Feminizing genitoplasty in congenital adrenal hyperplasia: A new method for clitoral reduction

Yogender S Kadian; Kajal Pradeep; Vikas Verma

doi:10.4103/2278-9596.202368

ORIGINAL ARTICLE

Year : 2016 | Volume : 6 | Issue : 3 | Page : 153-157

Feminizing genitoplasty in congenital adrenal hyperplasia: A new method for clitoral reduction

Yogender S Kadian, Kajal Pradeep, Vikas Verma
Department of Paediatric Surgery, Pt. B. D. Sharma PGIMS, Rohtak, Haryana, India

Date of Web Publication

17-Mar-2017

Correspondence Address:
Yogender S Kadian
Department of Paediatric Surgery, Pt. B. D. Sharma PGIMS, Rohtak, Haryana - 124 001
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2278-9596.202368

Abstract

Background: Congenital adrenal hyperplasia (CAH) is a disorder wherein female babies are born with masculinized external genitalia characterized by hyperpigmented or fused labioscrotal tissue, a urogenital sinus, and clitoromegaly. Feminizing genitoplasty (clitoroplasty, labioplasty, and vaginoplasty) is necessary to make the patient consistent with gender identity and to ensure adequate sexual function in pubertal age.
Patients and Method: We performed feminizing genitoplasty in which reduction clitoroplasty was done by a ventral approach in 6 patients of CAH, with a mean age of 3.8 years. Excessive erectile tissue was excised by giving incisions into Buck's fascia at 8 and 4 o'clock position on the clitoral shaft and Byar's flaps created from the degloved clitoral shaft were used for labial and vaginal reconstruction. The glans clitoris was left as such and anchored to proximal 1–1.5 cm clitoral shaft.
Results: All patients had good cosmetic female appearance of external genitalia. In 5 patients, the clitoroplasty was done along with labioplasty and vaginal introitus exteriorization. However, in 1 patient, the vaginal confluence into urogenital sinus was high and hence only clitoral reduction as well as labioplasty was done and the urogenital sinus opening was left as such. The maximum follow-up is up to 6 years with good result.
Conclusion: The reduction clitoroplasty by present approach of 8 and 4 o'clock incision into the Buck's fascia is a good option for clitoral girth and length reduction as it preserves approximately two-third of Buck's fascia with intact neuromuscular bundle and glans. Postoperatively, patients have good vascularity and sensation of the glans clitoris and acceptable cosmetic outcome. However, the long-term results are difficult to comment as no patient of the present study achieved puberty.

Keywords: Clitoroplasty, congenital adrenal hyperplasia, feminizing, genitoplasty

How to cite this article:
Kadian YS, Pradeep K, Verma V. Feminizing genitoplasty in congenital adrenal hyperplasia: A new method for clitoral reduction. Arch Int Surg 2016;6:153-7

How to cite this URL:
Kadian YS, Pradeep K, Verma V. Feminizing genitoplasty in congenital adrenal hyperplasia: A new method for clitoral reduction. Arch Int Surg [serial online] 2016 [cited 2023 Dec 3];6:153-7. Available from: https://www.archintsurg.org/text.asp?2016/6/3/153/202368

Introduction

Congenital adrenal hyperplasia (CAH) is a disorder wherein female babies are born with masculinized external genitalia because of excessive in utero androgen exposure. This masculization is characterized by hyperpigmented or fused labioscrotal tissue, a urogenital sinus, and clitoromegaly.^[1],[2] Feminizing genitoplasty (clitoroplasty, labioplasty, and vaginoplasty) is necessary to make these patients consistent with gender identity and to ensure adequate sexual function in pubertal age. The surgical treatment of clitoromegaly has undergone evolutionary changes. Initial efforts to correct clitoromegaly consisted of its amputation.^[3] However, the realization of the role of clitoris in the development of female sexuality stimulated the necessity for clitoral preservation. This prompted the development of techniques to bury or recess the clitoris.^[4] Although it preserves the clitoral innervations and vascularity, it is associated with painful erections. In 1970, Randolph and Hung devised a new technique for correction of enlarged clitoris and named it reduction clitoroplasty. This procedure involves the excision of a portion or the entire shaft with preservation of glans.^[5] Kogan et al. described a subtunical excision of the erectile tissue via lateral or dorsal incision into corpora with the preservation of the dorsal neurovascular structures.^[6] However, recently Baskin et al. described a detailed description of the dorsal nerve anatomy of clitoris and found that more concentration of these dorsal nerves are at 11 and 1 o'clock position.^[7] Based on this concept, Poppas et al. described a nerve sparing ventral approach for clitoral reduction wherein the clitoral reduction was done by making incisions into Buck's fascia just lateral to the 6 o'clock position, and the excised erectile tissue was examined to quantify the nerves.^[8] This ventral approach has been reported to give better long-term cosmetic and functional results.^[9] Taking into consideration the above concepts, the authors of the present report have performed clitoral reduction in 6 patients with CAH by ventral approach, however, the incisions sites were at 8 and 4 o'clock positions of the corporal shaft rather than just lateral to 6 o'clock position, as described by Poppas et al.^[8] Moreover, the authors also discussed the advantage of this approach. Along with the clitoral reduction, the glans was also preserved as such and the labia minora reconstruction was done, as well as exteriorization of vaginal introitus was done when it was feasible.

