- Email: [email protected]
A new and simplified method for concealing the hypertrophied clitoris
A New and Simplified Method for Concealing the Hypertrophied Clitoris By Julian S. Ansell and Jacob Rajfer
Seattle, Washington 9 Current surgical procedures that are utilized in the management of patients with an enlarged clitoris are both complex and challenging. W e report a n e w method of concealing the hypertrophied clitoris between the labia majora instead of resorting to the more difficult clitoral amputation or recession. This concealment of the clitoris is simple, m a y be done on an outpatient basis, and accomplishes the goal of creating normal-appearing female external genitalia. INDEX WORDS: Clitoroplasty; intersexuality; ambiguous genitalia.
HE E V A L U A T I O N , diagnosis, and subsequent medical and surgical treatment of patients with ambiguous external genitalia still continues to engender a certain amount of confusion among most physicians. Although this condition is relatively rare, the vast majority of these patients are seen in infancy and are usually diagnosed as having either congenital virilizing adrenal hyperplasia or mixed gonadal dysgenesis, the two most common causes of ambiguous genitalia in the newborn period. ~ Although patients with either of these two disorders may present morphologically anywhere along the spectrum of the normal phenotypic male with bilateral cryptorchidism to the normal phenotypic female with a slightly enlarged clitoris, almost all of these patients are subsequently reared as females 2 and ultimately present to the surgeon for some form of genital reconstruction. Since the goals of any surgical reconstruction of the external genitalia are the achievement of normal appearance and function, the present recommendation for these patients to be reared as females is to reconstruct the hypertrophied clitoris at an early age and postpone the creation of a vaginal canal until after puberty. Currently, there are a variety of surgical procedures that are utilized in the reconstruction of the patient with an enlarged clitoris. These methods range from total amputation to partial amputation to recession of the organ. Recently, while planning to perform a total clitoridectomy on a 4-mo-old patient with a diagnosis of congenital adrenal
T
Journal of Pediatric Surgery, Vol. 16, No. 5 (October), 1981
hyperplasia who presented with an enlarged clitoris (Fig. 1), it became apparent to us that instead of resorting to recession or resection of the clitoris, it might be possible to conceal the enlarged organ between the labia majora. PROCEDURE
The patient is placed in the dorsal lithotomy position and stay sutures of 2-0 silk are placed in each labium majora alongside the tip of the glans clitoris (Fig. 2). Following this, an inverted U incision is then made through the skin and subcutaneous tissue, lateral to, and above the glans clitoris. The incision is then widened subcutaneously in order to allow adequate closure of each side of the inverted U in three successive layers thereby concealing the hypertrophied clitoris. Each of the two layers of skin is closed with running absorbable subcuticular suture of 4-0 dexon and the subcutaneous fat is closed with interrupted absorbable 4-0 dexon suture. The postoperative appearance of the external genitalia is depicted in frame 6 of Fig. 2. A 3-mo follow-up of this patient reveals a satisfactory short-term cosmetic result (Fig.
3). DISCUSSION
Ideally, a n y procedure that is utilized in the surgical reconstruction of the patient with ambiguous genitalia should create a normalappearing and functioning external genitalia. Based on these two tenets, various methods of reconstructing the hypertrophied clitoris have been described. In 1937, Young 3 described the first method for correcting this anomaly. His procedure involved amputating only the shaft of the clitoris in an attempt to preserve the supposedly sensitive glans clitoris. However, most of these operations ended in failure when the glans clitoris sloughed because of an impaired vascular supply. Fourteen years later, Jones and Jones 4 described their method of total clitoridectomy and the formation of a pseudoclitoris from an From the Department of Urology, University o f Washington, School o f Medicine, Seattle, Wash. Address reprint requests to Dr. Julian S. Ansell, Department o f Urology, University o f Washington School o f Medicine, Seattle, Wash. 98105. 9 1981 by Grune & Stratton, Inc. 0022-3468/81/1605-0006501.00/0
681
Fig. 1, Genital morphology of patient with congenital virilizing adrenal hyperplasia at (A) 25 days of age {prior to cortisol therapy) and (B) 3 mo of age (following cortisol therapy). Note the marked reduction in phallic and scrotal size following cortisol treatment.
nli;ie n'unStS' ))j
,i
4. Underside of skin sutured by subc u t i c u l a r stitch.
5. Middle layer closed with inter' rupted sutures,
Fig. 2.
