Cloaca1 Malformations:
Experience
With 105 Cases
By W. Hardy Hendren Boston, Massachusetts l A personal experience with 105 cloaca1 malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 88 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloaca1 spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function. Copyright 8 1992 by W.B. Saunders Company INDEX WORDS: Cloaca1 malformations; imperforate posterior sagittal anorectoplasty; vaginoplasty.
anus;
C
LOACA, which is the Latin word for sewer, refers to that spectrum of pelvic malformations in female patients in which there is confluence of the urinary, genital, and gastrointestinal tracts. Although this is normal in birds, reptiles, and some fish, it can be disastrous in the human if not properly managed. Since our report of 64 cases in 1986,’ 41 more patients have been seen, giving an overall experience to date with evaluation and/or management of 105 cases. This paper will review results from the entire experience with 105 cases and will present 6 selected cases from among the 41 recent ones that illustrate the breadth of the cloaca1 spectrum. MATERIALS AND METHODS Records were reviewed of all 105 patients. There were 65 primary cases in which no reconstruction of the cloaca had been attempted before referral. There were 40 secondary cases. In most of those a rectal pull-through had been done previously without repairing the other aspects of the cloaca1 anomaly. Follow-up included 98 of the 105 patients. Seven patients were
excluded for the following reasons: 4 died preoperatively (dysplastic kidneys, dehydration, congenital heart disease, and hypematremia); 1 was seen in consultation in 1976, but disappeared; and 2 patients are preoperative, having had major urologic procedures performed and their cloacas will be repaired in the near future. Twenty patients in the series were from Massachusetts; the other 85 came from 12 foreign countries and 24 other states. Follow-up included personal examination or telephone contact with families and patients. The external appearance of the perineum varied widely (Figs 1 and 2). Similarly, there was a spectrum in the anatomy of the urogenital sinus (Fig 3). Vaginal anatomy was especially variable (Fig 4). In 12 patients there was no vagina; 42 had 2 vaginas; 51 patients had 1 vagina. Vaginal reconstruction required use of a bowel segment as an extention or substitute in 29 patients. Rectal anatomy varied as well (Fig 5). Twenty-seven patients had a urinary diversion when referred, which underscores the serious nature of urologic complications that occur in girls with cloacas. These included: 17 vesicostomy, 6 ureterostomy, and 4 ileal loop urinary diversions. The vagina commonly fills with urine in high cloaca1 malformations, pushing the bladder forward, thereby obstructing its emptying as well as drainage of the ureters (Fig 6). This can often be managed by intermittent catheterization of the vagina, using an 8F or 10F plastic catheter or a small metal catheterizing cannula. In retrospect, some of the secondary, previously diverted cases, could probably have been managed by prolonged catheterization instead of diversion. Fifty-seven patients had significant vesicoureteral reflux; most required ureteral reimplantation. In some severe cases a urologic operation was required before correcting the cloaca1 malformation, because obstruction or massive reflux were life-threatening and took precedence over the more elective aspects of anoplasty and vaginoplasty. In other cases in which reflux or obstruction were less severe it was corrected either at the time of the cloaca1 repair or later. Many other malformations were encountered. In the skeletal system they included: hemivertebra, short sacrum, absent sacrum, sacrococcygeal teratoma, presacral cyst, and absent leg. In the gastrointestinal tract they included: esophageal atresia, duodenal atresia, ileal atresia, gastroesophageal reflux, bowel duplication, Meckels diverticulum, and neuronal dysplasia of the colon. In the urinary tract they included: crossed renal ectopia, horseshoe kidney, ectopic ureter, ureteropelvic junction obstruction, dysplastic kidney, megaureter, ureterocele, and the prune-belly syndrome in two girls. Miscellaneous abnormalities included: congenital heart disease, aortoiliac vascular malformation, and tethering of the spinal cord.
Position for Operation From the Department of Sutgev, Children’s Hospital, Boston, MA. Presented at the 38th Annual International Congress of the British Association of Paediahic Surgeons, Budapest, Hungary. July 24-26, 1991. Address reprint requests to W. Hardy Hendren, MD, Department of Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. Copyright 0 I992 by W B. Saunders Company 0022-3468/92/2707-0027$03.00/O
890
In the first 35 cases prior to 19822-4cloacas were reconstructed using combined abdominal-perineal exposure with the legs sterilely prepped to allow lifting them into lithotomy position as needed. Since 1982 when Petia and DeVries described using the posterior sagittal approach for anorectoplasty,5s6 we incorporated it into the repair of cloaca1 malformations. In 43 primary cloaca1 reconstructions since 1982 the operation was completed using just the posterior sagittal approach in 12 patients. In 17 patients the
JournalofPediatric
Surgery, Vol27, No 7 (July), 1992: pp 890-901
REPAIR OF CLOACAL
MALFORMATIONS
891
augmentation in 1. Three patients have stress incontinence, but are currently content to live with it. Twenty-two patients empty their bladders by intermittent self-catheterization (ISC) and 18 are completely dry. Sixty void spontaneously with satisfactory control and are dry; in some the ability to void and be dry was present early, but in others that evolved slowly. Six patients are too recently operated to evaluate their urinary control. Four patients have urinary diversions, each with no bladder to reconstruct and each done prior to the era of continent diversion; this will be offered to each of them in the future.
Fig 1. The spectrum of enatomy in cloaca1 malformations from minimal to severe. (A) A 15.year-old girl with form fruste of cloaca1 malformation. Anus is further forward than normal, just behind urogenital sinus opening which had been previously cut back to enlarge it. (6) A Syear-old girl wlth anus even further forward, separated from the urogenital sinus by a thin urorectal septum. (C) A 4-year-old girl with one perineal opening, the urogenitel sinus, into which emptied the colon, vagina, and bladder. (D) A lg-month-old girl with more advanced cloaca1 anomaly. No opening evident in perineum. The urogenital sinus was located at the base of the phallic structure, which is surrounded by primitive labia minora.
operation began in the posterior sagittal position, but required turning the patient into the supine position as well to work in the abdomen. In 7 the reverse was done, commencing in the abdomen and turning prone to work from behind later. In 7 patients operation was performed through the belly and perineum, not using the posterior sagittal position at all. Similarly, the approach used in secondary cases varied according to what needed to be done. Therefore, circumferential prepping and draping of the patient as described previously was used routinely.’ RESULTS
Results are presented for 98 of 105 patients. Urinary Control Constant urinary wetting was seldom a problem, and was seen in only 3 patients. Further surgery for incontinence is planned for them, including bladder
Fig 2. Variations in the appearance of the perineum. (A) Age 1 year. Hemartome of perineum (arrow) and extensive capillary hemengioma. This patient had a wide urogenital sinus opening, at the top of which were three vertically ellgned ostia, the bladder, vagina, end rectum. (6) Neonate. Lipoma of perineum (arrow) and asymmetrical labia majora. The urogenital sinus opened et the base of the large phallic structure. (C) Age 6 months. Large penis-like phellic structure and single opening at its base. Note rounded perineum without intergluteal cleft. This typically indicates poor innervation of muscles and bodes poor bowel control. (D) Age 6 months. Prominent labia majora and perineal raphe. Patient had a high cloecal confluence, with two small vaginas that were extended to perineum with bowel segment. She also required ureteral reimplantetions and bilateral pyeloplesties.
W. HARDY HENDREN
UG SINUS
LONG UGS ENDING IN TIP OF CLITORIS
SUBCLITORAL MEATUS
Fig 5.
WIDE OPENING (Like a vagina) Fig 3.
WITH ACCESSORY TRACT
The spectrum of urogenital sinus anatomy. (A) Long urogen-
ital sinus opening at tip of clitoris. This is usually associated with high confluence of bladder, vagina, and rectum. A l-cm cut-back procedure can facilitate endoscopy and subsequent intermittent catheterization. The urogenital sinus should be preserved to serve as urethra after separating from it the rectum and vagina. Patient shown in Fig 1D had this type of urogenital sinus. (6) Urogenital sinus opening more posteriorly. (C) Wide urogenital sinus opening. Patient shown in Fig 1C had this type of urogenital sinus. (D) Accessory tract from urogenital sinus to phallus. The patient shown in Fig 18 had this type of anatomy. In repairing cloaca1 malformations if the urogenital sinus is not wide, it should be preserved to serve as the urethra. If it is wide it should be reduced in caliber. An accessory tract is not useful and should be marsupialized.
The spectrum of rectal anatomy.
patients use a soapsuds enema washout every day or every other day and are free from fecal soiling on that regime. Seven patients have a colostomy; 4 were performed because there was no pelvic muscle complex, making anorectoplasty inadvisable. Two colostomies were performed elsewhere because bowel control was imperfect after cloaca1 repair and the parents were unwilling to use an enema washout program. One patient has a temporary colostomy that will be closed in the future. Six patients were operated on too recently to assess. Seven patients have some fecal soiling and do not use enema washouts to control the problem. There were 4 patients with anterior position of the anus, but not a true cloaca, in whom repair of the urogenital sinus-vagina defect did not require moving the rectum. They started with normal bowel control and maintained it. Predictors of good bowel control were a good perineal raphe, a well-defined anal dimple, a normal sacrum and spine, a normal magnetic resonance imaging (MRI) examination, and a brisk muscle reflex during electrical stimulation.
Bowel Control Wide orifices
Forty-seven patients have normal bowel habits. Some needed a training period with enema washouts to learn bowel control. Others did not. Twenty-seven A catheter or
ED
Fig 4.
The spectrum of vaginal anatomy.
Fig 6. To show the mechanism of bladder outlet obstruction caused by the vagina distended with urine. Intermittent catheterlzation will usually enter the vagina, not the bladder which is displaced forward. Nevertheless, that can be effective in decomprassing the urinary tract.
REPAIR OF CLOACAL MALFORMATIONS
Poor bowel control correlated with a flat or rounded perineum and sacral and spinal defects.
893
A
SP tube (since bwth) Normal
ureters
Sexual Function Eight patients are now of adult age and report satisfactory coitus. Two have conceived and delivered infants by cesarean section. Two patients developed hematometrasalpix at puberty in one of two uteri and were treated by unilateral hysterosalpingectomy. It is likely that this will be seen in more patients who mature in the future who have a rudimentary uterus that has been joined to a bowel segment. Remarkably, two patients married for 4 and 6 years, respectively, were not having intercourse. In one case, the husband was reluctant to make the attempt. Counseling is planned for this couple. The second patient, recently examined, needs to have her introitus enlarged. Further, her husband has hypospadias in need of repair. Appropriate procedures are planned for this couple. SELECTED CASE REPORTS The following 6 cases from the 41 managed since 1986 will illustrate the wide range of problems the surgeon must solve in dealing with cloaca1 malformations.
Wide UGS
Cystic sacrococcygeal
teratoma
Ma& permeal body Pull through splemc flexure. enclosed in muscle complex
Removed colosfomy. ulled through proxinal limb. segment wwld not reach rrneum (short vessels).
