| Abstract|| |
To determine the incidence of rectourogenital fistulae and the possible role of fistulae on the mechanism of defecation in patients with high imperforate anus, we retrospectively studied 42 children, 38 males and four females, all with high imperforate anus presenting in the period from 1982 to1995. All children underwent radiological evaluation, to determine the level of the rectal pouch 18-24 hours after birth and to study the bony spine. A preliminary colostomy was performed in all patients after the diagnosis shortly after birth. Ultrasonography was performed in all the cases to assess the kidneys and ureters. All patients but one was operated on via a posterior sagittal anorectoplasty. Bowel control was assessed clinically regarding the presence of voluntary bowel movement, fecal soiling, incontinence and constipation. Eighteen (42.8%) patients were found to have rectourogenital fistulae; 15 rectourethral, two rectovaginal and one rectovesical. Outcomes were known for 36 out of 42 patients; 15(41.6 %) had voluntary bowel movements, nine (25 %) had soiling, six (16.6%) had constipation and six (16.6 %) had incontinence. In conclusion, the presence of rectourogenital fistula in patients with high imperforate anus is another new prognostic factor for anorectal function which needs further clarification.
Keywords: High imperforate anus, Rectourogenital fistulae, Bowel movements.
|How to cite this article:|
Daradka I, Hazza I. The Effect of Rectourogenital Fistula in High Imperforate Anus. Saudi J Kidney Dis Transpl 2007;18:186-90
|How to cite this URL:|
Daradka I, Hazza I. The Effect of Rectourogenital Fistula in High Imperforate Anus. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2015 Aug 1];18:186-90. Available from: http://www.sjkdt.org/text.asp?2007/18/2/186/32307
| Introduction|| |
Imperforate anus is a relatively common form of congenital anomaly in children that occurs in approximately one in every 5000 live births. It is caused by abnormal hindgut development of the embryo. It may be complicated by other congenital anomalies.
Anorectal malformation causes defecation disorder, which become a source of embarrassment, discomfort and stress to the patients. Many patients endure their affliction silently. Patients with imperforate anus are difficult to treat, especially those with high lesions.
Management of children with imperforate anus depends on accurate determination of the level and type of malformation, the developmental status of the sphincter muscle complex, the presence of associated anomalies and the type of fistula between the high blind end of the rectum and the urinary tract.
Following surgical correction of imperforate anus, voluntary bowel control is frequently poor because of abnormal anorectal function. ,
In this study, we report the incidence of recto-urogenital fistulae and possible prognostic relationships regarding fecal incontinence, soiling, and constipation in high imperforate anus patients
| Material and Methods|| |
We retrospectively studied 42 children, 38 males and 4 females, with high type imperforate anus treated at King Hussein Hospital from 1985 to 1995. All patients were diagnosed at birth as having a high imperforate anus. Following the diagnosis, all children underwent radiological evaluation to determine the level of the rectal pouch 18-24 hours after birth. Pouch-to perineum (p-p) distance measurements, using the lateral view x-ray of the lumbosacrum, while the patient was in the prone position with the pelvis elevated, were obtained. P-P distance of more than one centimeter was considered a high type anomaly. A preliminary colostomy (proximal descending colostomy) was performed in all patients after the diagnosis of high type anomaly shortly after birth.
Anterio-posterior and lateral sacral plain x - rays were used to evaluate the lumbo-sacral spine. Ultrasonography to assess the kidneys and ureters were performed in each case.
The recto-urogenital fistulae in patients with high anorectal atresia were diagnosed by using a pressure gastrographin enema through the distal colostomy opening, using standard techniques in which the contrast medium is forced by gentle pressure, filling the fistula.
All the patients but one underwent posteriorsagittal anorectoplasty (PSARP) by the age of 4-6 months. Adequate dilatation of the neoanus was performed using Hegar's dilators according to age, followed by closure of colostomy. Accordingly, the child born without an anus in the perineum should have been able to pass stool via the neo-anus by the age of seven to nine months.
