- Email: [email protected]
Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus
Journal of Pediatric Surgery (2008) 43, 1839–1843
www.elsevier.com/locate/jpedsurg
Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus Billur Demirogullari⁎, I. Onur Ozen, Ramazan Karabulut, Zafer Turkyilmaz, Kaan Sonmez, Nuri Kale, A. Can Basaklar Department of Pediatric Surgery, Gazi University Medical Faculty, Ankara, Turkey Received 24 December 2007; revised 26 January 2008; accepted 28 January 2008
Key Key words: words:
Anorectal Anorectal malformations; malformations; Colonic motility; motility; Colonic Colonic Colonic transit transit time; time; Krickenbeck Krickenbeck consensus; consensus; Voluntary Voluntary bowel bowel movements movements
Abstract Background/Purpose: In this study, the patients operated on for anorectal malformations (ARM) were evaluated in terms of segmental (SCTT) and total colonic transit times (TCTT) and clinical status according to Krickenbeck consensus before and after treatments. Methods: Forty-one patients with ARM (28 males/13 females) older than 3 years (median age, 7.7 years; range, 3-25) who had no therapy before were assessed for voluntary bowel movements (VBM), soiling (from 1 to 3), and constipation (from 1 to 3), retrospectively. Distribution of the patients were rectourethral fistula (17), perineal fistula (PF; 8), vestibular fistula (VF; 8), cloaca (3), rectovesical fistula (1), rectovaginal fistula (1), pouch colon with colovestibular fistula (1), no fistula (1), and unknown (1). The patients ingested daily 20 radiopaque markers for 3 days, followed by a single abdominal x-ray on days 4 and 7 if needed. The results were compared with the reference values in the literature. Results: Mean follow-up period was 36 months (range, 1-108.5 months). All patients but 1 had soiling in different degrees. Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). The longest TCTT and rectosigmoid SCTT were found in group 3 (69.5 and 35.2 hours, respectively). Group 1 had long SCTT in rectosigmoid but normal TCTT (27.8 and 47.4 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT, and there was no significant difference between them. After the appropriate treatment, of the patients, 45% (18/40) had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had unchanged soiling score, and 3 were excluded from the study because of follow-up problems. Half of the patients in group 3 (4 VF, 2 rectourethral fistula, PF) gained VBM without soiling after laxative treatment. Only four of 23 patients had decreased constipation score (2 cloaca, PF, VF). Conclusions: In this study, ARM patients complaining of constipation with or without VBM had prolonged SCTT in the rectosigmoid region. Percentage of the improvement in soiling scores was more conspicuous than that of constipation scores. The dismal figure observed at the first examination
⁎ Corresponding author. Gazi Üniversitesi Tıp Fakültesi, Çocuk Cerrahisi A.D. 12. Kat, Beşevler 06500, Ankara, Turkey. Tel.: +90 312 202 62 14; fax: +90 312 202 62 13. E-mail address: [email protected] (B. Demirogullari). 0022-3468/$ – see front matter © 2008 Published by Elsevier Inc. doi:10.1016/j.jpedsurg.2008.01.055
1840
B. Demirogullari et al. in the assessment of VBM was not associated with an unfavorable improvement with laxative treatment. So, it is suggested that assessment of VBM initially may be deceptive for clinical status. © 2008 Published by Elsevier Inc.
Anorectal malformations (ARM) has been an area of pediatric surgery interest for centuries. In the modern era, an International Conference for Development of Standards for the Treatment of ARM was organized at Krickenbeck Castle, Germany, in 2005. Recently, a new international classification and follow-up assessment for ARM and standards for surgical procedures have been reported under the name of the Krickenbeck consensus [1]. The patients in the present study were inspected in the light of this new insight, and in addition, their colonic motility was evaluated by total and segmental colonic transit time studies.