Case 1 A 15month-old girl was referred in 1986 with a cloaca1 malformation and a large cystic sacrococcygeal teratoma, principally within the pelvis (Fig 7A). A suprapubic tube had drained the bladder since birth. There was a loop colostomy in the left lower quadrant. Aortogram done previously showed splaying of the iliac vessels over the teratoma (Fig 8A). Preoperative radiographic study showed high confluence of the bladder, vagina, and rectum, emptying to the perineum through a wide urogenital sinus (Fig 8B). Under anesthesia electrical stimulation of the perineum showed brisk muscle contraction of the perineum at the site for the anus (Fig 9). Operation began in the prone, jackknifed position for posterior sagittal mobilization of the tumor, including excision of the coccyx. She was then turned to enter the abdomen to complete mobilization of the tumor, after which the cloaca1 anatomy was repaired. The patient could not void postoperatively and a catheter could not be passed from below. Therefore, 2% weeks later endoscopy was performed, incising a shelf of mucosa at the bladder neck which prevented passing of the catheter. Now age 5% years she has no spontaneous voiding, but she can tell when the bladder is full. It is emptied 5 times daily by catheterization. She does not move her bowels spontaneously. Enema washout of the colon daily gives satisfactory bowel emptying, and there is no soiling on that program. The continuing need for catheterization and enema washouts may represent denervation of the muscle complex secondary to the extensive dissection to remove the teratoma. Aside from enemas and catheterization, she is a normal little girl. Comment. The cloaca1 anatomy in this case was straightforward to repair. However, the need to remove a large teratoma simultaneously made this a long (15 hours) and complex operation. This undertaking could not be staged and required multiple position changes to work alternately through the abdomen and the posterior sagittal approach. The previous low loop colostomy complicated the repair because the lower colon was tethered
and teratoma
Fig 7.
Anatomy in case 1 (A) before and (B) after reconstruction.
upward and could only be brought down by taking down the colostomy. Therefore, the safest option was discarding the segment of colon distal to the colostomy, and pulling through the bowel proximal to the colostomy. A right transverse colostomy at birth would have allowed pull-through of the distal colon without sacrificing that segment. A pull-through procedure with a colostomy closure just above it would not have been safe. unless a
Fig 8. Roentgenograms in case 1. (A) Preoperative aortograms showing displacement of iliac vessels by large intrepelvic teratoma. (6) Preoperative study by simultaneous injection of contrast into the bladder (B) and the rectum (R) through the distal limb of the colostomy, filling the vagina (V) and the urogenital sinus below it.
W. HARDY HENDREN
Dysblastic L. kidney
Fig 9. Photographs of perineum in case 1 preoperatively. (A) Perineum in resting state. Note barely visible surface component of sacrococcygeal teratoma (arrow). (B) Electrical stimulation of perineum under anesthesia noting excellent contraction of muscle complex at future location for anus. Relaxant agents should be avoided lest this reflex be inhibited.
second complementary colostomy were performed. A right transverse colostomy would have avoided this problem.
Cutback
UGS
/
Urethral sphincter
Colostomy
2 layer closure of UGS hrectomy
Case 2 A 4-month-old girl was referred in 1990 (Fig 10). Endoscopic and radiographic evaluation showed a small urogenital sinus opening at the tip of the phallus, apparent absence of a vagina, and enormous dilatation of the rectum (Fig 11). There were several small pit-like defects in the mucosa of the urogenital sinus below the bladder neck, but none would admit a small ureteral catheter, ie, there was no visible entry to a vagina. When the rectum was filled with irrigating solution, the bladder trigone was displaced markedly forward. It would fall back into normal position on emptying the rectum. Operation began in the prone position through posterior sagittal exposure. The urogenital sinus was cut back about 15 mm, to a location appropriate for a normal urethral meatus. With a sound in the urogenital sinus, it was opened near its upper end to locate the rectal fistula. With a Foley catheter in the rectum, and another in the bladder, the two were separated. The entry of the rectum was high, at the level of the coccyx, and was impossible to mobilize completely from the posterior sagittal position. A small vagina was found on the right side filled with mucous. None was found on the left. the abdomen. There was The oatient was then turned to exDlore r-m-~ ~~~~ ~~~~~~~ ~~~-~-a normal tube, ovary, and small uterus on each side, wide apart, against each pelvic wall. On the right the uterus communicated with the vagina which had been previously mobilized from below. On the left there was only a fibrous cord below the uterus. Further, sections of the uterus disclosed no lumen. Therefore, it was removed. Probing the right side disclosed a patent cervix and endometrial cavity. By mobilizing the rectum further through the abdominal approach, it was possible to bring it to the perineum. Its large caliber made it more appropriate to use it as a vaginal extension than tapering it to become the rectum. The dysplastic left kidney was removed. The patient was turned prone once again, closing the urogenital sinus opening, and pulling down the rectum as a vagina. Once again in the supine position, the legs were picked up into lithotomy position to complete suturing the colon segment to the perineum. An opening was made in the anterior wall of this rectal segment, joining to it the right vagina, spatulating the anastomosis to make it as large as possible. The rectum was divided
17 mos
Fig 10. (A) Preoperative and (B) postoperative anatomy in case 2. Note absent vagina on left side and “occult” vagina on right side.
1 in above that, leaving a long enough segment of colon to have adequate depth to serve as a vagina. The next segment of colon above was removed, preserving its mesentery. The colon was divided above the sigmoid vessels to pull through the next higher segment based on left colic vessels. This segment was pulled through, to be dealt with later. The abdomen was closed. The patient was turned once again into the prone position, constructing a perineal body, and placing the rectum appropriately in the muscle complex. The rectum was tapered to the caliber of a size 10 Hegar dilator. The muscle complex was closed around it. The operation lasted 16 hours. The patient spent 2 days in the intensive
1
Fig 11. Preoperative roentgenograms in case 2. (A) Anteroposterior and (B) lateral views. Note bladder (B) displaced forward by dilated rectum (R).
REPAIR OF CLOACAL MALFORMATIONS
895
care unit and recovered uneventfully. Three months later neurosurgical release of the spinal cord was performed by Dr Michael Scott. Colostomy closure was performed 1 week later. The patient is now 6 months following cloaca1 reconstruction. She voids and stools intermittently, but it is too early to assess the degree of control of these functions. Commenr. This case illustrates the “occult” vagina, ie, a vagina was present but could not be seen endoscopically or by preoperative radiographs or ultrasound examination. The close proximity of the rectum to the bladder on preoperative radiographs (Fig 11) had been interpreted as absence of a vagina. However, this is not a valid radiographic finding if vaginas are duplicated and diverge laterally. When there is a blindly ending uterus as was seen on the left side, it is probably best removed. We have encountered hematometrium at puberty in two cloaca1 cases in which evidently there was no uterine canal to allow menstruation at puberty. In this particular case no uterine cavity was observed on serially sectioning the left uterus before its removal.
Case 3 In 1988 a girl was referred at the age of 2 months. Transverse colostomy had been performed at birth (Fig 12). There was a wide urogenital sinus opening. At its apex the bladder neck entered anteriorly, with no segment of urethra between the bladder neck and urogenital sinus. The ureteral orifices were close to the bladder neck. Two vaginal openings lay side by side with a septum between them. The vaginas were not large. The rectal fistula entered the septum anteriorly, not in the usual position at its base. The septum Ecfopic ureters I” bladder neck (reflux ,nto both) Normal
was incised to create one vaginal cavity. Intermittent catheterization was not needed because there was free drainage of urine from the urogenital sinus. At age 2 years cloaca1 reconstruction was performed. There were only two openings in the urogenital sinus, namely the bladder neck and the vagina just behind it. The rectum entered the septum between the vaginas at the point at which they diverged. By splitting the vaginas from behind, the rectum was visualized and mobilized up to the cul-de-sac peritoneum, actually detaching the cul-de-sac peritoneum from the rectum and dividing the sigmoid vessels to gain length. The two vaginas were then sewn together, to create a single one, and rotated in order to have intact vaginal wall over the urogenital sinus closure. Two skin flaps of labia majora tissue were created and rotated into the posterior vagina wall to avoid tension in bringing down the vaginas. Five months later the ureters were reimplanted for reflux and the colostomy was closed. Now 14 months following reconstruction, and age 3 years, the patient has urinary control and bowel control that her parents judge to be normal. Comment. The variable anatomy in cloacas is well illustrated by this case. For example, the bladder neck emptied almost directly into the urogenital sinus, and two refluxing ureters entered the bladder neck. The vaginal anatomy is of interest, because the two vaginas were not completely separate, as seen in some cases, nor were they side by side for their full length which is the most common type of vaginal duplication. Diverging vaginas can allow anterior displacement of the rectum. The very high position of the rectal pouch was initially thought to require laparotomy. By circumscribing the cul-de-sac peritoneum and dividing the rectosigmoid mesentery enough length was gained to bring down the colon. This case also illustrates the use of perineal flaps. When a vagina will not reach the perineum despite wide mobilization and the gap to be bridged is short, perineal flaps can be created to lay up into the vagina. There is usually ample labial scrotal tissue to do that. Local flaps should be based anteriorly and rotated upward. When the vagina is small or the gap to be bridged great, it is better to extend the vagina with a bowel segment. When two vaginas he side by side for their full length they can be converted into one by incising the common wall between them. When they are separate, or diverge as in this case, they must be anastomosed together to create a single vaginal passage. When the vaginas do not occupy the midline, the rectum may be positioned more forward than usual.
Case 4 Sacrum OK
MRI OK
B
Rsdum puffed thnqfh andencloaadinmusde
Fig 12.
(A) Preoperative
and (B) postoperative
anatomy in case 3.
An l&month-old girl was referred in 1988. At age 2 days abdominal exploration had been performed, inserting a suprapubic tube into a fluid-filled structure in the pelvis, thought to be the bladder, but it was the vagina. She did not do well and at age 5 weeks underwent reoperation. The bladder was exteriorized as a vesicostomy in the midline above the location of the previous tube vaginostomy. Left end colostomy was also performed, with turn in of the distal colon. It was noted that most of the urinary drainage came from the vaginostomy tube and that urine passed intermittently via the anus. Endoscopic and radiographic evaluation disclosed the anatomy depicted in Fig 13A. There was excellent contractility of the muscle complex on stimulating the perineum electrically, but the anal opening in the perineum was behind the point of maximum contractility. Endoscopy of the vagina showed the fistula in the rectum, and the endoscope could be passed through it. The urogenital sinus was strictured. Dilatation was performed, leaving a catheter stent in place. Reconstruction was performed 4 months later (Fig 13B). Ureteral catheters were placed through the rectal fistula, the vesicostomy, and the vaginostomy to aid in finding each of these passages during operation. The procedure began from above, in the supine position, to liberate the
896
W. HARDY HENDREN
sphincter Incomplete
/ sacrum
during infancy. However, it appeared that the urogenital sinus was tethered across the pubis by the bladder’s being pulled upward by the vesicostomy. We have seen this in another patient in whom vesicostomy had been performed; the bladder had been pulled up so tightly that a ureteral catheter could not be passed up the urogenital sinus. However, when the vesicostomy was taken down, allowing the bladder to descend, the caliber of the urogenital sinus became normal. The presence of a vesicostomy and a vaginostomy greatly complicated this child’s management and caused hydronephrosis which led to reimplantation of the right ureter. Intermittent catheterization from below during infancy might have prevented these complications. Another cloaca1 case had persisting sigmoid dilatation and was treated by sigmoid resection, which cured her constipation. Microscopic examination showed neuronal dysplasia of the colon.
