Paper - Hermaphroditism in man (1920)
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Hermaphroditism in Man
Hubert Sheppard Deparlmcnl of Anatomy, The University of Kansas
An opportunity to make a study of the anatomical structures of the genital organs of hermaphroditism in man is seldom found for two reasons: first, such irregularities seldom occur and, second, when they do occur the material is exceedingly difficult to obtain for laboratory purposes. As recently as 1911 it was asserted that "hermaphroditism in the sense that separate testicles and ovaries are found has not Ix'cn demonstrated in man, nor even in other mammals beyond a <luubt."" We thought it worth while, in the light of this and other in^•estigations, to report a study of the anatomical structures of an extreme case of hermaphroditism Avhich came to the dissecting room. The gross study is supplemented by microscopical examinations in so far as the condition of the material would permit.
The cadaver featured, objectively, both as a male and as a female subject. Hair was lacking on the face and scant around the genitals, the body was large and obese, with the mammae well developed, large and flabby, which in every way resembled a female rather than a male organ. One would have judged, in so far as the external genitals were concerned, that they were male rather than female genitalia. However, upon a closer examination, one could see that there were certain irregularities. The penis was small with a dilated urethral orifice three-fourths as large as the organ itself (fig. 1). The scrotum appeared to be abnormally large, although the testicles, upon palpation, were found to be normal and symmetrically formed. When the region posterior and beneath the scrotum was palpated, its apparent unusual size was found to be due to a large fold or ridge which extendeil from near the anus to the pubis on either side of the penis.
The Famale External Genitalia
After (lie skin and scrotum wore completely reflected from the urogenital triangle, the large ridge beneath the scrotum was found to l)e a structure which res(Mnl)Icd female external genitalia. Hiiili till- lai)ia niajoTa and minora were nearly nornial in size, the former extending to the posteiior connnissure, while the latter formed the fremdum. The skin and dartos of the scrotum di\ided into two lamina on reaching the postero-inferior part of the labia majora. The superficial layer continued as the skin and fascia of the f(>moral region, the imier lannna thickened into the labia majora. The nunora was two mendjranous folds within th(> majora and surrounding the penis or enlarged glans clitoris. M this stage of the dissection, if one would disregard the latter enlargetl organ, the cadaver was nearer a female than a male subject.
The Penis and Vagina
The penis in all subjects resembled a glans clitoris which had develoi)ed into an organ that closely figured externally as male genitalia with a cone-shaped dilated urethra (fig. 1). At the large or vaginal end of the urethra was an ojiening that extended back into the uterus through the prostatic-cervix of the uterus (fig. 2), which will be descril)ed later. The penis was small, mea.suring a little more (li;in 1' inch in length and \ inch in diameter. The glans had a pr('|)uce fuseil with the erectile tissue of the corpus cavernosum. and was only a rudhnentaiy fold at the end of the organ. The dorsal vein, arteries, and nerves were regular and similarly related as those found in a normal subject. Kxternally, the urethral orifice of the corpus cavernosum urethra (fig. 1), measured ^ inch in diameter. This opening gradually increased in diameter until it was a little more than an inch in diameter at the urogenital dia|)hragm. In so tar as we could note, there was no spongiosum tissue present in the mrtlnal i)art of the organ. Both the urethra and the vagina opened into this enlarged urethra. The true urethra itself was only about f inch in length. This duct passed through the upper part of the prostatic-cervix portion of the uterus, while the vagina was located in the lower two-thirds and extended up\\ard and backward into the uterus proper. Posteriorly, the corpus cavernosum penis divided into two short crura (fig. l) f inch in length.
The body of the uterus was separated from the bladder by the vesico-uterine fold of peritoneum in the usual manner. However, the uterus as a whole was somewhat lower down in the pelvic cavity than is ordinarily found in normal cases. This was due possibly to the development of the organs — the fusing of one genital system with the other. The greatest width of the uterus was 1.5 cm., with a total length of 5.5 cm.; the body was 4 cm., and the prostatico-cervix 1.5 cm. A uterine body cavity was perfectly developed, and measured nearly 5 cm. This cavity extended into the uterus with little demarcation between the two organs. The entire cervix was fused with the prostate; in fact, the prostate was a mere enlargement of the cervix of the inferior extremity of the uterus. The uterus held the same relation to the prostate that utriculus prostaticus holds in a normal male subject. Not only the lumen, but the uterine glands and muscular walls could be easily defined (fig. 3). The ductus deferens entered the prostatico-uterine canal of the cervLx by piercing its posterior wall (fig 2). A broad hgament was well developed, resembling in every detail a normal female subject except the course of the ductus deferens, which will be described later, and it was a little thicker and wider, due to the fact the uterus was a little lower in the pelvis as has been previously described.
