Agenesis of the female urethra, trigone and the anterior anorectal wall (a congenital anorectourogenital cloaca)

Agenesis of the Female Urethra, Trigone and the Anterior Anorectal Wall (A Congenital Anorectourogenital Cloaca) A NEW

PROCEDURE

FOR THE CORRECTION MONROE WOLF,

From the Cburity

Hospital

of New Orleans,

M.D.,

INCONTINENCE

New Orleans, Louisiana

Louisiuna

THOROUGH search of the literature has failed to reveaI a singIe case which was simiIar in any respect to the case presented herein. Two cases of urethral absence are cited: Petit’s [8] case was that of “a four year old female sans urethra, chtoris and nymphae.” The second case, reported by FraenkeI [5], was that of a twenty year oId femaIe unable to prevent discharge of urine since chiIdhood. the vagina1 introitus was normaI, the ;I*** ymen was intact and so narrow as not to permit the passage of the tip of the littIe finger. ImmediateIy above it was an opening with a reddish mucous membrane into which the tip of the IittIe finger could be introduced. From the aperture urine was continuousIy discharged. At this point there was not, as might be supposed, a deficient urethra, but the bladder opened into the vagina1 vestibule.” CompIete cure was obtained by the technic of Martius using the hymen and the bulbo- and ischiocavernosus muscles. The foIlowing two cases are brought to the reader’s attention, ahhough I do not pIace them in the same category as the aforementioned cases. Langenbeck’s [8] case was that of a nineteen year old girl whose “ . . . bladder and vagina formed a common cana1, gained contro1 afterwards.” There is no mention of surgery. This couId have been a case of hypospadias. Emmet [4] recorded the case of “ . . . a tweIve year oId femaIe with a cIeft of the symphysis pubis and an absence of a portion of the urethra, so that the gir1 couId not retain her urine Ionger than 15 minutes.” There is no

A

American Journal of Surgery, Volume 9% June, rw7

OF URINARY

1030

mention of the outcome of this case, which was probably an epispadias. The case to be presented herein is simiIar to the case of Fraenkel with the additiona pathoIogic condition described by Browne [J] in his 1950 Hunterian Lecture as “a shot-gun perineum.” It is not the intention of this report to discuss the embryology of the alimentary and urogenita1 tracts, but rather to place on record the only case of a congenital anorectourogenita1 cIoaca and the author’s modification of Bricker’s [2] application of a Ioop of the iIeum to form an artificial hIadder. CASE

REPORT

Because the hospital records of this case were mispIaced, the following was acquired from the autopsy protocol. This patient was a sixty-eight year old Negro woman with a very Iow I.Q. As a result the cIinica1 and socia1 history is poor and unreliable. The patient was admitted to the hospital in 1939 for treatment of syphiIis and IymphogranuIoma venereum. The Frei test and the serologic test for syphilis were positive. She was readmitted in 1943 for treatment of a probable vesicovaginal fistuIa. Her next admission was in 1949, when compIete absence of the urethra was noted. No mention of the anorectogenital defect was made at this or the Surgery was refused on previous admissions. this admission. On the next admission in August, 1953, a diagnosis of congenita1 absence of the urethra and proIapse of the bIadder was made.

CongitaI On

the

AnorectourogenitaI

present

admission, November 16, with only this admission), the patient compJained of urinary incontinence of approximately three years’ duration; there had been no Joss of feces. She had been married for seventeen years and had never been pregnant; intercourse had been normal (?). There had been previous antisyphiJitic treatment with arsenicals and bismuth. The past history was otherwise irreJevant except as mentionecl on tJle previous admissions. On physical examination the patient’s J~Iood pressure was 148/72 mm. Hg. Deep reflexes were normal, as were the extremities. There was no adenopathy. Eyes, ears, nose and throat were normal. There were no adventitious sounds in the heart and lungs. The electrocardiogram was bvithin normal limits. Inspection and palpation of the abdomen revealed no abnormalities. Inspection of the external genitalia revealed absence of the perineum with a definite inverted V cleft involving the anus, rectum ancl vagina for approsimatell\ 4 cm. into the vagina. This deformity similated a-hat Denis Browne cJass&etJ as a shotgun perineum. Urine appeared intermittently. On separating the Jabia a small, atrophic uterine cervix was seen which was without an anterior vaginal waJ1; in pIace of this wall there was a beefy, red tissue which slightly protrudecl postpubically. From the posterior angJes of this tissue at approximately 5 and 7 o’clock there were small apertures (ureteral orifices) from which cJear fluid (urine) was intermittently ejaculated. There was no semblance of a urethra or a previous structure present. (Fig. I.) The physica findings similated what one wouJd expect to find as the result of complete tears associated with parturition. As previously stated, the patient had never been pregnant, nor was there a history of injury or undue trauma to the parts involved. Therefore this condition can be classified only as a congenital anorectourogenital cloaca. The compJete blood count was within normal limits; the hemoglobin was 12 gm. per cent and the hematocrit 33 per cent. The serologic test for syphilis was positive. Urea nitrogen was 18 mg. per IOO cc. Admission urinalysis revealed no abnormalities. On November 17th a Dosteroanterior roentgenogram of the chest with the patient erect showed several old calcific opacities near the 1953 (I am familiar

