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Posterior cloaca: A unique defect
Journal of Pediatric Surgery MARCH
VOL 33, NO 3
Posterior
Cloaca:
By Albert0
Peiia
A Unique and
Oded
1998
Defect
Kessler
New Hyde Park, New York and Bronx,
New York
The authors present nine cases of a unique defect in which urethra and vagina are fused together forming a urogenital sinus that deviates posteriorly and opens In the anterior rectal wall at the anus or immediately anterior to it. The rectum is essentially normal or may be minimally anteriorly mislocated. The authors call this defect posterior cloaca. The diagnosis requires a suspicious observer and a meticulous examination of the female genitalia. A complete urologic evaluation is mandatory because 88% of these patients have important associated urologic defects. Our patients were operated on via a posterior sagittal transanorectal approach,
which allowed a full dissection and mobilization of urethra and vagina together to be placed in a normal location. Rectal dissection and mobilization was avoided to preserve bowel control. Seven patients enjoy bowel and urinary control. One patient was lost to follow-up, and one patient with a severely dysplastic sacrum suffers from fecal and urinary incontinence. J Pediatr Surg 33:407-412. Copyright o 1998 by W.B. Saunders Company.
A
and the patients and their parents were interviewed. The mean age at operation was 36 months (range, 9 months to 9 years). Six patients were younger than 3 years of age at the time of operation Five patlents had a colostomy done very early in life. Therefore, we knew about preoperative bowel control in only two patients: they were totally continent. The follow-up ranges from 2 months to 8 years with an average of 4 years.
TYPICAL CLOACA is a defect in which urethra, vagina, and rectum fuse and form a common channel that opens as a single orifice where the urethra is normally located.’ The malformation that we present is unique in that the urogenital sinus deviates posteriorly and opens in the anterior rectal wall, at the anus, or immediately anterior to it. Therefore, we call it a posterior cZoaca. An important feature of this defect is that the rectum is essentially normal or may be slightly anteriorly mislocated. Since our first publication regarding this condition,2 eight additional patients with this rare condition have been operated on by us. Because of its unique anatomic characteristics, repairing this defect requires special surgical maneuvers to achieve good functional results. In our review of the English-language literature, we were unable to find any previous publications specifically related to this defect as a separate entity. However, in Hendren’s publications,3-10 one can read descriptions of at least 10 cases with anatomic characteristics of what we call posterior cloaca. MATERIALS
AND
METHODS
Smce 1982. 170 primary cases of persistent cloaca have been operated on by the senior author. From these, nine cases were selected for review because they shared the anatomic features consistent with what we call posterior cloaca. The clinical and radiological records of the patients were reviewed, JournalofPediatric
Surgery,
Vol33,
No 3 (March),
1998: pp 407-412
INDEX
Anatomy
WORDS:
Posterior
and Description
cloaca,
urethral
vaginal
fusion.
of the Defect
Figure 1 is a diagram of the anatomy of a female patlent with a posterior cloaca. The most conspicuous anatomic features of a posterior cloaca mclude a well-located or minimally anteriorly mislocated rectum. Both urethra and vagina form a urogenital sinus that is postenorly deviated and opens in the anterior rectal wall, in the anus, or in the permeum immechately antenor to the anus. The external appearance of the permeum in these girls is bizarre because they may have labia but no urethral or vaginal opening. Three anatomic variants of this defect were found: (1) The urogenital Smus opens into the anterior rectal wall or mto the anus and is visible only endoscopically (Figs 1 and 2, five cases). (2) The urogenital Smus opens mto the perineum immediately anterior to the anus (Fig 3, four
Fmrn the Dwzsion of Pediatric Surgery, Departments of Surgery and Urology, Long Island Jewish Medical Centel; New Hyde Park, NY and the Clmical Campus of the Albert Emstein College of Medicme, Bronx, NY Address reprint requests to Albert0 Peiia, MD, Chief; Division oj Pediatric Surgev, Schneider Children’s Hospital, Long Island Jewish Medical Centel; 269-01 76th Ave, New Hyde Park, NY 11040. Copyright 0 1998 by WB. Saunders Company 0022.3468/98/3303-0001$03.00/0
407
408
PENA AND
Normally
Fig 1. posterior
Diagram cloaca.
shows
KESSLER
focafed-anus
a sagittal
view
of the anatomy
of a case of a
cases). (3) The urogemtal sinus opens either in the rectum or the perineum and there is a rudimentary urethra opening at the tip of a pseudophallus (three cases).