There were 36 (87.6%) patients available for long-term assessment, following a PSARP procedure, from three to 12 years. Bowel control was assessed through the presence of voluntary bowel movements, fecal soiling, constipation or incontinence. Voluntary bowel movements were defined as the act of feeling the urge to use the toilet to have a bowel movement, and the ability to hold the bowel movement until the bathroom is reached. Soiling was defined as the involuntary leaking of small amounts of stool that lead to smearing of the underwear. Constipation was considered as the inability to empty the rectum spontaneously on a daily basis. Patients with incontinence were those who were unable to hold their feces. 
| Results|| |
[Table - 1] summarizes the results of our study. Among 38 boys who had complete data, 16 (42%) patients had recto-urological fistulae; 15 patients with recto urethral fistulae, and one with recto-vesicle fistula. Two out of four (50%) females had rectovaginal fistulae.
The sacrum was normal in 36 (87.6%) patients. Six boys (15.8%) had defects of their sacrum on the lumbosacral spine x-ray with 1-4 sacral segments missing. Four boys with sacral abnormalities that were associated with recto-urethral fistulae developed incontinence, while the other two developed soiling; urinary bladder dysfunction was observed in three patients.
Fifteen (35.9%) boys had voluntary bowel movements of which eight had rectourinary fistulae. Soiling involving both genders was seen in nine (22%) patients with no differences in relation to fistula type, and only two patients had sacral anomalies. Constipation was reported by six (14.2%) patients; five were boys but neither had association with fistulae, nor sacral anomalies. Incontinence was detected in six (14.2%) patients (5M. 1F), 4 of the five boys had combined sacral and fistula abnormalities. Incontinence was also seen in a boy who had neither fistula nor sacral abnormality.
Medical treatment with diet and bulk laxatives and rectal enemas successfully relieved constipation in all but one patient. Six children with incontinence who were unresponsive to conventional medical treatment were subjected to a bowel training program on a daily basis. Children with soiling achieved some degree of clinical improvement with time. Overall, 66.6% reported improvement in their bowel function with occasional soiling; the remaining three patients were subjected to a bowel training program.
All patients are now over 12 years old. We consider that 26 out of 36 (73%) patients had a satisfactory outcome regarding their bowel movements, and the percentage of patients with good fecal continence increased from 41.6% to 72%. Mortality occurred in three boys who had no fistula association; death was due to acute myelocytic leukemia (AML) in one Down's syndrome patient, respiratory failure in another and myelodysplasia and hydrocephalus in the third case. Three other patients were lost during follow-up.
| Discussion|| |
Major development of the anorectal canal occurs between the fifth and sixth weeks of fetal life. However, abnormalities during this period may occur. Decisions concerning initial management of anorectal fistulae can be made only after accurate determination of the level and type of malformation, the developmental status of the sphincter muscle complex, the presence of associated anomalies and the type of fistulae.
Fistulous communications between the blind rectum and urinary tract are common in boys with high anomalies. Lately because of the importance of these fistulae, lesions were classified into major clinical groups based on the fistulae location. Little research has been conducted regarding the presence of these fistulae in children with these disorders, and their effects, either on the ability to pass stool or to control defecation. In a span of a 10- year period, we had 42 high imperforate anus cases with 42.8 % association of recto urogenital fistulae (RUGF) in comparison with 58.6% to 90% reported elsewhere. ,,, This low association was probably due to operative technical or radiological errors. Nevertheless, the presence of RUGF either alone or in combination with congenital defects of the sacrum plays an important role in the results of bowel continence.
It is well known that abnormalities of the spine or spinal cord are common and found in patients with normal plain films. Accordingly, spinal ultrasonography or magnetic resonance imaging should be performed in patients with anorectal anomalies to rule out occult spinal pathology such as lipoma of the cord. Such conditions can mistakenly be attributed to the reconstructive procedure of the anorectum. ,,
The low incidence (15.8%) of sacral defects in our series in comparison to the 42% reported in the literature probably indicates that these abnormalities were underestimated due to inadequate investigations. Routine screening in the first six months for spinal pathology and surgical intervention may maximize anal sphincter function, and prevent or reduce secondary urologic morbidity that may result in the loss of renal function, as was seen in two of our patients.
Chronic constipation occurred in 16.6% of our patients postoperatively in comparison to 10 % reported by others., Among the reasons of constipation are iatrogenic extramural nerve injuries, malformation of intramural nerve or plexus such as hypo-ganglionosis or neuronal intestinal dysphasia, and dysganglionosis which cause an abnormal innervation pattern in the rectal pouch in children with high anomalies.