Patients and Methods

We have retrospectively reviewed the case records of 6 female patients of CAH who underwent the surgery for enlarged clitoris in the institution. The age ranged from 2–6 years (mean of 3.8 years) and all patients were evaluated by a team of endocrinologist, child psychologist, and surgeon. Their hormonal status was normalized before surgery. Preoperative genitogram cystoscopic assessment of the urogenital sinus were done and confluence of vagina into it was visualized.

Surgical technique

All patients were operated under general anesthesia and the procedure was done in a lithotomy position and an indewelling catheter was inserted into the bladder. Then, a stay suture was applied on the glans and subcoronal incision was made 5 mm proximal to the glans all around the shaft; urethral plate was also divided and the clitoral shaft was degloved with the dorsal skin divided into two equal flaps (Byars flap) [Figure 1] and [Figure 2]. Then, Bucks fascia was incised by two parallel incisions on 8 o'clock and 4 o'clock positions. The erectile tissue was excised from corporal bodies from the glans distally and proximally leaving a 1.0–1.5 cm clitoris stump just distal to the crural bifurcation. This stump was oversewn with a 4 0 vicryl suture. Afterwards, the glans clitoris was anchored with the remaining clitoral shaft. The dorsal flaps were rotated and placed on the sides to create labia minora, and the vaginal opening was exteriorized with the help of local skin flaps. Urinary catheter was removed on the 5^th postoperative day and then the patient was discharged home.

Figure 1: The clinical photograph showing enlarged clitoris as well as the vaginal opening

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Figure 2: The degloved clitoral shaft with the created Byar's flap

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Results

In all 6 patients the clitoral size was reduced and it was visible only after separation of the reconstructed labial flaps [Figure 3] and [Figure 4]. However, in one patient the vaginal confluence into the urogenital sinus was high, and it was left as such without vaginal reconstruction; however, only labioplasty was done. In the remaining 5 patients, the clitoroplasty was associated with labioplasty and vaginal introitus exteriorization. The maximum follow-up is up to 6 years, and the external genitalia appearance was of the female type [Figure 5]. However, no patient of the present study achieved the pubertal age.

Figure 3: The completed repair clinical photograph with labia separated

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Figure 4: The completed repair clinical photograph with labia apposed

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Figure 5: The follow-up clinical photograph with a near normal appearance of female external genitalia

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Discussion

Virilization of female external genitalia is commonly observed in CAH, and feminizing genitoplasty is needed for surgical correction of this anomaly. Feminizing genitoplasty essentially consists of clitoroplasty, labioplasty, and vaginoplasty. Historically, clitoral surgery has passed through evolutionary changes ranging from amputation of the organ to its complete preservation (clitoral recession), wherein the clitoris is embedded near symphysis pubis.^[3],[4] However, both the approaches for an enlarged clitoris have been abandoned because of the realization of the importance of clitoris in female sexual function and painful erections associated with its recession. Therefore, the choice of technique for clitoral surgery has remained a subject of debate and various procedures have been described in literature for reducing the its size with acceptable cosmetic and functional results.^[5],[6],[10] The procedure of removing the erectile tissue along with preservation of neurovascular bundle has been named reduction clitoroplasty, however, there is still controversy regarding its method and approach.

Dorsal nerve preservation is vital to future sexual function because it mediates clitoral somatosensory function. From an experimental study in female animals, wherein pelvic nerve stimulation caused genital swelling, increased clitoral pressure, engorgement and increase in corporal diameter and length, and the importance of dorsal nerve in clitoral somatosensory function has been realized.^[11],[12] The course of these dorsal nerve has been recently described in detail by Baskin et al.^[7] Hence, reduction clitoroplasty by the ventral approach rather than the dorsal or lateral approach causes minimal damage to the clitoral nerves. Poppas et al. described a new technique of reduction clitoroplasty wherein erectile tissue was excised by incising the Buck's fascia just lateral to the 6 o'clock position (ventral approach).^[8] They reported very minimal loss of nerve tissue, which was confirmed by immunoperoxidase staining of the excised erectile tissue in the study. In the present study, the authors realized clitoral reduction by incising at 8 and 4 o'clock position rather than just lateral to 6 o'clock, as described earlier. This approach leaves approximately two-third of Buck's fascia intact along with intact neuromuscular bundle. Moreover, it is technically easy, causes no blood loss, and can be done without loop magnification, as compared to the procedure described by Poppas et al. However, the risk of damage to dorsal nerve may be more in comparison to the study of Poppas et al. because the incision into Buck's fascia is given at 8 and 4 o'clock position rather than just lateral to the 6 o'clock position. Further, in the present study, the excised erectile tissue was not examined for the presence of nerves by immunoperoxidase staining, and hence, it is difficult to exactly quantify the nerves lost. Moreover, it must be mentioned here that the maximal density of the dorsal nerve has been documented at the 11 and 1 o'clock position, which area is left untouched in the present approach.