Associated Defects Seven patients had associated defects that mcluded uterine septation disorders (hemivagmas and hemmten),
vagmal and four patients:
Fig 3. Urogenital the anus.
Perineum of sinus opens
a patient with a posterior cloaca type into the perineum, immediately anterior
B. to
hydrocolpos, four patients; vesicoureteral reflux, three patients; hydronephrosis, four patients; absent kidney, three patients; esophageal atresia and tracheoesophageal fistula, two patlents: thoracic hermvertebrae, two patients; ureteral duplication, one patient; ventricular septal defect, one patient; malrotation, one patient; bladder diverticulum. one patient; and ureteropelvic obstruction, one patient. Chromosomal studies were not performed m our patients.
Management All patients underwent at least one surgical procedure at another institution before correction of the mam anomaly by the authors. Three had a vesicostomy and three had a colostomy. One patient underwent a nephroureterectomy of a poorly functioning kidney, and another patient underwent bilateral ureteral reimplantation. Because the rectum was either normally located or minimally tmslocated, there was a normal-appearing anal canal and a normalsphincteric mechanism (except in one patient with a severe sacral anomaly). and thus we assumed that the patients would have bowel control, as long as a perirectal dissection was avoided. This subsequently proved true, except m the patient with a severely dysplastic sacrum. Therefore. special emphasis was placed on not mobilizing or dissecting the rectum. All patients were operated on through a posterior sagittal transanorectal approach.
Surgical Technique
Fig 2. Perineum of a patient with Urogenital sinus opens into the anterior not seen in this picture.
a posterior rectal wall
cloaca type and therefore,
A. is
A posterior sag&al transanorectal approach was performed following similar prmciples previously established.“.‘2 Through a posterior midsagittal incision the entire bowel sphincteric mechanism posterior to the rectum was divided. The strength of the muscle contraction at both sides of the midline was frequently tested with an electrical stimulator
POSTERIOR
CLOACA
to be sure that the incision was located exactly in the nudline. The posterior rectal wall was opened. A Weitlander retractor was used to expose the rectal lumen. In those cases in which the urogenital sinus opened in the anterior rectal wall, the orifice communicating with the urogenital sinus was clearly exposed. Multiple 5-O silk sutures were. placed circumferentially in the edge of the sinus to exert uniform traction and facilitate its dissection. A circumferential incision was made around the sinus orifice, and the anterior rectal wall was divided. including the sphincteric mechanism located anterior to the rectum. The incision through the rectum was extended anteriorly through the permeum to reach the pubis symphysis. The entire urogenital sinus was dissected, mobilized, and switched forward to be placed immechately behind the pubic bone as in normal individuals. For this, it was essential to perform a dissection around the entire urogenital Smus (urethra and vagina together) while applying traction on the multiple silk stitches. The dissection was camed out until there was adequate length to allow the vagina to be sutured to the perineum without tension (Fig 4). A new vulva was created leaving a remarkably normal-looking appearance. With the help of the electrical stimulator, the rectal sphmcteric mechanism was meticulously reconstructed, anterior and posterior to the rectum, as were the anterior and posterior walls of the rectum. In those patients who presented with two orifices in the perineum (Type B, Fig 3), the operation was started by placing multiple 6-O silk sutures around the edge of the urogenital sinus to apply a uniform traction (Fig 5). The dissection of the urogenital sinus can be perfonned through the perineum before the opening of the rectum (Fig 6). Conceivably in a case of a low confluence of urethra and vagina (short
r.:\
Posterior
limit Fig 5. lntraoperative Multiple silk stitches sinus for traction.