The Ductus Deferens
The ductus (vas) deferens differed in no respect from a normal male subject until it passed through the annulus inguinalis abdominis in connection with a very rudimentary ligamentum teres uteri (fig. 2). It then coursed alongside, and posterolateral to the oviduct, at tii>;l encircliiif; iIk- ovaries. When it readied the level of the uterus, it made a quick S-.shaped turn, and entered the .superior part of the cervix of the prostatico-uterus. The hi.stolopical structures of the duct are veiy well developed. The epithelium surrounding the lumen is slightly disintegrated, a.s is shown by the photograph The circular and longitudinal muscle layers are clearly defined, and in a number of sections the tunica projiria and the inner longitudinal layer are also easily recognized (fig. 4).
The Ovaries and Oviduct
The ovaries measured about 1 inch in length and ' (> inch in breadth. They were attached to the mesovarium in the usual way. However, the ovaries were found to be in a poor state of preservation for histological purposes; nevertheless, some of the materials stained sufficently well to demonstrate the ovarian tissue. One of the larger follicles as well as a number of smaller are shown in figure 5. A little below the follicles is a light area where the corpus lutoum has disintegrated from the rest of the tissue.
Each oviduct took a nornuil course to the proximal opening into the uterus (fig. 2). The course of each duct was at first almost horizontal, lateral, and posterior from its attachment to the uterus until it reached an inferior lateral portion of the pelvic wall where it came into relation with the uterine extremity of the ovaries. Then it coursed at right angles, and passed almost vertically upward along the mesovarial border of the ovary to the mouth of the infundibulum and the fimbriated extremity of the duct. Microscopically, the sections of the oviduct very clearly difTerentiated the various tunics; the serosa, the longitudinal, and the circular miiscle layers show with marked clearness (fig. ()). The ei)itliclium as well as the inner i)art of the mucosa has somewhat disintegrated from the lumen. It was jKissible in many of the sections to define the epithelial cell structures. .V lumen extended throughout the full extent of the duct.
The testes did not differ from a normal testicle, except in size. The right testis measured IJ inch in length, f inch in breadth and 1 inch in diameter anteriorposteriorly. The left testis was nearly the same size as the right except for length. It measured a little more than l-^- inch. Each testis lay upon and slightly laterally to the large ridge or fold beneath the scrotum. This arrangement gave the scrotum its extremely large appearance when viewed in its normal state. The funiculus spermaticus had all of its usual structures, even the pampiniform plexus was easily worked out. A small round ligament, before mentioned, was fused with the fimiculus as far as the point where the labia majora began. At this point it was lost and could not be traced any farther along the cord.
This tissue, like the ovaries, was in a poor state of preservation for histological study. However, in many of the sections the convoluted tubules could be easily differentiated (fig. 7). Only the shape of the tubule with its contents could be clearly defined. It was impossible to differentiate between sexual and sustentacular cells except in a few sections. In these better sections, a few interstitial cells could be observed under oil immersion.
According to Virchow, this individual subject would be an individuum uterusque generis, since both male and female organs are found almost equally developed. Klebs regards a hermaphroditismus verus as a subject who possesses both male and female genital organs united in it. In the spechnen under consideration, we find two set;; of reproductive glands. They were not united in the sense of ovitestes, but since both the ductus (vas) deferens and the oviduct (Fallopian tube) enter the uterus and, further, the round ligament and the spermatic funiculus have a union as well as a natural position and course, we can say that there was an indirect union. Even according to Kleb's definition this would be an hennaphroditisnms verus.
nuilornatsch says that "herinaphnMlitisni in the sense that separate* testicles and ovaries are formed has not been demon8trate<l Ix-yond doubt." Except for minor variations which we have previously describe<l, we find not only separate testicles and ovaries which are in their noniial position in the body, but also a complete male and female urogenital system with the exception of the urethro-vagina and the prostatico-cervix of the uterus. Here we have noted the fusion of the two systems into a single system where male and female are combined.
Such a finding as this substantiates the old theory of Waldeyer that there is a bi.sexual anlage of the genital ridge. We cannot quit« .SCO how Benda's theory, "that the primary anlage of the entire sexual system of the vertebrates must be regarded as female," would hannonize with facts now recorded. A separate devclo|)ment would seem to be further substantiated by the fact that in every case of hermaphroditism on record there is always a .sharp tlistinction between the two kinds of tissue, and never an undifTerentiated mixture of botli elements, as would be the ca.se if the germinal epithelium could produce either male or female reproductive tissue.
