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Journal of Indian Association of Pediatric Surgeons
Medknow Publications on behalf of the Indian Association of Pediatric Surgeons
ISSN: 0971-9261 EISSN: 1998-3891
Vol. 10, Num. 1, 2005, pp. 44-47

Journal of Indian Association of Pediatric Surgeons, Vol. 10, No. 1, January-March, 2005, pp. 44-47

Original Article

Urogenital anomalies associated with anorectal malformation

Srivastava Vaishali, Ray A.K., Patra R., Saha Basu K., Samanta N., Saha K.

Department of Pediatric Surgery, N.R.S. Medical College and Hospital, Kolkata
Correspondence Address:Department of Pediatric Surgery, N.R.S. Medical College and Hospital, Kolkata. E-mail: samantan4@vsnl.net

Code Number: ip05011

Abstract

Background: The objective of the paper is to review the incidence and types of associated urogenital anomalies (U.G.A.) we encountered in patients with anorectal malformations (A.R.M.) and compare the results with previously published world literature.
Materials and Methods: Retrospective review was done of 220 cases of A.R.M., treated from May 2002 to April 2003. All patients routinely underwent ultrasound (U.S.) study of the K.U.B. region and pelvis and lumbosacral radiography. Voiding cystourethrography (V.C.U.G.), nuclear renography and other investigations like buccal smear and karyotyping were done in selected cases only.
Results: Genital anomalies were found in 30 cases (13.63%) and urologic anomalies in 25 cases (11.36%), a direct correlation being found between the level of A.R.M. and the incidence of urogenital anomalies (U.G.A.).
Conclusion: The high incidence of associated anomalies (24.54%) makes careful clinical examination and evaluation of the urinary tract during the neonatal or early infantile period mandatory in all cases of A.R.M., particularly to avoid deterioration of renal function in future.

Key words: Urogenital anomalies, anorectal malformations, congenital

INTRODUCTION

Congenital anomalies of the urogenital tract are the leading associated anomalies in patients with ARM.[1] Previous studies show the incidence of UGA associated with high or intermediate forms of ARM to be approximately 50-60%[2] and with low anomalies to be 15-20%. Urologic abnormalities have been found to predominate in this group of anomalies.[3]

Usually the management of the ARM gets priority and the urological evaluation is not always given its due importance, though in many cases the urinary tract disorder is the primary cause of morbidity and mortality.[4]

Materials and Methods

The records of 220 patients with congenital anorectal malformations (ARM) who were admitted from May 2002 to April 2003 were reviewed retrospectively. Of these, 65 (29.65%) were follow-up patients who had already undergone a primary colostomy and were admitted for a revision of the colostomy, for definitive operation or for a colostomy closure following definitive operation. [Table - 1].The rest 70.45% (155 patients) were newly diagnosed cases of ARM [Table - 2]. All patients underwent ultrasound (US) studies of the KUB region and pelvis for detection of urogenital abnormalities and lumbosacral radiography. Voiding cystourethrography (VCUG) was done in patients diagnosed with an abnormality in USG and other investigations like nuclear renography, IVU, urodynamic studies, fistulography, buccal smear and karyotyping were done in appropriate cases. All follow-up patients had their serum urea and creatinine levels evaluated.

The patients were grouped into two categories. The patients with genital abnormalities were clinically diagnosed with external examinations followed by laboratory investigations. The urological anomalies were mainly detected by imaging studies (23 out of 25).

Results

In this study a total of 55 patients (24.54%), [10 (18%) females and 45 (81.82%) males], had an abnormality of the urogenital tract. There were 25 (45.45%) urinary and 30 (54.55%) genital abnormalities. [Table - 3] and [Table - 4]. The most common abnormalities were hypospadias (9 patients -16.36%) and penoscrotal transposition (7 patients -12.72%). Undescended testes were seen in 4 patients (7.27%), unilateral in 3 and bilateral in 1. Two patients (3.63%) had ambiguous genitalia. 1 patient had normally descended testes in the scrotal sac but no penis (urethral opening was seen in the anterior wall of rectum) while another had a pseudoduplication of the external genitalia with an accessory phallus and scrotal sac.1 patient had a complete duplication of external genitalia. Two patients had ovarian cysts (3.63%), 1 antenatally diagnosed had bilateral ovarian cysts while the other had a unilateral ovarian cyst. 1 patient had vaginal atresia.

The most common renal abnormality viz. renal ectopia, was diagnosed in 5 patients (9.09%). Of these 2 cases were of crossed renal ectopia and the rest were pelvic kidneys. Unilateral moderate to severe hydronephrosis, due to PUJ obstruction, was present in 4 patients (7.27%). Unilateral renal agenesis was detected in 3 patients (5.45%). Urethral stricture was also present in 3 patients (5.45%); of these, 1 had multiple urethral strictures [Figure - 1]. 3 patients (5.45%) were demonstrated to have VUR [Figure - 2] in VCUG which was performed in in 55 cases. Unilateral renal dysplasia was detected in 2 patients (2.63%). One female with bladder exstrophy presented at the age of 9 years with severe constipation due to a stenotic anterior ectopic anus, having had no past surgical treatment. 1 patient had a patent urachus and 1 had an ectopic urethra.

The incidence of urogenital anomalies was found to bear a direct correlation with the level of ARM. (Table 5). UGA were found in 5 out of 31 low lesions (16.12%), 39 out of 173 intermediate lesions, (22.54%) and 11 out of 16 high lesions (68.75%). Of the 16 high ARM cases, 13 were females and 3 were males. Of the low ARM cases 23 (74.19%) were males and 8 (25.81%) were females.

Discussion

Of all anomalies associated with ARM, urogenital anomalies are the most frequent.[1] The incidence of associated UGA. from different series varies widely from 25% to 60%[1],[4]-[13] reflecting the number of patients with higher versus lower malformations.