\ Tethered
cord
POSITIONS: supine
a
prone
3
supine
B Vesicostomy
Ureter reimplanted
Excision
stricture
Vagina pulled through (former stoma) 4 months Excised
later
Case 5 A girl was referred at birth in 1987 with imperforate anus, abdominal distention, and a funnel-shaped appearance to her vaginal introitus (Fig 14). No meconium had passed. Cystogram showed several diverticula arising from the bladder, shedding little light on what the anatomy might be. Endoscopy was performed before laparotomy to relieve bowel obstruction. There was a wide urogenital sinus with no structures entering it. There were multiple openings in the bladder including a patent urachus. Real atresia was found at laparotomy; below it there was a gap in the mesentery and a short segment of colon in the lower abdomen. The upper end of the colon was blind and the lower end connected with the
fistula
Rectum tapered
and
LATERAL VIEW
moved forward into muscle complex
Fig 13.
(A) Preoperative
and (8) postoperative
anatomy in case 4.
which were tethered upward. The right ureter was imbedded in scar surrounding the vaginostomy and required mobilization. The patient was then turned prone. The vagina was separated from the urogenital sinus, which was strictured. The etiology of the stricture appeared to be compression by the pubis because the bladder was pulled upward by the vesicostomy. Resection and reanastomosis of the urogenital sinus was performed. The rectovaginal fistula was divided. The part of the vagina that had been the stoma was brought to the perineum. This was possible because it had been mobilized from above. The rectum was tapered and placed within the muscle complex. The patient was then turned supine to reimplant the right ureter and place a suprapubic tube in the bladder. Endoscopy 2 weeks later showed satisfactory healing. A new urethral catheter was placed to allow further healing. MRI examination showed slight tethering of the spinal cord, but neurosurgical consultation felt that an untethering operation was not needed at that time. Three months later colostomy closure was performed. The patient voided well and intermittent catheterization was discontinued. During the next year constipation was a problem and was relieved by enema washouts. The anal opening became slightly stenotic and was enlarged by 2 z-plasties. Now age 4 years, the rectosigmoid remains dilated. Sigmoid resection will be performed. Comment. This is the only case we have seen with an H-fistula from the rectum to the vagina. Furthermore, posterior anal ectopia is extremely rare. When the rectal opening is ectopic, it nearly always lies forward of the point of maximum contractility of the sphincter complex. The cause for the urogenital sinus stricture may have been created by traumatic catheterization or endoscopy vagina and bladder
.-
Positions:
Good anal sph,ncter
supine *
6
prone *
supine
~- a
DivertMum
Fig 14.
(A) Preoperative and (6) postoperative
LATERAL VIEW
anatomy in case 5.
a97
REPAIR OF CLOACAL MALFORMATIONS
bladder. The urachus was closed. The heal atresia was exteriorized. The blindly ending colon was also exteriorized without knowing whether this was the end of bowel that might have been in continuity with the heal atresia or whether it was the end of bowel as usually seen in cloaca1 exstrophy. At age 7 months endoscopy was performed again, but the findings were difficult to decipher. At age 15 months endoscopy was repeated with uroradiologist, Dr Robert Lebowitz, present with a C-arm fluoroscopic unit. As each orifice was visualized, and catheterized, contrast medium was injected, obtaining fluoroscopy and spot films (Fig 15). The anatomy depicted in Fig 14A was derived in that fashion. There were two hemibladders, four ureters, two vaginas entering the bladders, and a bowel opening in the septum between the two hemibladders. There was a diverticulum at the apex of the right hemibladder with communicating openings. Repair was performed (Fig 14B) at age 2 years. After circumferentially preparing and draping the child, a midline laparotomy was performed to dissect out all of the anatomy. Biopsy specimens were taken from both ends of the distal defunctioned bowel segment because it was not possible from the orientation of its mesentery to tell which end was developmentally the distal one, ie, whether the blind end was what might have been in continuity with the ileum, or whether it was the blindly ending gut seen in cloaca1 exstrophy. Interestingly, there were no tinea on the colon. The bladder was opened and it was possible to locate the two vaginas which entered on each side of a midline septum. They were dissected free from the bladder, using the intravesical approach and were sewn together to create a single
vagina. The bowel segment was detached from the bladder. The ureters were reimplanted. The midline septum between the bladders was excised to make a single bladder. The patient was then turned into the prone position to make a posterior sagittal midline incision. The wide urogenital sinus was tapered into a urethra and closed with two layers. The two vaginas were rotated 90” and joined to the perineum. A perineal body was constructed. The appropriate end of the bowel segment was pulled through and enclosed in the muscle complex. The child was then turned supine to complete bladder closure and exteriorize the other end of bowel. The anesthesia time for this global reconstruction was 20 hours. The patient convalesced uneventfully. Endoscopy 2% weeks later showed satisfactory healing. Intermittent catheterization was begun and the suprapubic tube was soon removed. Four months later bowel continuity was restored by resecting the ileostomy and joining it to the defunctioned distal bowel. The patient could not empty her bladder completely and was maintained on intermittent catheterization. Surprisingly, at age 3 years she emptied her bowel 2 to 3 times daily voluntarily and without soiling. A minor degree of rectal prolapse was trimmed as an outpatient procedure. MRI of the spine showed no tethering of her spinal cord. She now voids spontaneously, does not wet, and is catheterized for residual twice daily as a precaution, usually obtaining about 100 mL. Comment. This case is of special interest because the anatomy initially appeared so complex that there seemed little likelihood that reconstruction would achieve continence. Those gloomy prospects proved incorrect. It was vital to thoroughly assess the anatomy both radiographically and endoscopically. This case seems to be a transition form between a usual cloaca and cloaca1 exstrophy, because it included hemibladders, vesicointestinal fissure, and a blindly ending segment of bowel hanging in the pelvis. However, there was no omphalocele or bladder exstrophy.
Case 6
Fii 15. Preoperative roentgenogram in case 5 showing bizarre constellation of structures produced by filling anatomy shown in Fig 14A. Prolonged endoscopic study in conjunction with simultaneous roentgen study with C-arm fluoroscopic unit in operating room was required to decipher the anatomy.
This patient was referred in 1989 at age 10 years (Fig 16A). Colostomy had been done at birth and a rectal pull-through at 1 year of age. The bowel had not been placed properly in the muscle complex; therefore, the patient was incontinent. A second colostomy was performed at age 3 years. Two small vaginas entered the urogenital sinus and there was recurrent urinary infection and dribbling. After consideration of various options a permanent urinary diversion was performed at age 4 years. Cystogram showed a small, elongated bladder, with two small vaginas filling from the urogenital sinus (Fig 17A). The defunctioned rectal segment was filled with old stool. MRI showed no spinal cord tethering. At a separate endoscopic procedure the anatomy was assessed and the defunctioned distal colon was emptied of a large amount of inspissated fecal material. Six months later reconstruction was undertaken (Fig 16B). Endoscopy of the right vagina showed a uterine cervix. The left vagina was smaller; a hole at its apex led to a tube but there was no uterus. The operation began in the prone position, mobilizing the previously pulled through rectum to gain access to the upper urogenital sinus and vaginas at the bladder neck. The right vagina was the better of the two, but was only 2 in long. The left vagina was smaller and no cervix could be seen at its apex. The urogenital sinus was repaired with two layers. The patient was turned. There were extensive intra-abdominal adhesions. There was no uterus on the left side, only a direct connection between the Fallopian tube and the vagina. Therefore, the left vagina and tube were removed, sparing the ovary. The right vagina was anastomosed to the segment of colon which had been previously pulled through. This anastomosis was accomplished from below, turning the patient prone once again. In many cases I have found it easier to do this from above. A perineal body was constructed. The patient was then turned supine once again to deal
898
W. HARDY HENDREN
A
I.
L_~
/
Colon conduit
Prior rectal pull through as infant, too anterior
-rum
Positions:
prone j
supine *
prone *
supine 3 prone
Gastrocystoplasty Psoas hitch
I
R. gastroepiploic pedicle Removed L. vagina
fvfdt OK
tapering, pit in center of muscle
anastomosed to colon
< Fig 16. Complex reoperative case (case 6) wtth colostomy and ureterostomy. (A) Preoperative and (6) postoperative. Note need to augment bladder, using stomach, because it was small after many years of diversion.
the urinary tract. The bladder was small, and smooth walled due to long time diversion. By stretching the bladder manually, it was possible to position it for a psoas hitch on the right side. The urinary conduit was removed and discarded because it was too with
small to use as an augmentation. The right ureter, which was the better of the two, was reimplanted into the bladder with a long tunnel. There was a kink in the right ureter of questionable significance. Therefore, the left-to-right transureteroureterostomy was located at that point to make certain that there was no functional obstruction at that level. A generous wedge of stomach, representing about one third of its volume, was mobilized on the right gastroepiploic pedicle and rotated down to augment the bladder. The colostomy was taken down, mobilizing the splenic flexure. To gain length, it was necessary to divide both the left colic and midcolic vessels and bring the left colon down on the vascular pedicle of right ileocolic vessels. The colon was pulled through to the perineum and fastened temporarily with a single suture to the adjacent perineum. The belly was then closed and the patient was turned for the final time into the prone position. The pulled through rectum was tapered in caliber and placed in the muscle complex. Blood replacement included 4 U of whole blood, 1 U of packed red blood cells, and 8 L of lactated Ringer’s solution during this 24-hour reconstruction. The patient was in excellent condition with a good urinary output at the end of this extensive surgical undertaking. Now 1% years later she is entirely well. She empties the bladder by ISC every 4 hours and is dry and free from urinary infection. The colon is emptied using a saline enema daily and is free from fecal soiling on this program. Postoperative cystogram showed normal bladder volume (Fig 17B). Comment. This case illustrates several important principles. First, colon pull-through should never be performed as an isolated procedure. Second, when pull-through is performed it is best done through the posterior sagittal approach with tapering of the rectum and accurate placement in the muscle complex. This enhances the likelihood of bowel control. Furthermore, correction of the urinary tract should not be “deferred until later.” The urinary diversion greatly complicated the reconstruction. It is tempting to think of a way in which a reconstruction such as this could be done in stages to avoid such an enormous global operation of this length. However, that is not practical. Fortunately, expert anesthesia care with intraoperative monitoring and postoperative intensive care make possible the safe conduct of such a formidable operation. This would have been impossible 2.5 years ago. Great credit is deserved by the anesthesiologists for making this possible. Bladder augmentation was essential in this case. Using stomach, as compared with small bowel or colon, has three advantages: (1) it does not form mucous and secondary stones; (2) it excretes chloride rather than absorbing chloride, which can aggravate acidosis; and (3) it creates an acid pH, which makes urinary infection less likely. DISCUSSION
Fig 17. Roentgenograms in case 6. (A) Preoperative cystogram showing small bladder with two small vaginas filling from it, one on each side. Flecks of calcium are in stool in colon segment defunctioned 6 years before. (B) Postoperative cystogram showing normal capacity of bladder. Patient empties by ISC. Wisp of reflux is in retained left ureterai stump, not a functional ureter.
