Congenital Malformation of the Genital Tract. Persistence of the Sinus Uro-genitalis as a Common Opening with the Urethra. Bicornate Uterus. BY C. P. STRONG, M.D., BOSTON, MASS. REPRINT FROM VOL. XVI. 1891. CONGENITAL MALFORMATION OF THE GENITAL TRACT: PERSISTENCE OF THE SINUS URO-GENITALIS AS A COMMON OPENING WITH THE URETHRA; BICORNATE UTERUS. BY C. P. STRONG, M.D., BOSTON ; ASSISTANT IN GYNECOLOGY, HARVARD MEDICAL SCHOOL; PHYSICIAN TO OUT-PATIENTS, MASSACHUSETTS GENERAL HOSPITAL J ASSISTANT-SURGEON, FREE HOSPITAL FOR WOMEN. REPRINTED FROM THE TRANSACTIONS OF THE AMERICAN GYNECOLOGICAL SOCIETY, SEPTEMBER. 1891. ■>' PHILADELPHIA; WM. J. DORNAN, PRINTER. 1891. [Reprinted from the Transactions of the American Gynecological Society, September, 1891.] CONGENITAL MALFORMATION OF THE GENI- TAL TRACT. PERSISTENCE OF THE SINUS URO-GENITALIS AS A COMMON OPEN- ING WITH THE URETHRA. BICORNATE UTERUS. By C. P. Strong, M.D. Boston, Mass. Mrs. M., an American, aged thirty-one, seven years married, entered my service at the Free Hospital for Women for relief from sterility, offensive discharge, dyspareunia. The history she gave me, in so far as it relates to the points of interest in this case, can be considered under the two heads of menstruation and marital relations. Menstruation. At the age of fifteen the patient first noticed pelvic pains located in a comparatively small area in the supra- pubic region. The pain she described as constant, but not severe, except the occurrence of monthly exacerbations, during which she was confined to bed, the pain at this time assuming a more " colicky " character. This condition lasted for eight months, when, in the midst of one of these periodical attacks, a sudden copious hemorrhage took place, with complete relief for a few weeks, when the pains began again, to be relieved in six months by another hemorrhage; and thus, with gradually decreasing intervals, it continued for four years, until she was twenty years old, since which time she has menstruated regularly and pain- lessly each month, the length of the period being seven days and the amount never excessive. The patient was married at twenty-three, entirely unconscious of anything abnormal in the condition of her genital organs. Attempts at coitus were for many months excessively painful 2 CONGENITAL MALFORMATION OF GENITAL TRACT. both to herself and her husband, and the act seemed incomplete. Latterly, however, it was apparently perfectly performed, al- though there has always been discomfort, amounting occasion- ally to pain on the patient's part and little satisfaction to the husband. Desire for coitus has never existed. Physical examination. Inspection showed absence of the clitoris and prepuce. Extending from the mons veneris on either side were small, rounded elevations, which occupied the position of the labia majora, terminating just below the level of the urethra, at the upper limit of the sphincter ani muscle. There were no labia minora, even in a rudimentary condition. Just below the pubes, with no appreciable amount of intervening tissue, was a funnel-shaped opening, its outer end ragged and irregular as though ruptured, its lining a distinct mucous membrane. Be- tween this opening and the upper margin of the sphincter ani Fig. 1. Represents thejaccurate size of the external genital organs 'muscle was a smooth surface of perhaps half an inch of appar- ently normal skin unmarked by any depressions. The total dis- tance from the pubes'to the sphincter ani muscle was barely more than one inch. The funnel-shaped opening mentioned above terminated internally'in a large cavity, into which the finger was easily and painlessly; introduced, encountering only slight C. P. STRONG. 3 tonic resistance at one point, which was apparently the entrance to the bladder. I could nowhere touch the walls of this cavity except in front, where they came against the posterior part of the symphysis pubis. No fluid escaped by the side of the exam- ining finger, but with the catheter a pint or more of clear urine was withdrawn, without, however, bringing the vesical walls in contact with the finger-i. e., the bladder did not contract. By combined recto-abdominal and recto-vesical examination it was evident that the vagina did not occupy its normal position. Also there was distinctly revealed a mass about the size of an ox-heart drawn closely back against the sacrum and lying in the median line. The upper part of this mass had a distinct central division, the lateral portions being each about the size of a hen's egg, but fusing together lower down into a single uniform globular shape. The existence of the ovaries was also determined. Further examination was negative in its results. With these facts as a basis, I deemed it advisable to wait until the occurrence of the next menstrual period, that the discharge might aid in discovering the channel through which the flow escaped from the uterus to the external genitals, and so indicate the best method of operative procedure for relief. On March 24,1891, the second day of free menstrual flow, a careful search showed that the funnel-shaped opening previously mentioned was the channel for the escape of the discharge externally. The urine drawn was perfectly clear; therefore it was a necessary in- ference that the opening must be anterior to the bladder, and finally a drop of blood was detected oozing through a delicately fine opening on the posterior floor of the urethra, about half an inch from the external orifice. A very fine probe was introduced into this opening, and, after passing along a canal which lay just beneath the urethral mucous membrane, entered at the depth of two inches a cavity of considerable size. Using this probe as a guide to avoid wounding the urethra, and the finger in the rec- tum to protect that organ, a