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Urologic problems associated with imperforate anus
UROLOGIC
PROBLEMS
IMPERFORATE PETER
JOHN
V. SANTULLI,
K. LAITIMER,
WITH
ANUS*
J. PUCHNER,
THOMAS
ASSOCIATED
M.D. M.D. M.D.
From the Departments of Urology and Surgery, College of Physicians and Surgeons, Columbia University; the Urological and Surgical Services of the Babies Hospital, and the Squier Urological Clinic, Columbia-Presbyterian Medical Center, New York
ABSTRACT - A total of 120 children with both high, intermediate, and low types of imperforate anus were evaluated urologically. Genitourinary anomalies were associated with all types of imperforate anus but were found more often in children with high rectal deformities. Because the over-all incidence of genitourinary anomalies was high, early and complete urologic evaluation is necessary to achieve the best functional result.
The association of genitourinary anomalies with all forms of imperforate anus has been noted by l-5 More recently, Belman many investigators. and King,’ and Wiener and Kiesewetter’ stressed the importance of urologic evaluation with surgical correction of the anomaly. Santulli et al. * reported significant urologic anomalies in 26 per cent of 1,166 patients surveyed from members of the Surgical Section of the American Academy of Pediatrics in a five-year period, 1965 to 1969. The international classification of anorectal anomalies suggested in 1970 is used herea The anomalies are divided into three general types: high, intermediate, and low. In high lesions there is rectal agenesis or the rectum ends above the levator ani muscle, either without a fistula or with a rectourethral or rectovesical fistula in the male, and a high rectovaginal, rectocloacal, or rectovesical (extremely rare) fistula in the female. In low lesions the bowel traverses the puborectalis sling of the levator ani muscle and the abnormal anus presents at the normal anal site or in *Supported in part by Irene LaPorte Given Fund for Research Urology.
UROLOGY
Heinz Given and John and Teaching of Pediatric
/ AUGUST 1975 / VOLUME
VI, NUMBER
2
an ectopic perineal location. In intermediate lesions, the abnormalities of the bowel occur at levels between the high and low lesions. All forms of imperforate anus (high, low, and intermediate) in both male and female patients are associated with genitourinary anomalies. The following review of 120 children admitted to the Babies Hospital was done to delineate more precisely the specific types of genitourinary problems, their severity, and over-all management. Material
and Methods
One-hundred twenty children with imperforate anus admitted for the first time to the Babies Hospital between 1964 and 1972 were studied. Their ages ranged from newborn to fourteen years. There were 71 males; 40 had high lesions, 4 intermediate, and 27 low. Of the 49 females, 7 had high lesions, 14 intermediate, and 28 low. Male patients
with imperforate
anus
High. Five of the 40 boys had some form of hypospadias, and 5 had either uni- or bilateral undescended testes (Table I). Ten boys had persistently abnormal findings on intravenous pyelogram, exhibiting such major abnormalities as
205
TABLE I.
Genitourinary anomalies in 120 children with high, intermediate, and low fbrn~ of imperjiiate
YImperforate Conditions
High (40)* No. %
Abnormal genitalia 10 Hypospadias 5 Undescended testes 5 Cloaca (urogenital sinus) . . Abnormal IVP 10 Abnormal bladder Vesicoureteral reflux Abnormal sacrum Urinary diversion Antireflux surgery
6 15 25 4 1
-1mperforate
Anus (Male)Intermediate (4)f Low (27)$ No. % No. %
25 0 0
0 0 0
0 0 0
25 15 37.5 62.5 10 2.5
.. 0 0 1 0 0 0
.. 0 0 25 0 0 0
3 2
15
1 .. 8 3 5 8 1 0
0
High (7)Q No. %
0
&!I 11 18 30 40 0
anus
Anus (Femaleb Intermediate 04) 11 Low (28)ll No. % No. %
5 ..
71 ..
1 ..
7 ..
1 ,.
4 ..
