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Optimal management of cloacal exstrophy
Optimal Management of Cloacal Exstrophy By C Howell, A Caldamone, H Snyder, M Ziegler, and J Duckett
Philadelphia, Pennsylvania 9 Classic management of cloacal e x s t r o p h y has emphasized primary closure of the omphalocele, and ileostomy, and delayed genitourinary reconstruction. The resultant m o r t a l i t y f r o m " s h o r t - b o w e l synd r o m e " and urinary sepsis, and m o r b i d i t y f r o m inappropriate gender assignment has p r o m p t e d us to r e e x a m i n e the operative approach t o this problem. Our experience involves 15 cases f r o m 2 days to 21 years after repair. All had initial closure of their omphalocele. Nine of t h e 15 had closure of the vasicointestinal fistula w i t h preservation of t h e distal colon segment and creation of an end colostomy; 2 had an initial ileostomy w i t h later conversion t o an end colostomy; and 2 have p e r m a n e n t ileostomies. Nine of t h e 15 w o r e genetic males; six w e r e conv e r t e d t o female, five during the n e w b o r n period and one at 9 months. The hemibladders w e r e approximated in 13 of 15 and t w o had a p r i m a r y turn-in of the exstrophied bladder. Three had bladder closure w i t h iliac osteotomies delayed beyond t h e n e w b o r n period. Urinary diversion has been utilized in 11 patients, 7 ileal conduits, 1 vesicostomy, 2 cutaneous ureterostomies, and 2 t e m p o r a r y pyalostomias; only one patient remains w i t h an exstrophied bladder. Three patients w i t h closure of the bladder remain incontinent. Of our patients, 13 of 15 are alive and well. One died at age 2 days because of renal agenesis and one was 19 years old w h e n found dead of an u n k n o w n cause. There have been no deaths due t o short-bowel syndrome or sepsis, and no patients have had upper urinary-tract deterioration. These results p r o m p t us t o recommend t h e following t h e r a p y for cloacal exstrophy: (1) early closure of the omphalocele; (2) closure of the vesicointestinal fistula w i t h creation of an end colostomy at the distal end of the blind pouch; (3) r e a p p r o x i m a t i o n of t h e hemibladders; and (4) gender assignment t o female. Subsequent procedures in the stable infant would include bladder closure and later reconstruction for continence if the bladder is suitable or appropriate p e r m a n e n t diversion. INDEX WORDS: Cloacal e x s t r o p h y ; vesicointastinal fissure; short-bowel syndrome; omphalocoele.
TO A REVIEW of cloacal exstrophy p RIOR in 1960 by Rickham, ] infants with this devastating problem were usually left to die. Rickham's recommendation that total correction of the infant's problem should be attempted was supported in 1965 when Spencer 2 summarized 47 cases of cloacal exstrophy and recommended that infants with this problem be given an opportunity to survive. Occasional references3 have Journal of PediatricSurgery, Vol. 18, No. 4 (August), 1983
appeared suggesting that the expense incurred in the management of these problems may outweigh the potential for ultimate survival and imply that no therapy is indicated. Despite these negative reports, in general, cloacal exstrophy is considered a major operative challenge to modern pediatric surgery and is now accepted as a tragic but potentially salvageable problem. The surgical management of this complex problem is clearly variable. 4-s Prompted by results reported from various institutions~-8 in regard to urinary sepsis, short-bowel syndrome, ileostomy management, and inappropriate gender assignment, we have reviewed our experience with this problem. Furthermore, our most recent cases have encouraged us to preserve intestinal length at all costs, to reapproximate the symphysis pubis during the initial operative intervention and to reapproximate the hemibladders allowing for a more secure abdominal-wall closure either primarily or with temporary prosthetic material. MATERIALS A N D METHODS A g e , Sex, a n d Birth W e i g h t In this retrospective review the age range of these 15 patients was from two days to 21 years. The average followup was 91/2years. Six of the patients now range from 10 to 21 years of age. There were nine genotypic males and six genotypic females in this group of patients. Female gender assignments were given to 6 of the genotypic males, five in the newborn period and one at 9 months of age. The average weight of 10 of these 15 infants was 2.1 kilograms (five unknown).