1031

CIoaca

hiJum of both lungs. The heart appeared normal in size, shape and position. On November zoth, the patient was started on a regimen of flo-ciJlin,@ 2 cc. intramuscularly twice daily. SulfathaIidine@ therapy, 2 gm. four times a da\-, was initiated on November

FIG. I. Congenit:lI anorectourogenit;ll cloac:~. 25, 1953. The patient was given a transfusion of 500 cc. of whole blood on November 27, 1953 and on the same day the administration of I gm. intramuscularly clihydrostreptomycin, claily, was begun. The patient underwent surgery on November 30th. After the usual right paramedian abdomina1 incision, the patient was placed in the Trendelenburg position, and the small bowel and sigmoid coJon were placed in the upper part of the abdomen and heJd there with steriJe gauze Japarotomy sponges. The ureters were then identified Jow in the peJvis where they passed through the broad Jigaments. The posterior parieta1 peritoneum with the ureters incorporated was grasped with a pair of Allis forceps near the broad ligaments. Longitudinal incisions were made in the posterior parietal peritoneum I to 1.5 cm. medial and lateral to the ureters near the base of the broad ligaments. The ureter was elevatecl by passing a pair of curved KelJy forceps posterior to the ureter at the lowermost angles of the incisions in the peritoneum. After the Kelly forceps passed through both points of the incised peritoneum, the strip of peritoneum overlying the ureters was incised transversely at the lowermost angle. The ureter was dis-

WoIf sected into the broad ligament, at which point the ureter was grasped with a pair of Kocher forceps and Iigated with No. z chromic catgut. The ureter was incised transverseIy just proximal to the point where it had been grasped with the Kocher forceps. The proximal end of the

FIG.

2.

Exposure

to 24 cm. from the iIeoceca1 vaIve, was isoIated to construct an artificia1 bIadder employing the author’s modification of the Bricker procedure. After the desired loop of ileum had been acquired between intestina1 clamps, the con20

of the right ureter.

ureter with the overlying peritoneum was picked up with a pair of AIIis forceps in order to eIiminate Ieakage of urine and to avoid trauma to the termina1 end of the severed ureter unti1 No. 8 uretera catheters couId be passed folIowing eIongation 8 to IO cm. upward of the incisions in the posterior parieta1 peritoneum on both sides of the ureters. In this wav it was DossibIe to eIevate the ureters with the aitached peritoneum without disturbing the circuIation and innervation. The posterior parieta1 peritoneum was cIosed with No. oooo atraumatic chromic catgut, beginning at the Iowermost point of the broad Iigament and continuing upward to enclose the ureter compIeteIy within the strio of Deritoneum: its attachment to the peritoneum was never disturbed. (Figs. 2 and 3.) After both ureters had been deaIt with in this manner, the gauze Dacks were removed from the abdomina1 cavity. The terminal iIeum was isoIated and a section of the bowe1 measuring approximateIy 20 cm. in Iength, as suggested by Rieger and Harris [T], and approximateIy I

FIG. 3. A, elevation of ureter and attached peritoneum. B. closure of varietal Deritoneum with encIosure of the uieter. Allis forceps s’hould grasp the ureter with the peritoneum.