Partridge and Gough, in 1961, reported 9% incidence of urologic anomalies in low ARM and 30% in high ARM.[5] McLorie et al noted incidence of 20% and 60% respectively.[11] Parrot reported figures of 14% with low, 21% with intermediate and 40% with high anomalies.[12] Rich et al showed 25%, 42% and 71% associations.[6] Tohda et al found UGA in 38.1% of low, 65.5% of intermediate and 85.7% of high ARM.[13] Our study shows figures of 16.12%, 22.54% and 68.75% respectively in low, intermediate and high ARM cases. Genital anomalies were found in 14% and urologic anomalies in 25.6% by Sangkhathat et al .[14] The most common abnormality in our study was hypospadias, seen in 9 cases (4.09% overall). Hoekstra et al reported a 6% incidence of hypospadias.[7] Renal agenesis, reported to be the most common UGA in literature[4],[6] was encountered in only 3 patients in our study. Instead the most common renal anomaly seen was renal ectopia, present in 5 patients (2.27%). VUR was seen only in 3 patients of the 55 who underwent VCUG (5.45% of patients investigated and 1.36% overall) as against 47% by Narasimha Rao et al[16], 14% by Rich et al[6] and 8.6% by Sangkhathat et al .[14]

Ideally, the ARGUS protocol devised by Boemer et al[15] should be used for evaluating newborns with ARM. However, the overwhelming number of patients we deal with(as shown in this study) and the poor socioeconomic condition of most of these patients, precludes the use of all investigations suggested by Boemer et al to be mandatory in all patients with ARM. We routinely performed USG and X-ray spine (to detect vertebral anomalies) in all cases, and VCUG was done only in patients detected with an UGA. Thus, the actual incidence of reflux is probably higher than diagnosed.

Conclusion

ARM is associated in a large number of cases with urogenital anomalies (UGA) of a wide diversity ranging from an undescended testis to duplication of the external genitalia and from renal agenesis to multiple urethral strictures.

While many of these are obvious on inspection, especially the genital abnormalities, many others are clinically not apparent but have the potential of causing major morbidity and even mortality ,though the incidence of such anomalies in our study was low. These require extensive investigations for diagnosis. It is imperative that a thorough clinical examination and systematic investigations are carried out to diagnose those anomalies early in life to avoid future complications.

Hence, in the management of ARM, not only should we consider the different techniques suitable for repair of different types of ARM, but for better results we should be very meticulous in ruling out or diagnosing and treating appropriately associated anomalies of the urogenital tract which may pose a serious problem in post-operative long term follow-up.

Reference

1.	Gupta DK. Management of associated anomalies with anorectal malformations. Presented at A.R.M. Varanasi: Update & Workshop; 2001. Back to cited text no. 1
2.	Rowe MI, O'Neill JA Jr, Grosfeld JL, et al . Anorectal Disorders. Essentials of Pediatric Surgery. Missouri: Mosby-Year Book Inc.; 1995. p. 601-9. Back to cited text no. 2
3.	Kiely EM, Pena A. Anorectal Malformations. In O'Neill JA (Jr), Rowe MI, Grosfeld JL, editors. Pediatric Surgery, Vol. 2, 5th Ed., Missouri: Mosby-Year Book Inc.; 1998. p. 1425-48. Back to cited text no. 3
4.	Belman BA, King LR. Urinary tract abnormalities associated with imperforate anus. J Urol 1982;108:823-4. Back to cited text no. 4
5.	Partridge JP, Gough MH. Congenital abnormalities of the anus and rectum. Br J Surg 1961;49:37. Back to cited text no. 5
6.	Rich MA, Brock WA, Pena A. Spectrum of genitourinary malformations in patients with imperforate anus. Pediatr Surg Int 1988;3:110-3. Back to cited text no. 6
7.	Hoekstra WJ, Scholtmeijer RJ, Molenar JC, et al . Urogenital tract abnormalities associated with congenital anorectal anomalies. J Urol 1983;130:962-3. Back to cited text no. 7
8.	Munn R, Schillinger JF. Urologic abnormalities found with imperforate. Anus Urol 1983;21;260-4. Back to cited text no. 8
9.	Tank SE. Latent anomalies associated with imperforate anus. Arch Surg 1970;100:512-4. Back to cited text no. 9
10.	Wiener ES, Kiesewetter WB. Urologic abnormalities associated with imperforate anus. J Pediatr Surg 1973;8:151-7. Back to cited text no. 10
11.	McLorie G, Sheldon M, Fleisher M, et al . The genitourinary system in patients with imperforate anus. J Pediatr Surg 1987;22:1100. Back to cited text no. 11
12.	Parrot TS. Urologic implications of anorectal malformations. Urol Clin North Am 1985;12:13-21. Back to cited text no. 12
13.	Tohda A, Hosokawa S, Shimada K. Urinary tract abnormalities associated with anorectal malformations. Nippon Hinyokika Gakkai Zasshi 1995;86:1388-93. Back to cited text no. 13
14.	Sangkhathat S, Patrapinyokul S, Tadtayathikom K. Associated genitourinary tract anomalies in anorectal malformations: A thirteen year review. J Med Assoc Thai 2002;84:289-96. Back to cited text no. 14
15.	Boemers TML, Beek FJA, Bax NMA. Guidelines for the urological screening and initial management of lower urinary tract dysfunction in children with anorectal malformations; the ARGUS protocol. BJU Int 1999;83:662-71. Back to cited text no. 15
16.	Rao KLN, Prasad GR, Mukhopadhyay B, et al . Vesicoureteric reflux in neonates with anorectal anomalies. Br J Urol 1975;55:268-270. Back to cited text no. 16

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