The presence of recto- urogenital fistulae clearly favors better prognosis concerning bowel movement, constipation and patient survival. However, it has a worse prognosis regarding incontinence, especially if associated with sacral defects.
In conclusion, the presence of recto- urogenital fistulae is another prognostic factor for continence in high imperforate anus. The relationship of fistulae with prognosis requires further study.
| References|| |
|1.||Tunnel WP, Austin JC, Barnes PD, Reynolds A. Neuroradiologic evaluation of sacral abnormalities in imperforate anus complex. J Pediatr Surg 1987;22:58-61. |
|2.||Kumar V, Rao PL, Vepakomma D. Low anorectal malformation associated with ano-urethra- cutaneous fistula. Pediatr Surg Int 2005; 21: 829-30. [PUBMED] [FULLTEXT]|
|3.||Brisseau GF, Langer JC. Surgical approaches to pediatric defecatory disorders. Curr Gastroenterol Rep 2000; 2:241-7. [PUBMED] |
|4.||Nievelstein RA, Vos A, Valk J. MR imaging of anorectal malformations and associated anomalies. Eur Radiol 1998;8(4):573-81. |
|5.||Heikenen JB, Werlin SL, Di Lorenzo C, et al. Colonic motility in children with repaired imperforate anus. Dig Dis Sci 1999; 44:1288-92. [PUBMED] [FULLTEXT]|
|6.||Endo M, Hayashi A, Ishihara M, et al. Analysis of 1,992 patients with anorectal malformations over the past two decades in japan. J Pediatr surg, 1999;34:435-41. |
|7.||Torres R, Levitt MA, Tovilla JM, Rodriguez G, Pena A. Anorectal malformations and Down's syndrome. J Pedioctr Surg 1998;33:2 pp: 194-7. |
|8.||Moazam F, Talbert JL. Congenital anorectal malformations. Arch Surg 1985;120:858-9. |
|9.||Nelson RL. Epidemiology of fecal incontinence. Gastroenterology 2004; 126:s3-7. [PUBMED] [FULLTEXT]|
|10.||Holschneider A, Hutson J, Pena A, et al. Preliminary report on the international conference for the development of standards for the treatment of anorectal malformations. J Pediatr Surg 2005;40:1521-6. [PUBMED] [FULLTEXT]|
|11.||Black CT, Sherman JO. The association of low imperforate anus and Down's syndrome. J Pediatr Surg 1989;24:92-4. [PUBMED] [FULLTEXT]|
|12.||Tsakayannis DE, Shamberger RC, Dimitris et al. Association of imperforate anus with occult spinal dysraphism. J Pediatr Surg 1995; 30:1010-2. |
|13.||Hoekstra WJ, Scholtmeijer R J, Molenaar J C, Schreeve R H, Schroeder FH. Urogenital tract abnormalities associated with congenital anomalies. J urol 1983;130:962-3. |
|14.||Shaul DB, Harrison EA. Classification of anorectal malformations-initial approach, diagnostic tests and colostomy. Semin Pediatr Surg 1997;6(4):187-95. |
|15.||Rivosecchi M, Lucchetti MC.Zaccara A, De Gennaro M, Fariello G. Spinal dysraphism detected by magnetic resonance imaging in patients with anorectal anomalies: incidence and clinical significance. J Pediatr Surg1995;30:488-90. |
|16.||Boemers TM, de Jong TP, van Gool JD, Bax KM. Urologic problems in anorectal malformations. Part 2: functional urologic sequelae. J Pediatr Surg 1996;31:634-7. |
|17.||Holschneider AM, Koebke J, MeierRuge W, Land N, Jesch NK, Pfrommer W. Pathophysiology of chronic constipation in anorectal malformations. Long - term results and preliminary anatomical investigations.Eur J Pediatr Surg 2001;11: 305-10. |
|18.||Bhat NA, Mathur M, Bhatnagar V. Sacrococcygeal teratoma with anorectal malformation. Indian J Gastroenterol. 2003;22: 27. [PUBMED] [FULLTEXT]|
|19.||Holschneider AM, Ure BM, Pfrommer W, Meier-Ruge W. Innervation patterns of the rectal pouch and fistula in anorectal malformations:a preliminary report. J Pediatr Surg 2000;31: 357-62. |
Department of Pediatrics, King Hussein Medical Center P.O. Box 540997, Amman11937
[Table - 1]