SURGICAL CORRECTION OF FEMALE PSEUDOHERMA- PHRODITISM DUE TO ADRENAL HYPERPLASIA By A. J. C. HUFFSTADT, M.D. Department of Plastic Surgery, University Hospital, Groningen, The Netherlands SINCE the work of Wilkins (I965), the p~ediatricians understand much more about female pseudohermaphroditism. He showed the role of the adrenals in this syndrome. He also introduced treatment with cortisone to stop the process of virilisation. Recently Visser (1966) reviewed several aspects of congenital adrenal hyperplasia (CAH). Because of these developments surgeons will be asked more often for corrective operations in these patients, and I shall discuss some points of the surgical treatment of the deformed genitals. FIG. I FIG. 2 The increased elaboration of androgenic substances during embryonic life in female patients with CAH results in hypertrophy of the clitoris (Fig. I) and variable fusion of the labioscrotal folds (female pseudohermaphroditism). A high degree of labioscrotal fusion results in the formation of a long, narrow urogenital sinus opening in a small round or slit-like meatus at the base of the phallus (Fig. 2). These forms particularly are sometimes diagnosed as hypospadias and the baby will be registered as male. In a few cases a complete penile urethra has been described. With a lesser degree of fusion, there is a shallow vulva with a funnel-shaped orifice at the posterior end. The urethra opens into this just above the orifice. In some cases there is no fusion with separate vaginal and urethral orifices (Wilkins, I965). The internal duct system has developed normally and normal infantile ovaries are present. q~o
360 BRITISH JOURNAL OF PLASTIC SURGERY Most authors agree that the best age of the patient at the time of operation should be during the first four years of life. In my opinion it is often wise not to operate in the first year of life, because of technical difficulties and the risks of surgical stress. A large clitoris often diminishes in size after medical treatment and operation should not be performed too early. On the other hand, from a psychological point of view, it is advisable not to postpone operations on the genitalia for too long. I prefer to operate in the second year of life. :.( ---),/ FIG. 3 ---) FIG. 4 If should be emphasised that patients with CAH, like patients with Addison's disease, should be treated shortly before and a few days after surgery with a two- to tenfold increase in the maintenance dose of glucocorticoids. Details of corrections of labioscrotal fusion have been discussed by Jones and Jones (I954) and Jones and Wilkins (I96I). The simplest way to deal with the enlarged clitoris is to amputate it. Jones and Jones support this idea and add a little flap procedure to form a " cosmetic clitoris ". A better treatment of the enlarged clitoris has been described by Lattimer (I96I) ; this author suggested a procedure with preservation of the glans, which may be important for normal sexual experience in later life. Recently Stefan (I966) has described a slightly different method for reconstruction of the vulva. It should be emphasised that during surgical correction of the external genitalia in female patients with CAH, clitorectomy should be definitely avoided: reconstruction should be the aim. There is one exception to this rule. When this operation is performed on an older girl preservation of the large clitoris, although tunnelled,
SURGICAL CORRECTION FIG. 5 FIG. 7 OF FEMALE PSEUDOHERMAPHRODITISM FIG. 6 FIG. 8 361
362 BRITISH JOURNAL FIG. 9 FIG. I I OF P L A S T I C SURGERY FIG. IO FIG. 12
SURGICAL CORRECTION OF FEMALE PSEUDOHERMAPHRODITISM 363 might produce embarrassment by too forceful erections. Partial amputation might be necessary. I would still prefer to do this partial amputation in a second operation. The newly formed clitoris can be left alone while the partial excision takes place on the buried shaft. Figure 3 shows the preserving and relocating of the enlarged clitoris (following the directions of Lattimer). The skin of the phallus has been removed except for a small island on the dorsal side of the glans. A tunnel has been created from the base of this denuded phallus to the ultimate location of the glans, where a sort of buttonhole is made. Then, after complete severance of the suspensory ligaments, the phallus is pulled through and the preserved island of skin on the glans is stitched into the buttonhole. The skin defect at the base of the phallus can easily be sutured directly, after a little undermining of the surrounding skin. Correction of the labioscrotal fusion is shown in Figure 4 in which the vaginal orifice has been externalised and a normal vulva and introitus have been created according to H. W. Jones' method. Figures 5 and 6 show a severe case of labioscrotal fusion--forming a long urogenital sinus-- while in Figure 7 the inkmark shows how far the probe has been introduced. The sinus has been opened in the midtine, the vaginal mucosa was freed on the rectal side for about 2 cm. and by stitching mucosa to skin the externalisation of the vaginal orifice was completed as is shown in Figure 8 (a catheter has been introduced in the urethra and forceps into the vagina). The next step will be the correction of the hypertrophic clitoris (Fig. 9). In Figure IO the clitoris is shown after the skin has been excised except for a small round island on the dorsal side of the glans. The inkmark shows where this skin island will be fixed after the denuded clitoris has been pulled through a tunnel from its base to the new location of the clitoris. In Figure II the result at the end of the operation is clear. Stefan advises the use of a urinary catheter for 7 to IO days. When meticulous suturing of the wound has been done this is not necessary and there is less risk of infection. We never use a catheter in the postoperative period. Figure I2 shows the situation after the wound has healed. Six months later it is hardly possible to distinguish the reconstructed vulva from a normal one. This surgical method can be recommended as being simple and reconstructive, with very little stress for the patient. SUMMARY Surgical correction of female pseudohermaphroditism consists of two parts : I. Correction of the hypertrophic clitoris. 2. Reconstruction of a normal vulva. The best age for the operation is in the second year of life. The clitoris should not be amputated but relocated. Close co-operation with the pa:diatricians is necessary for the treatment with glucocorticoids. REFERENCES JONES, H. W., and JONES, G. E. S. (1954). Am. J. Obstet. Gynec., 68, 133o. JONES, H. W., and WILKINS, L. (I96I). Am. J. Obstet. Gynec., 82, 1142. LATTIMER, J. K. (I96I). J. Urol., 8o~ II3. STEFAN, H. (I966). Z. Kinderheilk., 3, 249. VlSSER, H. K. A. (1966). Archs Dis. Childh., 41, 2. WILKINS, L. (I965). " The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence," 3rd ed. Springfield, II1. : C. C. Thomas.