picture had been
showing a type B posterior cioaca. placed at the edge of the urogenital
common channel), this amount of dissection could be enough to a&eve a satisfactory reconstruction. However. in all the cases presented here, it was necessary to dissect the urogenital sinus much more, and for that it was necessary to spht the anorectum (transanorectal approach, Fig 7). The dissection of the urogenital Smus is completed until the vagina reaches the perineum, and the surgeon can create a new urethra and vaginal opemngs (Figs 8 and 9). The perineal body. anterior and posterior rectal walls, and sphincter mechanism are meticulously reconstructed. The first five patients received a protective colostomy before the main repair. The last four patients were operated on without a colostomy. They were instead subjected to a strict preoperative total bowel preparation with balanced electrolyte solution given through a nasogastric tube. In ad&ion, a central venous line was Inserted dunng the operation to administer parenteral nutrition for 7 to 10 days, and intravenous antibiotics for 72 hours. All patients recovered uneventfully and there were no cases of wound infection. In the three cases with an accessory urethra in the tip of the pseudophallus. this structure was resected because It had a diameter of about 1 to 2 mm, which was considered functionally inadequate
RESULTS
Fig 4. lntraoperative picture showing the urogenital sinus completely mobilized and sutured in normal position. The rectum is shown here split into two halves.
Postoperative evaluation was performed only clinically. Seven patients are able to have voluntary bowel movements. Voluntary bowel movements are defined as the capacity to verbalize the desire to have a bowel movement and to hold the stool until reaching the toilet and evacuating the rectum voluntarily. One patient was lost to follow-up and one patient with a very dysplastic
410
PENA AND
KESSLER
impression of dealing with two separate conditions. We look forward to a better term that includes both variants. Concerning the embryopathogenesis of these anomalies, we were unable to find a plausible explanation. We asked Dr Dietrich Kluth, a well-known authority on the field, about a speculative explanation without a positive answer. The initial diagnosis can be missed in cases of posterior cloaca as exemplified by our first patient.2 This is because of the fact that in some cases the external genitalia look almost normal. It is only after the labia of the external genitalia are manually separated that one can appreciate that there is no urethra and no vaginal opening. In most cases, however, the perineum is clearly abnormal (Figs 2 and 3). In our review of the English-language literature, we were unable to find publications specifically referring to this entity. However, a careful review of the anatomic descriptions of Hendren’s cases3-loconvinced us that he has been treating patients in at least 10 similar cases. In those publications, we found descriptions of eight patients suffering from what we call type B posterior cloaca (two orifices in the perineum, urogenital sinus opening Fig 6. urogenital
lntraoperative sinus before
picture showing the transanorectal
the initial incision.
dissection
of the
sacrum is fecally incontinent. One of these patients suffers from occasional soiling; the remaining six never soiled. Six patients are toilet trained for urine. One patient suffers from some urine dribbling in between voluntary voiding episodes. One patient with a very dysplastic sacrum has a continent diversion. DISCUSSION
We created the term posterior cloaca to emphasize the fact that the single orifice or the urogenital sinus opening is located posteriorly next to the anus or into the rectum. In a typical cloaca, on the other hand, the single orifice is located where the urethra normally opens. A single perineal opening at the anal site or a little anterior to it is called by us posterior cloaca type A. The finding of two perineal orifices, one being the rectum and a second one being the urogenital sinus located immediately anterior to the anus, was arbitrarily called posterior cloaca type B. Perhaps this latter type of defect should be called posterior urogenital sinus because strictly, it is not a cloaca. However, in looking at the characteristics and associated defects of both groups (A and B), it certainly looks as though they are all part of the same spectrum. We are aware of the controversial potential of these denominations. Using the name posterior cloaca for the type A and posterior urogenital sinus for type B would be better from the descriptive point of view but also could give the
Fig 7. lntraoperative picture showing the dissection of the urogenital sinus after the rectum has been divided longitudinally in the same patient shown in Fig 6.
POSTERIOR
411
CLOACA
Fig 8. lntraoperative view. The urogenital sinus has been fully mobilized and switched forward. The urethra is now located approximately 1.5 cm posterior to the clitoris, and the vaginal wall will be sutured to the labia without tension.