Cloaca1 anomalies are much more variable in their anatomic form than most other congenital malformations. Nevertheless, certain principles have evolved that can lead to a high rate of success in managing these children. The prognosis should no longer be poor as it was three decades ago.7-10 At birth colostomy should be done. I prefer a divided loop transverse colostomy. This will preserve intact the left colic blood supply in case separate colon segments with their blood supply will be needed in later reconstruction. It is not necessary to do a wide abdominal exploration in these infants to inspect pelvic organs which can be studied later endoscopitally and radiographically. When there is a large bladder or vagina filling the lower abdomen, routine vesicostomy or vaginostomy is not needed. Often the
REPAIR OF CLOACAL
MALFORMATIONS
large viscus can be decompressed by intermittent catheterization. The catheter or catheterizing sound will usually enter the vagina, not the bladder that is displaced forward. If intermittent catheterization does not relieve lower urinary tract obstruction, cystostomy or vaginostomy can be considered. That can complicate later reconstruction by tethering these organs upward. When the urogenital sinus extends to the phallic tip it may prove helpful to cut it back a few millimeters to facilitate catheterizing. Soon, complete anatomic study should be performed. This includes: (1) upper tract evaluation by ultrasonography or intravenous pyelogram; and (2) contrast study of the lower tract by filling the urogenital sinus and the distal limb of the colostomy and any other orifice that may be present such as a vesicostomy or vaginostomy. Results of radiographic evaluation have been recently described.” MRI of the spine is now routine for cloaca patients to rule out tethering of the spinal cord.12 MRI studies were performed on 44 of these patients. Findings were normal in 29 and abnormal in 15. Three of the latter showed a syrinx of the spinal cord; 12 had tethering of the cord below its normal level of the first lumbar vertebra. Six patients have undergone detethering operations to date. The rest are being followed. Vertebral anomalies and missing sacral segments are common in these children and often accompany urological malformations. If there is a life-threatening urologic problem such as massive reflux, megaureter, ureteropelvic junction obstruction, etc, it is best to deal with that early before serious renal damage occurs.13J4 It is most important to emphasize that such conditions should not be ignored while waiting for the baby to grow old enough for a definitive total reconstruction. Furthermore, our experience with secondary cases has provided convincing evidence that the rectal component of a cloaca, the easiest task to repair, should never be operated on first as a separate procedure, leaving the most difficult parts of this malformation, the urogenital sinus and vagina repair, to be done later. Reflux, present in 55% of cases, if not corrected early may feasibly be corrected at the time of cloaca1 repair or later as a separate operation. Thus, the timing of antireflux surgery should depend on the severity of the reflux and whether the kidneys are normal or show scarring. Repair of the primary cloaca is usually done between the ages of 9 and 18 months. Endoscopic evaluation is always carried out several days before cloaca1 reconstruction to become familiar with the anatomy to be reconstructed and to wash out any remaining mucous or old fecal matter in the distal colon. Often, the endoscope can be introduced into
the rectal fistula to irrigate saline and antibiotic solution from below and out the colostomy. It can be helpful to insert an endoscope into the distal colostomy stoma to irrigate to and fro using an Ellyk evacuator to remove remaining fecal material. Cloacal reconstruction should never be undertaken until the bowel is absolutely clean. These operations are long and difficult. An experienced anesthesiologist and full intraoperative monitoring are mandatory. Blood loss and fluid loss can be large and must be replaced appropriately. Unless there is a particular reason to enter the belly first, such as the presence of a vaginostomy or vesicostomy that must be released, the operation usually begins in the prone, jackknife position for posterior sagittal approach. Electrical stimulation of the perineum is mandatory to assess muscle contractility. This precludes use of muscle relaxant anesthesia technique. The special advantages of the posterior sagittal approach include ideal access to separate the three organ systems and visualization of the muscle complex for accurate placement of the rectum within it. It is important to dissect in the exact anatomic midline while approaching the rectum, vagina(s) and urogenital sinus from behind. A sound in the urogenital sinus, lifted upward by an assistant, is a useful maneuver to facilitate opening the urogenital sinus exactly in the posterior midline. If the urogenital sinus is wide, it is opened completely so that it can be tapered in caliber to make a urethra. If the urogenital sinus is the correct size to serve as a urethra it is unnecessary to open its distal end. Instead, it is opened at the level where the vagina and rectum converge. Opening the rectum and placing multiple sutures on its edge can facilitate its mobilization away from the vagina(s). Alternatively, a Foley catheter can be placed into the distal rectum to provide atraumatic traction during its mobilization. In some cases the rectum can be freed extensively from below, even circumscribing the cul-de-sac peritoneum which encircles it above the level of the lower sacrum. When dissecting the rectum from the vagina it is better to avoid entry into the anterior wall of the rectum; however, entry into the posterior wall of the vagina is not so apt to lead to trouble later. The most difficult technical aspect of cloaca1 reconstruction is dissection of the vagina(s) from the region of the bladder neck because the vaginal wall often encircles the posterior aspect of the upper urogenital sinus and the bladder neck. I avoid use of cautery in these dissections to prevent possible thermal injury to these thin structures that are adherent to each other. It is useful to fill the bladder with saline and/or air to define its wall. Every possible effort is made to avoid inadvert-
W. HARDY HENDREN
900
ent entry to the urinary tract, which can lead to a urinary fistula postoperatively. If a hole is made in the vagina during its mobilization (which is common) that can be managed by rotating the vagina during its pull-through. The urogenital sinus closure is done with a running inverting continuous suture, over a small, straight, plastic catheter that is anchored with a suture at the clitoris. A second layer of interrupted sutures is placed, with particular care at the upper end of the closure. A Foley catheter is not used to drain the bladder in order to avoid possible compression by the balloon. Also, if the balloon catheter were pulled inadvertently the balloon could disrupt the urogenital sinus closure. In some the vagina comes easily to the perineum. In others, it is too small or too short despite extensive mobilization. Turning the vagina 90” will place its intact lateral wall over the urogenital sinus suture line, which is better than the anterior or posterior wall, each of which may have a hole in it after mobilization. Perineal flaps based anteriorly and rotated into the vaginal orifice can compensate for slight shortness of the vagina. If the distance from the vagina to the perineum is great, a bowel segment should be used to bridge the gap, preferably a segment of colon because a segment of small bowel tends to become tortuous. The technical aspects of placing a colon extension have been published previously.’ The rectum is usually dilated and should be trimmed in caliber if very large, or plicated if only moderately wide, in order to fit it within the muscle complex. A perineal body is constructed between the vagina and the anus and the levator and sphincter muscles are closed around the rectum, with a Hegar dilator in place, to prevent making the closure too tight. These operations are often lengthy and the children are usually in an intensive care unit for a day or two postoperatively. Initially there can be facial swelling from the prone, jackknife position. The patients are kept flat, not partially upright postoperatively, for several days so that the pelvis will not be below the level of the heart which would increase pelvic venous pressure and aggravate wound edema.
Postoperatively, if the patient can not void, intermittent catheterization is begun, using a soft plastic catheter, either straight or with a coude tip. Temporary suprapubic drainage of the bladder can be useful until this can be accomplished. It can be helpful to bring a parent to the operating room to observe endoscopy of the new urethra via a video monitor and the endoscope, to teach the parent how to pass the catheter, ie, in what direction and how to circumvent an obstacle such as a prominent bladder neck by turning a coude tip catheter appropriately. We routinely use dilatation of the new rectum and vaginal passages postoperatively to avoid contraction and stenosis. This begins about 2 weeks after surgery and is done at home by the parents, using Hegar dilators of appropriate size, which are provided for them. If a colostomy remains, it is closed 2 to 3 months later. If bowels do not move spontaneously, enema washouts can be used. Long-term follow-up is essential for patients who have had cloaca1 reconstruction. Some will develop minor prolapse of the rectum or of bowel used as vagina. This is easily trimmed. Anal stenosis at the mucocutaneous junction can be corrected by a simple z-plasty. The appearance of the perineum can be improved greatly in some by shifting labioscrotal tissue more posteriorly to surround the vagina. Vaginal stenosis can occur and may be corrected by z-plasty or an inlay flap from the buttock. Most patients with a cloaca can be corrected to give them satisfactory urinary and bowel control and genital function if they are managed properly. In 1959 the late Dr Willis J. Potts wrote? In general, atresia of the rectum is more poorly handled than any other congenital anomaly of the newborn. A properly functioning rectum is an unappreciated gift of greatest price. The child who is so unfortunate as to be born with an imperforate anus may be saved a lifetime of misery and social seclusion by the surgeon who with skill, diligence, and judgement performs the first operation on the malformed rectum.
These words ring true today even more poignantly for the female with a cloaca1 malformation, which is the most severe degree of the spectrum of imperforate anus.
REFERENCES 1. Hendren WH: Repair of cloaca1 anomalies: Current techniques. J Pediatr Surg 21:1159-1176,1986 2. Hendren WH: Surgical management of urogenital abnormalities. J Pediatr Surg 12:339-357,1979
sinus
3. Hendren WH: Urogenital sinus and anorectal malformation: Experience with 22 cases. J Pediatr Surg 15:628-644,198O 4. Hendren WH: Further experience in reconstructive surgery for cloaca1 anomalies. J Pediatr Surg 17:695-717,1982 5. deVries PA, Pena A: Posterior Pediatr Surg 17:638-643,1982
sagittal anorectoplasty.
