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Fecal continence in patients having undergone posterior sagittal anorectoplasty procedure for a high anorectal malformation improves at adolescence, as constipation disappears
Fecal Continence in Patients Having Undergone Posterior Sagittal Anorectoplasty Procedure for a High Anorectal Malformation Improves at Adolescence, as Constipation Disappears By R.J. Rintala and H.G. Lindahl Helsinki, Finland
Background/Purpose: Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient’s growth period. Methods: The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord.
before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result.
Conclusion: In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome. J Pediatr Surg 36:1218-1221. Copyright © 2001 by W.B. Saunders Company.
Results: At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated
INDEX WORDS: Anorectal malformations, imperforate anus, fecal continence, long-term outcome, constipation.
T
childhood of patients who have undergone PSARP procedure as a definitive repair is largely lacking. Constipation is a major postoperative functional complication in patients having undergone PSARP procedure.5,6 The incidence of constipation has been reported to range between 10% to 73%.6,7 In constipated patients, overflow incontinence is the main cause of fecal soiling. The aim of this study was to outline the natural history of constipation in patients who have undergone PSARP procedure for a high anorectal malformation and relate this to the functional outcome at the end of the patients’ growth period.
HE OVERALL FECAL continence outcome in patients with severe anorectal malformations has been dismal after repair with traditional methods. Only a small percentage of patients have resumed normal bowel habits without any soiling.1-3 Since the 1980s posterior sagittal anorectoplasty (PSARP) has gained acceptance as a standard procedure for the repair of high anorectal malformations, and short and medium-term functional outcome appear to be promising.4,5 However, reliable data concerning long-term fecal continence beyond
From Children’s Hospital, University of Helsinki, Helsinki, Finland. Presented at the 2000 Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, Chicago, Illinois, October 28November 1, 2000. Address reprint requests to R.J. Rintala, Professor of Pediatric Surgery, Children’s Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Finland. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3608-0024$35.00/0 doi:10.1053/jpsu.2001.25766 1218
MATERIALS AND METHODS Between 1984 and 1999 169 patients with high or intermediate anorectal malformations have undergone PSARP procedure by the authors. Of these patients, 22 have reached adolescence and puberty and were beyond their main growth period at the time of the current study. Nine of these patients were girls and 13 boys. The median age at the time of the study was 15 years (range, 13 to 25 years). The types of the anomalies of the patients are summarized in Table 1. All the patients have been followed-up regularly since birth. Data concerning the bowel function of the patients during childhood Journal of Pediatric Surgery, Vol 36, No 8 (August), 2001: pp 1218-1221
PSARP PROCEDURE: OUTCOME AT ADOLESCENCE
1219
Table 1. Patient Data Male (n ⫽ 13)
Rectourethral fistula Rectobulbar fistula No fistula, high anomaly Cloaca Rectovestibular fistula Rectovaginal fistula
Female (n ⫽ 9)
9 2 2 4 4 1
was retrieved retrospectively from hospital records. Histology reports of patients who had undergone late rectal biopsy for recalcitrant constipation also were reviewed retrospectively. All patients were interviewed and had a clinical examination during 1998 and 1999. The parents were interviewed without the patient’s presence to ascertain the functional outcome indicated by the patient. All patients had anorectal manometry and underwent sacral x-ray and magnetic resonance imaging (MRI) of the spine and spinal cord. The manometric variables recorded were anorectal resting pressure, presence of rectoanal relaxation reflex (RAR), voluntary sphincter squeeze, and rectal sensibility. The manometric techniques have been described recently elsewhere.8 As controls we used a population of children who had undergone anorectal manometry for constipation (n ⫽ 17; age range, 8 to 17 years). Clinically, the fecal continence was graded as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas or the antegrade colonic enema (ACE) procedure. Constipation was defined as a need to use medical treatment or regular special diet to ensure bowel emptying. Spearman’s correlation coefficient was used to test the correlation between the functional outcome and manometric variables. MannWhitney U test was used to compare the manometric findings of the patient to those of the controls. A P value less than .05 was considered statistically significant.