Management of Omphalocele All 15 patients had an omphalocele at the time of presentation. In all but two, the omphalocele was closed at the time of
From the Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Penn. Presented before the 31st Annual Meeting of the Surgical Section of the American Academy of Pediatrics, New York, New York, October 23-24, 1982. Address reprint requests to CG Howell, MD, Division of Pediatric Surgery, Medical College of Georgia, Augusta, GA 30912, (formerly the Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA). 9 1983 by Grune & Stratton, Inc. 0022-3468/83/1804~)006/$01.00/0 365
HOWELL ET AL
366 Table 1. Management of Bowel Anomalies
Table 2. Types of Urinary Anomalies No. of
No. of
Anomaly Anoplasty Anteriorly placed anus Ineostomy Permanent ileostomy with colon resection (newborns--done elsewhere) Temporary Ileostomy (done elsewhere) (converted to colostomies at chop) Colostomy Closure of bowel exstrophy End colostomy (microcolon) Duplicated colon (both used) ( 1) Total Patients
Anomaly
Patients 2
2
2
9
15
presentation. Two had a ruptured omphalocele and one of these was a craniopagus Siamese twin. Thirteen of the omphaloceles were closed primarily while two required a temporary prosthetic pouch with subsequence closure.
Management of Intestine Table ] summarizes the management of the intestinal anomalies. Of the 15 patients two had an anteriorly placed anus which now functions satisfactorily after an anoplasty. Two, operated on elsewhere, had permanent ileostomies in the newborn period with resection of the colon. Nine patients had an initial permanent end colostomy with primary reconstruction of the exstrophied ileocecal area. Two patients had an initial ileostomy done elsewhere and developed symptoms of short-bowel syndrome with subsequent malnutrition. We successfully converted them to a permanent end colostomy by identifying and salvaging the blind colon segment. In the operative procedure for conversion of one of these two infants, the colon was found to be duplicated and short. Therefore, the two ends of the colon were anastomosed in continuity (one in an isoperistaltic fashion and one with retrograde peristalsis) and brought out as an end colostomy. None of our 15 patients had evidence of congenital short intestine and none of our patients had an acquired short-bowel syndrome from iatrogenie resections. Ileostomy management problems were, however, present in two patients with permanent ileostomies. Both of these patients were plagued with intermittent diarrhea and chronic malnutrition. The two patients that were converted from temporary ileostomies to a permanent end colostomy both had resolution of their ileostomy management problems, subsequent weight gain and the development of formed stools.
Findings of the Genitourinary Tract Table 2 summarizes the specific urinary anomalies identified in this review. Thirteen of the 15 patients were found at presentation to have the classic hemibladder on each side of the exstrophied bowel. Two patients had a single exstrophied bladder off to one side. In 4 of the 15 patients, agenesis of the left kidney was also identified, 4 had crossed ectopia, 1 had
Split-Bladder exstrophy Single bladder exstrophy Bilateral renal agenesis Agenesis left kidney Crossed Fused-Ectopia Hydro Lower Pole ( 1) Ectopic to Vagina ( 1) Megaureters Cystic Kidney ( 1)
Patients 13 2 1
4 4
5
bilateral renal agenesis, and 1 had severe left ureterovesical obstruction with a hydronephrotic left kidney. Of the patients with ectopia, one had a solitary right kidney with a duplication of the ureters with a hydroureteronephrosis of the lower pole to the right hemibladder with a crossed ectopia of the right upper pole to the left hemibladder. The other patient had a right double collecting system and hydronephrosis with the ureter ectopic to the vagina and the left upper pole ureter traversing to the opposite bladder. Initial and subsequent management of the urinary anomalies is outlined in Table 3. Separation of the bowel from the bladder and reapproximation of the hemibladders was initially performed in 13 patients. In two patients a primary closure of the bladder exstrophy was accompanied. Three patients had later bladder closure with lilac osteotomies. Of the patients undergoing GU tract reconstruction, seven have had ileal loop diversion. Four patients were managed by temporary cutaneous pyelostomy (three renal units) or ureterostomy (four units). Only one subsequent nephrectomy was required. One closed bladder had to be converted to a vesicostomy. Three other patients who underwent bladder closure remain incontinent, one despite a Young-DeesLedbetter procedure. One recent patient has had reapproximation of the bladder halves only, and further management is still pending.
