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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406 EISSN: 2008-2150
Vol. 18, Num. 2, 2008, pp. 171-174
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Iranian Journal of Pediatrics, Vol. 18, No. 2, June, 2008, pp. 171-174
Urogenital Tract Abnormalities Associated with Congenital Anorectal
Malformations
Alireza Mirshemirani*1, MD; Javadghorobi 1, MD; Mohsen Roozroukh1; Sina Sadeghiyan1, MD; Jaefar Kouranloo1, MD
1Department of Pediatric Surgery, shaheed beheshti
University of Medical Sciences, iran
* Correspondence author;
Address: Mofid Childre'n Hospital, Dr Sharati St, Tehran, Iran
E-mail: almirshemirani@yahoo.com
Received: 09/08/07; Revised: 28/12/07;
Accepted: 12/02/08
Code Number: pe08028
Abstract
Objective:Genitourinary anomalies in patients with imperforate anus
are a frequent source of significant morbidity. Variability of reports on the
incidence of associated anomalies with imperforate anus mandates investigation
on this issue.
Material & Methods:The case records and imaging studies of 105 patients who underwent
surgery for imperforate anus over a 10-year period are retrospectively reviewed.
Voiding cystouretherography, intra venous pyelography (IVP) and ultrasound were
performed in patients with intermediate or high level anorectal lesions.
Findings:During
10 years, there were 48 boys (45.7%) and 57 girls (54.3%) with imperforate anus.
Genitourinary anomalies were seen in 34 (48.6%) patients with intermediate or high level anorectal
lesions. Eighteen of
them (52.9%) were shown to have vesicouretral
reflux, only 6 of them required surgical correction. Vesicoureteral reflux was
the most prominent urologic anomaly; other anomalies such as ureteropelvic
junction (UPJ) stenosis, hydronephrosis, hypospadias, renal agenesis and
undescended testis were seen in these series.
Conclusion:Patients with anorectal malformations should be evaluated
for urinary tract and spinal anomalies.
Key Words:Imperforate anus; Anorectal
malformation; Urinary tract anomaly
Introduction
Anorectal malformations are a complex group of
malformations diagnosed at birth because of absent or ectopic anus[1].
There is variable data, however the incidence is approximately 1:5000 in live
births. Genitourinary anomalies occur frequently in the patients with congenital anorectal
malformation. Urinary tract problems are common in these patients with a
reported incidence of 26% to 50% in several large series[1,2]. Most
of the genital anomalies are visible on clinical examination, but urological
anomalies need investigations for their detection[3].
The
purpose of this study was to evaluate the frequency of coexisting urinary
malformations in a single population of patients with imperforate anus in our
children's medical center.
Material & Methods
During 10 years, from 1996 till 2005 in Mofid Children's
Hospital totally 105 children with congenital anorectal malformations retro- spectively
were studied. All patients with apparent diagnosis of imperforate anus were
included in this study.
The patient's sex, anorectal lesion level and
the presence of urinary tract, genital or spinal anomalies were recorded. Level
of anorectal lesion was determined by radiographic evaluation.
Renal ultrasound or intra venous pyelography
(IVP) and Voiding cystouretherography (vcug)
were performed for urinary tract malformations in all cases with intermediate or
high level anorectal lesions but not in low level anal lesion. In patients with
low level imperforate anus, genital malformations were defined as any anomaly
of the penis, testis or scrotum in boys and vagina, cervix or uterus in girls.
Findings
During 10 years, there were 48 boys (45.7%) and 57
girls (54.3%) with imperforate anus. Of the 105 patients, 35 patients (34.3%)
had low level lesions. Totally, 70 (66.6%) of patients had intermediate or high
level anorectal lesions, thats included 38 boys (54.2%) and 32 girls (45.7%). Ten
boys (28.6%) and 25 girls (71.4%) girls had low anomalies.