5 3 4 2 4
‘b 43 57 29 57 00 14
1: 4 4 3 6
1: 29 29 21 43 014 7
1: 4 0 6 6
:: 14 0 21 21
1
4
1
1
*Cystograms done on 34; sacral x-ray studies on 40. Wystograms and sacral x-ray studies done on all. $Cystograms done on 21; sacral x-ray studies on 27. QCystograms and sacral studies done on 6. IlCystograms done on 10. TlCystograms done on 16; sacral x-ray studies done on all.
atretic or solitary kidneys and progressive uni- or bilateral hydronephrosis, often with abnormal bladders. Cystograms were done on 34 of the 40 children. Six boys exhibited grossly abnormal bladders which after evaluation were believed to be neurogenic bladders. Sacral anomalies were common and were seen in 25 of the 40 boys. Eight of the 10 patients with abnormal findings on intravenous pyelograms had abnormal sacrums. All 6 boys with neurogenic bladders had sacral abnormalities. Four of the six boys with both abnormal bladders and sacrums did poorly and eventually required urinary diversion because of continued infection, persistent reflux, and deterioration in renal function. The remaining 2 boys with large neurogenic bladders are, at the present time, doing well without reflux or infection. Fifteen children exhibited either uni- or bilateral vesicoureteral reflux. Six of the patients with reflux had neurogenic bladders. However, of the remaining 9 boys with reflux all but one have done well; he required ureteral reimplantation because of persistent, massive reflux and pyelonephritis. Intermediate. There were 4 boys in this group, and none exhibited penile or testicular abnormalities (Table I). Findings on intravenous pyelogram and x-ray films of the sacrum were normal in all 4 boys. One boy had mild bilateral vesicoureteral reflux which so far has not required surgery and has not been associated with recurrent urinary tract infection. None had abnormal or neurogenic bladders.
Low. There were 27 boys with genitourinary abnormalities in this group (Table I). All had some form of anal anomaly with or without perineal fistulas; none had fistulous connection with the urinary tract. Two children had hypospadias, and one had an undescended testis. Intravenous pyelograms were done on all but one. The findings on 8 intravenous pyelograms were abnormal, ranging from solitary, atretic, or malrotated kidneys to marked unilateral or bilateral hydronephrosis. Cystograms on 21 of the 27 children revealed abnormal neurogenic bladders in 3. One with marked bilateral ureterovesical reflux with progressive hydronephrosis required urinary diversion in the form of an ileal conduit; one with massive unilateral reflux had a nephroureterectomy and is currently doing well; and the third boy so far is doing well despite a large neurogenic bladder without reflux. Three other boys with mild, unilateral reflux, no infection, and with normal upper tracts did not require antireflux surgery. Sacral anomalies were seen in 8 boys, 6 of whom had associated abnormal findings on intravenous pyelogram .
206
UROLOGY
Female
patients
with imperforate
anus
Seven had anomalies consistent with High. high imperforate anus (Table I). One girl had rectal agenesis without fistula, one rectovaginal fistula, and 5 rectocloacal fistula with urogenital sinus. One girl with a cloaca1 fistula died after
/
AUGUST 1975 /
VOLUME VI, NUMBER 2
abdominal exploratory surgery for meconium peritonitis. At autopsy she was found to have one multicystic and one hydronephrotic kidney. Five of the 7 children had abnormal external genitalia consistent with urogenital sinus. In 3 of 6 living girls abnormal findings on intravenous pyelograms exhibited either unilateral or bilateral hydronephrosis. All 6 living children had cystograms, and 2 were found to have vesicoureteral reflux. One girl with reflux has had persistent pyelonephritis and has required bilateral ureteral reimplantation because of recurrent sepsis. After surgery she has been well despite a large atonic neurogenic bladder. Three other girls also have abnormal-appearing, mildly enlarged bladders which are not neurogenic. All 6 had sacral radiologic studies, and 4 showed abnormalities. However, only 1 girl with findings on intravenous pyelogram had an associated sacral anomaly. Intermediate. Fourteen female patients were found to have an intermediate type of imperforate anus (Table I). All girls had either low vaginal or vestibular fistulas. Thirteen children were evaluated urologically. One child died after attempt at correction of coexisting esophageal atresia with tracheoesophageal fistula and before complete urologic evaluation could be accomplished. All children had normal genitalia, except for one who had a urogenital sinus. Four had abnormal findings on intravenous pyelograms, ranging in severity from normal solitary kidney to moderate progressive bilateral hydronephrosis. Ten girls had cystograms, 4 of which revealed abnormally large bladders. Three girls had vesicoureteral reflux. However, only one had a mild neurogenic bladder without reflux. Because of massive bilateral reflux and progressive hydrone1 girl required bilateral ureteral phrosis, reimplantations. Sacral anomalies were found in 6 children, and 3 of these had abnormal findings on intravenous pylograms. Low. Of 28 female children only one had an abnormality of the genitalia, a urogenital sinus (Table I). Four children had abnormal findings on intravenous pyelograms. Two showed progressive bilateral hydronephrosis, and one of these required bilateral ureteral reimplantation because of persistent massive reflux and sepsis. The other child with marked bilateral hydronephrosis has subsequently undergone nephroureterectomy and now has a cutaneous ureterostomy from her solitary, still mildly hydronephrotic kidney. Two others with abnormal findings on intravenous pyelogram had associated reflux. With con-