J
6. Pefia A, deVries PA: Posterior sagittal anorectoplasty: Important technical considerations and new applications. J Pediatr Surg 17:796-809,1982 7. Gough MH: Anorectal agenesis with persistence of cloaca. Proc R Sot Med 52~886~889,1959 8. Johnson RJ, Palken M, Derrick W, et al: The embryology of high anorectal and associated genitourinary anomalies in the female. Surg Gynecol Obstet 135:759-762,1972 9. Palken M, Johnson RJ, Derrick W, et al: Clinical aspects of female patients with high anorectal agenesis. Surg Gynecol Obstet 135:411-416, 1972
REPAIR OF CLOACAL MALFORMATIONS
10. Snyder WH Jr: Some unusual forms of imperforate anus in female infants. Am J Surg 111:319-325, 1966 11. Jaramillo D. Lebowitz RL, Hendren WH: The cloaca1 malformation: Radiologic findings and imaging recommendations. Radiology 177:441-448,199O 12. Sato Y, Pringle KC, Bergman RA, et al: Congenital anorectal anomalies: MR imaging. Radiology 168:157-162, 1988
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13. Hendren WH, Oesch I, Tschaeppeler H, et al: Repair of cloaca1 malformation using combined posterior sagittal and abdominal perineal approaches. Z Kinderchir 42:115-119. 1987 14. Hendren WH: Urological aspects of cloaca1 malformations. J Urol140:1207-1213,1988 15. Potts WJ: The Surgeon and the Child. Philadelphia. PA, Saunders, 1959, p 213
Experience
With 105 Cases
By W. Hardy Hendren Boston, Massachusetts l A personal experience with 105 cloaca1 malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 88 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloaca1 spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function. Copyright 8 1992 by W.B. Saunders Company INDEX WORDS: Cloaca1 malformations; imperforate posterior sagittal anorectoplasty; vaginoplasty.
anus;
C
LOACA, which is the Latin word for sewer, refers to that spectrum of pelvic malformations in female patients in which there is confluence of the urinary, genital, and gastrointestinal tracts. Although this is normal in birds, reptiles, and some fish, it can be disastrous in the human if not properly managed. Since our report of 64 cases in 1986,’ 41 more patients have been seen, giving an overall experience to date with evaluation and/or management of 105 cases. This paper will review results from the entire experience with 105 cases and will present 6 selected cases from among the 41 recent ones that illustrate the breadth of the cloaca1 spectrum. MATERIALS AND METHODS Records were reviewed of all 105 patients. There were 65 primary cases in which no reconstruction of the cloaca had been attempted before referral. There were 40 secondary cases. In most of those a rectal pull-through had been done previously without repairing the other aspects of the cloaca1 anomaly. Follow-up included 98 of the 105 patients. Seven patients were
excluded for the following reasons: 4 died preoperatively (dysplastic kidneys, dehydration, congenital heart disease, and hypematremia); 1 was seen in consultation in 1976, but disappeared; and 2 patients are preoperative, having had major urologic procedures performed and their cloacas will be repaired in the near future. Twenty patients in the series were from Massachusetts; the other 85 came from 12 foreign countries and 24 other states. Follow-up included personal examination or telephone contact with families and patients. The external appearance of the perineum varied widely (Figs 1 and 2). Similarly, there was a spectrum in the anatomy of the urogenital sinus (Fig 3). Vaginal anatomy was especially variable (Fig 4). In 12 patients there was no vagina; 42 had 2 vaginas; 51 patients had 1 vagina. Vaginal reconstruction required use of a bowel segment as an extention or substitute in 29 patients. Rectal anatomy varied as well (Fig 5). Twenty-seven patients had a urinary diversion when referred, which underscores the serious nature of urologic complications that occur in girls with cloacas. These included: 17 vesicostomy, 6 ureterostomy, and 4 ileal loop urinary diversions. The vagina commonly fills with urine in high cloaca1 malformations, pushing the bladder forward, thereby obstructing its emptying as well as drainage of the ureters (Fig 6). This can often be managed by intermittent catheterization of the vagina, using an 8F or 10F plastic catheter or a small metal catheterizing cannula. In retrospect, some of the secondary, previously diverted cases, could probably have been managed by prolonged catheterization instead of diversion. Fifty-seven patients had significant vesicoureteral reflux; most required ureteral reimplantation. In some severe cases a urologic operation was required before correcting the cloaca1 malformation, because obstruction or massive reflux were life-threatening and took precedence over the more elective aspects of anoplasty and vaginoplasty. In other cases in which reflux or obstruction were less severe it was corrected either at the time of the cloaca1 repair or later. Many other malformations were encountered. In the skeletal system they included: hemivertebra, short sacrum, absent sacrum, sacrococcygeal teratoma, presacral cyst, and absent leg. In the gastrointestinal tract they included: esophageal atresia, duodenal atresia, ileal atresia, gastroesophageal reflux, bowel duplication, Meckels diverticulum, and neuronal dysplasia of the colon. In the urinary tract they included: crossed renal ectopia, horseshoe kidney, ectopic ureter, ureteropelvic junction obstruction, dysplastic kidney, megaureter, ureterocele, and the prune-belly syndrome in two girls. Miscellaneous abnormalities included: congenital heart disease, aortoiliac vascular malformation, and tethering of the spinal cord.
Position for Operation From the Department of Sutgev, Children’s Hospital, Boston, MA. Presented at the 38th Annual International Congress of the British Association of Paediahic Surgeons, Budapest, Hungary. July 24-26, 1991. Address reprint requests to W. Hardy Hendren, MD, Department of Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. Copyright 0 I992 by W B. Saunders Company 0022-3468/92/2707-0027$03.00/O
890
In the first 35 cases prior to 19822-4cloacas were reconstructed using combined abdominal-perineal exposure with the legs sterilely prepped to allow lifting them into lithotomy position as needed. Since 1982 when Petia and DeVries described using the posterior sagittal approach for anorectoplasty,5s6 we incorporated it into the repair of cloaca1 malformations. In 43 primary cloaca1 reconstructions since 1982 the operation was completed using just the posterior sagittal approach in 12 patients. In 17 patients the
JournalofPediatric
Surgery, Vol27, No 7 (July), 1992: pp 890-901
REPAIR OF CLOACAL
MALFORMATIONS
891
augmentation in 1. Three patients have stress incontinence, but are currently content to live with it. Twenty-two patients empty their bladders by intermittent self-catheterization (ISC) and 18 are completely dry. Sixty void spontaneously with satisfactory control and are dry; in some the ability to void and be dry was present early, but in others that evolved slowly. Six patients are too recently operated to evaluate their urinary control. Four patients have urinary diversions, each with no bladder to reconstruct and each done prior to the era of continent diversion; this will be offered to each of them in the future.
Fig 1. The spectrum of enatomy in cloaca1 malformations from minimal to severe. (A) A 15.year-old girl with form fruste of cloaca1 malformation. Anus is further forward than normal, just behind urogenital sinus opening which had been previously cut back to enlarge it. (6) A Syear-old girl wlth anus even further forward, separated from the urogenital sinus by a thin urorectal septum. (C) A 4-year-old girl with one perineal opening, the urogenitel sinus, into which emptied the colon, vagina, and bladder. (D) A lg-month-old girl with more advanced cloaca1 anomaly. No opening evident in perineum. The urogenital sinus was located at the base of the phallic structure, which is surrounded by primitive labia minora.
operation began in the posterior sagittal position, but required turning the patient into the supine position as well to work in the abdomen. In 7 the reverse was done, commencing in the abdomen and turning prone to work from behind later. In 7 patients operation was performed through the belly and perineum, not using the posterior sagittal position at all. Similarly, the approach used in secondary cases varied according to what needed to be done. Therefore, circumferential prepping and draping of the patient as described previously was used routinely.’ RESULTS
Results are presented for 98 of 105 patients. Urinary Control Constant urinary wetting was seldom a problem, and was seen in only 3 patients. Further surgery for incontinence is planned for them, including bladder
Fig 2. Variations in the appearance of the perineum. (A) Age 1 year. Hemartome of perineum (arrow) and extensive capillary hemengioma. This patient had a wide urogenital sinus opening, at the top of which were three vertically ellgned ostia, the bladder, vagina, end rectum. (6) Neonate. Lipoma of perineum (arrow) and asymmetrical labia majora. The urogenital sinus opened et the base of the large phallic structure. (C) Age 6 months. Large penis-like phellic structure and single opening at its base. Note rounded perineum without intergluteal cleft. This typically indicates poor innervation of muscles and bodes poor bowel control. (D) Age 6 months. Prominent labia majora and perineal raphe. Patient had a high cloecal confluence, with two small vaginas that were extended to perineum with bowel segment. She also required ureteral reimplantetions and bilateral pyeloplesties.
W. HARDY HENDREN
UG SINUS
LONG UGS ENDING IN TIP OF CLITORIS
SUBCLITORAL MEATUS
Fig 5.
WIDE OPENING (Like a vagina) Fig 3.
WITH ACCESSORY TRACT
The spectrum of urogenital sinus anatomy. (A) Long urogen-
ital sinus opening at tip of clitoris. This is usually associated with high confluence of bladder, vagina, and rectum. A l-cm cut-back procedure can facilitate endoscopy and subsequent intermittent catheterization. The urogenital sinus should be preserved to serve as urethra after separating from it the rectum and vagina. Patient shown in Fig 1D had this type of urogenital sinus. (6) Urogenital sinus opening more posteriorly. (C) Wide urogenital sinus opening. Patient shown in Fig 1C had this type of urogenital sinus. (D) Accessory tract from urogenital sinus to phallus. The patient shown in Fig 18 had this type of anatomy. In repairing cloaca1 malformations if the urogenital sinus is not wide, it should be preserved to serve as the urethra. If it is wide it should be reduced in caliber. An accessory tract is not useful and should be marsupialized.
The spectrum of rectal anatomy.
patients use a soapsuds enema washout every day or every other day and are free from fecal soiling on that regime. Seven patients have a colostomy; 4 were performed because there was no pelvic muscle complex, making anorectoplasty inadvisable. Two colostomies were performed elsewhere because bowel control was imperfect after cloaca1 repair and the parents were unwilling to use an enema washout program. One patient has a temporary colostomy that will be closed in the future. Six patients were operated on too recently to assess. Seven patients have some fecal soiling and do not use enema washouts to control the problem. There were 4 patients with anterior position of the anus, but not a true cloaca, in whom repair of the urogenital sinus-vagina defect did not require moving the rectum. They started with normal bowel control and maintained it. Predictors of good bowel control were a good perineal raphe, a well-defined anal dimple, a normal sacrum and spine, a normal magnetic resonance imaging (MRI) examination, and a brisk muscle reflex during electrical stimulation.
Bowel Control Wide orifices
Forty-seven patients have normal bowel habits. Some needed a training period with enema washouts to learn bowel control. Others did not. Twenty-seven A catheter or
ED
Fig 4.
The spectrum of vaginal anatomy.
Fig 6. To show the mechanism of bladder outlet obstruction caused by the vagina distended with urine. Intermittent catheterlzation will usually enter the vagina, not the bladder which is displaced forward. Nevertheless, that can be effective in decomprassing the urinary tract.
REPAIR OF CLOACAL MALFORMATIONS
Poor bowel control correlated with a flat or rounded perineum and sacral and spinal defects.
893
A
SP tube (since bwth) Normal
ureters
Sexual Function Eight patients are now of adult age and report satisfactory coitus. Two have conceived and delivered infants by cesarean section. Two patients developed hematometrasalpix at puberty in one of two uteri and were treated by unilateral hysterosalpingectomy. It is likely that this will be seen in more patients who mature in the future who have a rudimentary uterus that has been joined to a bowel segment. Remarkably, two patients married for 4 and 6 years, respectively, were not having intercourse. In one case, the husband was reluctant to make the attempt. Counseling is planned for this couple. The second patient, recently examined, needs to have her introitus enlarged. Further, her husband has hypospadias in need of repair. Appropriate procedures are planned for this couple. SELECTED CASE REPORTS The following 6 cases from the 41 managed since 1986 will illustrate the wide range of problems the surgeon must solve in dealing with cloaca1 malformations.
Wide UGS
Cystic sacrococcygeal
teratoma
Ma& permeal body Pull through splemc flexure. enclosed in muscle complex
Removed colosfomy. ulled through proxinal limb. segment wwld not reach rrneum (short vessels).
