Posterior perineal flap vaginoplasty is highly successful in preventing later vaginal stenosis. However, regular follow up is recommended for calibration and ...
The Surgical Management of Ambiguous Genitalia Secondary to Congenital Adrenal Hyperplasia: Alexandria Experience. Sameh Shehata,1 Ashraf T. Soliman,2 and Said Bedair3 From the departments of Pediatric Surgery, Pediatrics and Radiology, Faculty of Medicine, University of Alexandria, Egypt
Abstract: Congenital adrenal hyperplasia is the most common cause of female pseudohermaphroditism all-over the world. The excessive androgenic intermediates released by the diseased adrenal gland virilize the external female genitalia during the early in-utero life to a variable degree. Medical treatment is mandatory to correct the life-threatening metabolic derangement leading to salt-losing crises and surgical correction of the genitalia is essential for the psychologic and functional integrity of the female patient. We operated on 12 girls with CAH to correct their genitalia. The surgical procedure included: [1]. Reduction clitoroplasty: after complete degloving of the hypertrophied phallus, mobilization of the roots of the corpora to their attachment at the pubic arch. The neurovascular bundle is dissected and preserved to keep supply of the glans. The corporal bodies are dissected from their attachment to the pubic bone to their termination at the glans. Adequate hemostasis is performed and the glans is fixed with sutures to the roots of the corpora. [2]. Vaginoplasty: A posterior perineal flap is fashioned by drawing an inverted u incision centered on anal verge and reaching to the posterior limit of urogenital sinus anteriorly. [3].Labioplasty: the skin of the phallus, after degloving, is used for replacing the labia minora after division in the midline. The results of this operative procedure proved very effective with excellent cosmetic appearance of the genitalia and good vaginal patency in all the cases followed-up for three years. Mild vaginal stenosis was encountered only in three girls but none had tight stenosis. In conclusion, our data proved that complete correction in one stage is the treatment of choice. Reduction clitoroplasty is the best procedure for the treatment of phallic enlargement. Posterior perineal flap vaginoplasty is highly successful in preventing later vaginal stenosis. However, regular follow up is recommended for calibration and dilatation of the vagina and adjustment of the doses of steroid to assure good suppression of adrenal androgens in these girls.
Introduction: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease due to defective biosynthesis of the adrenal glucocorticoid and mineralocorticoid. The commonest enzymatic defect is 21-hydroxylase deficiency. The compensatory accumulation of androgenic intermediate metabolites leads to variable degrees of androgenization of the genotypic female. This is usually in the form of clitoral enlargement together with fused labioscrotal folds and retention of the urogenital sinus. It is considered as a medical and social emergency. Management of patients with CAH includes medical treatment in the form of glucocorticoid and mineralocorticoid hormonal replacement, to correct the metabolic abnormality with suppression of the suprarenal production of the androgenic compounds. The female with adrenogenital syndrome is subject to a variable degree of masculinization; surgery should be directed at three goals [1]. Clitoroplasty: removing the corpora while preserving the glans
sensation and vascularity. [2]. Labioplasty: to achieve normal appearing introitus. [3]. Vaginoplasty: to provide adequate opening for the vagina onto the perineum and avoiding the problem of stenosis. There has been some controversy between different centers regarding the technique and timing of each of these items. In this paper we present our technique in correction of CAH in the Pediatric Surgical Center, University of Alexandria.
Subjects and methods: Twelve girls with CAH, 21-hydroxylase deficiency, were operated on . Cases were referred from the pediatric endocrinology unit after performing diagnostic tests and prescribing the proper hormonal treatment. All patients were diagnosed during the neonatal period or shortly after. The diagnostic biochemical criteria included high basal serum 17-hydroxyprogesterone, testosterone and dehydroepiandrostedione (DHEA) concentrations. All patients were started on medical treatment with glucocorticoid (Hydrocortisone 15-20 mg/m2 surface area daily) and mineralocorticoid ( fludrocortisol 50
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[2]. Vaginal patency: Minimum follow up period was 6 months and longest was 3 years. Three cases were lost for follow-up. The patency was excellent in 6/12 and mild stenosis was encountered in 3 cases. None of the cases had tight stenosis. No case had residual corporal bodies or persistent erections after surgery. The most troublesome difficulty during surgery was bleeding during resection of the highly vascular corpora bodies which occurred in 4/12 cases but none needed blood transfusion. None of the patients had signs of virilization or advancing bone age during the period of the study. Figure 1 shows the preoperative appearance. Note the size of the phallus and scrotalization of the labial folds. Fig. 2 shows the postoperative appearance with restoration of the female phenotype.
– 150 μg daily). The doses were appropriately adjusted based on serum 17-hydroxyprogesterone concentrations and plasma renin activity performed every 2 months. Prior to surgery an ascending genitogram was performed to assess the level of vaginal entry into the urogenital sinus. The age at operation ranged between one year and three years, with a mean of 1.8 years. Preoperatively, the patients had threefold increase in their regular hydrocortisone dose . The surgical technique involved: [1]. Clitoroplasty: we used the technique of reduction clitoroplasty. After complete degloving of the hypertrophied phallus, mobilization of the roots of the corpora to their attachment at the pubic arch. The neurovascular is dissected and preserved to keep supply of the glans. The corporal bodies are dissected from their attachment to the pubic bone to their termination at the glans. Adequate hemostasis is performed and the glans is fixed with sutures to the roots of the corpora.
Discussion: Congenital adrenal hyperplasia is the commonest cause of ambiguity of the genitalia all-over the world. Early diagnosis & proper medical treatment is man-datura to avoid metabolic derangement including salt losing crisis as well as extensive virilization.(1,2,3) Surgical correction of the virilized genitalia is an essential step to create a functioning female.