UROLOGY
/ AUGUST 1975 I
VOLUME
VI, NUMBER
2
servative management, the reflux disappeared spontaneously, and their intravenous pyelograms now show normal findings. Two other children initially had reflux (with normal findings on intravenous pyelograms) which also has disappeared spontaneously. No neurogenic bladders were found in this group, and only 6 of 28 children had abnormal sacrums. Only one with an abnormal sacrum had an abnormal finding on intravenous pyelogram; this is the only child in the group to undergo urinary diversion. Comment In both males and females with imperforate anus, the frequency of genitourinary tract anomalies is high. Although the incidence of urinary tract problems appears to be less with low lesions, the number of problems is still significant, especially in girls with intermediate lesions. In a previous report’ it was stated that no spinal or genitourinary abnormalities were seen in females with low lesions. We since have found significant urinary tract problems associated with this group of girls. One child eventually required permanent urinary diversion. Those boys with high and low lesions who had grossly abnormal neurogenic bladders did poorly. Of 9 boys with neurogenic bladders, 5 to date have required permanent diversion despite concerted efforts to keep their lower urinary tracts intact. Approximately 1 in 4 children have shown vesicoureteral reflux. Despite this significant number, only 4 children with normal, nonneurogenic bladders have required antireflux surgery. In the remainder of children, spontaneous gradual disappearance of the reflux was seen. When reflux was associated with large neurogenic bladders, antireflux surgery was not effective in preventing upper tract deterioration, and subsequent permanent supravesical diversion was often required. Many children with high lesions required temporary suprapubic cystostomies at the time of initial colostomy and/or correction of fistulas between bowel and urinary tract. This form of temporary diversion, usually performed in infants, created no significant long-term complications. Many children, in all groups except for boys with intermediate lesions, showed sacral anomalies. Again, boys with high lesions and neurogenic bladders had a high associated incidence of deformed sacrums. This anomaly was frequently seen in females with high and inter-
207
mediate lesions. In females with low lesions there was no significant correlation between the presence of genitourinary abnormalities and sacral problems; however, the one girl with the most severe urinary tract problems did have a markedly abnormal sacrum. The importance of early urologic evaluation in children with imperforate anus cannot be too strongly emphasized. Although we have had no urologic-related deaths, unrecognized urologic problems, particularly ureterovesical reflux and neurogenic bladder, with associated infection, loom as real threats to the children - especially during infancy. In the newborn period we routinely obtain x-ray films of the sacral spine and voiding cystometrogram at the time of primary radiologic investigation of the imperforate anus anomaly. An intravenous pyelogram is done over the next three to seven days. This basic urologic evaluation is mandatory in children to obtain the best over-all result. An intravenous pyelogram alone is not adequate. Any child found to have a genitourinary abnormality must be followed closely with repeat diagnostic studies. Summary One-hundred twenty children, both male and female, with imperforate anus were studied. Male and female patients were classified according to high, intermediate, and low anomalies. High rectal anomalies were associated with a higher incidence of significant short- and longterm urologic problems. The majority of genitourinary anomalies were discovered early in the evaluation of these children. A small number of these children, especially those with high lesions, showed deterioration of their upper tracts as a result of persistent reflux and urinary tract infection. The presence of neurogenic bladder either on initial or subsequent urologic evaluation
208
was generally associated with a poorer prognosis. These patients often exhibited progressive deterioration of the upper tracts and loss of renal function, requiring permanent urinary diversion. The high incidence of genitourinary tract anomalies with all forms of imperforate anus makes early urologic evaluation with intravenous pyelogram, voiding cystourethrogram, and sacral x-ray studies mandatorv. , Intravenous nvelogram alone is not sufficient to rule out all abnormalities. L.