posteriorly, very close to the anus, and this last one minimally anteriorly mislocated). Three of these patients also had an accessory urethra. In addition, we also found in Hendren’s reports two cases anatomically consistent with what we called posterior cloaca type A (single orifice located where the anus normally opens or slightly anterior) and one of them with an accessory urethra. Before 1986 Hendren3-5 repaired cloaca1 defects by using abdominal and perineal approaches and applying different technical variants depending of the specific type of defect. Since 19866-10Hendren included the posterior sagittal incision as part of his approach to cloacas. In cases of patent rectum he was able to repair those defects without splitting the rectum. We present this maneuver as another alternative that we think improves the exposure. The transanorectal approach does not compromise bowel control as shown in experimental animals in our laborat0l-p and as demonstrated by our previous clinical experience.i1*i2 Long-term evaluation of results is not yet available. This approach also provides excellent exposure and can be particularly useful in cases of a high vaginal confluence. We are aware of the fact that most urogenital
sinuses can be repaired without splitting the rectum or else dividing only the anterior part of the sphincter with or without the anterior rectal wall. However, we also feel that surgeons dealing with this kind of problem should consider our approach as another alternative to higher vaginal confluences. Figure 6 is meant to illustrate the fact that the operation can be started in the perineum, giving the patient the opportunity to have her defect repaired without dividing the rectum. In the particular case illustrated in that figure. the reader can see that the exposure becomes limited. Figure 7 shows the improved exposure after the rectum has been divided. Conceivably, some cases could be repaired by using only an incision anterior to the rectum as shown in Fig 6. In cases of typical persistent cloaca, the malformation is repaired via the posterior sagittal approach.’ During the repair, the rectum is dissected and separated from the urogenital sinus, and then the rectum is switched back to be placed within the limits of the sphincteric mechanism. In cases of posterior cloaca, however, we propose to leave the rectum in place and to approach the urogenital sinus through a sagittal incision through the rectum. The sacrum, which has important prognostic implications in terms of fecal and urinary control, was very dysplastic in one patient, and as expected, that patient suffers from fecal and urinary incontinence. Urologic anomalies were present in 88% of these patients, which represents a frequency similar to the one we observed in cloacas.t4 We describe an unusual group of patients with posterior cloaca. The single specific finding in this group is the normally or almost normally located anus and rectum into which the urogenital sinus opens. The diagnosis requires a suspicious observer, particularly in those cases with normal labia. The female genitalia must routinely be
Fig 9. midsagittal
Diagram section.
showing
the
completed
operation
seen
in
a
PENA AND
412
meticulously examined in newborns. The clinical diagnosis of a posterior cloaca mandates a complete urologic evaluation based on the high incidence of urologic defects found in our series. We believe that during the repair of these defects the
KESSLER
rectum must remain in place. The transanorectal appreach is presented here as another alternative to improve the exposure. while approaching the urogenital sinus, particularly when dealing with very high vaginal confluences or in cases of posteriorly located urogenital sinuses.
REFERENCES 1. Pefia A: The surgical management of persistent cloaca: Results in 54 patients treated wtth the posterior sagittal approach. .I Pediatr Surg 24:590-598, 1989 2. Leditschke JF, Peiia A: An unusual cloaca1 anomaly-Late recognitron and operative correction. Pediatr Surg Int 6:52-55. 1991 3. Hendren WH: Surgical management of urogenital smus abnormalities. J Pediatr Surg 12:339-357, 1977 4. Hendren WH: Urogenital sinus and anorectal malformation: Experience with 22 cases. .I Pediatr Surg 15:628-641. 1980 5. Hendren WH: Further experience in reconstructive surgery of cloaca1 anomalies. J Pediatr Surg 17:695-717, 1982 6. Hendren WH: Repair of cloaca1 anomahes: Current techniques. J Pediatr Surg 21:1159-1176. 1986 7. Hen&en WH: Urological aspects of cloaca1 malformations. J Urol 140:1207-1213, 1988 8. Jaramtllo D, Lebowitz RL, Hendren WH: The cloacal malforma-
tion: Radiologic findings and imaging recommendations. Radiology 177:441-449, 1990 9. Hendren WH: Cloacal malformations: Experience with 105 cases. J Pediatr Surg 27:890-901, 1992 10. Hendren WH: Urogemtal sinus and cloacal malformations. J Pelvic Surg 1:149-160, 1995 11. Pefia A, Filmer B: Urogenital smus surgery in pediatric patients. Infections in Urology. May/June 1993. pp 87-93 12. Pefia A, Filmer B. Bomlla E, et al: Transanorectal approach for the treatment of urogenital sinus: Preliminary report. J Pediatr Surg 27:681-685, 1992 13. Pefia A, Amroch D, Baeza C, et al: The effects of the posterior sagittal approach on rectal function (experimental study). J Pediatr Surg 28:773-778, 1993 14. Rich MA, Brock WA. Peria A: Spectrum of genitourmaty malformations m patients with imperforate anus. Pediatr Surg Int 3:110-113,1988
VOL 33, NO 3
Posterior
Cloaca:
By Albert0
Peiia
A Unique and
Oded
1998
Defect
Kessler
New Hyde Park, New York and Bronx,
New York
The authors present nine cases of a unique defect in which urethra and vagina are fused together forming a urogenital sinus that deviates posteriorly and opens In the anterior rectal wall at the anus or immediately anterior to it. The rectum is essentially normal or may be minimally anteriorly mislocated. The authors call this defect posterior cloaca. The diagnosis requires a suspicious observer and a meticulous examination of the female genitalia. A complete urologic evaluation is mandatory because 88% of these patients have important associated urologic defects. Our patients were operated on via a posterior sagittal transanorectal approach,
which allowed a full dissection and mobilization of urethra and vagina together to be placed in a normal location. Rectal dissection and mobilization was avoided to preserve bowel control. Seven patients enjoy bowel and urinary control. One patient was lost to follow-up, and one patient with a severely dysplastic sacrum suffers from fecal and urinary incontinence. J Pediatr Surg 33:407-412. Copyright o 1998 by W.B. Saunders Company.
