DEVELOPMENTAL ANOMALIES OF THE EXTERNAL GENI TAL ORGANS. .
The reason is apparent, for in the first mon strosity the entire body remains in a very primitive stage of development, and in the latter the lower extremities are not normally applied, nor is the lower segment of the body well developed. Naturally, therefore, both the external and the internal genitals are wanting.
On the other hand those observations are of special interest where in the presence of well-formed internal genitals there was no trace of the external. There does not really exist a single authentic instance of such an anomaly. Such cases have certainly been described, but in many it is apparent that the condition was not absence but union between the ex ternal genitals. We recall Magee's case, cited by Kiwisch, where the urethra was lacking and the urine was excreted by the nrachus at the um bilicus. The young girl was aged eighteen, and suffered intensely from dysmenorrhea, for the relief of which Magee made an opening at the site where the vulva appeared to be and gave exit to the retained blood. We consider that in this case there existed simply union of the parts, dating from the latter part of intrauterine life, and leading to patency of the urachus.
There are instances recorded whero only a funnel-shaped depression was present at the site of the labia, or else a small urinary meatus, or not even this. Where it is question of absolute absence of the external geni tals it has never been proved with certainty that the internal genitals were normally developed. Riolan has recorded two cases where the left labium majus was lacking, and yet there was no trace of cicatrix. Kussmaul records in his classic work on absence, etc., of the uterus, a case of Rossi's, where the vagina, however, existed as a minute opening. The other geni tals are not referred to. Certainly the most recent case, that of Foville's, does not belong in the category of absence of the external and develop ment of the internal genital organs. The description concerns an indi vidual at maturity, who menstruated through the urethra, and thence it was inferred that the internal genitals were completely developed. The
clitoris, labia, nymplue were absent, the vestibule was closed in front. Only a minute opening was present, which was recognized as the outlet of the uro-genital canal, from the fact that both urine and menstrual blood issued from it. Between symphysis and anus there existed a species of raphe in the mid-line, and the above-mentioned minute opening. In the explanation of this case, Forster states that it is important as proving that we are dealing with fusion of the labia majors, while glob claims that there was fusion at the raph(.. The latter view is the most plausible, since a raphe is the result of the union of two previously separate parts, as, for example, the raphe of the scrotum. The same explanation will apply to Ashwell's case. Meckel has cited old cases of absence of all the genital organs. At the site of the external genitals, there existed either a depression, or else an elevation, or else the skin covered the site unal tered. We refer to these instances since we wish to point out the contrast between them and Foville's case. If his case is not a genuine one of the kind we are speaking of, there is recorded none other where the urethra and internal genitals were present, and the external genitals absolutely absent. This is so much the more important, seeing that the genital tubercle is never absent (except in sirens and sympodiEe), and that the malformations of the external genitals are to be explained by the greater or less development of the genital furrow, or else by fusion When the external genitals are entirely absent in the foatus, then natu rally the canals, which normally arise from the genital furrow, cannot open at the surface, but must remain as in the embryonal stage, inter communicating. Such a great malformation must be incompatible with life. As a rule, it is found only in premature foetuses, which are either still-born or succumb shortly after birth. Certain questionable cases have, however, been recorded where individuals without anus or meatus urinarius have survived. I believe them to be so unauthentic that I make no further reference to them.