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Postmedj00529-0038.Pdf .........M...... I . ' . - HERMAPHRODITISM A Critical Survey By HUGH JOLLY, M.D., M.R.C.P., D.C.H. Consultant Paediatrician, Plymouth Clinical Area Wo-ff ,.,/i/, decttPcradft$ Wrolff LO,^ uct Lsb(oastrhhpv ~ -.I ,~o5: U-e^latiit Iws l FIG. I.-The undifferentiated stage of urogenital development. The problem of hermaphroditism is one which the complicated method by which the male and has always attracted attention so that much has female genital tracts and external genitalia are been written on the subject. One of the finest derived, and particularly to the fact that this works appeared in 1937, with the publication of development is common to both in early embryonic Young's monograph1 and this has become a classic. life (Fig. I). In recent years, Lawson Wilkins and his co- workers at Johns Hopkins have, more than anyone The Gonads else, furthered our knowledge of the subject and Up to the sixth week, therefore, the gonads are much of the information in the present article is identical in both sexes and the embryo potentially derived from their writings2-15 and from personal bisexual. Changes in the gonad take place communication with Dr. Wilkins. Liberal use earliest if the embryo is to become male when, at also is made of the present author's publica- the seventh week, the elements of the testes appear, tions16-18 on the subject as well as more recent being derived from the medulla of the gland. personal experience. If the foetus is to become female no distinctive features appear until the tenth week when the Embryology germinal epithelium, consisting of epithelial The occurrence of hermaprhoditism is due to nests and primordial follicles containing ova, is 590 POSTGRADUATE MEDICAL JOURNAL December 1956 '.... ....... .... E ·:iSpL E,& · ::: ~': FgE~'.· '~ ~"':'" ~,~ggpp,~i?-~'-..?'ii,?'·'· ii.~.'.· .~ C' ~ PA kP~:- :.F:~.:::'.'"'....~. ::? ..:.ii !~ ..:..... .::..:i::-:;~'-.iij.. *ii 1.·::::..,::: :: i i.~ Charli :B 1111~l i iN~ eriF.¢ei-· ·;;·2||W ..... "I. KIDNEY '?''!.,,.'.:.'jS:.' i:...p RC!: ILI '~:~'::- s ?,?!::AL LO0Pf~A N T t ~~....i Ard:: :::::::::::::::::: :::.. .... -'..::::::::::::::::::::::::::: ...~' PLATE I.-P.M. specimen showing congenital adrenal hyperplasia. The hyperplastic adrenal glands are seen to be larger than the kidneys. The infantile uterus tubes and ovaries are well shown. derived from the cortex of the gland. It is part ovary and part testis or that one gonad apparent therefore, that the testis develops from becomes an ovary and the other a testis. the medulla and the ovary from the cortex of the gonad, but even this adult differentiation is not The Genital Ducts complete since the normal testis contains' female ' In the undifferentiated stage (Fig. I) two pairs (cortical) components and the ovary contains of genital ducts lead from the gonad to the ' male' (medullary) components. In this light it urogenital sinus-the male or Wolffian ducts and is understandable that on very rare occasions the the female or Mullerian ducts. These ducts foetal gonad may develop into an ovo-testis, being are not ambisexual and remnants of the pair December 1956 JOLLY: Hermaphroditism 591 belonging to the opposite sex are to be found in age. The problems of homosexuality and trans- adult life. The Wolffian ducts become the vestism will not be discussed in this paper since epididymis, vas deferens and seminal vesicles in they are psychological problems only. the male, whereas, in the female, their remnants exist as the epo6pheron, paro6pheron and Gartner's (I) Female Pseudohermaphroditism with Adrenal ducts. The Mullerian ducts become the Fallopian Hyperplasia tubes, uterus and vagina, the latter two structures This is the commonest of all types of her- being formed by fusion of the two ducts. Their maphrodites and Money quoted by Wilkins et al. remnants in the male are the appendix testis, (I955)11 in a recent survey of published cases utricle and verumontanum. betweren I895 and I95o has shown that the condition occurs as frequently as all the types of The Urogenital Sinus intersex. In the undifferentiated stage the bladder and The hyperplasia of the adrenal cortex (Plate I) both sets of genital ducts open into the urogenital commences in early foetal life and produces a sinus. In the male the urogenital sinus becomes massive masculinizing stimulus from the out- the lower part of the prostatic urethra and penile pouring of androgens. This action begins in the urethra. In the female this sinus becomes the fourth month by which time the gonads have shallow vestibule which contains the orifices of already differentiated to form ovaries but the the urethra and vagina. This is separated from Mullerian ducts have only developed as far as the the vagina by the hymen whose inner surface is formation of Fallopian tubes and uterus. The lined by vaginal epithelium derived from the further development of the genital ducts now takes Mullerian duct and outer surface