A
and the patients and their parents were interviewed. The mean age at operation was 36 months (range, 9 months to 9 years). Six patients were younger than 3 years of age at the time of operation Five patlents had a colostomy done very early in life. Therefore, we knew about preoperative bowel control in only two patients: they were totally continent. The follow-up ranges from 2 months to 8 years with an average of 4 years.
TYPICAL CLOACA is a defect in which urethra, vagina, and rectum fuse and form a common channel that opens as a single orifice where the urethra is normally located.’ The malformation that we present is unique in that the urogenital sinus deviates posteriorly and opens in the anterior rectal wall, at the anus, or immediately anterior to it. Therefore, we call it a posterior cZoaca. An important feature of this defect is that the rectum is essentially normal or may be slightly anteriorly mislocated. Since our first publication regarding this condition,2 eight additional patients with this rare condition have been operated on by us. Because of its unique anatomic characteristics, repairing this defect requires special surgical maneuvers to achieve good functional results. In our review of the English-language literature, we were unable to find any previous publications specifically related to this defect as a separate entity. However, in Hendren’s publications,3-10 one can read descriptions of at least 10 cases with anatomic characteristics of what we call posterior cloaca. MATERIALS
AND
METHODS
Smce 1982. 170 primary cases of persistent cloaca have been operated on by the senior author. From these, nine cases were selected for review because they shared the anatomic features consistent with what we call posterior cloaca. The clinical and radiological records of the patients were reviewed, JournalofPediatric
Surgery,
Vol33,
No 3 (March),
1998: pp 407-412
INDEX
Anatomy
WORDS:
Posterior
and Description
cloaca,
urethral
vaginal
fusion.
of the Defect
Figure 1 is a diagram of the anatomy of a female patlent with a posterior cloaca. The most conspicuous anatomic features of a posterior cloaca mclude a well-located or minimally anteriorly mislocated rectum. Both urethra and vagina form a urogenital sinus that is postenorly deviated and opens in the anterior rectal wall, in the anus, or in the permeum immechately antenor to the anus. The external appearance of the permeum in these girls is bizarre because they may have labia but no urethral or vaginal opening. Three anatomic variants of this defect were found: (1) The urogenital Smus opens into the anterior rectal wall or mto the anus and is visible only endoscopically (Figs 1 and 2, five cases). (2) The urogenital Smus opens mto the perineum immediately anterior to the anus (Fig 3, four
Fmrn the Dwzsion of Pediatric Surgery, Departments of Surgery and Urology, Long Island Jewish Medical Centel; New Hyde Park, NY and the Clmical Campus of the Albert Emstein College of Medicme, Bronx, NY Address reprint requests to Albert0 Peiia, MD, Chief; Division oj Pediatric Surgev, Schneider Children’s Hospital, Long Island Jewish Medical Centel; 269-01 76th Ave, New Hyde Park, NY 11040. Copyright 0 1998 by WB. Saunders Company 0022.3468/98/3303-0001$03.00/0
407
408
PENA AND
Normally
Fig 1. posterior
Diagram cloaca.
shows
KESSLER
focafed-anus
a sagittal
view
of the anatomy
of a case of a
cases). (3) The urogemtal sinus opens either in the rectum or the perineum and there is a rudimentary urethra opening at the tip of a pseudophallus (three cases).