v
620 West 168th Street New York, New York 10032
(DR. PUCHNER) References 1. SPENCE, H. M.: Anomalies and complications of .the urogenital tract associated with congenital imperforate anus, J. Urol. 71: 453 (1954). 2. BERDON, W. E., et al. : The association of lumbosacral spine and genitourinary anomalies with imperforate anus, Am. J. Roentgenol. 98: 181 (1966). Urinary anomalies and complications in 3. SMITH, E. D.: imperforate anus and rectum, J. Pediatr. Surg. 3: 337 (1968). 4. HALL, J. W., TOUK, E. S., and LAPIDES, J.: Urogenital anomalies and complications associated with imperforate anus, J. Urol. 103: 810 (1970). 5. STEPHENS, F. D., and SMITH, E. D.: Ano-rectal Malformations in Children, Chicago, Year Book Medical Publishers, Inc., 1971, p. 289. 6. BELMAN, A. B., and KING, L. R.: Urinary tract abnormalities associated with imperforate anus, J. Urol. 108: 823 (1972). 7. WIENER, E. S., and KIESEWETTER, W. B.: Urologic abnormalities associated with imperforate anus, J. Pediatr. Surg. 8: 151 (1973). 8. SANTULLI, T. U., SCHULLINGER, J. N., KIESEWETTER, W. B., and BILL, A. H., JR.: An imperforate anus: a survey from the members of the surgical section of the American Academy of Pediatrics, ibid. 6: 484 (1971). 9. SANTULLI, T. U., KIESEWET~ER, W. B., and BILL, A. H., JR. : Anorectal anomalies: a suggested international classification, ibid. 5: 281 (1970).
UROLOGY
/
AUGUST 1975 /
VOLUME VI, NUMBER 2
PROBLEMS
IMPERFORATE PETER
JOHN
V. SANTULLI,
K. LAITIMER,
WITH
ANUS*
J. PUCHNER,
THOMAS
ASSOCIATED
M.D. M.D. M.D.
From the Departments of Urology and Surgery, College of Physicians and Surgeons, Columbia University; the Urological and Surgical Services of the Babies Hospital, and the Squier Urological Clinic, Columbia-Presbyterian Medical Center, New York
ABSTRACT - A total of 120 children with both high, intermediate, and low types of imperforate anus were evaluated urologically. Genitourinary anomalies were associated with all types of imperforate anus but were found more often in children with high rectal deformities. Because the over-all incidence of genitourinary anomalies was high, early and complete urologic evaluation is necessary to achieve the best functional result.
The association of genitourinary anomalies with all forms of imperforate anus has been noted by l-5 More recently, Belman many investigators. and King,’ and Wiener and Kiesewetter’ stressed the importance of urologic evaluation with surgical correction of the anomaly. Santulli et al. * reported significant urologic anomalies in 26 per cent of 1,166 patients surveyed from members of the Surgical Section of the American Academy of Pediatrics in a five-year period, 1965 to 1969. The international classification of anorectal anomalies suggested in 1970 is used herea The anomalies are divided into three general types: high, intermediate, and low. In high lesions there is rectal agenesis or the rectum ends above the levator ani muscle, either without a fistula or with a rectourethral or rectovesical fistula in the male, and a high rectovaginal, rectocloacal, or rectovesical (extremely rare) fistula in the female. In low lesions the bowel traverses the puborectalis sling of the levator ani muscle and the abnormal anus presents at the normal anal site or in *Supported in part by Irene LaPorte Given Fund for Research Urology.
