Paper - Imperforate anus with rectovaginal cloaca

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Stone HB. Imperforate anus with rectovaginal cloaca. (1936) Ann Surg. 104(4): 651-61. PMID 17856859

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This historic 1936 paper by Stone describes a congenital abnormality of cloacal development.


See also Florian J. The early development of man, with special reference to the development of the mesoderm and cloacal membrane. (1933) J. Anat., 67(2): 263-76. PMID 17104422


Modern Notes: cloaca | cloacal membrane | Human Abnormal Development


Kruepunga N, Hikspoors JPJM, Mekonen HK, Mommen GMC, Meemon K, Weerachatyanukul W, Asuvapongpatana S, Eleonore Köhler S & Lamers WH. (2018). The development of the cloaca in the human embryo. J. Anat. , 233, 724-739. PMID: 30294789 DOI.

"Subdivision of cloaca into urogenital and anorectal passages has remained controversial because of disagreements about the identity and role of the septum developing between both passages. This study aimed to clarify the development of the cloaca using a quantitative 3D morphological approach in human embryos of 4-10 post-fertilisation weeks. ...Our main finding was a pronounced difference in growth between rapidly expanding central and ventral parts, and slowly or non-growing cranial and dorsal parts. The entrance of the Wolffian duct into the cloaca proved a stable landmark that remained linked to the position of vertebra S3. Suppressed growth in the cranial cloaca resulted in an apparent craniodorsal migration of the entrance of the Wolffian duct, while suppressed growth in the dorsal cloaca changed the entrance of the hindgut from cranial to dorsal on the cloaca. Transformation of this 'end-to-end' into an 'end-to-side' junction produced temporary 'lateral (Rathke's) folds'. The persistent difference in dorsoventral growth straightened the embryonic caudal body axis and concomitantly extended the frontally oriented 'urorectal (Tourneux's) septum' caudally between the ventral urogenital and dorsal anorectal parts of the cloaca. The dorsoventral growth difference also divided the cloacal membrane into a well-developed ventral urethral plate and a thin dorsal cloacal membrane proper, which ruptured at 6.5 weeks. The expansion of the pericloacal mesenchyme followed the dorsoventral growth difference and produced the genital tubercle. Dysregulation of dorsal cloacal development is probably an important cause of anorectal malformations: too little regressive development may result in anorectal agenesis, and too much regression in stenosis or atresia of the remaining part of the dorsal cloaca."


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Imperforate Anus with Rectovaginal Cloaca

Harvey B. Strong, M.D.

Baltimore, Mp.


The various anomalies in the development of the rectum and anus are well known to the student of embryology and of interest to the surgeon. In some of them complete obstruction of the bowel exists and requires prompt intervention to save the infant’s life. In others the rectum is not completely blind, but instead of opening through the anus normally, the anus is absent and the rectum opens into some other hollow viscus. This paper concerns itself particularly with those cases in the female in which the rectum opens into the vagina. These organs normally communicate or form a common cloaca during one period of embryonic development, but later the hindgut becomes separated from the urogenital sinus by the development of a septum between these two passages, and the former, the hindgut, opens to the surface by its fusion with the proctodeum that dips inward from the perineum, thus leading to the formation of the normal anus. Sometimes both these processes, that is, the separation of rectum from vagina and the opening of proctodeum into rectum, fail to occur and the incomplete embryonic state persists. This leads to a condition of imperforate anus and rectovaginal fistula or cloaca.

The occurrence of such an anomaly has been long known. Bodenhamer,’ in his interesting monograph entirely devoted to congenital anomalies of the rectum and anus, cites references dating back to classic antiquity and mentions records of similar lesions in the dog and cow. He states, however, that this is a rare condition, and cites all of the few instances to which he could find reference. Since his time there have, of course, been other cases reported, but one is struck by the scantiness of discussion of the subject in any of the works consulted. Many of the text-books on surgery omit mention of it or barely state that such an abnormality exists. Even with volumes devoted to the rectum and anus the subject is dealt with, if at all, in very cursory fashion. This is, of course, entirely proper. The rarity of the condition does not justify extensive discussion in general treatises. Furthermore, as several authorities point out, this particular form of imperforate anus is apt to be less serious than most of the other varieties because the rectal orifice into the posterior vaginal wall is usually of sufficient size to permit adequate emptying of the bowel, or if it 1s not large enough it may be easily found and readily enlarged by stretching or incision. Hence, life-threatening obstruction 1s rare. It does occur, however, when the communication is very small and high in the vaginal vault. Such cases may require colostomy to save the child’s life, and are apt to be associated with double uterus. The writer has seen one such case. Much more commonly the rectum opens into the posterior vaginal wall lower down, and indeed it has been said that the commonest location of the opening is in the fourchette.