tinuity of the iIeum was re-established with a round Murphy button. A doubIe barre1 gun effect was estabIished in the proxima1 end of the isolated Ioop of iIeum by pIacing a mattress suture of No. o black siIk from the convex (free) surface to the mesenteric attachment approximateIy $5 cm. from the free edge. The urothelium (MaIuf [6]) of the right ureter was sutured to the mucosa of the anterior opening and the Ieft ureter to the posterior opening, in the doubIe barrel created in the isoIated Ioop, with interrupted No. oooo atraumatic chromic catgut. The attached ureters were invaginated into the proxima1 end of the isoIated loop of ileum with interrupted sutures of No. o bIack silk apptied to the serosa of the bowel and to the peritoneum encIosing the ureters. The uretera catheters were Ieft in the ureters and pIaced through the

1

1032

CongitaI

AnorectourogenitaI

isoIated Ioop of the iIeum before impIantation of the ureters. (Fig. 4.) The distal end of the isoIated loop of iIeum was brought out to the skin surface through a smaI1 incision at McBurney’s point. The mucosa was sutured to the skin edge with No. oooo atraumatic

Cloaca

cation with bismuth subsaIicyIate administered intramuscuIarIy. On December 4th the patient had some abdominal distention for which a Levin tube was passed through her nose to the stomach. A portabIe x-ray fiIm of the chest, taken on the

FIG. 4. A, gun barrel cIosure of proximal iIea1 Ioop with attachment of ureters. B, invagination of ureters into iIea1 Ioop (faIse bladder).

chromic catgut. The uretera catheters were brought to the surface with the isolated Ioop of ileum and a 22 F. Bardex 5 cc. bag catheter was pIaced through the externa1 ostium into the artificia1 bIadder to care for any leakage around the uretera catheters. The serosa of the ileal Ioop was attached with interrupted sutures of No. o bIack silk to the peritoneum at its exit from the peritonea1 cavity and to the pelvic floor. The opening in the mesentery was closed in Iike manner and the peritoneal covering of the ureters were sutured to the posterior parieta1 peritoneum, leaving no apertures for a loop of bowe1 to enter and eIiminating the possibility of an incarceration and stranguIation. (Fig. 5.) The abdomen was cIosed in Iayers without drainage and the patient was taken from the operating room in good condition. During the postoperative course the catheters drained we11 with satisfactory output. UreteraI catheters were removed after fortyeight hours. On December 2nd the urea nitrogen was 63 mg. per IOO cc.; carbon dioxide was 35 VQIumes per cent. On December 3rd retrograde pyeloureterogram reveaIed the upper urinary tract to be within normal Iimits. Heavy metal deposits were noted in both buttocks. (Fig. 6A.) This was the resuIt of previous anti-syphilitic medi1033

FIG. 5. DistaI end of iIea1 loop attached with indwelling catheters.

to skin margin

same day, was not suffIcientIy satisfactory for interpretation. A portabIe excretory urogram revealed a dense, spheric opaque shadow 34 mm. in diameter in the right iIiac fossa, apparentIy the anastomosing Murphy button. The Levin tube could be seen in the stomach. The kidneys were not satisfactoriIy visuaIized, although there was a good concentration of the opaque materia1 in the artificia1 bladder ten minutes after intravenous administration. The opaque heavy meta previousIy mentioned, was present in both buttocks. (Fig. 6B.) On December 5th there was no improvement in abdomina1 distention. Blood chemistry showed mild azotemia. Late in the afternoon the patient was returned to surgery for expIoration to determine whether or not there was a leak at the point of intestinal anastomosis and/or the uretera implants. The patient died, supposedly from cardiac arrest, before compIete surgical anesthesia was obtained. In spite of morbidity, the abdomen was opened. The peritonea1 cavity appeared dry and cIean; there was no evidence of Ieakage, aIthough at the point of anastomosis of the iIeum the bowel appeared thinned over the Murphy button. The autopsy report was as foIIows: The congenital defect was as stated heretofore; Ieft

6A

6B

FIG. 6A. Retrograde pyeIoureterogram taken on December 3, 1953, reveals the upper urinary tract to be within normal limits. Heavy metal deposits can be seen in both buttocks.