RESULTS
Fifteen of the 22 patients (68%) had suffered from constipation before the onset of puberty. Six of these 15 patients had required long-term bowel enema regimen throughout the childhood to treat the constipation and to avoid overflow incontinence. Further, 2 patients had used enemas during their early childhood. The remaining 7 patients had used stimulant or bulk laxatives regularly. Twelve of these 15 patients had undergone rectal biopsy for the detection of innervation abnormalities at the age of 2 to 6 years. One boy had a short segment aganglionosis and underwent a pull-through operation. No neuronal abnormalities were found in any of the remaining 11 patients. At the time of the study 2 of the 15 previously constipated patients (9%) still suffered from constipation. A boy of 14 years of age managed to maintain bowel function most of the time by using stimulant laxatives but used enema courses when he had more soiling or symptoms of fecal impaction. A 16-year-old girl used regular enemas. Originally, she had a high rectovaginal fistula, severe hemisacral defects, and a huge presacral teratoma compatible with the Currarrino
triad. Despite good clinical and manometric anal tone and voluntary sphincter function she remains unable to empty her bowel because of completely lacking rectal sensation. Eleven (50%) patients were fully continent (grade 1) with voluntary bowel movements. At the time of the study none of them had constipation, but 6 required medical therapy for constipation and occasional overflow soiling earlier during childhood. Three patients (14%) had grade 2 continence outcome; they were socially continent with fecal staining less than once a week. None of them required treatment for constipation at the time of the study. However, 2 of them had constipation and occasional episodes of overflow soiling before the onset of puberty. Five patients (23%) had grade 3 continence outcomes with staining episodes more than once a week. All these patients had had long-term constipation during childhood. In 4 patients, constipation requiring medical treatment had disappeared at puberty; in 1 patient constipation had resolved at the age of 7 years. Three patients (14%) had a poor (grade 4) functional outcome. Two of these required continuous enema regimen to empty their bowel and stay socially clean. The cause for poor outcome in these patients was recalcitrant constipation in 2 and severe sphincter insufficiency in 1 who is scheduled for an ACE procedure. The development of continence in relation to constipation is summarized in Table 2. The results of anorectal manometry are summarized in Table 3. The control patients had significantly higher anal resting and squeeze pressures than the patients. All controls and 17 of the 22 patients (77%) had positive rectoanal relaxation reflex (RAR). The anal resting pressure was significantly higher in patients with positive RAR (median resting pressure for those with RAR, 47 cm H2O; range, 25 to 60 cm H2O; median resting pressure for those without RAR, 32 cm H2O; range, 30 to 40 cm H2O; P ⬍ .02). Among the patients, the only manometric parameter that correlated with the continence outcome was the voluntary sphincter squeeze (P ⬍ .05). Bony sacral anomalies were detected in 15 patients. In 4, the anomalies were severe with more than 2 sacral vertebrae missing. Four patients had intraspinal abnorTable 2. Development of Continence and Constipation
Continence (n ⫽ 22) Normal bowel habits Occasional staining Frequent staining Poor outcome (regular enemas) Constipation (n ⫽ 15) Regular enema regimen Occasional enemas Regular laxatives
Before Puberty
After Puberty
5 8 6 3
11 3 5 3
6 2 7
2 0 0
1220
RINTALA AND LINDAHL
Table 3. Results of Anorectal Manometry
Median resting pressure, cm H2O (range) Median voluntary squeeze, cm H2O (range) Rectoanal inhibitory reflex (RAR), % Median sensory threshold, mL (range)
Patients (n ⫽ 22)
Controls (n ⫽ 17)
P Value
41 (25-60) 98 (60-160) 77 25 (15-45)
60 (45-80) 120 (80-164) 100 25 (10-60)
.0001 .001
malities (tethering in 2, intraspinal lipomas in 2); all of them had sacral anomalies also. In a small patient series such as this the variability of intraspinal and sacral abnormalities preclude statistical comparisons in terms of correlations between presence of abnormality and continence outcome. DISCUSSION
The PSARP procedure appears to offer best chances for a reasonable fecal continence in patients with severe anorectal malformations,4,5,8 although contradictory results also have been reported.7,9,10 Constipation is a major functional problem after PSARP procedure and often is associated with overflow soiling. The cause of constipation in these patients is unclear. Its incidence has been reported to vary between 10% and 73%.5-7 The constipation rarely is associated with anal stenosis or stricture.5,6 Neuronal abnormalities of the rectal blind pouch have been suggested as a cause of constipation.11 We also have shown earlier that the region of the rectourogenital fistula, the ectopic anus, is neuronally abnormal,12 but these changes appear to disappear as this outlet channel resumes its normal function. Later in childhood, 12 of the 15 constipated patients of the current study population had rectal biopsy, which showed