UROLOGY
Heinz Given and John and Teaching of Pediatric
/ AUGUST 1975 / VOLUME
VI, NUMBER
2
an ectopic perineal location. In intermediate lesions, the abnormalities of the bowel occur at levels between the high and low lesions. All forms of imperforate anus (high, low, and intermediate) in both male and female patients are associated with genitourinary anomalies. The following review of 120 children admitted to the Babies Hospital was done to delineate more precisely the specific types of genitourinary problems, their severity, and over-all management. Material
and Methods
One-hundred twenty children with imperforate anus admitted for the first time to the Babies Hospital between 1964 and 1972 were studied. Their ages ranged from newborn to fourteen years. There were 71 males; 40 had high lesions, 4 intermediate, and 27 low. Of the 49 females, 7 had high lesions, 14 intermediate, and 28 low. Male patients
with imperforate
anus
High. Five of the 40 boys had some form of hypospadias, and 5 had either uni- or bilateral undescended testes (Table I). Ten boys had persistently abnormal findings on intravenous pyelogram, exhibiting such major abnormalities as
205
TABLE I.
Genitourinary anomalies in 120 children with high, intermediate, and low fbrn~ of imperjiiate
YImperforate Conditions
High (40)* No. %
Abnormal genitalia 10 Hypospadias 5 Undescended testes 5 Cloaca (urogenital sinus) . . Abnormal IVP 10 Abnormal bladder Vesicoureteral reflux Abnormal sacrum Urinary diversion Antireflux surgery
6 15 25 4 1
-1mperforate
Anus (Male)Intermediate (4)f Low (27)$ No. % No. %
25 0 0
0 0 0
0 0 0
25 15 37.5 62.5 10 2.5
.. 0 0 1 0 0 0
.. 0 0 25 0 0 0
3 2
15
1 .. 8 3 5 8 1 0
0
High (7)Q No. %
0
&!I 11 18 30 40 0
anus
Anus (Femaleb Intermediate 04) 11 Low (28)ll No. % No. %
5 ..
71 ..
1 ..
7 ..
1 ,.
4 ..
5 3 4 2 4
‘b 43 57 29 57 00 14
1: 4 4 3 6
1: 29 29 21 43 014 7
1: 4 0 6 6
:: 14 0 21 21
1
4
1
1
*Cystograms done on 34; sacral x-ray studies on 40. Wystograms and sacral x-ray studies done on all. $Cystograms done on 21; sacral x-ray studies on 27. QCystograms and sacral studies done on 6. IlCystograms done on 10. TlCystograms done on 16; sacral x-ray studies done on all.
atretic or solitary kidneys and progressive uni- or bilateral hydronephrosis, often with abnormal bladders. Cystograms were done on 34 of the 40 children. Six boys exhibited grossly abnormal bladders which after evaluation were believed to be neurogenic bladders. Sacral anomalies were common and were seen in 25 of the 40 boys. Eight of the 10 patients with abnormal findings on intravenous pyelograms had abnormal sacrums. All 6 boys with neurogenic bladders had sacral abnormalities. Four of the six boys with both abnormal bladders and sacrums did poorly and eventually required urinary diversion because of continued infection, persistent reflux, and deterioration in renal function. The remaining 2 boys with large neurogenic bladders are, at the present time, doing well without reflux or infection. Fifteen children exhibited either uni- or bilateral vesicoureteral reflux. Six of the patients with reflux had neurogenic bladders. However, of the remaining 9 boys with reflux all but one have done well; he required ureteral reimplantation because of persistent, massive reflux and pyelonephritis. Intermediate. There were 4 boys in this group, and none exhibited penile or testicular abnormalities (Table I). Findings on intravenous pyelogram and x-ray films of the sacrum were normal in all 4 boys. One boy had mild bilateral vesicoureteral reflux which so far has not required surgery and has not been associated with recurrent urinary tract infection. None had abnormal or neurogenic bladders.
Low. There were 27 boys with genitourinary abnormalities in this group (Table I). All had some form of anal anomaly with or without perineal fistulas; none had fistulous connection with the urinary tract. Two children had hypospadias, and one had an undescended testis. Intravenous pyelograms were done on all but one. The findings on 8 intravenous pyelograms were abnormal, ranging from solitary, atretic, or malrotated kidneys to marked unilateral or bilateral hydronephrosis. Cystograms on 21 of the 27 children revealed abnormal neurogenic bladders in 3. One with marked bilateral ureterovesical reflux with progressive hydronephrosis required urinary diversion in the form of an ileal conduit; one with massive unilateral reflux had a nephroureterectomy and is currently doing well; and the third boy so far is doing well despite a large neurogenic bladder without reflux. Three other boys with mild, unilateral reflux, no infection, and with normal upper tracts did not require antireflux surgery. Sacral anomalies were seen in 8 boys, 6 of whom had associated abnormal findings on intravenous pyelogram .