Findings of the Genitalia Of the 15 patients, 9 were genetic males and 6 were genetic females. All males had severe anomalies of the genitalia including diminutive and separated corporal bodies, bifid scrotum, and often, undescended testes. Today, all males with Table 3. GU-Tract Management Initial Management Reapproximation of hemibladders
Primary Closure Exstrophy
No. of Patients
13 2
Later Management Ileal Conduits Vesicostomy
7 1
Temporary Cutaneous Pyelostomies (4) Cutaneous Ureterostomy Bladder Closure
2 3
(lliac Osteotomies)
Bladder Closure ( 1~)
2
CLOACAL EXSTROPHY
367
Table 4. Associated Anomalies
Anomaly Myelomeningocoele Thoracic ( 1) Sacral ( 1) Lumbar (5) Scoliosis Bilateral Club Feet Siamese Twin Left Facial Paralysis Factor 8 Deficiency Atretic Right Upper Lobe Bronchus
No. of Patients
7
cloacal exstrophy are gender converted to female because of the difficulty in reconstructing a workable male phenotype. Six of the severely deformed males were later converted to females, five in the newborn period and one at 9 months of age. Five of the six females were noted to have a bifid uterus and one had a double vagina as well. Three of the six had an exstrophied vagina and two had a covered vaginal opening.
Associated Anomalies Table 4 summarizes the anomalies associated with cloacal exstrophy in this review. Eleven of the 15 patients had significant associated anomalies. Twenty-one specific anomalies were identified. Myelomeningocele, the most common associated anomaly, was present in seven of these patients. One was in the thoracic region, one in the sacral region and five in the lumbar region. Vertebral anomalies were common to all. Six patients had severe scoliosis, and four patients had .severe bilateral club feet. Also, there was one Siamese twin, one child with left facial paralysis, one with factor 8 deficiency, and one with an atretie right upper lobe bronchus. The patient with the factor 8 deficiency was a genetic male and during gender conversion in the newborn period had no hemostatic problems. At subsequent operative procedures the patient bled excessively and having been converted to a female, the diagnosis of factor 8 deficiency was unfortunately overlooked. Subsequent tests confirmed the factor 8 deficiency.
report comprise the recorded total world experience. The total number of survivors is only approximately 35, 17 in the period from 1968 to 1976, a 50% survival rate) This is in contrast to our 86% survival. There has been general agreement regarding primary or prosthetic closure of the omphalocele and approximation of the hemibladders. In almost all series, including our own, this appears to have been accomplished successfully even in cases of ruptured omphalocele. Management of the intestine as well as the genitourinary tract and the genitalia has yet to reach a consensus. An understanding of the anatomical abnormality is vital to the proper surgical reconstruction. This complex anomaly involves both the intestinal and genitourinary tracts. The classic cloacal exstrophy consists of: exstrophic bowel of the ileocecal region, imperforate anus, two exstrophic hemibladders, omphalocele in approximately 90% of the cases, vertebral anomalies, and anomalies of external genitalia. Less commonly seen are anomalies of the upper urinary tract and lower extremities. A diagram of the usual findings is depicted in Fig. 1. As early as two decades ago Rickham ~emphasized the need to salvage the entire intestine of infants with cloacal exstrophy to prevent the devastating problems of ileostomy management. Early in our series of patients the creation of an ileostomy was associated with this problem and these two patients were subsequently converted to an end colostomy with resolution of their symptoms. Our most recent patient was referred for evaluation of failure to thrive after an ileosto-
Current Status Thirteen of these patients are alive and doing well (86% survival). One patient died in the newborn period of renal agenesis and one died at age 19, a probable suicide. The nutritional status of ten of these patients was noted to be good. The two patients with permanent ileostomies have been ileostomy cripples with marginal weight gains and severe diarrhea with associated viral illnesses in childhood. The two patients with temporary ileostomies who were done elsewhere were converted to permanent colostomies with the resolution of their diarrhea, inability to gain weight, and chronic malnutrition. None of these patients had a congenital short intestine. They did, however, behave similar to the patient with short-bowel syndrome and an ileostomy.
Smaller/ intesti-ne~ / ~X~ Appendix~ ~ Right ~ hemibladder- .
~
~'~
~
Exomphalos '
~ ~ ~ ~ ~ ~ - ~ ~
Distal/ bowel ...
~ Proxima bowel orifice Appendix
Left
hemibladder
~~ r" I \ Bhnd . large /
DISCUSSION
Since the report by Littre9 in 1709 of the first case of cloacal exstrophy, 157 cases prior to this
Fig. 1.
A n a t o m y of a classic cloacal exstrophy.