Fig. 1. — Sagittal section of imperforate anus and rectovaginal cloaca.


So far as symptoms are concerned they are the obvious result of the abnormal location of the rectal orifice. Absence of the anus or a rudimentary dimple-like depression is the external evidence of the condition, combined with escape of meconium or stool from the vaginal outlet. There is usually an accompanying incontinence although sometimes there seems to be a partially effective sphincter-like power in the tissues about the rectovaginal opening. In social groups that are careless or unobservant, it is said that the condition may even escape detection and there are reports of women who had reached adult life, and had even borne children, without themselves being aware of their abnormal condition. As a rule, however, these patients suffer the disagreeable results of fecal incontinence and not infrequently the partial obstruction that goes with an inadequate rectal orifice. They have soiling, skin irritation, constipation, straining, and may develop one form of giant colon. Such a state of affairs clearly calls for efforts at cure or improvement.

So far as the writer has been able to discover, in a fairly extensive, but by no means exhaustive, search of the literature, there is only one form of operation described and utilized for the treatment of these lesions—that of Rizzoli.2, In this procedure, an incision is made backward in the midline through the posterior vaginal wall, the fourchette, perineum and skin, from the edge of the rectovaginal fistula to the position that the anus would normally occupy. The rectum is detached from the vagina and drawn backward through the cleft perineum to its new position where it is sutured to the skin. The divided perineum and posterior vaginal wall are then sutured together in front of it, in order to reconstruct the normal relations. The results of this type of operation are generally described as good, but in some cases the repaired perineum has not held well and control has been unsatisfactory.

The writer offers a somewhat different technical attack with the same objectives—closure of the fistula into the vagina, restoration of the anus to its proper location and restoration of sphincteric control.


OPERATIVE TECHNIC. — With retractors in the vaginal outlet to expose the rectovaginal fistula, a circular incision is made about this opening, separating the rectal and vaginal mucous membranes from each other. This incision is deepened about the rectal wall and the dissection is carried upward about the rectum on all sides until it is freely mobilized. As the anterior rectal and posterior vaginal walls often are in very close contact, meticulous care in dissection is sometimes needed to avoid making a hole in either viscus. The mobilization should be carried as high as possible without opening the peritoneum of the cul-de-sac of Douglas, which is to be avoided if possible. After this dissection is completed, the rectum lies free and separated in the space between the vagina in front and the sacrum behind, while below it the levator ani muscle forms a continuous layer across the perineal floor. The next step is to make a small oval removal of skin in the position where the anus should be. Sometimes this will be indicated by a dimple, or the sphincter may be palpable under the skin. Often neither of these aids is present and the operator selects the anal location without special guidance. Through the skin wound that is to form the new anus a closed straight hemostat is thrust and worked, by blunt dissection, upward through the fascia and muscle of the levator until it penetrates into the free dissected space about the rectum. The passage through which the hemostat has made its way is stretched and enlarged carefully, without division of muscle fibers, until the index finger can be passed through it. Through this passage a clamp is then introduced upward from the perineum, until one can grasp the free end of the mobilized rectum, which is drawn downward and outward to the skin surface of the new anus. To do this properly without strain or tension, the rectum must first have been thoroughly mobilized high up, and the opening through the levator stretched enough to allow the gut to pass without difficulty. The rectum is now anchored in its new position with four quadrant submucoussubcutaneous sutures of catgut, and the free end of the gut sutured to the skin of the perineum with interrupted sutures of fine silk. By this procedure the perineal body and such muscle as exists in the pelvic floor have been preserved intact. The levator closes snugly around the rectum that has been pulled through it, and while the hole in the posterior vaginal wall is still open, a few catgut sutures may be taken in the levator and fascial structures in front of the rectum in its new position, to further build up a perineal body and rectovaginal septum. lastly, the hole in the posterior vaginal wall is closed.







