Sex Reassignment: A Challenging Problem—Current Medical and Islamic Guidelines

Abstract

Sexual ambiguity is a complex and often confusing medical problem. In addition to the life-threatening adrenal crises which may accompany some forms, ambiguity of the genitalia may lead to incorrect sex assignment by parents and/or health personnel. Children who present to medical attention beyond the neonatal period constitute a challenging problem due to the grave consequences of this condition. Thirty cases in whom sex reassignment was indicated were seen at King Khalid University Hospital, Riyadh, over a 10-year period. Of these, 27 (90%) were genetic females (20 were 21-and seven were 11-hydroxylase deficient) and three (10%) were genetic males (two partial androgen insensitive and one 5α-reductase deficient). All genetic males who were incorrectly assigned as females accepted reassignment. But 9 out of 27 (33%) of the genetic females who were incorrectly assigned as males refused reassignment. Preference for male sex assignment, delayed diagnosis and sociocultural circumstances seem to be the contributing factors for refusal. A national consensus about this important issue strengthened by the existing religious recommendations is demanded. Ambiguous genitalia are a social and medical emergency.1,2 Sex assignment of the newborn is usually based on the external genitalia. If this is abnormal, it may be difficult to correctly assign the sex. An incorrect decision can lead to a major dilemma for the child, parents and other members of the family, friends and health personnel, who find themselves confused and in a difficult and unimaginable situation.3 In a community where this condition is. not uncommon, 1,4-7 health personnel will continue to face improperly sex-assigned children and young adults at different ages and circumstances. In this study, we retrospectively reviewed 30 cases in which sex reassignment was indicated. We also discuss the present Islamic guidelines regarding this matter and call for an urgent and unified national consensus, based on the basic scientific knowledge available and supported and approved by the existing Islamic recommendations. Material and MethodsThe medical records of 30 cases in whom sex reassignment was indicated, seen at King Khalid University Hospital (KKUH), Riyadh, over a 10-year period starting in September 1984, were analyzed. Genetic sex was based on chromosomal studies done on lymphocytes. Additional tests included genitogram and pelvic ultrasonography. Definitive etiological diagnosis was based on elevated 17 α-hydroxy, progesterone in 21-hydroxylase (P450C21) deficiency, elevated 11-deoxycortisone (compound S) in 11β-hydroxylase (P450C11) deficiency, elevated baseline and human chorionic gonadotropin (HCG) stimulated testosterone (T) to dihydrotestosterone (DHT) ratio in 5α-reductase deficiency and satisfactory penile length response to HCG and T in the presence of normal T/DHT ratio in partial androgen insensitivity (Figure 1). In view of the medical recommendation and the available Islamic guidelines, a final decision for the final diagnosis, outcome and recom...

Cytogenetics and etiology of ambiguous genitalia in 120 pediatric patients

“…The topic has been debated extensively and specific guidelines are proposed to manage cases of traumatized or ambiguous genitalia. 27 , 28 These guidelines take into consideration how the patient will develop post-puberty and adapt as a sexually active person. Accordingly, rearing as males is recommended in 1) 46,XY patients with partial AIS, 2) hypospadias, 3) micropenis with testes, because these children should develop a satisfactory male gender identity and sexual function; 29 and 4) 5 α-reductase deficiency.…”

Prevention of Ambiguous Genitalia in Congenital Adrenal Hyperplasia

“…Side effects of dexamethasone to the mothers are almost unavoidable. 26 This was the case in the patient who presented. She manifested excessive weight gain, increased hair growth, hypertension and soft tissue edema.…”

“…[1][2][3] This will minimize the confusion which usually accompanies these cases when only faced at birth. 25,26 It will also ease the amount of surgical correction indicated in these cases. 29 Furthermore, the long-term function of the genitalia may be better.…”

“…On the contrary, ignorance or mismanagement can lead to devastating results, in particular, incorrect sex assignment. 25,26 It is therefore extremely important to find a preventive method to this problem, even if the cause of the disorder has no permanent cure. Upon exposure to androgens, virilization of the genitalia occurs very early during the intrauterine life, as the genitalia, the labioscrotal fold fusion in particular, is sensitive and responsive to androgen stimulation.…”

Global Application of Assessment of Competencies of Paediatric Endocrinology Fellows in the Management of Differences of Sex Development (DSD) Using the ESPE e-learning.org Portal

“…A religious official may play a crucial role in management in terms of decision making and with respect to openness on informing family and friends [16,17]. Options regarding genital surgery involving gonadectomy, hysterectomy or fertility preservation procedures may be subject to religious recommendations [18]. In some countries, management of DSD is seriously hampered by lack of access to essential medications such as hydrocortisone and to specialized (paediatric) urosurgical expertise [15,19].…”