368
HOWELL ET AL
my. Closure of her ileocecal plate and ileostomy with conversion to an end colostomy by anastomosing the ileum to the residual blind-ending colon restored her to a normal nutritional status. Even today, however, there are those 4'5'6 who recommend ileostomy for the initial management of the intestine with subsequent maintenance on long-term hyperalimentation and enteral feedings until adaptation of the small intestine occurs. These authors 4'5"6 desire to salvage portions of the colon for reconstruction of the genitourinary tract as well as the genitalia. We do not feel these considerations take precedence over the prevention of life-threatening short-bowel syndrome, a problem in approximately 50% of the patients in some series. In our experience this can be avoided by closing the vesicointestinal fissure and preserving the distal colon. More than a decade ago Tank and Lindenauer recommended conversion of males to females rather than trying to reconstruct inadequate genitalia into a workable male phenotype. Our series supports this approach. The three reconstructed males were left with inadequate genitalia; one probably committed suicide. Subsequent patients 6 have all been converted to females in early life. Still, despite recommendations 4'5 that most if not all of these infants should be reared as females, there are vague recommendations t~ for assessing potential penile length implying that reconstruction in the male phenotype is desirable. We strongly believe it would be rare to find a genetic male that should not be converted to a female phenotype. It is tragic to "condemn" a boy to life with inadequate genitalia. Management of the urinary tract should begin with a primary separation of the bowel from the bladder plate and a joining of the two bladder halves. The child may then be followed until her general condition indicates that she can tolerate further surgery. Closure of the exstrophied blad-
der will permit the option of later attempts to achieve continence with clean intermittent catheterization. In virtually all cases this will require additional surgery and may require intestinal augmentation of the bladder, tl We do not feel bowel should be so used unless the child can be shown--usually by the passage of formed stools--to have sufficient bowel to spare. Cutaneous ureterostomy with a small cuff of bladder to help form a stoma is an acceptable alternative. Ileal conduit has also been a successful mode of urinary diversion. Meticulous follow-up, however, must be emphasized. In summary, we support an initial closure of the omphalocele if possible, separation of the ileocecal connection from the exstrophied bladder plate, reapproximation of the separated bladder halves, and construction of an end colostomy with preservation of all bowel. We feel strongly that the excellent survival rate in this series reflects this approach to bowel management. We support reapproximation of the symphysis pubis in the newborn period as we 12 have previously demonstrated in exstrophy of the bladder that this can be performed in the first 72 hours of life. This simple maneuver makes abdominal wall closure easier and also provides an inferior buttress for the use of prosthetic materials in children with a much more difficult abdominal wall closure. We believe that except for the obvious male, genetic males should usually be given a female gender assignment and undergo appropriate surgery early in life. Further genitourinary tract management may involve closure of the bladder and later surgery to achieve continence or establish urinary diversion. In the future the use of artificial sphincter mechanisms to establish urinary continence, and the exploration of possible intestinal continence mechanisms by the posterior sagittal anorectoplasty as described by Pefia and deVries 13 may eliminate the lifelong "double stoma."
REFERENCES
1. Rickham PO: Vesico-intestinal fissure. Arch Dis Child 35:97-102, 1960 2. Spencer RW: Exstrophia splanchnica (Exstrophyofthe cloaca). Surgery 57:751-766, 1965 3. Hayden PW, Chapman WH, Stevenson JK: Exstrophy of the cloaca. Am J Dis Child 125:879-883, 1973 4. Sukarochana K, Sieber WK: Vesicointestinal fissure revisited. J Pediatr 13:713-719, 1978
5. Welch K J, Cloacal exstrophy, in Ravitch MM, Welch K J, Benson CD, et al (eds): Pediatric Surgery (Vol II). Chicago, Year Book Medical, 1979, pp 802-808 6. Tank ES, Lindenauer SM: Principles of management of exstrophy of the cloaca. Am J Surg 119:95-98, 1970 7. Fonkalsrud EW, Linde LM: Successful management of vesicointestinal fissure: Report of two cases. J Pediatr Surg 5:309-314, 1970
CLOACAL EXSTROPHY
8. Soper RT, Kilger K: Vesico-intestinal fissure. J Urol 92:490-501, 1964 9. Littre A: Med Acad R Sci 4:9, 1709, cited by Duncan 10. Jeffs RD: Exstrophy and cloacal exstrophy. Urol Clin North Am 5:127-140, 1978 11. Arap S, Giron AM, deGoes G: Initial results of the