1. Methods Of 60 patients, 41 with ARM older than 3 years who completed their surgical procedures were retrospectively evaluated according to the Krickenbeck consensus recommendations. Median age of the patients was initially 7.7 years (range, 3-25 years). Distribution of the types of anomalies and surgical procedures is shown in Table 1. There was no obstructive problem at the anorectal region in any of the patients studied. Before evaluation, clinical status of the patients were scored for voluntary bowel movements (VBM), soiling, and constipation (Table 2). Feeling of urge, capacity to verbalize, and holding the bowel movement were described as VBM. Soiling was graded as 1, occasionally (once or twice per week); 2, everyday without social problem; and 3, constant with social problem. Constipation was graded according to the treatment as 1, manageable only
Table 1 Distribution of the types of the anomalies and surgical procedures Types of the anomalies
Types of surgical procedures
PF RUF RVF VF CL NF PC RVGF UN Total
Perineal operation Anterior sagittal approach Sacroperineal procedure PSARP Abdominosacroperineal pull through Abdominoperineal pull through
8 17 1 8 3 1 1 1 1 41
6 7 9 14 4 1
41
with diet; 2, requirement of laxatives; and 3, resistance to diet and laxatives. Colonic motility was assessed according to the study reported by Bautista et al [2]. During the course of the motility study, the patients continued to eat a normal diet and did not take any medication. They ingested 20 radiopaque markers at the same time daily for 3 days. On day 4 and, if needed, on day 7, a single high-voltage abdominal radiograph was obtained. Total (TCTT) and segmental (right-left-rectosigmoid colon) colonic transit time (SCTT) was calculated and compared with the reference values in the literature [2]. Colonic anatomy of the patients was obtained with barium enema studies, and rectal index was calculated to establish if there was megarectum [3]. The patients with any abnormality on the sacral bone were evaluated with spinal magnetic resonance imaging. The patients with constipation received a fiber diet and hyperosmolar (Duphalac, Solvay Company, İstanbul, Turkey) or stimulant laxative (X-M laxative solution, Yenisehir Company, Ankara, Turkey). In contrast, the patients with rapid TCTT received a constipating diet, loperamide HCl (Lopermid, Saba Company, İstanbul, Turkey), and enemas, if needed. All patients, were encouraged to eat and visit the toilet regularly during the day. After appropriate therapy protocol, clinical status was noted at the last control. Kruskal-Wallis and Mann-Whitney U tests were used for statistical analysis of TCTT and SCTT, with a P b .05 accepted as statistically significant. Spearman rank test was used for correlation analysis.
2. Results Mean follow-up period of the patients was 36 months (range, 1-108.5 months). Almost all patients except one (rectourethral fistula [RUF]) had soiling, so, these patients were grouped as to presence of VBM with or without constipation (Table 3). Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). Results for TCTT and SCTT are shown in Table 4. Although group 1 had normal TCTT (47.4 hours) but prolonged SCTT in the rectosigmoid region (27.8 hours), group 3 had both prolonged TCTT and SCTT in the rectosigmoid region (69.5 and 35.2 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT values, and there was no significant difference between them.
Patients with anorectal malformations
1841
Table 2 Method for assessment of outcome established in Krickenbeck 2005 (patient age N3 years, no therapy) [1] 1. VBMs Feeling of urge Capacity to verbalize Hold the bowel movement 2. Soiling Grade 1, occasionally (once or twice per wk) Grade 2, everyday, no social problem Grade 3, constant, social problem 3. Constipation Grade 1, manageable by changes in diet Grade 2, requires laxatives Grade 3, resistant to diet and laxatives
Yes/no
Group 1 Right CTT
Yes/no
Yes/no
12 (0-25.2) Left CTT 12.8 (0-27) Rectosigmoid 27.8 CTT (7-46) ⁎ TCTT 47.4 (7.2-74.4)
Group 2
Group 3
Group 4
7 (0-22.8) 4 (0-19.2) 13.8 (0-31.2) 23.9 (3.6-58.8)
17.5 (3.6-43.2) 16.8 (2.4-36) 35.2 (13.2-100.8) ⁎ 69.5 (38.4-106.8) †
10 (4.8-14.4) 6.4 (2.4-9.6) 14.7 (7.2-24) 31.2 (18-42)
CTT indicates colonic transit time. ⁎ P b .05, prolonged SCTT vs reference literature (2) and other groups, but there is no significant difference between group 1 and 3. † P b .05, prolonged TCTT vs reference literature (2) and other groups.