206
UROLOGY
Female
patients
with imperforate
anus
Seven had anomalies consistent with High. high imperforate anus (Table I). One girl had rectal agenesis without fistula, one rectovaginal fistula, and 5 rectocloacal fistula with urogenital sinus. One girl with a cloaca1 fistula died after
/
AUGUST 1975 /
VOLUME VI, NUMBER 2
abdominal exploratory surgery for meconium peritonitis. At autopsy she was found to have one multicystic and one hydronephrotic kidney. Five of the 7 children had abnormal external genitalia consistent with urogenital sinus. In 3 of 6 living girls abnormal findings on intravenous pyelograms exhibited either unilateral or bilateral hydronephrosis. All 6 living children had cystograms, and 2 were found to have vesicoureteral reflux. One girl with reflux has had persistent pyelonephritis and has required bilateral ureteral reimplantation because of recurrent sepsis. After surgery she has been well despite a large atonic neurogenic bladder. Three other girls also have abnormal-appearing, mildly enlarged bladders which are not neurogenic. All 6 had sacral radiologic studies, and 4 showed abnormalities. However, only 1 girl with findings on intravenous pyelogram had an associated sacral anomaly. Intermediate. Fourteen female patients were found to have an intermediate type of imperforate anus (Table I). All girls had either low vaginal or vestibular fistulas. Thirteen children were evaluated urologically. One child died after attempt at correction of coexisting esophageal atresia with tracheoesophageal fistula and before complete urologic evaluation could be accomplished. All children had normal genitalia, except for one who had a urogenital sinus. Four had abnormal findings on intravenous pyelograms, ranging in severity from normal solitary kidney to moderate progressive bilateral hydronephrosis. Ten girls had cystograms, 4 of which revealed abnormally large bladders. Three girls had vesicoureteral reflux. However, only one had a mild neurogenic bladder without reflux. Because of massive bilateral reflux and progressive hydrone1 girl required bilateral ureteral phrosis, reimplantations. Sacral anomalies were found in 6 children, and 3 of these had abnormal findings on intravenous pylograms. Low. Of 28 female children only one had an abnormality of the genitalia, a urogenital sinus (Table I). Four children had abnormal findings on intravenous pyelograms. Two showed progressive bilateral hydronephrosis, and one of these required bilateral ureteral reimplantation because of persistent massive reflux and sepsis. The other child with marked bilateral hydronephrosis has subsequently undergone nephroureterectomy and now has a cutaneous ureterostomy from her solitary, still mildly hydronephrotic kidney. Two others with abnormal findings on intravenous pyelogram had associated reflux. With con-
UROLOGY
/ AUGUST 1975 I
VOLUME
VI, NUMBER
2
servative management, the reflux disappeared spontaneously, and their intravenous pyelograms now show normal findings. Two other children initially had reflux (with normal findings on intravenous pyelograms) which also has disappeared spontaneously. No neurogenic bladders were found in this group, and only 6 of 28 children had abnormal sacrums. Only one with an abnormal sacrum had an abnormal finding on intravenous pyelogram; this is the only child in the group to undergo urinary diversion. Comment In both males and females with imperforate anus, the frequency of genitourinary tract anomalies is high. Although the incidence of urinary tract problems appears to be less with low lesions, the number of problems is still significant, especially in girls with intermediate lesions. In a previous report’ it was stated that no spinal or genitourinary abnormalities were seen in females with low lesions. We since have found significant urinary tract problems associated with this group of girls. One child eventually required permanent urinary diversion. Those boys with high and low lesions who had grossly abnormal neurogenic bladders did poorly. Of 9 boys with neurogenic bladders, 5 to date have required permanent diversion despite concerted efforts to keep their lower urinary tracts intact. Approximately 1 in 4 children have shown vesicoureteral reflux. Despite this significant number, only 4 children with normal, nonneurogenic bladders have required antireflux surgery. In the remainder of children, spontaneous gradual disappearance of the