Case 1 A 15month-old girl was referred in 1986 with a cloaca1 malformation and a large cystic sacrococcygeal teratoma, principally within the pelvis (Fig 7A). A suprapubic tube had drained the bladder since birth. There was a loop colostomy in the left lower quadrant. Aortogram done previously showed splaying of the iliac vessels over the teratoma (Fig 8A). Preoperative radiographic study showed high confluence of the bladder, vagina, and rectum, emptying to the perineum through a wide urogenital sinus (Fig 8B). Under anesthesia electrical stimulation of the perineum showed brisk muscle contraction of the perineum at the site for the anus (Fig 9). Operation began in the prone, jackknifed position for posterior sagittal mobilization of the tumor, including excision of the coccyx. She was then turned to enter the abdomen to complete mobilization of the tumor, after which the cloaca1 anatomy was repaired. The patient could not void postoperatively and a catheter could not be passed from below. Therefore, 2% weeks later endoscopy was performed, incising a shelf of mucosa at the bladder neck which prevented passing of the catheter. Now age 5% years she has no spontaneous voiding, but she can tell when the bladder is full. It is emptied 5 times daily by catheterization. She does not move her bowels spontaneously. Enema washout of the colon daily gives satisfactory bowel emptying, and there is no soiling on that program. The continuing need for catheterization and enema washouts may represent denervation of the muscle complex secondary to the extensive dissection to remove the teratoma. Aside from enemas and catheterization, she is a normal little girl. Comment. The cloaca1 anatomy in this case was straightforward to repair. However, the need to remove a large teratoma simultaneously made this a long (15 hours) and complex operation. This undertaking could not be staged and required multiple position changes to work alternately through the abdomen and the posterior sagittal approach. The previous low loop colostomy complicated the repair because the lower colon was tethered
and teratoma
Fig 7.
Anatomy in case 1 (A) before and (B) after reconstruction.
upward and could only be brought down by taking down the colostomy. Therefore, the safest option was discarding the segment of colon distal to the colostomy, and pulling through the bowel proximal to the colostomy. A right transverse colostomy at birth would have allowed pull-through of the distal colon without sacrificing that segment. A pull-through procedure with a colostomy closure just above it would not have been safe. unless a
Fig 8. Roentgenograms in case 1. (A) Preoperative aortograms showing displacement of iliac vessels by large intrepelvic teratoma. (6) Preoperative study by simultaneous injection of contrast into the bladder (B) and the rectum (R) through the distal limb of the colostomy, filling the vagina (V) and the urogenital sinus below it.
W. HARDY HENDREN
Dysblastic L. kidney
Fig 9. Photographs of perineum in case 1 preoperatively. (A) Perineum in resting state. Note barely visible surface component of sacrococcygeal teratoma (arrow). (B) Electrical stimulation of perineum under anesthesia noting excellent contraction of muscle complex at future location for anus. Relaxant agents should be avoided lest this reflex be inhibited.
second complementary colostomy were performed. A right transverse colostomy would have avoided this problem.
Cutback
UGS
/
Urethral sphincter
Colostomy
2 layer closure of UGS hrectomy
Case 2 A 4-month-old girl was referred in 1990 (Fig 10). Endoscopic and radiographic evaluation showed a small urogenital sinus opening at the tip of the phallus, apparent absence of a vagina, and enormous dilatation of the rectum (Fig 11). There were several small pit-like defects in the mucosa of the urogenital sinus below the bladder neck, but none would admit a small ureteral catheter, ie, there was no visible entry to a vagina. When the rectum was filled with irrigating solution, the bladder trigone was displaced markedly forward. It would fall back into normal position on emptying the rectum. Operation began in the prone position through posterior sagittal exposure. The urogenital sinus was cut back about 15 mm, to a location appropriate for a normal urethral meatus. With a sound in the urogenital sinus, it was opened near its upper end to locate the rectal fistula. With a Foley catheter in the rectum, and another in the bladder, the two were separated. The entry of the rectum was high, at the level of the coccyx, and was impossible to mobilize completely from the posterior sagittal position. A small vagina was found on the right side filled with mucous. None was found on the left. the abdomen. There was The oatient was then turned to exDlore r-m-~ ~~~~ ~~~~~~~ ~~~-~-a normal tube, ovary, and small uterus on each side, wide apart, against each pelvic wall. On the right the uterus communicated with the vagina which had been previously mobilized from below. On the left there was only a fibrous cord below the uterus. Further, sections of the uterus disclosed no lumen. Therefore, it was removed. Probing the right side disclosed a patent cervix and endometrial cavity. By mobilizing the rectum further through the abdominal approach, it was possible to bring it to the perineum. Its large caliber made it more appropriate to use it as a vaginal extension than tapering it to become the rectum. The dysplastic left kidney was removed. The patient was turned prone once again, closing the urogenital sinus opening, and pulling down the rectum as a vagina. Once again in the supine position, the legs were picked up into lithotomy position to complete suturing the colon segment to the perineum. An opening was made in the anterior wall of this rectal segment, joining to it the right vagina, spatulating the anastomosis to make it as large as possible. The rectum was divided
17 mos
Fig 10. (A) Preoperative and (B) postoperative anatomy in case 2. Note absent vagina on left side and “occult” vagina on right side.
1 in above that, leaving a long enough segment of colon to have adequate depth to serve as a vagina. The next segment of colon above was removed, preserving its mesentery. The colon was divided above the sigmoid vessels to pull through the next higher segment based on left colic vessels. This segment was pulled through, to be dealt with later. The abdomen was closed. The patient was turned once again into the prone position, constructing a perineal body, and placing the rectum appropriately in the muscle complex. The rectum was tapered to the caliber of a size 10 Hegar dilator. The muscle complex was closed around it. The operation lasted 16 hours. The patient spent 2 days in the intensive
1
Fig 11. Preoperative roentgenograms in case 2. (A) Anteroposterior and (B) lateral views. Note bladder (B) displaced forward by dilated rectum (R).
REPAIR OF CLOACAL MALFORMATIONS
895
care unit and recovered uneventfully. Three months later neurosurgical release of the spinal cord was performed by Dr Michael Scott. Colostomy closure was performed 1 week later. The patient is now 6 months following cloaca1 reconstruction. She voids and stools intermittently, but it is too early to assess the degree of control of these functions. Commenr. This case illustrates the “occult” vagina, ie, a vagina was present but could not be seen endoscopically or by preoperative radiographs or ultrasound examination. The close proximity of the rectum to the bladder on preoperative radiographs (Fig 11) had been interpreted as absence of a vagina. However, this is not a valid radiographic finding if vaginas are duplicated and diverge laterally. When there is a blindly ending uterus as was seen on the left side, it is probably best removed. We have encountered hematometrium at puberty in two cloaca1 cases in which evidently there was no uterine canal to allow menstruation at puberty. In this particular case no uterine cavity was observed on serially sectioning the left uterus before its removal.
Case 3 In 1988 a girl was referred at the age of 2 months. Transverse colostomy had been performed at birth (Fig 12). There was a wide urogenital sinus opening. At its apex the bladder neck entered anteriorly, with no segment of urethra between the bladder neck and urogenital sinus. The ureteral orifices were close to the bladder neck. Two vaginal openings lay side by side with a septum between them. The vaginas were not large. The rectal fistula entered the septum anteriorly, not in the usual position at its base. The septum Ecfopic ureters I” bladder neck (reflux ,nto both) Normal
was incised to create one vaginal cavity. Intermittent catheterization was not needed because there was free drainage of urine from the urogenital sinus. At age 2 years cloaca1 reconstruction was performed. There were only two openings in the urogenital sinus, namely the bladder neck and the vagina just behind it. The rectum entered the septum between the vaginas at the point at which they diverged. By splitting the vaginas from behind, the rectum was visualized and mobilized up to the cul-de-sac peritoneum, actually detaching the cul-de-sac peritoneum from the rectum and dividing the sigmoid vessels to gain length. The two vaginas were then sewn together, to create a single one, and rotated in order to have intact vaginal wall over the urogenital sinus closure. Two skin flaps of labia majora tissue were created and rotated into the posterior vagina wall to avoid tension in bringing down the vaginas. Five months later the ureters were reimplanted for reflux and the colostomy was closed. Now 14 months following reconstruction, and age 3 years, the patient has urinary control and bowel control that her parents judge to be normal. Comment. The variable anatomy in cloacas is well illustrated by this case. For example, the bladder neck emptied almost directly into the urogenital sinus, and two refluxing ureters entered the bladder neck. The vaginal anatomy is of interest, because the two vaginas were not completely separate, as seen in some cases, nor were they side by side for their full length which is the most common type of vaginal duplication. Diverging vaginas can allow anterior displacement of the rectum. The very high position of the rectal pouch was initially thought to require laparotomy. By circumscribing the cul-de-sac peritoneum and dividing the rectosigmoid mesentery enough length was gained to bring down the colon. This case also illustrates the use of perineal flaps. When a vagina will not reach the perineum despite wide mobilization and the gap to be bridged is short, perineal flaps can be created to lay up into the vagina. There is usually ample labial scrotal tissue to do that. Local flaps should be based anteriorly and rotated upward. When the vagina is small or the gap to be bridged great, it is better to extend the vagina with a bowel segment. When two vaginas he side by side for their full length they can be converted into one by incising the common wall between them. When they are separate, or diverge as in this case, they must be anastomosed together to create a single vaginal passage. When the vaginas do not occupy the midline, the rectum may be positioned more forward than usual.
Case 4 Sacrum OK
MRI OK
B
Rsdum puffed thnqfh andencloaadinmusde
Fig 12.
(A) Preoperative
and (B) postoperative
anatomy in case 3.
An l&month-old girl was referred in 1988. At age 2 days abdominal exploration had been performed, inserting a suprapubic tube into a fluid-filled structure in the pelvis, thought to be the bladder, but it was the vagina. She did not do well and at age 5 weeks underwent reoperation. The bladder was exteriorized as a vesicostomy in the midline above the location of the previous tube vaginostomy. Left end colostomy was also performed, with turn in of the distal colon. It was noted that most of the urinary drainage came from the vaginostomy tube and that urine passed intermittently via the anus. Endoscopic and radiographic evaluation disclosed the anatomy depicted in Fig 13A. There was excellent contractility of the muscle complex on stimulating the perineum electrically, but the anal opening in the perineum was behind the point of maximum contractility. Endoscopy of the vagina showed the fistula in the rectum, and the endoscope could be passed through it. The urogenital sinus was strictured. Dilatation was performed, leaving a catheter stent in place. Reconstruction was performed 4 months later (Fig 13B). Ureteral catheters were placed through the rectal fistula, the vesicostomy, and the vaginostomy to aid in finding each of these passages during operation. The procedure began from above, in the supine position, to liberate the
896
W. HARDY HENDREN
sphincter Incomplete
/ sacrum
during infancy. However, it appeared that the urogenital sinus was tethered across the pubis by the bladder’s being pulled upward by the vesicostomy. We have seen this in another patient in whom vesicostomy had been performed; the bladder had been pulled up so tightly that a ureteral catheter could not be passed up the urogenital sinus. However, when the vesicostomy was taken down, allowing the bladder to descend, the caliber of the urogenital sinus became normal. The presence of a vesicostomy and a vaginostomy greatly complicated this child’s management and caused hydronephrosis which led to reimplantation of the right ureter. Intermittent catheterization from below during infancy might have prevented these complications. Another cloaca1 case had persisting sigmoid dilatation and was treated by sigmoid resection, which cured her constipation. Microscopic examination showed neuronal dysplasia of the colon.