Associated Defects Seven patients had associated defects that mcluded uterine septation disorders (hemivagmas and hemmten),
vagmal and four patients:
Fig 3. Urogenital the anus.
Perineum of sinus opens
a patient with a posterior cloaca type into the perineum, immediately anterior
B. to
hydrocolpos, four patients; vesicoureteral reflux, three patients; hydronephrosis, four patients; absent kidney, three patients; esophageal atresia and tracheoesophageal fistula, two patlents: thoracic hermvertebrae, two patients; ureteral duplication, one patient; ventricular septal defect, one patient; malrotation, one patient; bladder diverticulum. one patient; and ureteropelvic obstruction, one patient. Chromosomal studies were not performed m our patients.
Management All patients underwent at least one surgical procedure at another institution before correction of the mam anomaly by the authors. Three had a vesicostomy and three had a colostomy. One patient underwent a nephroureterectomy of a poorly functioning kidney, and another patient underwent bilateral ureteral reimplantation. Because the rectum was either normally located or minimally tmslocated, there was a normal-appearing anal canal and a normalsphincteric mechanism (except in one patient with a severe sacral anomaly). and thus we assumed that the patients would have bowel control, as long as a perirectal dissection was avoided. This subsequently proved true, except m the patient with a severely dysplastic sacrum. Therefore. special emphasis was placed on not mobilizing or dissecting the rectum. All patients were operated on through a posterior sagittal transanorectal approach.
Surgical Technique
Fig 2. Perineum of a patient with Urogenital sinus opens into the anterior not seen in this picture.
a posterior rectal wall
cloaca type and therefore,
A. is
A posterior sag&al transanorectal approach was performed following similar prmciples previously established.“.‘2 Through a posterior midsagittal incision the entire bowel sphincteric mechanism posterior to the rectum was divided. The strength of the muscle contraction at both sides of the midline was frequently tested with an electrical stimulator
POSTERIOR
CLOACA
to be sure that the incision was located exactly in the nudline. The posterior rectal wall was opened. A Weitlander retractor was used to expose the rectal lumen. In those cases in which the urogenital sinus opened in the anterior rectal wall, the orifice communicating with the urogenital sinus was clearly exposed. Multiple 5-O silk sutures were. placed circumferentially in the edge of the sinus to exert uniform traction and facilitate its dissection. A circumferential incision was made around the sinus orifice, and the anterior rectal wall was divided. including the sphincteric mechanism located anterior to the rectum. The incision through the rectum was extended anteriorly through the permeum to reach the pubis symphysis. The entire urogenital sinus was dissected, mobilized, and switched forward to be placed immechately behind the pubic bone as in normal individuals. For this, it was essential to perform a dissection around the entire urogenital Smus (urethra and vagina together) while applying traction on the multiple silk stitches. The dissection was camed out until there was adequate length to allow the vagina to be sutured to the perineum without tension (Fig 4). A new vulva was created leaving a remarkably normal-looking appearance. With the help of the electrical stimulator, the rectal sphmcteric mechanism was meticulously reconstructed, anterior and posterior to the rectum, as were the anterior and posterior walls of the rectum. In those patients who presented with two orifices in the perineum (Type B, Fig 3), the operation was started by placing multiple 6-O silk sutures around the edge of the urogenital sinus to apply a uniform traction (Fig 5). The dissection of the urogenital sinus can be perfonned through the perineum before the opening of the rectum (Fig 6). Conceivably in a case of a low confluence of urethra and vagina (short
r.:\
Posterior
limit Fig 5. lntraoperative Multiple silk stitches sinus for traction.
