities of the vagina and perineum.J Pediatr Surg. 1980 Dec; 15: 751-763
- Transanorectal approach for the treatment of urogenital sinus: preliminary report.J Pediatr Surg. 1992 Jun; 27: 681-685
- Anterior sagittal transanorectal approach to the urogenital sinus in adrenogenital syndrome: preliminary report.J Pediatr Surg. 1997 May; 32: 714-716
- Surgical treatment of high urogenital sinuses using the anterior sagittal transrectal approach: a useful strategy to optimize exposure and outcomes.J Urol. 2012 Jan; 18
- The role of estrogen deficiency in skin ageing and wound healing.Biogerontology. 2012 Feb; 13: 3-20
- Primary caregivers of children affected by disorders of sex development: mental health and caregiver characteristics in the context of genital ambiguity and genitoplasty.Int J Pediatr Endocrinol. 2010; 2010: 690674
- A long-term outcome study of intersex conditions.J Pediatr Endocrinol Metab. 2005 Jun; 18: 555-567
- A long-term view of hypospadias.Br J Plast Surg. 1989 May; 42: 251-255
- Proximal hypospadias repair outcomes in patients with a specific disorder of sexual development diagnosis.Adv Urol. 2012; 2012: 708301
- Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia. American Academy of Pediatrics.Pediatrics. 1996 Apr; 97: 590-594
- Is there an ideal age for hypospadias repair? A pilot study.J Pediatr Urol. 2009 Oct; 5: 345-350
- Outcomes of hypospadias repair in older children: a prospective study.J Urol. 2011 Jun; 185: 2483-2485
- Hypospadias surgery: a 10-year review.Pediatr Surg Int. 2004 Mar; 20: 200-203
- Outcomes of delayed hypospadias repair: implications for decision making.J Urol. 2007 Jul; 178: 278-281
- Early hypospadias surgery may lead to a better long-term psychosexual outcome.J Urol. 2009 Oct; 182: 1744-1749
- Hypospadias repair in adults: adventures and misadventures.J Urol. 2001 Jan; 165: 77-79
- The Cleveland clinic experience with adult hypospadias patients undergoing repair: their presentation and a new classification system.BJU Int. 2011 Apr; 107: 1142-1146
- Age and pro-inflammatory cytokine production: wound-healing implications for scar-formation and the timing of genital surgery in boys.J Pediatr Urol. 2011 Jun; 7: 324-331
- Gonadal pathology and tumor risk in relation to clinical characteristics in patients with 45, X/46, XY mosaicism.J Clin Endocrinol Metab. 2011 Jul; 96: E1171-E1180
- Current management of the undescended testicle.Semin Pediatr Surg. 2007 Feb; 16: 64-70
- 46, XY pure gonadal dysgenesis: clinical presentations and management of the tumor risk.J Pediatr Urol. 2011 Feb; 7: 72-75
- Clinical and gonadal features and early surgical management of 45, X/46, XY and 45, X/47, XYY chromosomal mosaicism presenting with genital anomalies.J Pediatr Urol. 2012 Jan 24;
- Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.Clin Endocrinol (Oxf). 2012 Jan 2;
- Puberty in subjects with complete androgen insensitivity syndrome.Horm Res. 2006; 65: 126-131
Article Info
Publication History
Published online: November 12, 2012
Accepted:
October 1,
2012
Received:
September 6,
2012
Identification
Copyright
© 2012 Journal of Pediatric Urology Company. Published by Elsevier Inc. All rights reserved.