\ Tethered
cord
POSITIONS: supine
a
prone
3
supine
B Vesicostomy
Ureter reimplanted
Excision
stricture
Vagina pulled through (former stoma) 4 months Excised
later
Case 5 A girl was referred at birth in 1987 with imperforate anus, abdominal distention, and a funnel-shaped appearance to her vaginal introitus (Fig 14). No meconium had passed. Cystogram showed several diverticula arising from the bladder, shedding little light on what the anatomy might be. Endoscopy was performed before laparotomy to relieve bowel obstruction. There was a wide urogenital sinus with no structures entering it. There were multiple openings in the bladder including a patent urachus. Real atresia was found at laparotomy; below it there was a gap in the mesentery and a short segment of colon in the lower abdomen. The upper end of the colon was blind and the lower end connected with the
fistula
Rectum tapered
and
LATERAL VIEW
moved forward into muscle complex
Fig 13.
(A) Preoperative
and (8) postoperative
anatomy in case 4.
which were tethered upward. The right ureter was imbedded in scar surrounding the vaginostomy and required mobilization. The patient was then turned prone. The vagina was separated from the urogenital sinus, which was strictured. The etiology of the stricture appeared to be compression by the pubis because the bladder was pulled upward by the vesicostomy. Resection and reanastomosis of the urogenital sinus was performed. The rectovaginal fistula was divided. The part of the vagina that had been the stoma was brought to the perineum. This was possible because it had been mobilized from above. The rectum was tapered and placed within the muscle complex. The patient was then turned supine to reimplant the right ureter and place a suprapubic tube in the bladder. Endoscopy 2 weeks later showed satisfactory healing. A new urethral catheter was placed to allow further healing. MRI examination showed slight tethering of the spinal cord, but neurosurgical consultation felt that an untethering operation was not needed at that time. Three months later colostomy closure was performed. The patient voided well and intermittent catheterization was discontinued. During the next year constipation was a problem and was relieved by enema washouts. The anal opening became slightly stenotic and was enlarged by 2 z-plasties. Now age 4 years, the rectosigmoid remains dilated. Sigmoid resection will be performed. Comment. This is the only case we have seen with an H-fistula from the rectum to the vagina. Furthermore, posterior anal ectopia is extremely rare. When the rectal opening is ectopic, it nearly always lies forward of the point of maximum contractility of the sphincter complex. The cause for the urogenital sinus stricture may have been created by traumatic catheterization or endoscopy vagina and bladder
.-
Positions:
Good anal sph,ncter
supine *
6
prone *
supine
~- a
DivertMum
Fig 14.
(A) Preoperative and (6) postoperative
LATERAL VIEW
anatomy in case 5.
a97
REPAIR OF CLOACAL MALFORMATIONS
bladder. The urachus was closed. The heal atresia was exteriorized. The blindly ending colon was also exteriorized without knowing whether this was the end of bowel that might have been in continuity with the heal atresia or whether it was the end of bowel as usually seen in cloaca1 exstrophy. At age 7 months endoscopy was performed again, but the findings were difficult to decipher. At age 15 months endoscopy was repeated with uroradiologist, Dr Robert Lebowitz, present with a C-arm fluoroscopic unit. As each orifice was visualized, and catheterized, contrast medium was injected, obtaining fluoroscopy and spot films (Fig 15). The anatomy depicted in Fig 14A was derived in that fashion. There were two hemibladders, four ureters, two vaginas entering the bladders, and a bowel opening in the septum between the two hemibladders. There was a diverticulum at the apex of the right hemibladder with communicating openings. Repair was performed (Fig 14B) at age 2 years. After circumferentially preparing and draping the child, a midline laparotomy was performed to dissect out all of the anatomy. Biopsy specimens were taken from both ends of the distal defunctioned bowel segment because it was not possible from the orientation of its mesentery to tell which end was developmentally the distal one, ie, whether the blind end was what might have been in continuity with the ileum, or whether it was the blindly ending gut seen in cloaca1 exstrophy. Interestingly, there were no tinea on the colon. The bladder was opened and it was possible to locate the two vaginas which entered on each side of a midline septum. They were dissected free from the bladder, using the intravesical approach and were sewn together to create a single
vagina. The bowel segment was detached from the bladder. The ureters were reimplanted. The midline septum between the bladders was excised to make a single bladder. The patient was then turned into the prone position to make a posterior sagittal midline incision. The wide urogenital sinus was tapered into a urethra and closed with two layers. The two vaginas were rotated 90” and joined to the perineum. A perineal body was constructed. The appropriate end of the bowel segment was pulled through and enclosed in the muscle complex. The child was then turned supine to complete bladder closure and exteriorize the other end of bowel. The anesthesia time for this global reconstruction was 20 hours. The patient convalesced uneventfully. Endoscopy 2% weeks later showed satisfactory healing. Intermittent catheterization was begun and the suprapubic tube was soon removed. Four months later bowel continuity was restored by resecting the ileostomy and joining it to the defunctioned distal bowel. The patient could not empty her bladder completely and was maintained on intermittent catheterization. Surprisingly, at age 3 years she emptied her bowel 2 to 3 times daily voluntarily and without soiling. A minor degree of rectal prolapse was trimmed as an outpatient procedure. MRI of the spine showed no tethering of her spinal cord. She now voids spontaneously, does not wet, and is catheterized for residual twice daily as a precaution, usually obtaining about 100 mL. Comment. This case is of special interest because the anatomy initially appeared so complex that there seemed little likelihood that reconstruction would achieve continence. Those gloomy prospects proved incorrect. It was vital to thoroughly assess the anatomy both radiographically and endoscopically. This case seems to be a transition form between a usual cloaca and cloaca1 exstrophy, because it included hemibladders, vesicointestinal fissure, and a blindly ending segment of bowel hanging in the pelvis. However, there was no omphalocele or bladder exstrophy.
Case 6
Fii 15. Preoperative roentgenogram in case 5 showing bizarre constellation of structures produced by filling anatomy shown in Fig 14A. Prolonged endoscopic study in conjunction with simultaneous roentgen study with C-arm fluoroscopic unit in operating room was required to decipher the anatomy.
This patient was referred in 1989 at age 10 years (Fig 16A). Colostomy had been done at birth and a rectal pull-through at 1 year of age. The bowel had not been placed properly in the muscle complex; therefore, the patient was incontinent. A second colostomy was performed at age 3 years. Two small vaginas entered the urogenital sinus and there was recurrent urinary infection and dribbling. After consideration of various options a permanent urinary diversion was performed at age 4 years. Cystogram showed a small, elongated bladder, with two small vaginas filling from the urogenital sinus (Fig 17A). The defunctioned rectal segment was filled with old stool. MRI showed no spinal cord tethering. At a separate endoscopic procedure the anatomy was assessed and the defunctioned distal colon was emptied of a large amount of inspissated fecal material. Six months later reconstruction was undertaken (Fig 16B). Endoscopy of the right vagina showed a uterine cervix. The left vagina was smaller; a hole at its apex led to a tube but there was no uterus. The operation began in the prone position, mobilizing the previously pulled through rectum to gain access to the upper urogenital sinus and vaginas at the bladder neck. The right vagina was the better of the two, but was only 2 in long. The left vagina was smaller and no cervix could be seen at its apex. The urogenital sinus was repaired with two layers. The patient was turned. There were extensive intra-abdominal adhesions. There was no uterus on the left side, only a direct connection between the Fallopian tube and the vagina. Therefore, the left vagina and tube were removed, sparing the ovary. The right vagina was anastomosed to the segment of colon which had been previously pulled through. This anastomosis was accomplished from below, turning the patient prone once again. In many cases I have found it easier to do this from above. A perineal body was constructed. The patient was then turned supine once again to deal
898
W. HARDY HENDREN
A
I.
L_~
/
Colon conduit
Prior rectal pull through as infant, too anterior
-rum
Positions:
prone j
supine *
prone *
supine 3 prone
Gastrocystoplasty Psoas hitch
I
R. gastroepiploic pedicle Removed L. vagina
fvfdt OK
tapering, pit in center of muscle
anastomosed to colon
< Fig 16. Complex reoperative case (case 6) wtth colostomy and ureterostomy. (A) Preoperative and (6) postoperative. Note need to augment bladder, using stomach, because it was small after many years of diversion.
the urinary tract. The bladder was small, and smooth walled due to long time diversion. By stretching the bladder manually, it was possible to position it for a psoas hitch on the right side. The urinary conduit was removed and discarded because it was too with
small to use as an augmentation. The right ureter, which was the better of the two, was reimplanted into the bladder with a long tunnel. There was a kink in the right ureter of questionable significance. Therefore, the left-to-right transureteroureterostomy was located at that point to make certain that there was no functional obstruction at that level. A generous wedge of stomach, representing about one third of its volume, was mobilized on the right gastroepiploic pedicle and rotated down to augment the bladder. The colostomy was taken down, mobilizing the splenic flexure. To gain length, it was necessary to divide both the left colic and midcolic vessels and bring the left colon down on the vascular pedicle of right ileocolic vessels. The colon was pulled through to the perineum and fastened temporarily with a single suture to the adjacent perineum. The belly was then closed and the patient was turned for the final time into the prone position. The pulled through rectum was tapered in caliber and placed in the muscle complex. Blood replacement included 4 U of whole blood, 1 U of packed red blood cells, and 8 L of lactated Ringer’s solution during this 24-hour reconstruction. The patient was in excellent condition with a good urinary output at the end of this extensive surgical undertaking. Now 1% years later she is entirely well. She empties the bladder by ISC every 4 hours and is dry and free from urinary infection. The colon is emptied using a saline enema daily and is free from fecal soiling on this program. Postoperative cystogram showed normal bladder volume (Fig 17B). Comment. This case illustrates several important principles. First, colon pull-through should never be performed as an isolated procedure. Second, when pull-through is performed it is best done through the posterior sagittal approach with tapering of the rectum and accurate placement in the muscle complex. This enhances the likelihood of bowel control. Furthermore, correction of the urinary tract should not be “deferred until later.” The urinary diversion greatly complicated the reconstruction. It is tempting to think of a way in which a reconstruction such as this could be done in stages to avoid such an enormous global operation of this length. However, that is not practical. Fortunately, expert anesthesia care with intraoperative monitoring and postoperative intensive care make possible the safe conduct of such a formidable operation. This would have been impossible 2.5 years ago. Great credit is deserved by the anesthesiologists for making this possible. Bladder augmentation was essential in this case. Using stomach, as compared with small bowel or colon, has three advantages: (1) it does not form mucous and secondary stones; (2) it excretes chloride rather than absorbing chloride, which can aggravate acidosis; and (3) it creates an acid pH, which makes urinary infection less likely. DISCUSSION
Fig 17. Roentgenograms in case 6. (A) Preoperative cystogram showing small bladder with two small vaginas filling from it, one on each side. Flecks of calcium are in stool in colon segment defunctioned 6 years before. (B) Postoperative cystogram showing normal capacity of bladder. Patient empties by ISC. Wisp of reflux is in retained left ureterai stump, not a functional ureter.