369
complete reconstruction of bladder exstrophy. Urol Clin North Am 7:477-491, 1980 12. Duekett JD: Newborn Exstrophy Closure, presented at the American Urological Association, 1982 13. deVries PA, Peita A: Posterior Sagittal Anorectoplasty, presented at the American Pediatric Surgical Association Meeting, Phoenix, 1982
Philadelphia, Pennsylvania 9 Classic management of cloacal e x s t r o p h y has emphasized primary closure of the omphalocele, and ileostomy, and delayed genitourinary reconstruction. The resultant m o r t a l i t y f r o m " s h o r t - b o w e l synd r o m e " and urinary sepsis, and m o r b i d i t y f r o m inappropriate gender assignment has p r o m p t e d us to r e e x a m i n e the operative approach t o this problem. Our experience involves 15 cases f r o m 2 days to 21 years after repair. All had initial closure of their omphalocele. Nine of t h e 15 had closure of the vasicointestinal fistula w i t h preservation of t h e distal colon segment and creation of an end colostomy; 2 had an initial ileostomy w i t h later conversion t o an end colostomy; and 2 have p e r m a n e n t ileostomies. Nine of t h e 15 w o r e genetic males; six w e r e conv e r t e d t o female, five during the n e w b o r n period and one at 9 months. The hemibladders w e r e approximated in 13 of 15 and t w o had a p r i m a r y turn-in of the exstrophied bladder. Three had bladder closure w i t h iliac osteotomies delayed beyond t h e n e w b o r n period. Urinary diversion has been utilized in 11 patients, 7 ileal conduits, 1 vesicostomy, 2 cutaneous ureterostomies, and 2 t e m p o r a r y pyalostomias; only one patient remains w i t h an exstrophied bladder. Three patients w i t h closure of the bladder remain incontinent. Of our patients, 13 of 15 are alive and well. One died at age 2 days because of renal agenesis and one was 19 years old w h e n found dead of an u n k n o w n cause. There have been no deaths due t o short-bowel syndrome or sepsis, and no patients have had upper urinary-tract deterioration. These results p r o m p t us t o recommend t h e following t h e r a p y for cloacal exstrophy: (1) early closure of the omphalocele; (2) closure of the vesicointestinal fistula w i t h creation of an end colostomy at the distal end of the blind pouch; (3) r e a p p r o x i m a t i o n of t h e hemibladders; and (4) gender assignment t o female. Subsequent procedures in the stable infant would include bladder closure and later reconstruction for continence if the bladder is suitable or appropriate p e r m a n e n t diversion. INDEX WORDS: Cloacal e x s t r o p h y ; vesicointastinal fissure; short-bowel syndrome; omphalocoele.
TO A REVIEW of cloacal exstrophy p RIOR in 1960 by Rickham, ] infants with this devastating problem were usually left to die. Rickham's recommendation that total correction of the infant's problem should be attempted was supported in 1965 when Spencer 2 summarized 47 cases of cloacal exstrophy and recommended that infants with this problem be given an opportunity to survive. Occasional references3 have Journal of PediatricSurgery, Vol. 18, No. 4 (August), 1983
appeared suggesting that the expense incurred in the management of these problems may outweigh the potential for ultimate survival and imply that no therapy is indicated. Despite these negative reports, in general, cloacal exstrophy is considered a major operative challenge to modern pediatric surgery and is now accepted as a tragic but potentially salvageable problem. The surgical management of this complex problem is clearly variable. 4-s Prompted by results reported from various institutions~-8 in regard to urinary sepsis, short-bowel syndrome, ileostomy management, and inappropriate gender assignment, we have reviewed our experience with this problem. Furthermore, our most recent cases have encouraged us to preserve intestinal length at all costs, to reapproximate the symphysis pubis during the initial operative intervention and to reapproximate the hemibladders allowing for a more secure abdominal-wall closure either primarily or with temporary prosthetic material. MATERIALS A N D METHODS A g e , Sex, a n d Birth W e i g h t In this retrospective review the age range of these 15 patients was from two days to 21 years. The average followup was 91/2years. Six of the patients now range from 10 to 21 years of age. There were nine genotypic males and six genotypic females in this group of patients. Female gender assignments were given to 6 of the genotypic males, five in the newborn period and one at 9 months of age. The average weight of 10 of these 15 infants was 2.1 kilograms (five unknown).