from urogenital place along male lines as a result of the androgenic sinus epithelium. stimulus, so that the urogenital sinus persists as External Genitalia in Fig. 2, type A, instead of giving way to a separate The genital tubercle enlarges in both sexes to urethra and vagina. This figure shows the form the phallus and as it does so a median groove commonest anatomical arrangement in these appears on its caudal surface. The raised margins patients, there being one external orifice only so of this groove are the urethral folds. In the male that the vagina appears to open into the floor of the the phallus lengthens to become the penis and as it urethra. The phallus steadily enlarges instead of does so the urethral folds fuse round the urogenital remaining small as a normal clitoris and the labio- sinus from behind forwards in such a way as to scrotal folds fuse to some extent and thereby give carry the penile urethra, which has formed from the impression of a scrotum rather than labia the urogenital sinus, to the tip of the penis. The majora. The ovaries however do not descend so two labioscrotal folds which have appeared on that the ' scrotum' is always empty. each side of the base of the phallus become fused Other varieties of this condition may occur, in the male to form the scrotum. the next most common being shown as type B in In the female the becomes the Fig. 2. Here the urogenital sinus opens externally phallus clitoris, through a funnel-like orifice which is larger than while the genital and labioscrotal folds do not fuse the previous type so that the urethra appears to but become the labia minora and majora open into the roof of the vagina. Three other respectively. much rarer types have occurred. In type C, there Classification is no communication between vagina and urethra; Two distinct groups of hermaphrodites should in type D the urethra has been carried forward first be separated on the basis of aetiology, the one by fusion of the urethral folds so that its orifice being due to an adrenal disorder and the other not. is at the tip of the clitoris. In type E the vagina Thus we have: and urethra have differentiated to become separate (I) Female pseudohermaphroditism due to as in the normal female, and one can only presume congenital adrenal hyperplasia. that in this case the androgenic stimulus did not (II) Intersex. commence until after the fifth month of foetal life. In this latter group there is no endocrine dys- At birth then, the child, who is of average function and the aetiology is unknown, although rather than large size, will be normal apart from a genetic factors play a part. It includes the remain- large clitoris, and in most cases will have a single ing varieties of pseudohermaphroditism and all external orifice serving both urethra and vagina. true hermaphrodites. A varying degree of fusion of the labioscrotal In female pseudohermaphroditism with adrenal folds will be present and the uterus can usually hyperplasia there is progressive virilization from be felt on rectal examination. After birth the birth, whereas in intersexes no further changes androgenic stimulus continues so that the child take place until puberty which occurs at the usual grows more rapidly than normal and the epiphysial 592 POSTGRADUATE MEDICAL JOURNAL December 1956 A -S C' £ FIG. 2.-Varieties of female pseudohermaphroditism with adrenal hyperplasia. :· ....:...:.. .... .;.... ;.... .. .......... i~ili~iiiiii.. .. ... .... ··:::i .... ....·: '' ·I .... .................... ii..:i :::: ............... ::,.i;:.... ...:I: ....... .. .... ..k PLATE 2.--Female pseudohermaphrodite with adrenal hyperplasia. These photographs were taken when the child was first seen at the age of five years and before cortisone therapy was commenced. The thickened and enlarged phallus can be appreciated, together with the growth of pubic hair and the single external orifice. December I956 JOLLY: Hermaphroditism 593 bone age is advanced. The phallus continues to of the diagnosis is required. The urethrQscope is increase in size, being much thicker than a normal passed into the capacious urogenital sinus until clitoris, but not so long as a penis. It is bound the vaginal opening is visible in the floor of the downwards in a curve-a condition known as sinus as a slit-like aperture surrounded by folds. chordee. Grossl9 states that the enlarged clitoris This opening is usually large enough toallow the can be differentiated from the penis by the two passage of the urethroscope into the vagina until small folds which run down on either side of its the cervix can be seen as a smaller slit and into midline to join the labia minora. In the penis which an ureteric catheter can usually be inserted. there is a single frenulum on the ventral surface. If necessary, a radio-opaque dye can be injected Erections of the enlarged phallus become increas- and watched under the X-ray screen as it passes ingly frequent and may be painful.
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