Cloaca1 anomalies are much more variable in their anatomic form than most other congenital malformations. Nevertheless, certain principles have evolved that can lead to a high rate of success in managing these children. The prognosis should no longer be poor as it was three decades ago.7-10 At birth colostomy should be done. I prefer a divided loop transverse colostomy. This will preserve intact the left colic blood supply in case separate colon segments with their blood supply will be needed in later reconstruction. It is not necessary to do a wide abdominal exploration in these infants to inspect pelvic organs which can be studied later endoscopitally and radiographically. When there is a large bladder or vagina filling the lower abdomen, routine vesicostomy or vaginostomy is not needed. Often the
REPAIR OF CLOACAL
MALFORMATIONS
large viscus can be decompressed by intermittent catheterization. The catheter or catheterizing sound will usually enter the vagina, not the bladder that is displaced forward. If intermittent catheterization does not relieve lower urinary tract obstruction, cystostomy or vaginostomy can be considered. That can complicate later reconstruction by tethering these organs upward. When the urogenital sinus extends to the phallic tip it may prove helpful to cut it back a few millimeters to facilitate catheterizing. Soon, complete anatomic study should be performed. This includes: (1) upper tract evaluation by ultrasonography or intravenous pyelogram; and (2) contrast study of the lower tract by filling the urogenital sinus and the distal limb of the colostomy and any other orifice that may be present such as a vesicostomy or vaginostomy. Results of radiographic evaluation have been recently described.” MRI of the spine is now routine for cloaca patients to rule out tethering of the spinal cord.12 MRI studies were performed on 44 of these patients. Findings were normal in 29 and abnormal in 15. Three of the latter showed a syrinx of the spinal cord; 12 had tethering of the cord below its normal level of the first lumbar vertebra. Six patients have undergone detethering operations to date. The rest are being followed. Vertebral anomalies and missing sacral segments are common in these children and often accompany urological malformations. If there is a life-threatening urologic problem such as massive reflux, megaureter, ureteropelvic junction obstruction, etc, it is best to deal with that early before serious renal damage occurs.13J4 It is most important to emphasize that such conditions should not be ignored while waiting for the baby to grow old enough for a definitive total reconstruction. Furthermore, our experience with secondary cases has provided convincing evidence that the rectal component of a cloaca, the easiest task to repair, should never be operated on first as a separate procedure, leaving the most difficult parts of this malformation, the urogenital sinus and vagina repair, to be done later. Reflux, present in 55% of cases, if not corrected early may feasibly be corrected at the time of cloaca1 repair or later as a separate operation. Thus, the timing of antireflux surgery should depend on the severity of the reflux and whether the kidneys are normal or show scarring. Repair of the primary cloaca is usually done between the ages of 9 and 18 months. Endoscopic evaluation is always carried out several days before cloaca1 reconstruction to become familiar with the anatomy to be reconstructed and to wash out any remaining mucous or old fecal matter in the distal colon. Often, the endoscope can be introduced into
the rectal fistula to irrigate saline and antibiotic solution from below and out the colostomy. It can be helpful to insert an endoscope into the distal colostomy stoma to irrigate to and fro using an Ellyk evacuator to remove remaining fecal material. Cloacal reconstruction should never be undertaken until the bowel is absolutely clean. These operations are long and difficult. An experienced anesthesiologist and full intraoperative monitoring are mandatory. Blood loss and fluid loss can be large and must be replaced appropriately. Unless there is a particular reason to enter the belly first, such as the presence of a vaginostomy or vesicostomy that must be released, the operation usually begins in the prone, jackknife position for posterior sagittal approach. Electrical stimulation of the perineum is mandatory to assess muscle contractility. This precludes use of muscle relaxant anesthesia technique. The special advantages of the posterior sagittal approach include ideal access to separate the three organ systems and visualization of the muscle complex for accurate placement of the rectum within it. It is important to dissect in the exact anatomic midline while approaching the rectum, vagina(s) and urogenital sinus from behind. A sound in the urogenital sinus, lifted upward by an assistant, is a useful maneuver to facilitate opening the urogenital sinus exactly in the posterior midline. If the urogenital sinus is wide, it is opened completely so that it can be tapered in caliber to make a urethra. If the urogenital sinus is the correct size to serve as a urethra it is unnecessary to open its distal end. Instead, it is opened at the level where the vagina and rectum converge. Opening the rectum and placing multiple sutures on its edge can facilitate its mobilization away from the vagina(s). Alternatively, a Foley catheter can be placed into the distal rectum to provide atraumatic traction during its mobilization. In some cases the rectum can be freed extensively from below, even circumscribing the cul-de-sac peritoneum which encircles it above the level of the lower sacrum. When dissecting the rectum from the vagina it is better to avoid entry into the anterior wall of the rectum; however, entry into the posterior wall of the vagina is not so apt to lead to trouble later. The most difficult technical aspect of cloaca1 reconstruction is dissection of the vagina(s) from the region of the bladder neck because the vaginal wall often encircles the posterior aspect of the upper urogenital sinus and the bladder neck. I avoid use of cautery in these dissections to prevent possible thermal injury to these thin structures that are adherent to each other. It is useful to fill the bladder with saline and/or air to define its wall. Every possible effort is made to avoid inadvert-
W. HARDY HENDREN
900
ent entry to the urinary tract, which can lead to a urinary fistula postoperatively. If a hole is made in the vagina during its mobilization (which is common) that can be managed by rotating the vagina during its pull-through. The urogenital sinus closure is done with a running inverting continuous suture, over a small, straight, plastic catheter that is anchored with a suture at the clitoris. A second layer of interrupted sutures is placed, with particular care at the upper end of the closure. A Foley catheter is not used to drain the bladder in order to avoid possible compression by the balloon. Also, if the balloon catheter were pulled inadvertently the balloon could disrupt the urogenital sinus closure. In some the vagina comes easily to the perineum. In others, it is too small or too short despite extensive mobilization. Turning the vagina 90” will place its intact lateral wall over the urogenital sinus suture line, which is better than the anterior or posterior wall, each of which may have a hole in it after mobilization. Perineal flaps based anteriorly and rotated into the vaginal orifice can compensate for slight shortness of the vagina. If the distance from the vagina to the perineum is great, a bowel segment should be used to bridge the gap, preferably a segment of colon because a segment of small bowel tends to become tortuous. The technical aspects of placing a colon extension have been published previously.’ The rectum is usually dilated and should be trimmed in caliber if very large, or plicated if only moderately wide, in order to fit it within the muscle complex. A perineal body is constructed between the vagina and the anus and the levator and sphincter muscles are closed around the rectum, with a Hegar dilator in place, to prevent making the closure too tight. These operations are often lengthy and the children are usually in an intensive care unit for a day or two postoperatively. Initially there can be facial swelling from the prone, jackknife position. The patients are kept flat, not partially upright postoperatively, for several days so that the pelvis will not be below the level of the heart which would increase pelvic venous pressure and aggravate wound edema.
Postoperatively, if the patient can not void, intermittent catheterization is begun, using a soft plastic catheter, either straight or with a coude tip. Temporary suprapubic drainage of the bladder can be useful until this can be accomplished. It can be helpful to bring a parent to the operating room to observe endoscopy of the new urethra via a video monitor and the endoscope, to teach the parent how to pass the catheter, ie, in what direction and how to circumvent an obstacle such as a prominent bladder neck by turning a coude tip catheter appropriately. We routinely use dilatation of the new rectum and vaginal passages postoperatively to avoid contraction and stenosis. This begins about 2 weeks after surgery and is done at home by the parents, using Hegar dilators of appropriate size, which are provided for them. If a colostomy remains, it is closed 2 to 3 months later. If bowels do not move spontaneously, enema washouts can be used. Long-term follow-up is essential for patients who have had cloaca1 reconstruction. Some will develop minor prolapse of the rectum or of bowel used as vagina. This is easily trimmed. Anal stenosis at the mucocutaneous junction can be corrected by a simple z-plasty. The appearance of the perineum can be improved greatly in some by shifting labioscrotal tissue more posteriorly to surround the vagina. Vaginal stenosis can occur and may be corrected by z-plasty or an inlay flap from the buttock. Most patients with a cloaca can be corrected to give them satisfactory urinary and bowel control and genital function if they are managed properly. In 1959 the late Dr Willis J. Potts wrote? In general, atresia of the rectum is more poorly handled than any other congenital anomaly of the newborn. A properly functioning rectum is an unappreciated gift of greatest price. The child who is so unfortunate as to be born with an imperforate anus may be saved a lifetime of misery and social seclusion by the surgeon who with skill, diligence, and judgement performs the first operation on the malformed rectum.
These words ring true today even more poignantly for the female with a cloaca1 malformation, which is the most severe degree of the spectrum of imperforate anus.
REFERENCES 1. Hendren WH: Repair of cloaca1 anomalies: Current techniques. J Pediatr Surg 21:1159-1176,1986 2. Hendren WH: Surgical management of urogenital abnormalities. J Pediatr Surg 12:339-357,1979
sinus
3. Hendren WH: Urogenital sinus and anorectal malformation: Experience with 22 cases. J Pediatr Surg 15:628-644,198O 4. Hendren WH: Further experience in reconstructive surgery for cloaca1 anomalies. J Pediatr Surg 17:695-717,1982 5. deVries PA, Pena A: Posterior Pediatr Surg 17:638-643,1982
sagittal anorectoplasty.
J
6. Pefia A, deVries PA: Posterior sagittal anorectoplasty: Important technical considerations and new applications. J Pediatr Surg 17:796-809,1982 7. Gough MH: Anorectal agenesis with persistence of cloaca. Proc R Sot Med 52~886~889,1959 8. Johnson RJ, Palken M, Derrick W, et al: The embryology of high anorectal and associated genitourinary anomalies in the female. Surg Gynecol Obstet 135:759-762,1972 9. Palken M, Johnson RJ, Derrick W, et al: Clinical aspects of female patients with high anorectal agenesis. Surg Gynecol Obstet 135:411-416, 1972
REPAIR OF CLOACAL MALFORMATIONS
10. Snyder WH Jr: Some unusual forms of imperforate anus in female infants. Am J Surg 111:319-325, 1966 11. Jaramillo D. Lebowitz RL, Hendren WH: The cloaca1 malformation: Radiologic findings and imaging recommendations. Radiology 177:441-448,199O 12. Sato Y, Pringle KC, Bergman RA, et al: Congenital anorectal anomalies: MR imaging. Radiology 168:157-162, 1988
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13. Hendren WH, Oesch I, Tschaeppeler H, et al: Repair of cloaca1 malformation using combined posterior sagittal and abdominal perineal approaches. Z Kinderchir 42:115-119. 1987 14. Hendren WH: Urological aspects of cloaca1 malformations. J Urol140:1207-1213,1988 15. Potts WJ: The Surgeon and the Child. Philadelphia. PA, Saunders, 1959, p 213