In patients with intermediate or high level
anorectal lesions renal ultrasound or excretory urography and vcug was performed for urinary tract
malformations. The incidence of urinary tractanomalies
was 48.6% in patients
with intermediate or high level anorectal lesions. The most frequently
encountered lesion was vesicoureteral reflux (VUR) which affected 18 (25.7%)of the patients, 8 of whom had
bilateral type of the lesion (table 1). One of these patients had bilateral
scar kidneys with chronic renal failure. Twelve patients with VUR were under medical supervision
and treatment for a period of 2 to 4 years, and only 6 patients required surgical correction, and others
are treated medically.
Table 1- Anomalies of urinary tract
Anomaly type |
Frequency |
Reflux |
18 (52.9%) |
Renal agenesis |
4 (11.8%) |
Hydronephrosis |
4 (11.8%) |
Renal hypoplasia |
3 (8.8%) |
UPJ* stenosis |
3 (8.8%) |
Duplication |
2 (5.9%) |
Total |
34
(100%) |
*Ureteropelvic junction
Genitalia anomalies were found only in male patients. The incidence of genitalia anomalies was 13 males (27.1%) boys. The
most frequently encountered anomaly was undecended testis (table 2).
Lumbosacral anomalies were found only in high type
lesion.Ten patients (9.5% all patients or
14.2% high type) had lumbosacral anomalies including sacral agenesis in 8 (7.6%)
and spina bifida in 2 (1.9%) cases. Two of them developed neurogenic bladder.
Table 2- Anomalies of the genitalia
Anomaly type |
Frequency |
Undecended testis |
7
(54%) |
Hypospadias |
3
(23%) |
Bifid scrotum |
3
(23%) |
Total |
13
(100%) |
Discussion
The association of genitourinary anomalies with
imperforate anus has been recently reviewed. In general, incidence of
associated genitourinary anomalies ranging from 26% to 50% has been reported
with an increased incidence of high (supralevator) as compared to low
(infralevator) lesions[1,3,4]. In our study, the incidence of
urinary tract anomalies was 48.6% in intermediate or high level anorectal lesions, and an overall rate was 32.4%. The
findings of this study are similar to urinary tract anomalies reported in other
studies[1,2,5]. The incidence of urinary tract anomalies increased
with a higher level of anorectal malformation[6].
VUR and renal agenesis are the most common
associated urinary tract anomalies with imperforate anus. In our studies,VUR was the most common anomalies in 25.7% patients
with high lesion and 52.9%
patients with urinary tractanomalies. VUR was reported the most common associated urinary
tract anomaly with imperforate anus[7]. The incidence of VUR in
patients with anorectal malformations was different in various studies form 19%
to 47.2%[2,4,8]. the
current practice is to perform ultrasound which can be used to image the
kidneys, bladder and to evaluate the spinal cord for tethering[9].
Metts and boemers
found VUR in 32% of their cases[7,8]. Misra et al reported that 7.5% of patients with low
deformity had VUR [10], but Tohda and Moore reported the incidence
of VUR only in 0.7% and 5.4% of their patients[6,11]. This wide
variation in incidence of VUR is related to the different methods of study. In
some studies VCUG was performed only when some graphic findings were abnormal[12].
In other study, hydronephrosis and renal
agenesis were the most common abnormalities of the upper urinary tract, and
neurovesical dysfunction is a frequent finding in children with anorectal
malformations [13]. We found hydronephrosis and renal agenesis as
two common of the upper urinary tract abnormalities. Neurovesical dysfunction commonly
is associated with sacrospinal deformities. Some authors recommend evaluation
of all patients with MRI, because spinal cord anomalies may occur without
obvious sacrospinal anomalies[9]. Urodynamic studies (UDS) are
reserved for those children with either a deformity of the spine or a spinal
cord defect[13].
Spine anomalies in other studies reported
between 16-27%)[2,6], but in our series were 14.2%. In our patients,
cryptorchidism and was the most common genital anomalies[14].
Conclusion
All patients with imperforate anus should be
investigated for urogenital and spinal anomalies. Every effort should be
unetaken to detect the associated urogenital anomalies, so that a better
outcome can be expected in anorectal malformation. There is also an intense
need to search for predisposing factors responsible for associated anomalies.
Acknowledgments
The authors
thank Mrs. M. Saeedi and A. Karimi for kind help and preparation of this
manuscript.
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