[Reprinted fi-om The American Journal of Obstetrics and Diseases of Women and Children, Vol. XVIII., March, 1885.] TWO CASES OF CONGENITAL IMPERFORATE ANUS WITH FECAL FISTULA IN THE ADULT FEMALE. BY CLARA MARSHALL, M.D., Professor of Materia Medica and General Therapeutics, in the Woman's Medical College of Pennsylvania ; one of the Accoucheurs to the Philadelphia Hospital. Congenital malformations of the rectum and anus, of such a nature as to interfere with the function of defecation, are, for- tunately, of rare occurrence. And yet by its very rarity such an unnatural conformation may entirely escape the notice of the accoucheur, or may tax to the utmost the resources of the surgeon. Among these malformations may be mentioned the simple occlusion of the anus by a thin layer of integument, or semi- mucous membrane, a rectum of unusually small calibre, or the still graver defect of an absence of a portion, or even the whole of the rectum, with or without a communication with the surface by some unnatural channel. These various devia- tions from nature have from time to time suggested a variety of surgical procedures, among which the simple crucial incision over the anus for the mildest type of deformity; the thrusting of a trocar into the perineum (in cases of imperforate rectum) in the hope of striking somewhere a bulging rectal cul-de-sac (" making an exploratory puncture into anatomical darkness "); the more careful dissections with the scalpel on a like voyage of discovery; or the still graver operations of inguinal and lumbar colotomy, are associated with the names of Littre, Callisen, Amussat, and others of high authority in the pro- fessional world. In this brief paper reference will be made only to such cases as are allied to those which have come un- der the personal observation of the writer, the general charac- ter of which is indicated in its title. Case I.-Ella H., set. 19; born in Ireland; domestic; mar- 2 Marshall : Two Cases of ried; primipara. Her family history is good, and she herself is stoutly built, and has always had good health. She was admitted to the Philadelphia Hospital, March 3d, 1884, and after a normal pregnancy was delivered, at full term, of a female child weighing six pounds. The labor was very slow, the head not being delivered until six hours after the membranes had ruptured. At the time when the messenger was sent for me, the condition, as described by the interne in charge, was as follows: "The pelvis is roomy, presentation that of the vertex with the occiput to the left and anterior, the os well dilated; but as the uterine contractions are very feeble, delivery by the forceps seems to be indicated." Upon arriving, I found the woman in good condition; the fetal pulsations were distinctly audible, and the head was upon the perineum. I noticed that, although the head was distending the perineum so that the anus seemed to be distinctly outlined, yet there was not that eversion of the mucous membrane of the rec- tum usually seen at this stage of labor. As the physician in charge of the case reported considerable progress in the labor during the absence of the messenger, a short time was allowed in which to see what could be accomplished without the aid of instruments. It was during this time, when introduction of the finger into the rectum, to favor extension of the head was suggested, that the deformity was discovered. The anus was found to be imperforate, and what appeared to be an anus was simply a depression surrounded by an imperfectly de- veloped sphincter. Upon introducing a finger within the vagina, and making pressure against the posterior wall, an opening was found about four inches from the vulva, leading directly into the rectum. When the child was born, its thighs and hips were cov- ered with fecal matter; much had also escaped with the liquor amnii. When questioned, the woman stated that the deformity was congenital, and had never given her any inconvenience ex- cept when constipated, at which times she had some difficulty in expelling the fecal mass-a state of affairs which might easily occur in one not so deformed. The labor was soon terminated without instrumental interference; the infant was well-formed in every respect, and the lying-in was normal. Some weeks after her delivery, the patient was examined by my colleague, Dr. Wm. H. Parish, and by Dr. Robert P. Harris; Drs. Bliss and Matson, who were with the patient during her con- finement, were also present. Upon this occasion, the fistulous opening between the vagina and rectum, which had so plainly existed during the labor, could not be found-the rectum open- ing upon the vulva just within the fourchette. This abnormal anus, which might better be described as a slit, had no sphincter in the strict sense of that term, and its anterior and posterior walls were in such close apposition as to render an examination by the touch alone quite deceptive, the finger readily passing- over the perineum and into the vaginal canal (after the usual Congenital Imperforate Anus. 3 method of making a digital examination) without detecting any- thing abnormal. Not in every case of imperforate anus does the onset of labor lead to the discovery of its existence, for Le Fort tells us of "A married woman who, upon rectal examination, was found to have an imperforate anus, the rectum opening obliquely into the back part of the vagina. The command of the feces was so perfect that no inconvenience