In general, after appropriate medical treatment, 45% (18/ 40) of the patients had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had an unchanged soiling score (Table 5). In group 1, 8 (89%) of 9 patients had decreased soiling score, and 3 of them (perineal fistula [PF], RUF, vestibular fistula [VF]) had no soiling. Megarectum was identified in 5 patients (3 PF, VF, cloaca [CL]). After a long period of stimulant laxative therapy (in high doses) without soiling, sigmoid resection was performed in 2 patients with PF and in a patient with CL who had a small neurogenic bladder with bilateral grade V vesicoureteric reflux during bladder augmentation. After sigmoid resection, constipation became manageable with diet in 2 patients (PF, CL) but required laxative again in the other PF patient but in lower doses. The constipation score did not change in the rest of group 1. Four patients (44%) (2 RUF, PF, VF) had prolonged SCTT only in the rectosigmoid region; 2 (2 PF), in the left colon; and 2 (VF, CL), in the right colon plus rectosigmoid had prolonged SCTT and/or TCTT. Interestingly, 1 patient with an RUF had normal SCTT and TCTT in association with megarectum. In group 2, all had a decrease in the grade of soiling, with soiling disappearing in half of the group (3 RUF, VF, PF, rectovaginal fistula [RVGF]) and decreasing to grade 1 in the other half (2 PF, 2 RUF, pouch colon [PC]) after treatment that included a constipating diet, toilet training, and Table 3
Table 4 Results on TCTT and SCTT of the groups (hours) (minimum-maximum)
loperamide HCl, plus a low volume enema in 1 (PC). Although TCTT was measured less than 24 hours in 7 (58%), 1 (PF) had longer TCTT when compared to normal values in the reference literature (58.8 vs 50) (ref) and benefited from only regular diet and toilet behavior. In group 3, there was a decrease in soiling score in 10 (71.4%) of 14 patients. Seven of the patients (50%) had no soiling and gained VBM after treatment (4 VF, 2 RUF, PF). On the other hand, all but 1 of these patients considered as pseudoincontinent did not show any changes in the grade of constipation and were still requiring laxative therapy at their last visit. Megarectum was found in 7 children (3 VF, 2 RUF, no fistula [NF], PF). Sigmoid resection was performed in 1 patient (VF) who gained VBM without soiling under laxative therapy, and she continued to receive stimulant laxative. Two patients (NF, VF) had normal TCTT, although both had megarectum and rectosigmoid SCTT was prolonged in 1 (NF). The rest of the group had prolonged SCTT and TCTT only in the rectosigmoid region in 3 (VF, RUF, unknown [UN]); in the right colon or in the left colon plus rectosigmoid in 3 (PF, VF, CL) and 2 (VF, CL), respectively; both in the right and left colon in 2 (2 RUF); and only in the right colon in 2 (2 RUF). We could not obtain follow-up information from 2 patients in this group (CL, UN). One patient (CL) who had no anorectal obstruction, required a redo-PSARP procedure because of mislocation of the
Groups of the patients having soiling as to presence of VBM with or without constipation
Soiling (+) (n = 40) VBM (+) (n = 21)
VBM (−) (n = 19)
Group 1, with constipation (n = 9) PF RUF VF CL
Group 2, without constipation (n = 12) 4 2 2 1
PF RUF VF RVGF PC
3 6 1 1 1
Group 3, with constipation (n = 14)
Group 4, without constipation (n = 5)
PF RUF VF CL NF UN
RUF RVF
1 4 5 2 1 1
4 1
1842
B. Demirogullari et al.
Table 5
Changes in grades of constipation and soiling
Groups
Grade of soiling (pre-/posttreatment no. of patients) NS
1
2
3
1
2
3
1 (n = 2 (n = 3 (n = 4 (n = Total
0/3 0/6 0/7 0/2 0/18 40/37 a
3/4 1/5 1/3 0/0 5/12
0/2 5/1 0/1 1/1 6/5
6/0 6/0 13/1 4/1 29/2
2/4
6/4
1/1
0/2
9/8
5/2
9) 12) 14) 5)
Grade of constipation (pre-/posttreatment no. of patients)
2/6 15/12 23/21 a
6/3
NS indicates no soiling. a Three missing patients (2 in group 3, 1 in group 4).