transverse incision was made in the skin just above the sphincter ani muscle, and a canal which would admit the finger was opened up into a cavity about one and a quarter inches in diameter, globular in shape, and filled 4 CONGENITAL MALFORMATION OF GENITAL TRACT. with offensive clots and debris. The fistulous opening from the urethra also terminated in this same cavity, which was evidently the dilated lower segment of the uterus. The tissue between the rectal and urethral mucous membranes was so very thin that, having provided a means of drainage and disinfection, I thought it prudent to postpone for a time the completion of an artificial vagina, which was subsequently formed by enlarging by lateral incisions the canal made at this time. This second operation was one of considerable difficulty, owing to the free hemorrhage which followed the opening up of the tissues at the sides of the pelvis, but was controlled by a firm tampon. During this inter- val of rest the uterus had emptied itself completely, the cervix remaining but little larger than normal, and the condition sug- gested above of uterus bicornis unicollis was even more dis- tinctly marked. After complete cicatrization of the new vaginal canal, the patient was discharged, with instructions to introduce daily a vaginal plug one and a quarter inches in diameter and two and a half inches long. To promote speedier healing and the formation of less cicatricial tissue, I should in another case try skin-grafting over the new vaginal surface. A letter received in July stated that she had experienced no difficulty in keeping the new vagina patent, that coitus now took place through it, and was attended by no pain. Reviewing the menstrual history of the patient's earlier years, it is evident that there was at first complete obstruc- tion to the escape of the periodical discharge until sufficient force was developed to overcome existing barriers, and then hemorrhage ensued. Latterly the patency of the opening from the uterus into the urethra being thoroughly established, menstruation occurred regularly and painlessly. The|consideration of anomalies as presented in adult life finds not the least of its interest in the possibility of tracing directly their development from variations in the normal embryological type. So I will very briefly add to the report of this case my explanation of the conditions which were here present. In the primitive stage of the embryo the genital cord is formed by four distinct structures-the Wolffian C. P. STRONG. 5 bodies and the ducts of Miiller. The ducts of Muller, two in number, form by their fusion the single uterus and vagina. It is conceded that fusion takes place about the middle of the ducts, and therefore for some time the uterus exists either wholly or in part as a double organ-uterus bicornis-which, in the case above cited, persisted, as is quite common, into adult life. The fusion of the lower part of the ducts of Miiller forms a canal, subsequently becoming the vagina, which terminates in a cavity of the allantois-the urachus. (Fig. 2.) The upper portion of the urachus becomes the Fig. 2. Ducts of Muller. Urachus? bladder. The lower part, including the point of entrance of the ducts of Muller, receives the special name of sinus uro- genitalis, and, continuing downward, forms with the termin- ation of the rectum a common cavity-the cloaca. (Fig. 2.) By the growth of new tissues, the cloaca is subdivided into two distinct cavities-the rectum posteriorly and the sinus uro- genitalis anteriorly (Fig. 3), at the same time that the urachus is subdivided transversely. There now exists three distinct cavities-the bladder, the rectum, and the sinus uro-genitalis. (Fig. 3.) The next subdivision to take place is by a down- ward growth of tissue, shutting off the urethra from the sinus uro-genitalis above and anterior to the entrance of the ducts of Miiller, while posteriorly the ducts of Muller persist as the vagina, and the sinus uro-genitalis as the vestibule. From this very brief resume of the normal embryological 6 CONGENITAL MALFORMATION OF GENITAL TRACT. formation, it is evident that failure of complete fusion of the Mullerian ducts may lead to persistence of two distinct utero- B/adder Fig. 3. Rectum. /Sinus Uro-genitalis. genital organs, either in whole or in part. In the case above reported it was limited to the body of the uterus, resulting in the not uncommon bicornate uterus with a single neck. Fig. 4. Fi i. 5. Tissue which <l\ows down -wards Urethral- The second variation was more rare. Should there be arrest of development in the tissues which separate the common cavity of the urachus into three distinct ones-the bladder, the urethra, and the sinus uro-genitalis (Figs. 4 C. P. STRONG. 7 and 5)-the lower end of the ducts of Muller which form the vagina would terminate not externally, but at some point within the cavity, along its posterior surface. This was the cause of the anomalous condition presented here. Develop- ment proceeded smoothly until the urachus was subdivided into the bladder and the sinus uro-genitalis, and the cloaca into the rectum and the sinus uro-genitalis. Then the tongue of tissue, which should have been prolonged downward pos- teriorly to shut otf the urethra, was not developed, and the sinus uro-genitalis persisted as a canal having a common opening, with the urethra externally, and through a very contracted channel with the uterus internally. Still further corroboration of this explanation is afforded by the small amount of tissue which existed between the rectum and the genital tract. There was no development of the muscular structures forming the posterior pelvic floor, and which are always present in those variations from the normal type due to, so to speak, accidental occlusion of the vagina, as by a simple imperforate hymen.