picture had been
showing a type B posterior cioaca. placed at the edge of the urogenital
common channel), this amount of dissection could be enough to a&eve a satisfactory reconstruction. However. in all the cases presented here, it was necessary to dissect the urogenital sinus much more, and for that it was necessary to spht the anorectum (transanorectal approach, Fig 7). The dissection of the urogenital Smus is completed until the vagina reaches the perineum, and the surgeon can create a new urethra and vaginal opemngs (Figs 8 and 9). The perineal body. anterior and posterior rectal walls, and sphincter mechanism are meticulously reconstructed. The first five patients received a protective colostomy before the main repair. The last four patients were operated on without a colostomy. They were instead subjected to a strict preoperative total bowel preparation with balanced electrolyte solution given through a nasogastric tube. In ad&ion, a central venous line was Inserted dunng the operation to administer parenteral nutrition for 7 to 10 days, and intravenous antibiotics for 72 hours. All patients recovered uneventfully and there were no cases of wound infection. In the three cases with an accessory urethra in the tip of the pseudophallus. this structure was resected because It had a diameter of about 1 to 2 mm, which was considered functionally inadequate
RESULTS
Fig 4. lntraoperative picture showing the urogenital sinus completely mobilized and sutured in normal position. The rectum is shown here split into two halves.
Postoperative evaluation was performed only clinically. Seven patients are able to have voluntary bowel movements. Voluntary bowel movements are defined as the capacity to verbalize the desire to have a bowel movement and to hold the stool until reaching the toilet and evacuating the rectum voluntarily. One patient was lost to follow-up and one patient with a very dysplastic
410
PENA AND
KESSLER
impression of dealing with two separate conditions. We look forward to a better term that includes both variants. Concerning the embryopathogenesis of these anomalies, we were unable to find a plausible explanation. We asked Dr Dietrich Kluth, a well-known authority on the field, about a speculative explanation without a positive answer. The initial diagnosis can be missed in cases of posterior cloaca as exemplified by our first patient.2 This is because of the fact that in some cases the external genitalia look almost normal. It is only after the labia of the external genitalia are manually separated that one can appreciate that there is no urethra and no vaginal opening. In most cases, however, the perineum is clearly abnormal (Figs 2 and 3). In our review of the English-language literature, we were unable to find publications specifically referring to this entity. However, a careful review of the anatomic descriptions of Hendren’s cases3-loconvinced us that he has been treating patients in at least 10 similar cases. In those publications, we found descriptions of eight patients suffering from what we call type B posterior cloaca (two orifices in the perineum, urogenital sinus opening Fig 6. urogenital
lntraoperative sinus before
picture showing the transanorectal
the initial incision.
dissection
of the
sacrum is fecally incontinent. One of these patients suffers from occasional soiling; the remaining six never soiled. Six patients are toilet trained for urine. One patient suffers from some urine dribbling in between voluntary voiding episodes. One patient with a very dysplastic sacrum has a continent diversion. DISCUSSION
We created the term posterior cloaca to emphasize the fact that the single orifice or the urogenital sinus opening is located posteriorly next to the anus or into the rectum. In a typical cloaca, on the other hand, the single orifice is located where the urethra normally opens. A single perineal opening at the anal site or a little anterior to it is called by us posterior cloaca type A. The finding of two perineal orifices, one being the rectum and a second one being the urogenital sinus located immediately anterior to the anus, was arbitrarily called posterior cloaca type B. Perhaps this latter type of defect should be called posterior urogenital sinus because strictly, it is not a cloaca. However, in looking at the characteristics and associated defects of both groups (A and B), it certainly looks as though they are all part of the same spectrum. We are aware of the controversial potential of these denominations. Using the name posterior cloaca for the type A and posterior urogenital sinus for type B would be better from the descriptive point of view but also could give the
Fig 7. lntraoperative picture showing the dissection of the urogenital sinus after the rectum has been divided longitudinally in the same patient shown in Fig 6.
POSTERIOR
411
CLOACA
Fig 8. lntraoperative view. The urogenital sinus has been fully mobilized and switched forward. The urethra is now located approximately 1.5 cm posterior to the clitoris, and the vaginal wall will be sutured to the labia without tension.