normal neuronal anatomy in all but one patient. The congenital rectosigmoid dilatation that is common in patients with anorectal malformations13 may have significance in the pathogenesis of constipation. It is important to note that the tapering, which is considered an essential part of the PSARP procedure, cannot be extended to the upper parts of the rectum and to the sigmoid through the standard sacroperineal approach. Therefore, at least the upper part of the rectum and sigmoid remain dilated. It may be speculated that the rectal sensibility is decreased because of extensive dissection required for rectal mobilization. This is apparent from the findings in this study. The sensory threshold was significantly higher than that of healthy subjects.14 The fact that no difference was noted between the patients and controls is because of abnormal sensory threshold of the constipated subjects.14-16 The preservation of functional internal sphincter also may contribute to development of constipation as it increases the resting pressure gradient between rectum and anal canal.8 The current study clearly shows that constipation dis-
Not significant
appears at puberty in the majority of patients. The cause of this, however, remains unclear. In the study population the change in bowel habits from constipation requiring treatment to more or less normal bowel frequency usually was quite abrupt. The time of disappearance of constipation was very uniform in the current patients and was clearly related to the growth spurt and sexual maturation of puberty. This feature is quite similar to what occurs in patients suffering from functional constipation: the symptoms usually disappear at the onset of puberty.17 On a speculative level this very uniform clinical progress in these patients suggests that the maturation of bowel function could be related to hormonal changes at puberty. Another explanation could be a significant enhancement of social motivation when the patients are reaching adolescence. However, it is difficult to understand how mental and social maturation would affect constipation. The disappearance of constipation at this age clearly is an open question that deserves further research. In the current series the percentage of patients gaining normal bowel function at the end of their growth period exceeds significantly what has been reported earlier in patients with high anorectal anomalies but are in line with the results reported in a recent review by Pen˜a and Hong.18 Overall, 37.5% of Pen˜a’s 1,192 patients of variable age distribution had normal bowel function. In the few true long-term follow-up reports the percentage of patients with voluntary and normal bowel function varies between 0 to 15%.1-3 Although most patients in these reports have been able to maintain some degree of social continence with adjunctive measures such as regular enemas, staying near toilets, wearing liners or diapers, or having dietary restrictions, objective evaluation of the data concerning bowel function provides a grim picture of the true level of fecal continence. Moreover, many of these patients have undergone secondary sphincter repairs to improve the defective bowel control. The percentage of patients having secondary sphincter surgery has varied between 30% to 68%.1-3 In the current series none of the patients underwent secondary sphincter repairs. Two patients underwent resection of the megarectosigmoid and one a pull-through procedure for associated Hirschsprung’s disease. The manometric findings in the study patients clearly were abnormal. All the recorded variables showed subnormal values. Abnormal manometric findings are not
PSARP PROCEDURE: OUTCOME AT ADOLESCENCE
1221
unexpected because repair of a high anorectal malformation includes major manipulation of the sphincter complex. Moreover, the voluntary sphincter complex is hypoplastic at least to some extent in most of the patients with high anorectal malformations. The findings concerning voluntary sphincter squeeze still are encouraging. Most patients were able to double the resting pressure of the anal canal; in healthy subjects this is considered as a sign of a well-functioning voluntary sphincter system. Voluntary sphincter squeeze was the only manometric parameter that correlated with good continence outcome with statistical significance. The anal resting pressure was higher in patients with normal continence, but this did not reach statistical significance. The spinal and intraspinal abnormalities were screened
in the patients of the study. Sacral anomalies were very common (68%) and intraspinal abnormalities also were relatively frequently detected (18%). The small number of patients and the great variability of the types of spinal and intraspinal defects preclude reliable statistical analysis of correlation between the presence of these anomalies and continence outcome. Constipation, which is a major source of functional problems in early childhood, disappears at adolescence in the majority of patients who have undergone PSARP procedure for a high anorectal malformation. The disappearance of constipation is associated with improved fecal continence outcome. The cause of this radical change of bowel function is unclear and warrants further studies.