resulted, and neither she, nor her husband, nor the accoucheur who had delivered her three, times, were aware that there was any peculiarity of the sexual organs." [Holmes' System of Surgery.] In the case now reported, the " anus " was drawn upwards dur- ing the labor, simulating a recto-vaginal fistula, the contents of the rectum being discharged into the vagina fully four inches above the entrance. In such a case as this, any obstetrical proce- dure requiring the introduction of the hand within the cavity of the uterus would be likely to result in the introduction of fecal matter also, which, by its absorption, might' endanger the safety of the patient. There is also the possibility of the feces becom- ing impacted, and acting as a serious obstruction to the pro- gress of the labor, as in the following case: M. Fournier (1813) was called in consultation in the case of a woman who had been in labor for five days, and whose bowels had not been moved for a week. The case proved to be one of im- perforate anus, the vagina being filled with hardened feces which had entered from an opening within the vulva. Copious injec- tions removed this hardened mass from the rectum and vagina, after which the labor was speedily and safely terminated ("Dic- tionnaire des Sciences Medicales," Tome IV., p. 155). Case II.-This case I was enabled to examine through the cour- tesy of Dr. D. D. Richardson and his assistant, Dr. Alice Avery, of the Insane Department of the Philadelphia Hospital. Dr. Avery has kindly furnished me the following notes from her case- book. Mary L , admitted to the hospital May 6th, 1884, was born in Philadelphia, is seventeen years of age, single, and without occupation. Her father is living and healthy, her mother died of phthisis pulmonalis, and a younger sister of convulsions. Her general physical condition is not good, as she is somewhat ane- mic, though the thoracic and abdominal viscera appear to be healthy; the pulse, temperature, and urine are normal. The mental condition is that of dementia. There exists a congenital malformation, by which the rectum terminates within the vagina. The recto-vaginal opening is low down upon the posterior wall of the vagina, of sufficient size to 4 Marshall : Two Cases of allow of free evacuation of the bowel, and surrounded by a sphincter, by means of which it is almost completely closed. When the finger is introduced into this "anus," it may be passed backward into a rectal cul-de-sac, the bottom of which is sepa- rated from the usual anal site by not more than a quarter of an inch of tissue. (In Case No. I. no such rectal pouch exists, but in both there is the same attempt at the development of a sphinc- ter ani.) The other pelvic organs are apparently normal, except that the uterus is unusually high (being partly au abdominal or- gan), and is displaced to the left. The control over the evacuation of the bowel posessed by both the above-mentioned patients may at first thought seem remarkable, but it is no more so than the fact of the small proportion of cases in which division of the sphincter ani for fistula in ano is followed by incontinence of feces; nor is it more remarkable than is the same state of affairs where colotomy has been performed for congenital malformations of the rectum. "The physiology of the act of defecation goes far to explain why there should be a certain warning of the approach of an evacuation. . . . The control spoken of will be found in most cases to mean rather a consciousness of an approaching movement, a warning given in sufficient time to allow the patient to make necessary arrangements, rather than an ability to absolutely prevent the evacuation which is about to take place " (Kelsey, on " Diseases of the Rectum and Anus"). There may arise a question as to the advisability of surgical interference. In case No. I., the patient is so per- fectly healthy in every respect that it would seem the most sensible course to " let well enough alone." The same conclu- sion is warranted in the case of the young girl in the insane department of the hospital. Indeed, it is obvious that opera- tive interference is not so imperatively demanded in this class of cases as it is in those in which there exists no outlet what- ever to the bowel. Yet even the more favorable cases, as a rule, suffer great discomfort, and in a certain proportion, sooner later death results. Even if trouble does not come on at the birth of the child, as soon as it begins to take solid food there is a liability to constipation of the most obstinate and dangerous character. Benivenius (1529) reports the case of a girl who had an imper- forate anus, with au opening between the rectum and vagina, through which the feces were expelled. This abnormal opening Congenital Imperforate Anus. 5 proved insufficient; the feces were usually voided only once in eight days, and death finally occurred at the age of sixteen from obstinate constipation with impaction of the feces ("Libellus de Abditis Nonnullis ac Mirandis Morborum Sanationum Causis," Cap. 86, Basil., 1529). In order to avoid so dangerous a complication, as well as to save the individual from the constant sense of what is in many instances a disgusting deformity, in the majority of cases operative interference is indicated, either in infancy (if the symptoms are urgent) or in early childhood. The method of operating can best be described by calling attention to some recorded cases