Management of Omphalocele All 15 patients had an omphalocele at the time of presentation. In all but two, the omphalocele was closed at the time of
From the Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Penn. Presented before the 31st Annual Meeting of the Surgical Section of the American Academy of Pediatrics, New York, New York, October 23-24, 1982. Address reprint requests to CG Howell, MD, Division of Pediatric Surgery, Medical College of Georgia, Augusta, GA 30912, (formerly the Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA). 9 1983 by Grune & Stratton, Inc. 0022-3468/83/1804~)006/$01.00/0 365
HOWELL ET AL
366 Table 1. Management of Bowel Anomalies
Table 2. Types of Urinary Anomalies No. of
No. of
Anomaly Anoplasty Anteriorly placed anus Ineostomy Permanent ileostomy with colon resection (newborns--done elsewhere) Temporary Ileostomy (done elsewhere) (converted to colostomies at chop) Colostomy Closure of bowel exstrophy End colostomy (microcolon) Duplicated colon (both used) ( 1) Total Patients
Anomaly
Patients 2
2
2
9
15
presentation. Two had a ruptured omphalocele and one of these was a craniopagus Siamese twin. Thirteen of the omphaloceles were closed primarily while two required a temporary prosthetic pouch with subsequence closure.
Management of Intestine Table ] summarizes the management of the intestinal anomalies. Of the 15 patients two had an anteriorly placed anus which now functions satisfactorily after an anoplasty. Two, operated on elsewhere, had permanent ileostomies in the newborn period with resection of the colon. Nine patients had an initial permanent end colostomy with primary reconstruction of the exstrophied ileocecal area. Two patients had an initial ileostomy done elsewhere and developed symptoms of short-bowel syndrome with subsequent malnutrition. We successfully converted them to a permanent end colostomy by identifying and salvaging the blind colon segment. In the operative procedure for conversion of one of these two infants, the colon was found to be duplicated and short. Therefore, the two ends of the colon were anastomosed in continuity (one in an isoperistaltic fashion and one with retrograde peristalsis) and brought out as an end colostomy. None of our 15 patients had evidence of congenital short intestine and none of our patients had an acquired short-bowel syndrome from iatrogenie resections. Ileostomy management problems were, however, present in two patients with permanent ileostomies. Both of these patients were plagued with intermittent diarrhea and chronic malnutrition. The two patients that were converted from temporary ileostomies to a permanent end colostomy both had resolution of their ileostomy management problems, subsequent weight gain and the development of formed stools.
Findings of the Genitourinary Tract Table 2 summarizes the specific urinary anomalies identified in this review. Thirteen of the 15 patients were found at presentation to have the classic hemibladder on each side of the exstrophied bowel. Two patients had a single exstrophied bladder off to one side. In 4 of the 15 patients, agenesis of the left kidney was also identified, 4 had crossed ectopia, 1 had
Split-Bladder exstrophy Single bladder exstrophy Bilateral renal agenesis Agenesis left kidney Crossed Fused-Ectopia Hydro Lower Pole ( 1) Ectopic to Vagina ( 1) Megaureters Cystic Kidney ( 1)
Patients 13 2 1
4 4
5
bilateral renal agenesis, and 1 had severe left ureterovesical obstruction with a hydronephrotic left kidney. Of the patients with ectopia, one had a solitary right kidney with a duplication of the ureters with a hydroureteronephrosis of the lower pole to the right hemibladder with a crossed ectopia of the right upper pole to the left hemibladder. The other patient had a right double collecting system and hydronephrosis with the ureter ectopic to the vagina and the left upper pole ureter traversing to the opposite bladder. Initial and subsequent management of the urinary anomalies is outlined in Table 3. Separation of the bowel from the bladder and reapproximation of the hemibladders was initially performed in 13 patients. In two patients a primary closure of the bladder exstrophy was accompanied. Three patients had later bladder closure with lilac osteotomies. Of the patients undergoing GU tract reconstruction, seven have had ileal loop diversion. Four patients were managed by temporary cutaneous pyelostomy (three renal units) or ureterostomy (four units). Only one subsequent nephrectomy was required. One closed bladder had to be converted to a vesicostomy. Three other patients who underwent bladder closure remain incontinent, one despite a Young-DeesLedbetter procedure. One recent patient has had reapproximation of the bladder halves only, and further management is still pending.