There are other reports in literature wherein the clitoral reduction has been reported with good results when it is done either by reducing clitoral girth or even by corporeal sparing technique.^[13],[14] Hence, it is difficult to standardize the clitoral reduction surgery, however, the final outcome of all the procedures is to have a short clitoris, just visible glans with good somatosensory function. In the present study, the clitoral size was reduced to a size which made it visible only after separation of the labial folds, and the external genitalia appearance was of the female type [Figure 4]. Along with clitoroplasty, labial as well as vaginal reconstruction should be done in patients of CAH.^[15] The advantage of this approach is that the prepuce skin is available to create labia minora as well as for exteriorization of vagina. In the present study, vaginoplasty was done in 5 out of 6 patients by using local skin flap because of low confluence of vagina into the urogenital sinus. However, in one patient, the vaginal opening into the urogenital sinus was high near the bladder neck so that only the clitoroplasty along with labioplasty was done, and the urogenital sinus opening was left as such because of the fear of urinary incontinence as authors did not have enough experience with urogenital sinus mobilization.

There are a number of studies available in the literature regarding the clitoral sensitivity after clitoroplasty, however, it is difficult to interpret them because of the heterogeneous nature of the data and the different techniques used for it.^[16],[17] In the present study, the maximum follow-up was for 6 years and no patient had entered the pubertal age, therefore, it is difficult to comment on sexual response following surgery; however, the cosmetic outcome was good, as observed on the appearance of external genitalia on follow up.

Conclusion

The reduction clitoroplasty by present approach of 8 and 4 o'clock incision into the Buck's fascia is a good option for clitoral girth and length reduction as it preserves approximately two-third of Buck's fascia with intact neuromuscular bundle and glans. Postoperatively, patients had good vascularity and sensation of the glans clitoris as well as acceptable cosmetic outcome. However, the long-term results are difficult to comment on as no patient of the present study has entered puberty.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Rink R, Kaefer M. Surgical Management of Intersexuality, Cloacal Malformations and other Abnormalities of the Genitalia in Girls. In: Kavoussi LR, Novick AC, Wein AJ, editors. Campbell-Walsh Urology. 9^th ed. Philadelphia: Elsevier; 2007. p. 3830.
2.	New MI. An update of congenital adrenal hyperplasia. Ann N Y Acad Sci 2004;1038:14-9.
3.	Gross RE, Randolph J, Crigler JF. Clitorectomy for sexual abnormalities: Indication and technique. Surgery 1966;59:300-4.
4.	Lattimer JK. Relocation and recession of enlarged clitoris with preservation of the glans: An alternative to amputation. J Urol 1961;86:113.
5.	Randolph JG, Hung W. Reduction clitoroplasty in female with hypertrophied clitoris. J Pediatr Surg 1970;5:126-8.
6.	Kogan SJ, Smey P, Levitt SB. Subtunical total reduction clitoroplasty: A safe modification of existing techniques. J Urol 1983;130:746-8.
7.	Baskin LS, Erol A, Li YW, Liu WH, Kurzrock E, Cunha GR. Anatomical studies of the human clitoris. J Urol 1999;162:1015-20.
8.	Poppas DP, Hochsztein AA, Baergen RN, Loyd E, Chen J, Felsen D. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol 2007;178:1802-6.
9.	Yang J, Felson D, Poppas DP. Nerve sparing clitoroplasty: Analysis of clitoral sensitivity and viability. J Urol 2007;178;1598-601.
10.	Rajfer J, Ehrlich RM, Goodwin WE. Reduction clitoroplasty via ventral approach. J Urol 1982;128:341-4.
11.	Giraldi A, Marson L, Nappi R, Pfaus J, Traish AL, Abdulmaged M, et al. Physiology of female sexual function: Animal models J Sex Med 2004;1:237-41.
12.	Baskin LS, Yucel S, Cunha GR, Glickmen SE, Place NJ. A neuroanatomical comparison of humans and hyena, a natural animal model for common urogenital sinus: Clinical reflection on feminizing genitoplasty. J Urol 2006;175:276-81.
13.	Hutson JM, Voigt RW, Luthra M, Kelly JH, Flower R. Girth-reduction clitoroplasty-a new technique: Experience with 37 patients. Pediatr Surg Int 1991;6:336-9.
14.	Pippi Salle JL, Braga LP, Macedo N, Rosito N, Bagli D. Corporeal sparing dismembered clitoroplasty: An alternative technique for feminizing genitoplasty. J Uro 2007;178:1796-801.
15.	Stikkelbroeck NM, Beerendonk CC, Willemsen WN, Schreuders-Bais CA, Feitz WF, Rieu PN, et al. 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;16:289-96.
16.	Frost-Arner L, Aberg M, Jacobsson S. Clitoral sensitivity after surgical correction in women with adrenogenital syndrome: A long term follow-up. Scand J Plast Reconstr Surg Hand Surg 2003;37:356-9.
17.	Minto CL, Liao LM, Woodhouse CR, Ransley PG, Creighton SM. The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: A cross-sectional study. Lancet 2003;361:1252-7.