posteriorly, very close to the anus, and this last one minimally anteriorly mislocated). Three of these patients also had an accessory urethra. In addition, we also found in Hendren’s reports two cases anatomically consistent with what we called posterior cloaca type A (single orifice located where the anus normally opens or slightly anterior) and one of them with an accessory urethra. Before 1986 Hendren3-5 repaired cloaca1 defects by using abdominal and perineal approaches and applying different technical variants depending of the specific type of defect. Since 19866-10Hendren included the posterior sagittal incision as part of his approach to cloacas. In cases of patent rectum he was able to repair those defects without splitting the rectum. We present this maneuver as another alternative that we think improves the exposure. The transanorectal approach does not compromise bowel control as shown in experimental animals in our laborat0l-p and as demonstrated by our previous clinical experience.i1*i2 Long-term evaluation of results is not yet available. This approach also provides excellent exposure and can be particularly useful in cases of a high vaginal confluence. We are aware of the fact that most urogenital
sinuses can be repaired without splitting the rectum or else dividing only the anterior part of the sphincter with or without the anterior rectal wall. However, we also feel that surgeons dealing with this kind of problem should consider our approach as another alternative to higher vaginal confluences. Figure 6 is meant to illustrate the fact that the operation can be started in the perineum, giving the patient the opportunity to have her defect repaired without dividing the rectum. In the particular case illustrated in that figure. the reader can see that the exposure becomes limited. Figure 7 shows the improved exposure after the rectum has been divided. Conceivably, some cases could be repaired by using only an incision anterior to the rectum as shown in Fig 6. In cases of typical persistent cloaca, the malformation is repaired via the posterior sagittal approach.’ During the repair, the rectum is dissected and separated from the urogenital sinus, and then the rectum is switched back to be placed within the limits of the sphincteric mechanism. In cases of posterior cloaca, however, we propose to leave the rectum in place and to approach the urogenital sinus through a sagittal incision through the rectum. The sacrum, which has important prognostic implications in terms of fecal and urinary control, was very dysplastic in one patient, and as expected, that patient suffers from fecal and urinary incontinence. Urologic anomalies were present in 88% of these patients, which represents a frequency similar to the one we observed in cloacas.t4 We describe an unusual group of patients with posterior cloaca. The single specific finding in this group is the normally or almost normally located anus and rectum into which the urogenital sinus opens. The diagnosis requires a suspicious observer, particularly in those cases with normal labia. The female genitalia must routinely be
Fig 9. midsagittal
Diagram section.
showing
the
completed
operation
seen
in
a
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meticulously examined in newborns. The clinical diagnosis of a posterior cloaca mandates a complete urologic evaluation based on the high incidence of urologic defects found in our series. We believe that during the repair of these defects the
KESSLER
rectum must remain in place. The transanorectal appreach is presented here as another alternative to improve the exposure. while approaching the urogenital sinus, particularly when dealing with very high vaginal confluences or in cases of posteriorly located urogenital sinuses.
REFERENCES 1. Pefia A: The surgical management of persistent cloaca: Results in 54 patients treated wtth the posterior sagittal approach. .I Pediatr Surg 24:590-598, 1989 2. Leditschke JF, Peiia A: An unusual cloaca1 anomaly-Late recognitron and operative correction. Pediatr Surg Int 6:52-55. 1991 3. Hendren WH: Surgical management of urogenital smus abnormalities. J Pediatr Surg 12:339-357, 1977 4. Hendren WH: Urogenital sinus and anorectal malformation: Experience with 22 cases. .I Pediatr Surg 15:628-641. 1980 5. Hendren WH: Further experience in reconstructive surgery of cloaca1 anomalies. J Pediatr Surg 17:695-717, 1982 6. Hendren WH: Repair of cloaca1 anomahes: Current techniques. J Pediatr Surg 21:1159-1176. 1986 7. Hen&en WH: Urological aspects of cloaca1 malformations. J Urol 140:1207-1213, 1988 8. Jaramtllo D, Lebowitz RL, Hendren WH: The cloacal malforma-
tion: Radiologic findings and imaging recommendations. Radiology 177:441-449, 1990 9. Hendren WH: Cloacal malformations: Experience with 105 cases. J Pediatr Surg 27:890-901, 1992 10. Hendren WH: Urogemtal sinus and cloacal malformations. J Pelvic Surg 1:149-160, 1995 11. Pefia A, Filmer B: Urogenital smus surgery in pediatric patients. Infections in Urology. May/June 1993. pp 87-93 12. Pefia A, Filmer B. Bomlla E, et al: Transanorectal approach for the treatment of urogenital sinus: Preliminary report. J Pediatr Surg 27:681-685, 1992 13. Pefia A, Amroch D, Baeza C, et al: The effects of the posterior sagittal approach on rectal function (experimental study). J Pediatr Surg 28:773-778, 1993 14. Rich MA, Brock WA. Peria A: Spectrum of genitourmaty malformations m patients with imperforate anus. Pediatr Surg Int 3:110-113,1988