FIG. 6B. Portable excretory urogram taken on December 4, 1953, revenIs a dense, spheric, opaque shadow 34 mm. in diameter in the right iliac fossa. The kidneys

ventricuIar hypertrophy; puImonary edema; biIatera1 pyelonephritis; jaundice; transpIanted ureters as described; anastomosis of the proxima1 and terminal ends of the ileum over a Murphy button (the latter appeared too Iarge for the bowe1 Iumen and the bowe1 wall appeared partIS necrotic). h&croscoprc sections confirmed the autopsy findings. Thus the exact cause of death during surgery cannot be determined. The pyeIonephritis was suppurative. This no doubt caused severe toxemia, if not septicemia, which wouId probabIy account for the jaundice. It is probable that this would have caused death even in the absence of surgery. The role of the surgery in precipitating the pyeIonephritis is diffIcuIt to assess. It is probabIe that the patient had had chronic pyeIonephritis preceding the surgery. However, one might have anticipated that the antibiotics used to prepare the bowel would have covered the infection. Apparently this was not the case. Perhaps this was due to a resistant organism which was invotved. It is probable that if the patient had 1034

are not satisfactorily visualized although there is good concentration of the opaque material in the artificial btadder ten minutes after intravenous administration. Note opaque heavy metal in both buttocks.

survived another few days, the bon-e1 would have perforated at the point of anastomosis due to necrosis of the waI1 because of anoxia caused by the pressure of the too Iarge Murphy button CONCLUSIONS

I am fulIy convinced that the ureters can be incorporated in the posterior parieta1 peritoneum to which they are attached, with preservation of their bIood suppIy and innervation when they are exposed for ureterointestina impIantation. This applies to either the coIon or a Ioop of bowe1 that is sectioned to be used as an artificial bladder. The peritonea1 covering of the freed ureter suppIies adventitia shouId the operating surgeon desire to invaginate the impIanted ureter. There is much Iess IikeIihood of a needIe entering the lumen of a norma ureter. Aunis and WeIIs [I] report the implantation of the Ieft ureter in the proxima1 end of a Ioop of ileum and the right ureter in the IateraI waI1.

CongitaI

Anorectourogenital

Azotemia does occur even though 20 cm. (Rieger and Harris) or Iess of smaI1 bowel are used to construct an artificial bladder. Jaundice in this patient was detected during postmortem examination. The scIerae should be inspected as a routine measure regardIess of the patient’s race or color.

Cloaca

made to expIore the abdomen the original operation.

five days after

Acknowledgment: I wish to express rn!. appreciation to the members of the Medical Art Department of Louisiana State Universit) for their generous cooperation and time spent in the preparation of the drawings and photographs.

SUMMARY

A report is presented of an only case of agenesis of the urethra, trigone and anorectovagina1 waIIs (shotgun perineum and an urogenital cloaca (congenital)). AIso presented is a new procedure for exposing the ureters without disturbing their bIood supply and innervation by not detaching them from the posterior parietal peritoneum. A modification of the Bricker technic of surgicaIIy making an artificial bIadder from a loop of iIeum by implanting the ureters in a double barrel effected in the proxima1 end of the freed Ioop of ileum has been empIoyed. Death occurred before an attempt couId be

REFERENCES I. AUNIS, H. and WELLS. Brit. J. Surg., 42: 173, 290, ‘954. 2. BKICKNER, E. Symposium on clinicat surgery; bladder substitution after oeIvic evisceration. S. Clin. North America, 30: 131 I, 1950. 3. BROWNE, D. Some congenita1 deformities of rectum, anus, vagina and urethra. Hunterian lectures. Ann. Rov. Cd. .%raeons. 8: 173-192. 105 I. 4. EMMET. J.“Obst., 5: 14;/, 18;2-1873. _ ” 3. FRAENKEL. West. J. Surg., 43: 8, 429, 1935. 6. MALUF. Surg., Gynec. c~ Obst., 100: I, 59, 1955. 7. RIEGER, I. T. and HARRIS, J. Experimental study on reduction of urctera1 rel%ux. Surf., Gynec. ~$7Obst., 96: 93, 1953. 8. SKENE. Wm. Wood & Cn. 1887.

RUCKENSTEINER’S studies in Germany have convinced him that radioactive iodine alone may be vaIuable in onIy approximateIy IO per cent of cases of thyroid maIignancy but that surgery and/or x-ray therapy is also needed in the vast majority of cases. In short, radioactive iodine is a heIpfu1 suppIementary procedure but is not a substitute for surgery or radiation. In fact the author positiveIy states that radioactive iodine effects onIy a paIIiative improvement but not a cure in cases of thyroid maIignancy. Hence he recommends its use in inoperabIe cases or postoperativeIy to try to reduce the rate of recurrences in thyroidectomy for cancer of this organ. (Richard A. Leonardo, M.D.)

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