REFERENCES 1. Rintala R, Mildh L, Lindahl H: Fecal continence and quality of life in adult patients with an operated high or intermediate anorectal malformation. J Pediatr Surg 29:777-780, 1994 2. Hassink EA, Rieu PN, Severijnen RS, et al: Are adults content or continent after repair for high anal atresia? A long-term follow-up study in patients 18 years of age and older. Ann Surg 218:196-200, 1993 3. Nixon HH, Puri P: The results of treatment of anorectal anomalies: A thirteen to twenty year follow-up. J Pediatr Surg 12:27-37, 1977 4. Rintala R, Lindahl H: Is normal bowel function possible after repair of intermediate and high anorectal malformations. J Pediatr Surg 30:491-494, 1995 5. Pen˜a A: Anorectal malformations. Semin Pediatr Surg 4:35-47, 1995 6. Rintala R, Lindahl H, Marttinen E, et al: Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations. J Pediatr Surg 28:1054-1058, 1995 7. Langemeijer RATM, Molenaar JC: Continence after posterior sagittal anorectoplasty. J Pediatr Surg 26:587-590, 1991 8. Rintala RJ, Lindahl HG: Posterior sagittal anorectoplasty is superior to sacroperineal- sacroabdominoperineal pull-through: A longterm follow-up study in boys with high anorectal anomalies. J Pediatr Surg 34:334-337, 1999 9. Mulder W, deJong, E, Wauters I, et al: Posterior sagittal anorectoplasty: Functional results of primary and secondary operations in comparison to the pull-through method in anorectal malformations. Eur J Pediatr Surg 5:170-173, 1995
10. Bliss DP, Tapper D, Anderson JM, et al: Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence. J Pediatr Surg 31:26-32, 1996 11. Holschneider AM, Pfrommer W, Gerresheim B: Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur J Pediatr Surg 4:303-309, 1994 12. Rintala R, Lindahl H, Sariola H, et al: The rectourogenital connection in anorectal malformations is an ectopic anal canal. J Pediatr Surg 25:665-668, 1990 13. Brent L, Stephens FD: Primary rectal ectasia: A quantitative study of smooth muscle cells in normal and hypertrophied human bowel. Progr Pediatr Surg 9:41-62, 1976 14. Holschneider AM: Elektromanometrie des Enddarms, MunichWien-Baltimore, Urban & Schwarzenberg, 1983 15. Meunier P, Marechal JM, de Beaujeu MJ: Rectoanal pressures and rectal sensitivity studies in chronic childhood constipation. Gastroenterology 77:330-336, 1979 16. Loening-Baucke V, Yamada T: Is the afferent pathway from the rectum impaired in children with chronic constipation and encopresis? Gastroenterology 109:397-403, 1995 17. Abrahamian FP, Lloyd-Still JD: Chronic constipation in childhood: A longitudinal study of 186 patients. J Pediatr Gastroenterol Nutr 3:460-467, 1984 18. Pen˜a A, Hong A: Advances in the management of anorectal malformations. Am J Surg 180:370-376, 2000 AQ1: Please spell out ACE
Background/Purpose: Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient’s growth period. Methods: The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord.
before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result.