6, Subcaticular closure of surface layer of skin.
Surgical method of concealing the hypertrophied clitoris (Frames 1 to 6).
CLITORAL CONCEALMENT
' Fig. 3. (A, B) Appearance of external genitalia 3 mo following concealment clitoroplasty,
adjacent skin flap. In 1961, Lattimer 5 described his procedure of trimming the glans clitoris to a size appropriate for the age of the patient and relocating the shaft of the clitoris under the skin in such a way that the glans protruded through a
683
new position at the top of the vaginal vestibule. In a long-term follow-up, the cosmetic result from this operation was deemed very satisfactory and the authors stressed that preserving the tactile and erectile function of the glans in this operation did contribute to the sexual gratification of their patients. 6 However, the belief that the glans clitoris is necessary for normal sexual gratification in the female has been challenged by Money et al. 7 They documented that patients with congenital adrenal hyperplasia who underwent total clitoridectomy during infancy and were raised as females were able to achieve orgasm at adulthood. Nevertheless, several years following this report, Randolph and Hung 8 described their technique of reduction clitoroplasty where the shaft of the clitoris was buried under the pubic bone and the mons veneris was reconstructed to cover the enlarged organ. Randolph and Hung realized, however, that not all patients with a hypertrophied clitoris are suitable for their procedure and, in fact, one of their patients with an unsatisfactory cosmetic result subsequently underwent a total clitoridectomy. It is evident from reviewing the literature and from our experience that the size of the hypertrophied clitoris is the major factor in deciding whether to amputate or relocate it. If a large clitoris is recessed or relocated, it is possible for the patient to present with a turgid and painful mass during sexual excitation in later life. 9'1~ HenCe, total clitoridectomy would be the procedure of choice in this situation. In most of the patients who present with less than a penis-like organ, the surgeon must then decide which procedure appears suitable. In most cases with moderate clitoral hypertrophy it may be possible to conceal the hypertrophied clitoris instead of resorting to amputation or recession of the organ. Although we have performed this concealment procedure in just one patient, we were impressed by its simplicity, ease of performance, and cosmetic result. In addition to this, the operative procedure may be undertaken early in infancy as soon as the patient's metabolic and hormonal status have been stabilized and the sex of rearing has been determined. Furthermore, in an uncomplicated situation, it may even be possible to perform this procedure on an outpatient basis as was done in our case.
684
ANSELL AND RAJFER
REFERENCES
1. Grumbach MM, Van Wyck J J: Disorders of sex differentiation, in Williams RH (ed): Textbook of Endocrinology (ed 5). Philadelphia, Saunders, 1974, pp 423-501 2. Federman DD: Abnormal Sexual Development. Philadelphia, Saunders, 1967 3. Young HH: Genital Abnormalities, Hermaphroditism and Related Adrenal Diseases. Baltimore, Williams & Wilkins, 1937, pp 273-276 4. Jones HW Jr, Jones GES: The gynecological aspects of adrenal hyperplasia and allied disorders. Am J Obstet Gynecol 68:1330-1361, 1954 5. Lattimer JK: Relocation and recession of the enlarged clitoris with preservation of the glans: An alternative to amputation. J Urol 86:113-116, 1961 6. Sotiropoulous A, Morishima A, Homsy Y, et al: Long-
term assessment of genital reconstruction in female pseudohermaphrodites. J Urol 115:599 601, 1976 7. Money J, Hampson JG, Hampson JL: Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management. Bull Johns Hopkins Hosp 97:284-300, 1955 8. Randolph JG, Hung W: Reduction clitoroplasty in females with hypertrophied clitoris. J Pediatr Surg 5:224331, 1970 9. Hendren WH, Crawford JD: Adrenogenital syndrome: The anatomy of the anomaly and its repair. Some new concepts. J Pediatr Surg 4:49-58, 1969 10. Gross RE, Randolph J, Crigler JF Jr: Clitorectomy for sexual abnormalities: Indications and technique. Surgery 59:300 308, 1966
Seattle, Washington 9 Current surgical procedures that are utilized in the management of patients with an enlarged clitoris are both complex and challenging. W e report a n e w method of concealing the hypertrophied clitoris between the labia majora instead of resorting to the more difficult clitoral amputation or recession. This concealment of the clitoris is simple, m a y be done on an outpatient basis, and accomplishes the goal of creating normal-appearing female external genitalia. INDEX WORDS: Clitoroplasty; intersexuality; ambiguous genitalia.