rectum, and her grade 3 constipation decreased to 1 after surgery. She was the single patient whose grade of constipation was changed in this group. In group 4, after 2 patients (2 RUF) received a bowel management program including constipating diet, lowvolume enemas, and loperamide HCl twice a day, grade 3 soiling was kept under control. The same therapy resulted in failure in 1 child (rectovesical fistula [RVF]) with sacral agenesis and in another (RUF) who lost his rectosigmoid region during pull-through procedure. The transabdominal route was used for sigmoid resection. Histopathologic findings of the resected specimens were found normal under light microscope. Twelve patients (5 in group 1 and 7 in group 3) had megarectum in total. The relation of megarectum to the type of malformation, severity of the constipation, and duration of the colonic transit time was evaluated; however, there was no correlation between these factors. Colonic transit time studies were associated with falsenegative results in 8.6% and 5.8% of the patients with and without constipation, respectively. Although different sacral abnormalities were seen in 68.2% (28/41) of the patients, tethered cord was found in 24.3% (10/41) of them (3 PF, 2 RUF, 2 VF, CL, NF), including the unique patient who was completely continent. The distribution of the patients with tethered cord was 2, 2, 3, and 2 in groups 1, 2, 3, and 4, respectively. We could not find any relationship of tethered cord with VBM and colonic transit time. Only 1 patient (VF) in group 1 was operated on for tethered cord before referral to our center.
3. Discussion Long-term outcomes of ARM have been reported by many authors. Different scoring methods have been used to assess clinical status in these series in which low and intermediate-high malformations have been inspected and compared [4]. To standardize the assessment of outcome
after ARM repair, the Krickenbeck consensus was achieved. In this study, the patients were evaluated regardless of the type of the anomalies. Soiling and VBM were the determinant factor to group the patients. The number of the patients was a drawback of this study. When they were divided into 4 groups, interpretation of the results according to the types of ARM was very difficult. However, it was not a problem related to this study because there was no anatomical consideration in Krickenbeck. Importance of VBM was emphasized by Peña [5] in 1995. Recently, Peña and Hong [6] reported that 75% of 1192 patients had VBM, with half of them still occasionally soiling. In the present study, almost all patients with VBM had soiling. Approximately half of the patients were referred from different centers because of their complaints, and that is why the surgical procedures (Table 1) are so varied, and this could be a reason for the high soiling ratio observed in this series. However, ratio of the patients with VBM increased to 68.2%, and soiling disappeared in 45% of the patients after specific treatment for each. Assessment of VBM before the treatment may be misleading, especially in patients with constipation, which has been previously described as pseudoincontinence [7]. Laxative therapy guided the decision about the grade of constipation, which is another difficulty at the beginning of our study. Most of the patients had grade 2 constipation (Table 5). Because of low response to diet and laxative therapy, the patients with grade 3 constipation may be ideal candidates for a bowel management program [8]. Rintala et al [3] did not find a correlation between rectal index and severity of constipation. However, they reported that there was a positive correlation between rectal index measured from the loopogram (which was performed before the closure of the protecting colostomy) and the severity of later symptoms of constipation. The patients with megarectum in this study did not differ from the others in terms of ARM type, constipation grade, and colonic transit time. Motility disturbances after repair of ARM have resulted in chronic constipation or fecal incontinence, and the former should be distinguished from the latter [1]. Both can cause soiling, and additional studies and clinical assessment may be required. The barium contrast enema may have a valuable place in demonstrating the anatomical appearance of the colon. However, the results of this and a previous study [3] showed that the barium enema was insufficient to assess severity of constipation; hence, we needed additional functional studies. Different methods for measuring colonic transit time have been used [9]. Today, ingestion of multiple radiopaque markers is the most preferred technique in children and adults [2,10]. Although there is a sparsity of transit time studies for ARM patients in the literature, Rintala et al [11] published their results in 1997. They noted that the patients with low anomalies had hypomotility in the rectosigmoid, but patients with high anomalies had more generalized motility disturbances, especially in the right colon. Colonic transit studies in this study were generally consistent with the symptoms. Although 2 patients with
Patients with anorectal malformations constipation had normal transit time, 1 patient without constipation had slow transit. The slowest region in patients with constipation was the rectosigmoid colon. Unfortunately, because of ethical problems, we could not collect data from our own control group. This study showed that one was not able to get much accurate information about VBM at the first examination. After the appropriate treatment, however, the grade of soiling significantly decreased, but improvement in the grade of constipation was not so apparent. In addition, the patients with constipation mostly had rectal stasis regardless of ARM type, and colonic transit time may be used as a complementary study to the clinical assessment.
