THE CONGENITAL MALFORMATIONS OF THE FEMALE URETHRA.
At the tenth weei occurs the separation of the intestine from the uro-genital system, by the formation of the recto-vaginal septum (perineum). The urethra, at first absent, is formed by the development of the parts constituting the neck of the bladder so as to form a canal, the septum urethro-vaginali being made in the same way. (Fig. 11.) E. Rose believes that the urethra is formed of three separate parts. The first is the neck of the bladder; the second an infolding of the external skin, forming the glandular part; and the third a projection from the posterior extremity of the alimentary canal, a vaginal piece, which projects to meet the anterior portion just as the allantois grows towards the excretory ducts of the primordial kidneys.
The congenital anomalies of the female urethra may, therefore, occur in various ways. Both portions, vaginal and glandular, may be absent, defectus urethras totalis; or the inner portion only may be absent, de fectus urethrae internus; or the invagination of the skin may not occur, defectus urethrce externus; or the vaginal and glandular pieces may miss each other, atresia urethrce. In the latter case there is a septum urethrte, which is oblique. If both portions simply develop insufficiently, the sep tum urethrte will be vertical.
Total absence of the female urethra, defectus urethra totalis, may occur in otherwise normal organs, or it may exist together with other congeni tal defects. The vestibule shows no meatus, while in the vagina near the symphysis, is a transverse slit, the opening of the bladder. Petit has re corded such a case in a girl four years old, in which clitoris, nymplun. and urethra were wanting; the vagina was large, and incontinence of urine existed. M. Langenbeck has reported a case of imperforatio hytnenis, in which in a girl nineteen years old, vagina and hltulder formed a com mon canal, and incontinence at first existed. Smith (See Hepner, 1. c.) found in a girl seven weeks old, a clitoris 1 inch long, under which was a triangular opening, which led into the vagina; and the vagina, 2 lines in diameter, had an opening in its anterior wall, which led into the bladder. Behncke's case belongs to this latter class.
Though Heppner classes his case, in which the vestibule showed traces of the anterior and lateral walls, as an example of true female hypospadia, it is to be doubted if Ile does so correctly, since the patient in her third year was operated upon for stone. The incontinence of urine from
which the patient suffered was not influenced by the operation. Hepp ner's drawing of the malformation gives one the idea that it might be due to a traumatic destruction of the urethra, rather than a congenital defect.
Congenital atresia urethrre occurs when the middle or vaginal portion of the urethra is wanting, or when the vaginal and glandular pieces miss each other, or are imperfectly developed. Duparcque found the first variety of atresia, defectus urethrte internus; the urethra was open to the bladder, the bladder was closed, and both that organ and the ureter were markedly dilated. A case of E. RON'S illustrates the second variety, in which the glandular and vaginal portion had missed each other, the urethra was crooked, was solid for a certain extent, and thus caused hydrops vesicle urinarire, hydro-necrosis and ascites. Besides this there was uterus hicornis septus, vagina septa. To this category belongs also Schatz's case: double uterus, double vagina, double bladder, double congenital vesico vaginal fistula, the first case on record of a complete division of the entire uro-genital system ! The external genitals were normal, and an opening leading into a i of an inch deep represented the meatus urina rine. This was the normally developed glandular portion of the urethra. Each bladder opened into the corresponding urethra. (Fig. 12.) Hypospadia is a further malformation of the female urethra. Van Mosengeil has described such a case. The girl was eight years old, and had a large clitoris with a well-developed prepuce. The meatus was not situated at the usual distance from the opening in the hymen, but formed a half canal, the lower wall of which was absent to a considerable extent. Deep down behind the orifice of the vagina was the completely closed portion of the urethra, about .4 of an inch in length. The bladder itself was large, and had many diverticula. Lebedeff's case, shown in Fig. 13, is still more instructive. Lebedeff succeeded in giving her the power to retain urine for a considerable time in any position. He did several plastic operations, and later used electricity, placing one electrode on the symphysis, and the other in contact with the vaginal scar.