Findings of the Genitalia Of the 15 patients, 9 were genetic males and 6 were genetic females. All males had severe anomalies of the genitalia including diminutive and separated corporal bodies, bifid scrotum, and often, undescended testes. Today, all males with Table 3. GU-Tract Management Initial Management Reapproximation of hemibladders
Primary Closure Exstrophy
No. of Patients
13 2
Later Management Ileal Conduits Vesicostomy
7 1
Temporary Cutaneous Pyelostomies (4) Cutaneous Ureterostomy Bladder Closure
2 3
(lliac Osteotomies)
Bladder Closure ( 1~)
2
CLOACAL EXSTROPHY
367
Table 4. Associated Anomalies
Anomaly Myelomeningocoele Thoracic ( 1) Sacral ( 1) Lumbar (5) Scoliosis Bilateral Club Feet Siamese Twin Left Facial Paralysis Factor 8 Deficiency Atretic Right Upper Lobe Bronchus
No. of Patients
7
cloacal exstrophy are gender converted to female because of the difficulty in reconstructing a workable male phenotype. Six of the severely deformed males were later converted to females, five in the newborn period and one at 9 months of age. Five of the six females were noted to have a bifid uterus and one had a double vagina as well. Three of the six had an exstrophied vagina and two had a covered vaginal opening.
Associated Anomalies Table 4 summarizes the anomalies associated with cloacal exstrophy in this review. Eleven of the 15 patients had significant associated anomalies. Twenty-one specific anomalies were identified. Myelomeningocele, the most common associated anomaly, was present in seven of these patients. One was in the thoracic region, one in the sacral region and five in the lumbar region. Vertebral anomalies were common to all. Six patients had severe scoliosis, and four patients had .severe bilateral club feet. Also, there was one Siamese twin, one child with left facial paralysis, one with factor 8 deficiency, and one with an atretie right upper lobe bronchus. The patient with the factor 8 deficiency was a genetic male and during gender conversion in the newborn period had no hemostatic problems. At subsequent operative procedures the patient bled excessively and having been converted to a female, the diagnosis of factor 8 deficiency was unfortunately overlooked. Subsequent tests confirmed the factor 8 deficiency.
report comprise the recorded total world experience. The total number of survivors is only approximately 35, 17 in the period from 1968 to 1976, a 50% survival rate) This is in contrast to our 86% survival. There has been general agreement regarding primary or prosthetic closure of the omphalocele and approximation of the hemibladders. In almost all series, including our own, this appears to have been accomplished successfully even in cases of ruptured omphalocele. Management of the intestine as well as the genitourinary tract and the genitalia has yet to reach a consensus. An understanding of the anatomical abnormality is vital to the proper surgical reconstruction. This complex anomaly involves both the intestinal and genitourinary tracts. The classic cloacal exstrophy consists of: exstrophic bowel of the ileocecal region, imperforate anus, two exstrophic hemibladders, omphalocele in approximately 90% of the cases, vertebral anomalies, and anomalies of external genitalia. Less commonly seen are anomalies of the upper urinary tract and lower extremities. A diagram of the usual findings is depicted in Fig. 1. As early as two decades ago Rickham ~emphasized the need to salvage the entire intestine of infants with cloacal exstrophy to prevent the devastating problems of ileostomy management. Early in our series of patients the creation of an ileostomy was associated with this problem and these two patients were subsequently converted to an end colostomy with resolution of their symptoms. Our most recent patient was referred for evaluation of failure to thrive after an ileosto-
Current Status Thirteen of these patients are alive and doing well (86% survival). One patient died in the newborn period of renal agenesis and one died at age 19, a probable suicide. The nutritional status of ten of these patients was noted to be good. The two patients with permanent ileostomies have been ileostomy cripples with marginal weight gains and severe diarrhea with associated viral illnesses in childhood. The two patients with temporary ileostomies who were done elsewhere were converted to permanent colostomies with the resolution of their diarrhea, inability to gain weight, and chronic malnutrition. None of these patients had a congenital short intestine. They did, however, behave similar to the patient with short-bowel syndrome and an ileostomy.
Smaller/ intesti-ne~ / ~X~ Appendix~ ~ Right ~ hemibladder- .
~
~'~
~
Exomphalos '
~ ~ ~ ~ ~ ~ - ~ ~
Distal/ bowel ...
~ Proxima bowel orifice Appendix
Left
hemibladder
~~ r" I \ Bhnd . large /
DISCUSSION
Since the report by Littre9 in 1709 of the first case of cloacal exstrophy, 157 cases prior to this
Fig. 1.
A n a t o m y of a classic cloacal exstrophy.