successfully treated by ope- rative measures. In 18-, in Philadelphia, Dr. J. Rhea Barton performed two operations upon the same child at the ages of six weeks and nine months; the first by incision through the anal depression upward into the rectum. This failed, as the parts soon united. He then introduced a director into the recto-vaginal opening, and cut downward, laying open the tissues to the anal depression, and then upward to the bowel. The newly formed route from the rectum to the anus was kept open, and the remainder healed by granulation, the result being a rectum and vagina each complete. Moreover, tlie absence of the sphincter-ani muscle did not pre- vent the patient's having control over the evacuations (Medical Recorder, Vol. VII., p. 357. Philadelphia, 1824). Following the example of Dr. Barton, Dr. Joseph Parish oper- ated with entire success Feb. 18th, 1823, upon achild aged fifteen months, who was suffering from the malformation under consid- eration. In this case, Prof. Dieffenbach performed the following opera- tions. He introduced a grooved director, considerably curved, into the recto-vaginal orifice, thrust a pointed bistoury imme- diately below the "fossanavicularis" outside of the vagina into the groove of the director, and divided all the cellular and muscular tissues between the point of the puncture and the coccyx. lie then dissected off the end of the rectum from the abnormal open- ing, and isolated it for some distance from the surrounding parts: this enabled him to draw down the free end of the bowel, and attach it to each edge of the cleft perineum. The cut edges of the rectum united to the skin, and the recto-vaginal aperture closed very completely, only having been occasionally touched with nitrate of silver. Three weeks later, by a second operation, the perineum was restored. The cure was complete. (Boden- hamer on " Congenital Malformations of the Rectum and Anus.") Other parallel cases are on record, among which are the following: 6 Marshall : Two Cases of M. J. Hgesbart saw a young woman, set. twenty years, with a similar malformation. Her health was excellent, and no opera- tive measures had ever been instituted ("Miscellanea curiosa decur," II., Ann. X., Observ. 75, p. 132, 1691). 1791. One of the most remarkable cases on record is that of a Jewess, a resident of Padua, described by Mercurialis and Morgagni. In this young woman, there existed the deformity already described, and we are told that she lived to the advanced age of one hundred years, without any aid from the stirgeon; in- deed, Mercurialis advised against operative procedure ("De Sedibus et Causis Morborum," Epist. xxxii., Art. 3, Venetiis, 1761, 2 Tome, Folio). 1785. Van Sweiter reports the case of a young woman with the same deformity (Boerhave, Book iv., 1340, p. 575). 1822. Mr. William Cooke, while attending a case of labor in a woman aged forty, discovered that the feces were escaping from the vagina; this led to the discovery of a congenital communica- tion between the rectum and vagina, large enough to admit of two fingers (English translation of Morgagni, Vol. ii., p. 110, Boston, 1824). 1826. Switzer reports the case of a woman who complained of no pain in defecation, and who claimed ability to secure entire cleanliness by sponging out the vulva after each fecal evacuation (" Annotationes in Colotomiam," p. 79, Hafnie, 1826). 1833. Ricord describes a most interesting case of deformity in a woman twenty-two years of age. Upon introducing the vaginal speculum, he was surprised to find it passed so far with- out meeting the neck of the uterus, when a lump of fecal matter was brought into view which he at first mistook for the os tineas; also some grape seeds, at first thought to be granulations; a more thorough examination, however, revealed the fact that the vagina was acul-de-sac, the uterus being absent, the anus imperforate, and the feces voided through a recto-vaginal opening, over which she had control, except so far as the expulsion of flatus was con- cerned. Menstruation had never occurred in any form. Injec- tions after defecation had secured cleanliness, and the deformity had never been suspected by others (Jour. Univer. et Hebdom. de Med., Tome xii., p. 167, Paris, Oct., 1883). 1860. Dr. Berrut operated successfully upon an infant with this deformity. Up to seven years of age, she was subject to diarrhea, but at twenty was healthy and well-developed, and had diurnal evacuations. The number of recorded cases of this deformity is, com- paratively speaking, very small, many cases being lost to statistics through the carelessness of midwives. Dr. Lohrer, of Vienna, met with only two cases of imperforate anus in fifty thousand new-born children. Mr. Collins, during his master- ship of the Dublin Lying-In Hospital, observed only one in- Congenital Imperforate Anus. 7 stance in 16,645 births. (West's lecture on "Diseases of In- fancy and Childhood.") But the rarity of these cases does not excuse the accoucheur from the important duty of examining every infant immedi- ately after birth, or from ascertaining, at a subsequent visit, whether there has been a passage of meconium. The nurse must be cautioned not to administer purgative medicines ex- cept under direction, and such direction .must not be given until all question of malformation is set at rest. If unhappily a deformity exist, fortunate is the obstetrician, who, being forewarned, is at the same time forearmed.