HE E V A L U A T I O N , diagnosis, and subsequent medical and surgical treatment of patients with ambiguous external genitalia still continues to engender a certain amount of confusion among most physicians. Although this condition is relatively rare, the vast majority of these patients are seen in infancy and are usually diagnosed as having either congenital virilizing adrenal hyperplasia or mixed gonadal dysgenesis, the two most common causes of ambiguous genitalia in the newborn period. ~ Although patients with either of these two disorders may present morphologically anywhere along the spectrum of the normal phenotypic male with bilateral cryptorchidism to the normal phenotypic female with a slightly enlarged clitoris, almost all of these patients are subsequently reared as females 2 and ultimately present to the surgeon for some form of genital reconstruction. Since the goals of any surgical reconstruction of the external genitalia are the achievement of normal appearance and function, the present recommendation for these patients to be reared as females is to reconstruct the hypertrophied clitoris at an early age and postpone the creation of a vaginal canal until after puberty. Currently, there are a variety of surgical procedures that are utilized in the reconstruction of the patient with an enlarged clitoris. These methods range from total amputation to partial amputation to recession of the organ. Recently, while planning to perform a total clitoridectomy on a 4-mo-old patient with a diagnosis of congenital adrenal
T
Journal of Pediatric Surgery, Vol. 16, No. 5 (October), 1981
hyperplasia who presented with an enlarged clitoris (Fig. 1), it became apparent to us that instead of resorting to recession or resection of the clitoris, it might be possible to conceal the enlarged organ between the labia majora. PROCEDURE
The patient is placed in the dorsal lithotomy position and stay sutures of 2-0 silk are placed in each labium majora alongside the tip of the glans clitoris (Fig. 2). Following this, an inverted U incision is then made through the skin and subcutaneous tissue, lateral to, and above the glans clitoris. The incision is then widened subcutaneously in order to allow adequate closure of each side of the inverted U in three successive layers thereby concealing the hypertrophied clitoris. Each of the two layers of skin is closed with running absorbable subcuticular suture of 4-0 dexon and the subcutaneous fat is closed with interrupted absorbable 4-0 dexon suture. The postoperative appearance of the external genitalia is depicted in frame 6 of Fig. 2. A 3-mo follow-up of this patient reveals a satisfactory short-term cosmetic result (Fig.
3). DISCUSSION
Ideally, a n y procedure that is utilized in the surgical reconstruction of the patient with ambiguous genitalia should create a normalappearing and functioning external genitalia. Based on these two tenets, various methods of reconstructing the hypertrophied clitoris have been described. In 1937, Young 3 described the first method for correcting this anomaly. His procedure involved amputating only the shaft of the clitoris in an attempt to preserve the supposedly sensitive glans clitoris. However, most of these operations ended in failure when the glans clitoris sloughed because of an impaired vascular supply. Fourteen years later, Jones and Jones 4 described their method of total clitoridectomy and the formation of a pseudoclitoris from an From the Department of Urology, University o f Washington, School o f Medicine, Seattle, Wash. Address reprint requests to Dr. Julian S. Ansell, Department o f Urology, University o f Washington School o f Medicine, Seattle, Wash. 98105. 9 1981 by Grune & Stratton, Inc. 0022-3468/81/1605-0006501.00/0
681
Fig. 1, Genital morphology of patient with congenital virilizing adrenal hyperplasia at (A) 25 days of age {prior to cortisol therapy) and (B) 3 mo of age (following cortisol therapy). Note the marked reduction in phallic and scrotal size following cortisol treatment.
nli;ie n'unStS' ))j
,i
4. Underside of skin sutured by subc u t i c u l a r stitch.
5. Middle layer closed with inter' rupted sutures,
Fig. 2.