Fig. 2. — Mobilization of rectum by dissection from surrounding structures. Upper insert shows beginning of dissection by separation of rectovaginal orifice. Lower insert shows drawing of mobilized rectum downward through perineum to oval wound at normal anal location. The vaginal opening is shown here closed by stitches.


This method of operative treatment has been employed in the three cases reported herewith. It has yielded uniformly good results. The vaginal closure has been firm, without a leak. The perineal body is substantial. The rectum opens in its normal position. The grasp of the levator on the rectum as it surrounds it has afforded excellent control for both gas and feces. A few comments may be made on certain points affecting the ease and success of the operation. It is best postponed until the age of puberty is approached, as the structures then are larger and much more satisfactorily handled than in very young infants. As was previously mentioned, this is possible as there is usually sufficient egress of bowel content to avoid obstructive symptoms in this particular form of anomaly, although sometimes a colostomy may be necessary. When the selected age is reached, a few days’ preliminary treatment in the hospital before operation, to empty the bowel completely, is very desirable. After operation, defecation is avoided if possible for seven to ten days, to permit the wound to solidify before subjecting it to the passage of feces. The daily administration of mild opiates effects this. After defecation has begun, warm sitz baths, twice daily, aid in the cleansing, healing and comfort of the wound. Before final dismissal, several digital examinations and gentle dilatations of the new anus and rectum should be carried out, but not earlier than the 14th day, in order that the newly healing wounds may not be damaged. A late survey of the cases, some months after operation, to determine the result, and to correct any tendency to stenosis, 1s desirable.




perineum. Lower insert is attempt to indicate relation

of levator muscle to new position of rectum.

Upper insert shows view of same from



Fig. 3.—Sagittal diagram of completed operation.



CASE REPORTS

Case 1.—No. 32812, Union Memorial Hospital. L. B., white girl, aged 13. Examination shows imperforate anus with a small scar, said to be due to an attempt to open the bowel by operation shortly after birth. When this failed, a colostomy was effected in the sigmoid, which is still open and functioning. In the posterior vaginal wall, one and one-half inches above the hymen, is a small opening marking the abnormal rectal orifice. The child is otherwise well developed, rather large for her age, and quite normal physically and mentally. The operation was performed as described June 27, 1934. On July 10, 1934, a digital dilatation was done. Convalescence was uneventful except for an intercurrent attack of pyelitis, and the patient was discharged after three weeks. She returned to the hospital one year later for closure of the colostomy, which was effected June 26, 1935, successfully and without incident. Examination at this time showed an excellent result. Normal appearing anus, good perineum, and excellent control of bowels without soiling.

Case 2.—No. 33117, Union Memorial Hospital. G. J. B., white girl, aged 13. Examination showed a small dimple where the anus should have been. The rectum opened into the posterior vaginal wall one inch above the hymen by an orifice, which had always been large enough to permit of easy defecation since birth. The child was otherwise normal, mentally and physically, well developed, and free from complaints, except for incontinence and irritation and excoriation about the vulva. The operation described was performed July 25, 1934. Wounds healed well, convalescence was uneventful, and the patient was discharged in three weeks. Examination some months later showed practically complete restitution of rectum, anus, perineum and vagina to normal with complete rectal continence.