References [1] Holschneider A, Hutson J, Pena A, et al. Preliminary report of the International Conference for the development of standards for the treatment of anorectal malformations. J Pediatr Surg 2005;40:1521-6.
1843 [2] Bautista CA, Varela CA, Villanueva J, et al. Measurement of colonic transit time in children. J Pediatr Gastroenterol Nutr 1991;13:42-5. [3] 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 1993;28:1054-8. [4] Davies MC, Creighton SM, Wilcox DT. Long-term outcomes of anorectal malformations. Pediatr Surg Int 2004;20:567-72. [5] Pena A. Anorectal malformations. Semin Pediatr Surg 1995;4:35-47. [6] Pena A, Hong A. Advances in the management of anorectal malformations. Am J Surg 2000;180:370-6. [7] Pena A, el Behery M. Megasigmoid: a source of pseudoincontinence in children with repaired anorectal malformations. J Pediatr Surg 1993; 28:199-203. [8] Pena A, Guardino K, Tovilla JM, et al. Bowel management for fecal incontinence in patients with anorectal malformations. J Pediatr Surg 1998;33:133-7. [9] Mulinos MG. The value of selective drugs in the treatment of constipation. Rev Gastroenterol 1935;2:292-301. [10] Bouchoucha M, Devroede G, Arhan P, et al. What is the meaning of colorectal transit time measurement? Dis Col Rectum 1992;35: 773-82. [11] Rintala RJ, Marttinen E, Virkola K, et al. Segmental colonic motility in patients with anorectal malformations. J Pediatr Surg 1997;32:453-6.
www.elsevier.com/locate/jpedsurg
Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus Billur Demirogullari⁎, I. Onur Ozen, Ramazan Karabulut, Zafer Turkyilmaz, Kaan Sonmez, Nuri Kale, A. Can Basaklar Department of Pediatric Surgery, Gazi University Medical Faculty, Ankara, Turkey Received 24 December 2007; revised 26 January 2008; accepted 28 January 2008
Key Key words: words:
Anorectal Anorectal malformations; malformations; Colonic motility; motility; Colonic Colonic Colonic transit transit time; time; Krickenbeck Krickenbeck consensus; consensus; Voluntary Voluntary bowel bowel movements movements
Abstract Background/Purpose: In this study, the patients operated on for anorectal malformations (ARM) were evaluated in terms of segmental (SCTT) and total colonic transit times (TCTT) and clinical status according to Krickenbeck consensus before and after treatments. Methods: Forty-one patients with ARM (28 males/13 females) older than 3 years (median age, 7.7 years; range, 3-25) who had no therapy before were assessed for voluntary bowel movements (VBM), soiling (from 1 to 3), and constipation (from 1 to 3), retrospectively. Distribution of the patients were rectourethral fistula (17), perineal fistula (PF; 8), vestibular fistula (VF; 8), cloaca (3), rectovesical fistula (1), rectovaginal fistula (1), pouch colon with colovestibular fistula (1), no fistula (1), and unknown (1). The patients ingested daily 20 radiopaque markers for 3 days, followed by a single abdominal x-ray on days 4 and 7 if needed. The results were compared with the reference values in the literature. Results: Mean follow-up period was 36 months (range, 1-108.5 months). All patients but 1 had soiling in different degrees. Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). The longest TCTT and rectosigmoid SCTT were found in group 3 (69.5 and 35.2 hours, respectively). Group 1 had long SCTT in rectosigmoid but normal TCTT (27.8 and 47.4 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT, and there was no significant difference between them. After the appropriate treatment, of the patients, 45% (18/40) had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had unchanged soiling score, and 3 were excluded from the study because of follow-up problems. Half of the patients in group 3 (4 VF, 2 rectourethral fistula, PF) gained VBM without soiling after laxative treatment. Only four of 23 patients had decreased constipation score (2 cloaca, PF, VF). Conclusions: In this study, ARM patients complaining of constipation with or without VBM had prolonged SCTT in the rectosigmoid region. Percentage of the improvement in soiling scores was more conspicuous than that of constipation scores. The dismal figure observed at the first examination
⁎ Corresponding author. Gazi Üniversitesi Tıp Fakültesi, Çocuk Cerrahisi A.D. 12. Kat, Beşevler 06500, Ankara, Turkey. Tel.: +90 312 202 62 14; fax: +90 312 202 62 13. E-mail address: [email protected] (B. Demirogullari). 0022-3468/$ – see front matter © 2008 Published by Elsevier Inc. doi:10.1016/j.jpedsurg.2008.01.055
1840
B. Demirogullari et al. in the assessment of VBM was not associated with an unfavorable improvement with laxative treatment. So, it is suggested that assessment of VBM initially may be deceptive for clinical status. © 2008 Published by Elsevier Inc.