Conclusion: In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome. J Pediatr Surg 36:1218-1221. Copyright © 2001 by W.B. Saunders Company.
Results: At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated
INDEX WORDS: Anorectal malformations, imperforate anus, fecal continence, long-term outcome, constipation.
T
childhood of patients who have undergone PSARP procedure as a definitive repair is largely lacking. Constipation is a major postoperative functional complication in patients having undergone PSARP procedure.5,6 The incidence of constipation has been reported to range between 10% to 73%.6,7 In constipated patients, overflow incontinence is the main cause of fecal soiling. The aim of this study was to outline the natural history of constipation in patients who have undergone PSARP procedure for a high anorectal malformation and relate this to the functional outcome at the end of the patients’ growth period.
HE OVERALL FECAL continence outcome in patients with severe anorectal malformations has been dismal after repair with traditional methods. Only a small percentage of patients have resumed normal bowel habits without any soiling.1-3 Since the 1980s posterior sagittal anorectoplasty (PSARP) has gained acceptance as a standard procedure for the repair of high anorectal malformations, and short and medium-term functional outcome appear to be promising.4,5 However, reliable data concerning long-term fecal continence beyond
From Children’s Hospital, University of Helsinki, Helsinki, Finland. Presented at the 2000 Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, Chicago, Illinois, October 28November 1, 2000. Address reprint requests to R.J. Rintala, Professor of Pediatric Surgery, Children’s Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Finland. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3608-0024$35.00/0 doi:10.1053/jpsu.2001.25766 1218
MATERIALS AND METHODS Between 1984 and 1999 169 patients with high or intermediate anorectal malformations have undergone PSARP procedure by the authors. Of these patients, 22 have reached adolescence and puberty and were beyond their main growth period at the time of the current study. Nine of these patients were girls and 13 boys. The median age at the time of the study was 15 years (range, 13 to 25 years). The types of the anomalies of the patients are summarized in Table 1. All the patients have been followed-up regularly since birth. Data concerning the bowel function of the patients during childhood Journal of Pediatric Surgery, Vol 36, No 8 (August), 2001: pp 1218-1221
PSARP PROCEDURE: OUTCOME AT ADOLESCENCE
1219
Table 1. Patient Data Male (n ⫽ 13)
Rectourethral fistula Rectobulbar fistula No fistula, high anomaly Cloaca Rectovestibular fistula Rectovaginal fistula
Female (n ⫽ 9)
9 2 2 4 4 1
was retrieved retrospectively from hospital records. Histology reports of patients who had undergone late rectal biopsy for recalcitrant constipation also were reviewed retrospectively. All patients were interviewed and had a clinical examination during 1998 and 1999. The parents were interviewed without the patient’s presence to ascertain the functional outcome indicated by the patient. All patients had anorectal manometry and underwent sacral x-ray and magnetic resonance imaging (MRI) of the spine and spinal cord. The manometric variables recorded were anorectal resting pressure, presence of rectoanal relaxation reflex (RAR), voluntary sphincter squeeze, and rectal sensibility. The manometric techniques have been described recently elsewhere.8 As controls we used a population of children who had undergone anorectal manometry for constipation (n ⫽ 17; age range, 8 to 17 years). Clinically, the fecal continence was graded as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas or the antegrade colonic enema (ACE) procedure. Constipation was defined as a need to use medical treatment or regular special diet to ensure bowel emptying. Spearman’s correlation coefficient was used to test the correlation between the functional outcome and manometric variables. MannWhitney U test was used to compare the manometric findings of the patient to those of the controls. A P value less than .05 was considered statistically significant.