6, Subcaticular closure of surface layer of skin.
Surgical method of concealing the hypertrophied clitoris (Frames 1 to 6).
CLITORAL CONCEALMENT
' Fig. 3. (A, B) Appearance of external genitalia 3 mo following concealment clitoroplasty,
adjacent skin flap. In 1961, Lattimer 5 described his procedure of trimming the glans clitoris to a size appropriate for the age of the patient and relocating the shaft of the clitoris under the skin in such a way that the glans protruded through a
683
new position at the top of the vaginal vestibule. In a long-term follow-up, the cosmetic result from this operation was deemed very satisfactory and the authors stressed that preserving the tactile and erectile function of the glans in this operation did contribute to the sexual gratification of their patients. 6 However, the belief that the glans clitoris is necessary for normal sexual gratification in the female has been challenged by Money et al. 7 They documented that patients with congenital adrenal hyperplasia who underwent total clitoridectomy during infancy and were raised as females were able to achieve orgasm at adulthood. Nevertheless, several years following this report, Randolph and Hung 8 described their technique of reduction clitoroplasty where the shaft of the clitoris was buried under the pubic bone and the mons veneris was reconstructed to cover the enlarged organ. Randolph and Hung realized, however, that not all patients with a hypertrophied clitoris are suitable for their procedure and, in fact, one of their patients with an unsatisfactory cosmetic result subsequently underwent a total clitoridectomy. It is evident from reviewing the literature and from our experience that the size of the hypertrophied clitoris is the major factor in deciding whether to amputate or relocate it. If a large clitoris is recessed or relocated, it is possible for the patient to present with a turgid and painful mass during sexual excitation in later life. 9'1~ HenCe, total clitoridectomy would be the procedure of choice in this situation. In most of the patients who present with less than a penis-like organ, the surgeon must then decide which procedure appears suitable. In most cases with moderate clitoral hypertrophy it may be possible to conceal the hypertrophied clitoris instead of resorting to amputation or recession of the organ. Although we have performed this concealment procedure in just one patient, we were impressed by its simplicity, ease of performance, and cosmetic result. In addition to this, the operative procedure may be undertaken early in infancy as soon as the patient's metabolic and hormonal status have been stabilized and the sex of rearing has been determined. Furthermore, in an uncomplicated situation, it may even be possible to perform this procedure on an outpatient basis as was done in our case.
684
ANSELL AND RAJFER
REFERENCES
1. Grumbach MM, Van Wyck J J: Disorders of sex differentiation, in Williams RH (ed): Textbook of Endocrinology (ed 5). Philadelphia, Saunders, 1974, pp 423-501 2. Federman DD: Abnormal Sexual Development. Philadelphia, Saunders, 1967 3. Young HH: Genital Abnormalities, Hermaphroditism and Related Adrenal Diseases. Baltimore, Williams & Wilkins, 1937, pp 273-276 4. Jones HW Jr, Jones GES: The gynecological aspects of adrenal hyperplasia and allied disorders. Am J Obstet Gynecol 68:1330-1361, 1954 5. Lattimer JK: Relocation and recession of the enlarged clitoris with preservation of the glans: An alternative to amputation. J Urol 86:113-116, 1961 6. Sotiropoulous A, Morishima A, Homsy Y, et al: Long-
term assessment of genital reconstruction in female pseudohermaphrodites. J Urol 115:599 601, 1976 7. Money J, Hampson JG, Hampson JL: Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management. Bull Johns Hopkins Hosp 97:284-300, 1955 8. Randolph JG, Hung W: Reduction clitoroplasty in females with hypertrophied clitoris. J Pediatr Surg 5:224331, 1970 9. Hendren WH, Crawford JD: Adrenogenital syndrome: The anatomy of the anomaly and its repair. Some new concepts. J Pediatr Surg 4:49-58, 1969 10. Gross RE, Randolph J, Crigler JF Jr: Clitorectomy for sexual abnormalities: Indications and technique. Surgery 59:300 308, 1966