Anorectal malformations (ARM) has been an area of pediatric surgery interest for centuries. In the modern era, an International Conference for Development of Standards for the Treatment of ARM was organized at Krickenbeck Castle, Germany, in 2005. Recently, a new international classification and follow-up assessment for ARM and standards for surgical procedures have been reported under the name of the Krickenbeck consensus [1]. The patients in the present study were inspected in the light of this new insight, and in addition, their colonic motility was evaluated by total and segmental colonic transit time studies.
1. Methods Of 60 patients, 41 with ARM older than 3 years who completed their surgical procedures were retrospectively evaluated according to the Krickenbeck consensus recommendations. Median age of the patients was initially 7.7 years (range, 3-25 years). Distribution of the types of anomalies and surgical procedures is shown in Table 1. There was no obstructive problem at the anorectal region in any of the patients studied. Before evaluation, clinical status of the patients were scored for voluntary bowel movements (VBM), soiling, and constipation (Table 2). Feeling of urge, capacity to verbalize, and holding the bowel movement were described as VBM. Soiling was graded as 1, occasionally (once or twice per week); 2, everyday without social problem; and 3, constant with social problem. Constipation was graded according to the treatment as 1, manageable only
Table 1 Distribution of the types of the anomalies and surgical procedures Types of the anomalies
Types of surgical procedures
PF RUF RVF VF CL NF PC RVGF UN Total
Perineal operation Anterior sagittal approach Sacroperineal procedure PSARP Abdominosacroperineal pull through Abdominoperineal pull through
8 17 1 8 3 1 1 1 1 41
6 7 9 14 4 1
41
with diet; 2, requirement of laxatives; and 3, resistance to diet and laxatives. Colonic motility was assessed according to the study reported by Bautista et al [2]. During the course of the motility study, the patients continued to eat a normal diet and did not take any medication. They ingested 20 radiopaque markers at the same time daily for 3 days. On day 4 and, if needed, on day 7, a single high-voltage abdominal radiograph was obtained. Total (TCTT) and segmental (right-left-rectosigmoid colon) colonic transit time (SCTT) was calculated and compared with the reference values in the literature [2]. Colonic anatomy of the patients was obtained with barium enema studies, and rectal index was calculated to establish if there was megarectum [3]. The patients with any abnormality on the sacral bone were evaluated with spinal magnetic resonance imaging. The patients with constipation received a fiber diet and hyperosmolar (Duphalac, Solvay Company, İstanbul, Turkey) or stimulant laxative (X-M laxative solution, Yenisehir Company, Ankara, Turkey). In contrast, the patients with rapid TCTT received a constipating diet, loperamide HCl (Lopermid, Saba Company, İstanbul, Turkey), and enemas, if needed. All patients, were encouraged to eat and visit the toilet regularly during the day. After appropriate therapy protocol, clinical status was noted at the last control. Kruskal-Wallis and Mann-Whitney U tests were used for statistical analysis of TCTT and SCTT, with a P b .05 accepted as statistically significant. Spearman rank test was used for correlation analysis.