RESULTS
Fifteen of the 22 patients (68%) had suffered from constipation before the onset of puberty. Six of these 15 patients had required long-term bowel enema regimen throughout the childhood to treat the constipation and to avoid overflow incontinence. Further, 2 patients had used enemas during their early childhood. The remaining 7 patients had used stimulant or bulk laxatives regularly. Twelve of these 15 patients had undergone rectal biopsy for the detection of innervation abnormalities at the age of 2 to 6 years. One boy had a short segment aganglionosis and underwent a pull-through operation. No neuronal abnormalities were found in any of the remaining 11 patients. At the time of the study 2 of the 15 previously constipated patients (9%) still suffered from constipation. A boy of 14 years of age managed to maintain bowel function most of the time by using stimulant laxatives but used enema courses when he had more soiling or symptoms of fecal impaction. A 16-year-old girl used regular enemas. Originally, she had a high rectovaginal fistula, severe hemisacral defects, and a huge presacral teratoma compatible with the Currarrino
triad. Despite good clinical and manometric anal tone and voluntary sphincter function she remains unable to empty her bowel because of completely lacking rectal sensation. Eleven (50%) patients were fully continent (grade 1) with voluntary bowel movements. At the time of the study none of them had constipation, but 6 required medical therapy for constipation and occasional overflow soiling earlier during childhood. Three patients (14%) had grade 2 continence outcome; they were socially continent with fecal staining less than once a week. None of them required treatment for constipation at the time of the study. However, 2 of them had constipation and occasional episodes of overflow soiling before the onset of puberty. Five patients (23%) had grade 3 continence outcomes with staining episodes more than once a week. All these patients had had long-term constipation during childhood. In 4 patients, constipation requiring medical treatment had disappeared at puberty; in 1 patient constipation had resolved at the age of 7 years. Three patients (14%) had a poor (grade 4) functional outcome. Two of these required continuous enema regimen to empty their bowel and stay socially clean. The cause for poor outcome in these patients was recalcitrant constipation in 2 and severe sphincter insufficiency in 1 who is scheduled for an ACE procedure. The development of continence in relation to constipation is summarized in Table 2. The results of anorectal manometry are summarized in Table 3. The control patients had significantly higher anal resting and squeeze pressures than the patients. All controls and 17 of the 22 patients (77%) had positive rectoanal relaxation reflex (RAR). The anal resting pressure was significantly higher in patients with positive RAR (median resting pressure for those with RAR, 47 cm H2O; range, 25 to 60 cm H2O; median resting pressure for those without RAR, 32 cm H2O; range, 30 to 40 cm H2O; P ⬍ .02). Among the patients, the only manometric parameter that correlated with the continence outcome was the voluntary sphincter squeeze (P ⬍ .05). Bony sacral anomalies were detected in 15 patients. In 4, the anomalies were severe with more than 2 sacral vertebrae missing. Four patients had intraspinal abnorTable 2. Development of Continence and Constipation
Continence (n ⫽ 22) Normal bowel habits Occasional staining Frequent staining Poor outcome (regular enemas) Constipation (n ⫽ 15) Regular enema regimen Occasional enemas Regular laxatives
Before Puberty
After Puberty
5 8 6 3
11 3 5 3
6 2 7
2 0 0
1220
RINTALA AND LINDAHL
Table 3. Results of Anorectal Manometry
Median resting pressure, cm H2O (range) Median voluntary squeeze, cm H2O (range) Rectoanal inhibitory reflex (RAR), % Median sensory threshold, mL (range)
Patients (n ⫽ 22)
Controls (n ⫽ 17)
P Value
41 (25-60) 98 (60-160) 77 25 (15-45)
60 (45-80) 120 (80-164) 100 25 (10-60)
.0001 .001
malities (tethering in 2, intraspinal lipomas in 2); all of them had sacral anomalies also. In a small patient series such as this the variability of intraspinal and sacral abnormalities preclude statistical comparisons in terms of correlations between presence of abnormality and continence outcome. DISCUSSION
The PSARP procedure appears to offer best chances for a reasonable fecal continence in patients with severe anorectal malformations,4,5,8 although contradictory results also have been reported.7,9,10 Constipation is a major functional problem after PSARP procedure and often is associated with overflow soiling. The cause of constipation in these patients is unclear. Its incidence has been reported to vary between 10% and 73%.5-7 The constipation rarely is associated with anal stenosis or stricture.5,6 Neuronal abnormalities of the rectal blind pouch have been suggested as a cause of constipation.11 We also have shown earlier that the region of the rectourogenital fistula, the ectopic anus, is neuronally abnormal,12 but these changes appear to disappear as this outlet channel resumes its normal function. Later in childhood, 12 of the 15 constipated patients of the current study population had rectal biopsy, which showed normal neuronal anatomy