ONTOGENESIS OF SEXUAL BEHAVIOR 



1407 



differentiation (Klebs, 1876). In his classi- 

 fication Klebs recognized true hermaphro- 

 dites, who possessed both ovarian and tes- 

 ticular tissue, male pseudohermaphrodites 

 with only testicular tissue and female pseu- 

 dohermaphrodites with only ovarian tissue. 

 In studying the determinants of the psycho- 

 logic |)henomena of sexual outlook and ori- 

 entation — gender role — Kleb's classification 

 has proven too anachronistic to be useful, 

 for modern discoveries in endocrinology, in- 

 cluding recent work in sex chromatin de- 

 termination (Moore and Barr, 1953; Moore, 

 (iraham, and Barr, 1953; Grumbach and 

 Barr, 1958) make it clear that multiple 

 variables are involved. The following list 

 of the varieties of ambisexual incongruities 

 was drawn up with these considerations in 

 mind and is based in part on Dr. .John 

 ^loney's unpublished doctoral thesis in 

 which he reviewed the medical literature of 

 over 300 cases of hermaphroditism. 



It would be inappropriate here to ex- 

 pound at length on the differential diagnosis 

 of the various clinical types of ambisexual 

 incongruities but it may be relevant to com- 

 ment briefly on the six varieties listed in 

 Table 23.1.^ 



Variety 1. Congenital hyperadrenocortical fe- 

 males. In this group of patients the external geni- 

 tals may appear almost normally female with 

 slight to medium enlargement of the clitoris; or 

 there may be a single urogenital orifice with en- 

 largement of the clitoris. Rarely, a penile urethra 

 and a fused empty scrotal sac are found. Without 

 l)enefit of suppressive cortisone therapy physical 

 growth and development is precocious and viriliz- 

 ing. Diagnosis is now possible during llic neonatal 

 [leriod by means of urinary IT-koto-lrionl assess- 

 ment, without laparotomy. Plural inciileiiee m a 

 family is common, but is usually restricted to a 

 single generation and a single marriage (Childs, 

 Grumbach and Van Wyk, 1956). The Miillerian 

 ?y.stem and ovaries are normal; the sex chromatin 

 l^attern is female. 



Variety 2. Hermaphrodites with ambiguous or 

 masculinized external genitals. Unlike females with 

 liyperadrenocorticism, the hermaphrodites in this 

 siroup, although born with varying degrees of am- 

 l)iguous or masculinized genitals, do not show pro- 

 gressive virilization. On the contrary, since the 

 ovaries and accessory internal reproductive struc- 

 tures are normal, secondary feminization at pu- 

 l)erty is the rule and reproduction is iio.-sil)le. The 

 enlarged phallus usually re.scnililes a liypospadic 

 penis with chordee. Labial fusion may be pro- 

 nounced or even complete. The vagina usually 

 opens into a urogenital sinus but occasionally opens 

 independently. The sex chromatin pattern is fe- 



male. Wilkins, Jones, Holman and Stempfel (1958) 

 have published a rei)ort on 21 such hermaphro- 

 dites; in all but 3 the mother had received pro- 

 gestinic medication (usually 17-ethinyltestosterone) 

 beginning in the first trimester of pregnancy. 



Variety 3. Classical true hermaphroditism. Ovar- 

 ian and testicular tissue is present, in one ovo- 

 testis, the other gonad being an ovary or a testis, 

 in two ovotestes, or in one ovary and one testis. 

 The internal genital structures as well as the ex- 

 ternal genitalia show various degrees of ambiguity. 

 Pubertal development of the secondary sexual 

 characteristics may be masculine, feminine, or 

 ambiguous. Diagnosis requires laparotomy and bi- 

 opsy of the gonads. In an unpublished study of 20 

 patients with classical true hermaphroditism, M. L. 

 Barr found that 15 had a female sex chromatin 

 pattern. 



Variety 4- Cryptorchid hermaphrodites with rel- 

 atively complete Miillerian differentiation. The 

 penis may be fully formed with a penile urethra 

 or it may be hypospadic. One or both testes may 

 by cryptorchid; one testis may be atrophic. Miil- 

 lerian structures not infrequently herniate into the 

 groin or scrotum in the company of one testis. At 

 puberty the secondary sexual development is 

 nearly always masculine although a eunuchoid hab- 

 itus is sometimes observed, especially in those in- 

 stances of bilateral cryptorchidism and hypospa- 

 dias. Diagnosis requires surgical exploration; the 

 sex chromatin pattern is probably always male al- 

 though it is conceivable that a female pattern may 

 be possible. 



Variety 5. Cryptorchid hermaphrodites with rel- 

 atively incomplete Miillerian differentiation. His- 

 torically, hypospadiacs have not been called her- 

 maphrodites imless the hypospadias is severe, the 

 scrotum bifid, and the testes undescended. Careful 

 examination, however, often reveals that such hy- 

 pospadiacs have a blind vaginal pouch hidden be- 

 yond the single external urogenital orifice ; or there 

 may be a separate external orifice for a blind vagi- 

 nal pouch. In some instances the phallus may be 

 only sHghtly larger than a clitoris. The Miillerian 

 and Wolffian systems are malformed or vestigial. 

 A virilizing puberty cannot be predicted; in some 

 patients pubertal development may be weakly 

 feminine resulting in breast enlargement. Plural 

 incidence within a family may occur. Diagnosis re- 

 quires testicular biopsy ; the sex chromatin pattern 

 is probably always male although future findings 

 may require a revision of this assumption. 



Variety 6. Simulant females with feminizing in- 

 guinal testes and vestigial Miillerian differentia- 

 tion. In this group of patients the external genital 

 appearance completely simulates the normal fe- 

 male. The vagina is a blind pouch and, with few 

 exceptions, the Miillerian system is a cord-like 

 vestige; the Wolffian system is malformed or 

 vestigial. The testes may remain intra-abdominal 

 or herniate into the groin; even in adults the mi- 

 croscopic appearance of the testes is best described 

 as immature and poorly differentiated. At puberty 

 the development of the female secondary sexual 

 characteristics is nearly always complete except 

 that menstruation does not occur. In about one- 