In every male subject the prostatic utriculus, a homologue of the vagina of the female, can be demonstrated. It would appear from what we know of the embry^ological development of the urogenital system that there would be a fusion of the prostate, vagina, and uterus in an hennaphroditismus verus. This would no doubt explain the variation or fusions of the two systems found in the cadaver we are considering.
Benda, C. 1895 Hermaphroditismus unci Missbildungcn mit Verwischung
(los Gesphlechtscharakters. Ergebn. d. allg. Path., Bd. 2, S. 627. C'oHUY, H. 1905 Removal of a tumor from a hcrmaplirodite. Brit. Med. J.,
vol. 2. GvDERN.^T.scH, J. T. 1911 Hermaphroditismus verus in man. .\ni. Jour.
Anat., vol. 11, pp. 267-78. H.\LB.\N, J. 1903 Die Entstehungder Geschlechtscharaktere. Arch. f. Gvnaek.,
Bd. 70, S. 205. IIiRSCHFELD, M. 1905 Ein seltener Fall von Hermaphroditismus. Monats.schr.
f. Harnkr. und sex. Hyg., Bd. 2, S. 202. Janosik, J. 18S7 Bemerkungen liber die Entwicklung des Genitalsj'stems.
Sitzungsber. Akad. Wiss. Wien, Bd. 99, 3. Abt., S. 260. Ldksh, F. 1900 i'ber einen neuen Fall vom weit entwickeltem Hermaphroditismus spurius masculinus internus. Ztschr. f. Hellk., .\bt. f. Path.,
Bd. 21,8.215. Meixner, K. 1905 Zur Frage des Hermaphroditismus verus. Ztschr. f.
Heilk., Abt. f. prakt. Anat., Bd. 26, S. 318. NBUGEBAtJER, E. 1908 Hermaphroditismus beim Menschcn. Leipzig. PiiiLiPPS, J. 1887 Four cases of spurious hermaphroditism in one family.
Trans. Obst. Soc. London, vol. 28, p. 158. Reizenstein, A. 1905 Uber pseudohermaphroditismus masculinus. Mlinchn.
med. Woch., Bd. 52, S. 1517. Salix, E. 1899 Ein Fall von Hermaphroditismus verus miilatcralis beim
Menschen. Verb. Deutsch. Path. Ges., Bd. 2, S. 241. ScHECKELE, G. 1906 Adenoma tubulare ovarie (testiculare). Hegar's Beitr.
z. Geburtsh. u. Gynack., Bd. 11, S. 263. Si.MON', W. Hermaphroditismus verus. Virchow's Arch. f. path. .\n., Bd. 172,
S. 1. TouRNEiTX, F. 1904 Hermaphroditisme de la glande genitale chex la taupe
femelle adulte et localisation des cellules interstitielles dans le segment
spermatique. (,'omp. rend, de I'assoc. des. anat., Toulouse, p. 49. Unger, E. 1905 Beitriige zur Lehre vom Hermaphroditismus. BerL klin.
Woch.,Bd. 42, S. 499. Waldeyer, W. 1870 Eierstock und Ei. Leipzig.
All the figures !»ro untoiiohiMl pliotoffaplis cif the organs desoribed in this paper. Figures 1 and 2 are niacroseopie photographs of the external and internal genital systems. The remainder of the figures are microscopic iihotographs.
EXI'LAX.^TION OF KHiUnKS
1 A photograph to show the external genital organs. The short penis has Ix'eii dissected out with its crura and laid upon the jiuhis. The dilated urethra has l)ecn split and pinned open to show the small opening into the vagina as it turns liackward to enlor the uterus.
2 A photograph of the same section from a pelvic view. The vagina and uterus have heen laid open and |iiniied backward to the l>road ligament. The ductus deferens can lie seen on the right side near the upper extremity as it enters the cervix of the uterus. Near the lower extremity of the uterus can lie seen both oviducts coming off from the angle of the uterus. The bladder could not be shown in the photograph.
3 A microscopic sectitm to show the lumen and nmscular wall of a portion of the uterus. This was taken from the right side 2 cm. from the cornu.
F.XI'LANATION OF FIGURES
4 A section of the ductus deferens to show the tunics. Considerable disintegnition 1ms taken place in the lumen. However, all the layers of the duet show clearly in the photograph.
5 A section of the ovary. A large follicle is seen near the top of the photograph. T«o smaller follicles to the right can also be seen. Helow and near the bottom of the photograph is a light area. This was an area of disintegrated corpus luteuni.
6 A section of the oviduct taken about half-way between the ovary and the uterus.
7 A low-power section of the testicle, to show the convoluted tubules and the connective tissue among the tubules.
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