in all but one patient. The congenital rectosigmoid dilatation that is common in patients with anorectal malformations13 may have significance in the pathogenesis of constipation. It is important to note that the tapering, which is considered an essential part of the PSARP procedure, cannot be extended to the upper parts of the rectum and to the sigmoid through the standard sacroperineal approach. Therefore, at least the upper part of the rectum and sigmoid remain dilated. It may be speculated that the rectal sensibility is decreased because of extensive dissection required for rectal mobilization. This is apparent from the findings in this study. The sensory threshold was significantly higher than that of healthy subjects.14 The fact that no difference was noted between the patients and controls is because of abnormal sensory threshold of the constipated subjects.14-16 The preservation of functional internal sphincter also may contribute to development of constipation as it increases the resting pressure gradient between rectum and anal canal.8 The current study clearly shows that constipation dis-
Not significant
appears at puberty in the majority of patients. The cause of this, however, remains unclear. In the study population the change in bowel habits from constipation requiring treatment to more or less normal bowel frequency usually was quite abrupt. The time of disappearance of constipation was very uniform in the current patients and was clearly related to the growth spurt and sexual maturation of puberty. This feature is quite similar to what occurs in patients suffering from functional constipation: the symptoms usually disappear at the onset of puberty.17 On a speculative level this very uniform clinical progress in these patients suggests that the maturation of bowel function could be related to hormonal changes at puberty. Another explanation could be a significant enhancement of social motivation when the patients are reaching adolescence. However, it is difficult to understand how mental and social maturation would affect constipation. The disappearance of constipation at this age clearly is an open question that deserves further research. In the current series the percentage of patients gaining normal bowel function at the end of their growth period exceeds significantly what has been reported earlier in patients with high anorectal anomalies but are in line with the results reported in a recent review by Pen˜a and Hong.18 Overall, 37.5% of Pen˜a’s 1,192 patients of variable age distribution had normal bowel function. In the few true long-term follow-up reports the percentage of patients with voluntary and normal bowel function varies between 0 to 15%.1-3 Although most patients in these reports have been able to maintain some degree of social continence with adjunctive measures such as regular enemas, staying near toilets, wearing liners or diapers, or having dietary restrictions, objective evaluation of the data concerning bowel function provides a grim picture of the true level of fecal continence. Moreover, many of these patients have undergone secondary sphincter repairs to improve the defective bowel control. The percentage of patients having secondary sphincter surgery has varied between 30% to 68%.1-3 In the current series none of the patients underwent secondary sphincter repairs. Two patients underwent resection of the megarectosigmoid and one a pull-through procedure for associated Hirschsprung’s disease. The manometric findings in the study patients clearly were abnormal. All the recorded variables showed subnormal values. Abnormal manometric findings are not
PSARP PROCEDURE: OUTCOME AT ADOLESCENCE
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unexpected because repair of a high anorectal malformation includes major manipulation of the sphincter complex. Moreover, the voluntary sphincter complex is hypoplastic at least to some extent in most of the patients with high anorectal malformations. The findings concerning voluntary sphincter squeeze still are encouraging. Most patients were able to double the resting pressure of the anal canal; in healthy subjects this is considered as a sign of a well-functioning voluntary sphincter system. Voluntary sphincter squeeze was the only manometric parameter that correlated with good continence outcome with statistical significance. The anal resting pressure was higher in patients with normal continence, but this did not reach statistical significance. The spinal and intraspinal abnormalities were screened
in the patients of the study. Sacral anomalies were very common (68%) and intraspinal abnormalities also were relatively frequently detected (18%). The small number of patients and the great variability of the types of spinal and intraspinal defects preclude reliable statistical analysis of correlation between the presence of these anomalies and continence outcome. Constipation, which is a major source of functional problems in early childhood, disappears at adolescence in the majority of patients who have undergone PSARP procedure for a high anorectal malformation. The disappearance of constipation is associated with improved fecal continence outcome. The cause of this radical change of bowel function is unclear and warrants further studies.