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HOWELL ET AL
my. Closure of her ileocecal plate and ileostomy with conversion to an end colostomy by anastomosing the ileum to the residual blind-ending colon restored her to a normal nutritional status. Even today, however, there are those 4'5'6 who recommend ileostomy for the initial management of the intestine with subsequent maintenance on long-term hyperalimentation and enteral feedings until adaptation of the small intestine occurs. These authors 4'5"6 desire to salvage portions of the colon for reconstruction of the genitourinary tract as well as the genitalia. We do not feel these considerations take precedence over the prevention of life-threatening short-bowel syndrome, a problem in approximately 50% of the patients in some series. In our experience this can be avoided by closing the vesicointestinal fissure and preserving the distal colon. More than a decade ago Tank and Lindenauer recommended conversion of males to females rather than trying to reconstruct inadequate genitalia into a workable male phenotype. Our series supports this approach. The three reconstructed males were left with inadequate genitalia; one probably committed suicide. Subsequent patients 6 have all been converted to females in early life. Still, despite recommendations 4'5 that most if not all of these infants should be reared as females, there are vague recommendations t~ for assessing potential penile length implying that reconstruction in the male phenotype is desirable. We strongly believe it would be rare to find a genetic male that should not be converted to a female phenotype. It is tragic to "condemn" a boy to life with inadequate genitalia. Management of the urinary tract should begin with a primary separation of the bowel from the bladder plate and a joining of the two bladder halves. The child may then be followed until her general condition indicates that she can tolerate further surgery. Closure of the exstrophied blad-
der will permit the option of later attempts to achieve continence with clean intermittent catheterization. In virtually all cases this will require additional surgery and may require intestinal augmentation of the bladder, tl We do not feel bowel should be so used unless the child can be shown--usually by the passage of formed stools--to have sufficient bowel to spare. Cutaneous ureterostomy with a small cuff of bladder to help form a stoma is an acceptable alternative. Ileal conduit has also been a successful mode of urinary diversion. Meticulous follow-up, however, must be emphasized. In summary, we support an initial closure of the omphalocele if possible, separation of the ileocecal connection from the exstrophied bladder plate, reapproximation of the separated bladder halves, and construction of an end colostomy with preservation of all bowel. We feel strongly that the excellent survival rate in this series reflects this approach to bowel management. We support reapproximation of the symphysis pubis in the newborn period as we 12 have previously demonstrated in exstrophy of the bladder that this can be performed in the first 72 hours of life. This simple maneuver makes abdominal wall closure easier and also provides an inferior buttress for the use of prosthetic materials in children with a much more difficult abdominal wall closure. We believe that except for the obvious male, genetic males should usually be given a female gender assignment and undergo appropriate surgery early in life. Further genitourinary tract management may involve closure of the bladder and later surgery to achieve continence or establish urinary diversion. In the future the use of artificial sphincter mechanisms to establish urinary continence, and the exploration of possible intestinal continence mechanisms by the posterior sagittal anorectoplasty as described by Pefia and deVries 13 may eliminate the lifelong "double stoma."
REFERENCES
1. Rickham PO: Vesico-intestinal fissure. Arch Dis Child 35:97-102, 1960 2. Spencer RW: Exstrophia splanchnica (Exstrophyofthe cloaca). Surgery 57:751-766, 1965 3. Hayden PW, Chapman WH, Stevenson JK: Exstrophy of the cloaca. Am J Dis Child 125:879-883, 1973 4. Sukarochana K, Sieber WK: Vesicointestinal fissure revisited. J Pediatr 13:713-719, 1978
5. Welch K J, Cloacal exstrophy, in Ravitch MM, Welch K J, Benson CD, et al (eds): Pediatric Surgery (Vol II). Chicago, Year Book Medical, 1979, pp 802-808 6. Tank ES, Lindenauer SM: Principles of management of exstrophy of the cloaca. Am J Surg 119:95-98, 1970 7. Fonkalsrud EW, Linde LM: Successful management of vesicointestinal fissure: Report of two cases. J Pediatr Surg 5:309-314, 1970
CLOACAL EXSTROPHY
8. Soper RT, Kilger K: Vesico-intestinal fissure. J Urol 92:490-501, 1964 9. Littre A: Med Acad R Sci 4:9, 1709, cited by Duncan 10. Jeffs RD: Exstrophy and cloacal exstrophy. Urol Clin North Am 5:127-140, 1978 11. Arap S, Giron AM, deGoes G: Initial results of the
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complete reconstruction of bladder exstrophy. Urol Clin North Am 7:477-491, 1980 12. Duekett JD: Newborn Exstrophy Closure, presented at the American Urological Association, 1982 13. deVries PA, Peita A: Posterior Sagittal Anorectoplasty, presented at the American Pediatric Surgical Association Meeting, Phoenix, 1982