Case 3.—No. 66891, Johns Hopkins Hospital. M. C., white girl, aged 10. Child was born with imperforate anus, the rectum opening through the posterior vaginal wall, low down. There were several other anomalies—an extra thumb on each hand, which was removed in early infancy without impairing the usefulness of hands, absence of coccyx, unilateral asymmetry of sacrum, fusion of several vertebrae, etc. For the past year or two, the child has had curious monthly attacks of mental disturbance, evidenced by confusion, irrational speech, and tendency to lapse into coma. These last several hours, and afterward she has no recollection of them. During the early years of her life, three or four attempts were made to correct the cloaca surgically, which succeeded only in bringing downward the inferior margin of the rectovaginal orifice so that it now lies practically in the fourchette. There is still incontinence except for solid stools, and vulval and perineal soiling and irritation. No sphincter muscle could be felt under the skin and there was no dimple to indicate the position of the absent anus. The operation described was performed December 30, 1935. During the first week there was some inflammation about the stitches closing the vaginal wound, but this subsided without breaking down of the ‘repair, and the rectal suture held also. The child was discharged in three weeks, all wounds practically healed, the rectal orifice and lumen adequate and with practically normal control.

SUMMARY AND CONCLUSIONS

Rectovaginal cloaca of congenital origin with imperforate anus is a well known but rare anomaly. It is associated with incontinence of feces and gas in many instances, with the distressing results of such incontinence, but rarely with high grade obstruction that calls for surgical relief in the early hours of life. It is amenable to very satisfactory surgical correction, and a method for such correction is herewith described, with a report of three successful cases. It is advisable to defer operative attack until the child approaches puberty, when the anatomic structures are easier to deal with than in the years of infancy.

References

Bodenhamer, Wm.: Congenital Malformations of Rectum and Anus. Samuel S. and Wm. Wood, N. Y., 1860.

Rizzoli, Francesco: Memorie dell’ Accademia delle Scienze dell’ Institute di Bologna, 1857. Idem, 297, 1874.

Discussion

Dr. WILLIAM E. Lower (Cleveland, Ohio.) — I should like to present a case which is somewhat similar to that reported by Doctor Stone, except that the fistula was between the rectum and the urinary bladder. The child was born with an imperforate anus. I established a colostomy to relieve the acute condition, and then made an artificial anus, closed the fistula between the rectum and the bladder, and then closed the colostomy. The child is now 12 years old; she has no vagina, is almost reaching the age of maturity, and the secondary sex characteristics are developing. The problem now is, what is the next step? Shall I sterilize by roentgen therapy or perform another operation removing the uterus and the ovaries? I am presenting this as a problem. If any one can tell me just what should be done, I should appreciate it very much.

Dr. Franz Torexk (Montclair, N. J.). — I had a case, similar to the one Doctor Stone presented, upon whom I operated when she was six months old. The fecal soiling was so disgusting that the parents could not tolerate it any longer. The procedure was the same that Doctor Stone has described. The fistula in the back portion of the vagina was closed; the rectum was moved backward and implanted at a new site.

That was 24 years ago. I never heard anything more of the patient until four months ago. She had in the meantime become married and divorced. The reason for the divorce was because of the absence of the perineal muscles, which resulted in loss of sphincteric control over the vaginal introitus.

On examination I found that at the operation when she was six months old, I had paid no attention whatever to the restoration of the perineal muscles. I told her that could be remedied. The operation was performed, and the result is perfect. There is a good sphincteric control. It was also possible to repair the sphincter ani, the anterior portion of which was open. She has since tested the local muscular functions with satisfaction to both parties.

Dr. VERNON C. Davip (Chicago, Ill.).—I think Doctor Stone’s operation is a very much better operation than that described by Rizzoli, Delket or de Kermisson, all of whom employed the longitudinal incision, because, as Ombédonne says in his text-book, the rectum in that type of operation tends to return to the abnormal place, so that after a few months or years the situation is very much as it was before operation. I have seen six of these patients, children or infants, with atresia and vaginitis, and have operated upon two of them according to the method described by Doctor Stone, with one or two additions to the technic. Before mentioning them I should like to say that I think it is very important to allow these children to go until they reach the age of five to seven, because in two of these six patients, the rectum and the vagina have spontaneously and naturally separated them selves from each other, so that a perineum has formed between the vaginal orifice and the rectum, and the bowel has carried with it the muscles that surrounded the abnormal opening.

In these six children, three have remained continent with the abnormal opening, and if one decides to do an operation to replace the end of the rectum into another situation, which would be considered the normal site, it is very important to be sure that the external sphincter is present, because if the bowel is transplanted into a new place and incontinence ‘develops, the patient will be a great deal worse off than she would have been with the abnormal opening and continence, and I really believe that continence is present in a number of these children.