REFERENCES 1. Rintala R, Mildh L, Lindahl H: Fecal continence and quality of life in adult patients with an operated high or intermediate anorectal malformation. J Pediatr Surg 29:777-780, 1994 2. Hassink EA, Rieu PN, Severijnen RS, et al: Are adults content or continent after repair for high anal atresia? A long-term follow-up study in patients 18 years of age and older. Ann Surg 218:196-200, 1993 3. Nixon HH, Puri P: The results of treatment of anorectal anomalies: A thirteen to twenty year follow-up. J Pediatr Surg 12:27-37, 1977 4. Rintala R, Lindahl H: Is normal bowel function possible after repair of intermediate and high anorectal malformations. J Pediatr Surg 30:491-494, 1995 5. Pen˜a A: Anorectal malformations. Semin Pediatr Surg 4:35-47, 1995 6. Rintala R, Lindahl H, Marttinen E, et al: Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations. J Pediatr Surg 28:1054-1058, 1995 7. Langemeijer RATM, Molenaar JC: Continence after posterior sagittal anorectoplasty. J Pediatr Surg 26:587-590, 1991 8. Rintala RJ, Lindahl HG: Posterior sagittal anorectoplasty is superior to sacroperineal- sacroabdominoperineal pull-through: A longterm follow-up study in boys with high anorectal anomalies. J Pediatr Surg 34:334-337, 1999 9. Mulder W, deJong, E, Wauters I, et al: Posterior sagittal anorectoplasty: Functional results of primary and secondary operations in comparison to the pull-through method in anorectal malformations. Eur J Pediatr Surg 5:170-173, 1995
10. Bliss DP, Tapper D, Anderson JM, et al: Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence. J Pediatr Surg 31:26-32, 1996 11. Holschneider AM, Pfrommer W, Gerresheim B: Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur J Pediatr Surg 4:303-309, 1994 12. Rintala R, Lindahl H, Sariola H, et al: The rectourogenital connection in anorectal malformations is an ectopic anal canal. J Pediatr Surg 25:665-668, 1990 13. Brent L, Stephens FD: Primary rectal ectasia: A quantitative study of smooth muscle cells in normal and hypertrophied human bowel. Progr Pediatr Surg 9:41-62, 1976 14. Holschneider AM: Elektromanometrie des Enddarms, MunichWien-Baltimore, Urban & Schwarzenberg, 1983 15. Meunier P, Marechal JM, de Beaujeu MJ: Rectoanal pressures and rectal sensitivity studies in chronic childhood constipation. Gastroenterology 77:330-336, 1979 16. Loening-Baucke V, Yamada T: Is the afferent pathway from the rectum impaired in children with chronic constipation and encopresis? Gastroenterology 109:397-403, 1995 17. Abrahamian FP, Lloyd-Still JD: Chronic constipation in childhood: A longitudinal study of 186 patients. J Pediatr Gastroenterol Nutr 3:460-467, 1984 18. Pen˜a A, Hong A: Advances in the management of anorectal malformations. Am J Surg 180:370-376, 2000 AQ1: Please spell out ACE