reflux was seen. When reflux was associated with large neurogenic bladders, antireflux surgery was not effective in preventing upper tract deterioration, and subsequent permanent supravesical diversion was often required. Many children with high lesions required temporary suprapubic cystostomies at the time of initial colostomy and/or correction of fistulas between bowel and urinary tract. This form of temporary diversion, usually performed in infants, created no significant long-term complications. Many children, in all groups except for boys with intermediate lesions, showed sacral anomalies. Again, boys with high lesions and neurogenic bladders had a high associated incidence of deformed sacrums. This anomaly was frequently seen in females with high and inter-
207
mediate lesions. In females with low lesions there was no significant correlation between the presence of genitourinary abnormalities and sacral problems; however, the one girl with the most severe urinary tract problems did have a markedly abnormal sacrum. The importance of early urologic evaluation in children with imperforate anus cannot be too strongly emphasized. Although we have had no urologic-related deaths, unrecognized urologic problems, particularly ureterovesical reflux and neurogenic bladder, with associated infection, loom as real threats to the children - especially during infancy. In the newborn period we routinely obtain x-ray films of the sacral spine and voiding cystometrogram at the time of primary radiologic investigation of the imperforate anus anomaly. An intravenous pyelogram is done over the next three to seven days. This basic urologic evaluation is mandatory in children to obtain the best over-all result. An intravenous pyelogram alone is not adequate. Any child found to have a genitourinary abnormality must be followed closely with repeat diagnostic studies. Summary One-hundred twenty children, both male and female, with imperforate anus were studied. Male and female patients were classified according to high, intermediate, and low anomalies. High rectal anomalies were associated with a higher incidence of significant short- and longterm urologic problems. The majority of genitourinary anomalies were discovered early in the evaluation of these children. A small number of these children, especially those with high lesions, showed deterioration of their upper tracts as a result of persistent reflux and urinary tract infection. The presence of neurogenic bladder either on initial or subsequent urologic evaluation
208
was generally associated with a poorer prognosis. These patients often exhibited progressive deterioration of the upper tracts and loss of renal function, requiring permanent urinary diversion. The high incidence of genitourinary tract anomalies with all forms of imperforate anus makes early urologic evaluation with intravenous pyelogram, voiding cystourethrogram, and sacral x-ray studies mandatorv. , Intravenous nvelogram alone is not sufficient to rule out all abnormalities. L.
v
620 West 168th Street New York, New York 10032
(DR. PUCHNER) References 1. SPENCE, H. M.: Anomalies and complications of .the urogenital tract associated with congenital imperforate anus, J. Urol. 71: 453 (1954). 2. BERDON, W. E., et al. : The association of lumbosacral spine and genitourinary anomalies with imperforate anus, Am. J. Roentgenol. 98: 181 (1966). Urinary anomalies and complications in 3. SMITH, E. D.: imperforate anus and rectum, J. Pediatr. Surg. 3: 337 (1968). 4. HALL, J. W., TOUK, E. S., and LAPIDES, J.: Urogenital anomalies and complications associated with imperforate anus, J. Urol. 103: 810 (1970). 5. STEPHENS, F. D., and SMITH, E. D.: Ano-rectal Malformations in Children, Chicago, Year Book Medical Publishers, Inc., 1971, p. 289. 6. BELMAN, A. B., and KING, L. R.: Urinary tract abnormalities associated with imperforate anus, J. Urol. 108: 823 (1972). 7. WIENER, E. S., and KIESEWETTER, W. B.: Urologic abnormalities associated with imperforate anus, J. Pediatr. Surg. 8: 151 (1973). 8. SANTULLI, T. U., SCHULLINGER, J. N., KIESEWETTER, W. B., and BILL, A. H., JR.: An imperforate anus: a survey from the members of the surgical section of the American Academy of Pediatrics, ibid. 6: 484 (1971). 9. SANTULLI, T. U., KIESEWET~ER, W. B., and BILL, A. H., JR. : Anorectal anomalies: a suggested international classification, ibid. 5: 281 (1970).
UROLOGY
/
AUGUST 1975 /
VOLUME VI, NUMBER 2