[2]. Vaginoplasty: A posterior perineal flap is fashioned by drawing an inverted u incision centered on anal verge and reaching to the posterior limit of urogenital sinus anteriorly. A wellvascularized flap is raised cautiously to avoid damage to the rectum. The vagina is identified by careful dissection and posterior wall incised. The perineal flap is laid inside the opened vagina and Vicryl 3/0 stitches inserted. [3]. Labioplasty: the skin of the phallus after degloving is used for replacing the labia minora after division in the midline. It is stretched on both sides of the reconstructed vagina and stitched in place with fine catgut stitches. Labia majora are mobilized as anteriorly based thick flaps and slid to give normal appearance of female genitalia and stitched with catgut. Post operatively the vagina is calibrated after one month and dilated regularly at decreasing intervals. The patients were followed-up regularly and doses of steroids adjusted to keep their 17hydroxyprogesterone and renin in the normal range.
Phallic enlargement: There were a variety of surgical procedures to correct phallic enlargement and restore a more normal female phenotype. Phallic enlargement was first dealt with by amputation clitorectomy. Master and Johnson have suggested that, while the clitoris may not the necessary for female orgasm, it certainty contributes, and thus surgery which completely ablates the glans altogether or does not preserve its blood supply is no longer accepted. This has led to the development of clitoral recession, the corpora in this procedure were folded a back beneath the symphysis pubis.(2,4) The disadvantage of these procedures may not become apparent until puberty, when sexual stimulation leads to painful enlargement of the recessed corporal bodies. Another approach has been to preserve the glans, which is the homologue of the clitoris, while resecting the corpora while preserving the dorsal neurovascular bundle to keep normal sensations. To create a more normal appearing introitus phallic skin and foreskin are used to create labia minora which are absent in these girls.(4,5,6) Vaginal surgery: Vaginal surgery initially consisted of a simple cut back into the vagina from the perineum, while sufficient for the rare low vagina, in many cases this
Results: During the follow-up period which ranged between 1 and 3 years we observed the following data. [1]. Cosmetic appearance: immediately after surgery the appearance was not usually accepted by the parents due to edema and ecchymosis. However, after subsidence of this reaction, 9 cases were described by the parents as being excellent, 3 cases described as acceptable, and none was described as unacceptable.
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approach led to stenosis of the vaginal introitus. A perineal flap following the principles of a v-y plasty was suggested by Fortunoff and associates this more satisfactorily opens the vagina because the flap of perineal skin is interposed in the posterior vaginal wall.(1,3,7)
preoperatively by ascending genitogram. In case of high vaginal entry, clitoroplasty and labioplasty can be performed initially and vaginoplasty delayed for a later age.(8,9,10)
Timing of surgery: As would be expected, the genital ambiguity is extremely distressing to the parents and to the child if left uncorrected. So, the tendency to early correction is well justified. It has been suggested that complete correction can be done at around the age of 6 months. We operated our cases at the age of 1-3 years with a mean of 1.8 years due to the age of presentation. We are tending to operate at progressively younger ages. The full correction can be done in one stage except for the rare high entry of vagina into urogenital sinus; this can be assessed
The most common type of anomaly in adrenogenital syndrome is medium entry of the vagina into the urogenital sinus. This can be confirmed before surgery through ascending genitogram. The recommended age of correction is at one year of age. Complete correction in one stage is the treatment of choice. Reduction clitoroplasty is the best procedure for the phallic enlargement. Posterior perineal flap vaginoplasty is highly successful in preventing later vaginal a stenosis. However, regular follow up is recommended for calibration and dilatation of the vagina.
Conclusion:
Figure 1
Figure 2
References: 1. Danahoe Pk, Hendren WH : perineal reconstruction in ambiguous genitalia in infants raised as females. Ann Surg 1984; 200:363-371. 2.Allen LE, Hardy BE, Churchill BM. The surgical management of the enlarged clitoris, J Urol 1982; 128: 351-354. 3. Danahoe PK. the diagnosis and treatment of infants with intersex abnormalities. Pediatr Clin North Am 1987; 34 (5): 1333-1348. 4. Pinter A, Newman K, Randolf j , Anderson K. The surgical management of infants and children with ambiguous genitalia: lessons learned from 25 years. Ann Surg 1992; 215(6): 644-653.
5. Randolph SG, Hung W. Reduction clitoroplasty in females with hypertrophied clitoris. J Ped Surg 1970; 5: 224-231. 6. Spence HM , Allen TD. Genital reconstruction in adrenogenital syndrome. Br J Urol 1973; 45: 126130. 7. Snyder HM, Retik AB, Bauer SB, et al. Feminizing genitoplasty: A synthesis J Urol 1983; 129: 1024. 8. Altwein JE, Homoki J. Feminization of children with adrenogenital syndrome using the nerve sparing technique: experience with 25 girls: in plastic surgery in sexually handicapped. Berlin and Heidelberg: Eider Springer Verlag 1989: 15-22. 9. Oesterling JE, Gerhart JP, Jeffs RD. A unified approach to early reconstructive surgery of the child
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with ambiguous genitalia. J Urol 1987; 138: 10791084.
10. Sharp RJ, Holder TM, Campbell et al. Neonatal genital reconstruction. J Ped Surg 1987; 22 168-171. 1987.
Correspondence: Sameh Shehata MD Associate Professor of Pediatric Surgery Alexandria University Children’s Hospital El-Shatby, Alexandria, EGYPT
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