In transplanting the rectum according to Doctor Stone’s technic, you will notice the mucosa is sutured to the skin, and that results in a situation that is commonly seen after opening an imperforate anus, namely, an extrophy of the mucosa results ; mucus covers the skin, and it is like a poorly performed Whitehead operation. I think that can be avoided. At least we did avoid it in two patients of this group by making skin flaps from the sides of the new opening of the bowel and allowing them to invert in this new position of the rectum, so that as the end of the bowel tends to retract, as it always does, it pulls these loose flaps of skin in with it. They can be turned in from the side, so that an anal canal lined with skin results, and if enough skin is turned in, it 1s a very helpful way to prevent stricture, which is another bad result one can obtain from transplantation of these abnormal openings to a new site.

Dr. Orro C. PickHarpt (New York). — Doctor Stone’s presentation has been interesting and instructive. I should imagine that in a good percentage of cases the procedure outlined would be sufficient. However, each one of these cases presents certain individual difficulties which have to be overcome, and where there is a question of a lack of length of the rectum, as in the appended case, which I would like to report, I think a more formidable procedure frequently has to be attempted.

Case Report. — A girl of seven was operated upon in 1928. The cloacal opening represented in Figure I is not quite correct. It was really situated well back in the posterior portion of the vagina. In this case also there was a dimple, but an entirely imperforate anus. The scar is the result of some previous operation (Fig. 1).



FIG, 1 It was felt that because of the distance between the cloacal opening and the anal opening, that the simple operation of mobilizing the rectum would not be sufficient, and that a modified Kraske operation would help. Therefore, the usual incision was made, also one around the anal dimple, taking care to leave a bridge of tissue in this particular situation (Fig. 2).


FIG. 2 With the coccyx and a small portion of the sacrum removed, it was an easy matter to mobilize the rectum and to obtain any desired length that seemed necessary at the time. The skin was removed over the anal dimple (Fig. 3). I think it is rather interesting that, in certainly all the cases that I have seen, and in most of those which have been reported, both the internal and external sphincter were almost invariably present.


FIG. 3 FIG. 4 After complete mobilization the rectum was brought out through the anal opening (Fig. 4). It is wise to leave a large cuff of mucous membrane in order to allow for the retraction that is sure to follow. (

The various incisions were then closed, and in this particular instance the opening in the vagina was so large that the sutures posteriorly could not be placed. The whole vaginal wall, therefore, was brought forward and sutured to the fourchette (Fig. 5).


Fig. 5 Another point of interest is the time when the first bowel movement should take place. I waited 13 days in this particular instance. The wounds healed kindly, without infection, and at the end of 30 days the patient had very definite sphincteric control. For the first two or three years, regularly, every month, bougies were passed. At present the child is in perfectly normal condition.


Dr. Harvey B. Stone (Baltimore, Md.) closing — I think Doctor Pickhardt is right when he says there are much more complicated cases than those I showed, that require extensive and elaborate operations. As a matter of fact I think some of them are probably insoluble.

Such a case, which I referred to briefly, was a patient who had a double uterus, with a very small tubular end of the rectum opening into the very apex of the vaginal vault, between the two cervices, with no gut below that point at all. I felt that case could not be corrected by any measures that I knew of, but I am sure that some less difficult ones might be corrected by the approach Doctor Pickhardt has described.


I have no answer to Doctor Lower’s problem. It would take a wiser man than I to tell him what to do in such a condition. I think Doctor Torek’s case emphasizes the advisability of deferring operation in these children until they are out of the infant class, certainly until they are four or five years old, and I believe, better, until they are approaching puberty.


I appreciate Doctor David’s suggestion for handling the suture line at the margin of the skin-mucosa anastomosis. I am quite sure that is a valuable addition, and shall employ it at the first opportunity.



Cite this page: Hill, M.A. (2019, November 17) Embryology Paper - Imperforate anus with rectovaginal cloaca. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Paper_-_Imperforate_anus_with_rectovaginal_cloaca

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