2. Results Mean follow-up period of the patients was 36 months (range, 1-108.5 months). Almost all patients except one (rectourethral fistula [RUF]) had soiling, so, these patients were grouped as to presence of VBM with or without constipation (Table 3). Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). Results for TCTT and SCTT are shown in Table 4. Although group 1 had normal TCTT (47.4 hours) but prolonged SCTT in the rectosigmoid region (27.8 hours), group 3 had both prolonged TCTT and SCTT in the rectosigmoid region (69.5 and 35.2 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT values, and there was no significant difference between them.
Patients with anorectal malformations
1841
Table 2 Method for assessment of outcome established in Krickenbeck 2005 (patient age N3 years, no therapy) [1] 1. VBMs Feeling of urge Capacity to verbalize Hold the bowel movement 2. Soiling Grade 1, occasionally (once or twice per wk) Grade 2, everyday, no social problem Grade 3, constant, social problem 3. Constipation Grade 1, manageable by changes in diet Grade 2, requires laxatives Grade 3, resistant to diet and laxatives
Yes/no
Group 1 Right CTT
Yes/no
Yes/no
12 (0-25.2) Left CTT 12.8 (0-27) Rectosigmoid 27.8 CTT (7-46) ⁎ TCTT 47.4 (7.2-74.4)
Group 2
Group 3
Group 4
7 (0-22.8) 4 (0-19.2) 13.8 (0-31.2) 23.9 (3.6-58.8)
17.5 (3.6-43.2) 16.8 (2.4-36) 35.2 (13.2-100.8) ⁎ 69.5 (38.4-106.8) †
10 (4.8-14.4) 6.4 (2.4-9.6) 14.7 (7.2-24) 31.2 (18-42)
CTT indicates colonic transit time. ⁎ P b .05, prolonged SCTT vs reference literature (2) and other groups, but there is no significant difference between group 1 and 3. † P b .05, prolonged TCTT vs reference literature (2) and other groups.
In general, after appropriate medical treatment, 45% (18/ 40) of the patients had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had an unchanged soiling score (Table 5). In group 1, 8 (89%) of 9 patients had decreased soiling score, and 3 of them (perineal fistula [PF], RUF, vestibular fistula [VF]) had no soiling. Megarectum was identified in 5 patients (3 PF, VF, cloaca [CL]). After a long period of stimulant laxative therapy (in high doses) without soiling, sigmoid resection was performed in 2 patients with PF and in a patient with CL who had a small neurogenic bladder with bilateral grade V vesicoureteric reflux during bladder augmentation. After sigmoid resection, constipation became manageable with diet in 2 patients (PF, CL) but required laxative again in the other PF patient but in lower doses. The constipation score did not change in the rest of group 1. Four patients (44%) (2 RUF, PF, VF) had prolonged SCTT only in the rectosigmoid region; 2 (2 PF), in the left colon; and 2 (VF, CL), in the right colon plus rectosigmoid had prolonged SCTT and/or TCTT. Interestingly, 1 patient with an RUF had normal SCTT and TCTT in association with megarectum. In group 2, all had a decrease in the grade of soiling, with soiling disappearing in half of the group (3 RUF, VF, PF, rectovaginal fistula [RVGF]) and decreasing to grade 1 in the other half (2 PF, 2 RUF, pouch colon [PC]) after treatment that included a constipating diet, toilet training, and Table 3
Table 4 Results on TCTT and SCTT of the groups (hours) (minimum-maximum)
loperamide HCl, plus a low volume enema in 1 (PC). Although TCTT was measured less than 24 hours in 7 (58%), 1 (PF) had longer TCTT when compared to normal values in the reference literature (58.8 vs 50) (ref) and benefited from only regular diet and toilet behavior. In group 3, there was a decrease in soiling score in 10 (71.4%) of 14 patients. Seven of the patients (50%) had no soiling and gained VBM after treatment (4 VF, 2 RUF, PF). On the other hand, all but 1 of these patients considered as pseudoincontinent did not show any changes in the grade of constipation and were still requiring laxative therapy at their last visit. Megarectum was found in 7 children (3 VF, 2 RUF, no fistula [NF], PF). Sigmoid resection was performed in 1 patient (VF) who gained VBM without soiling under laxative therapy, and she continued to receive stimulant laxative. Two patients (NF, VF) had normal TCTT, although both had megarectum and rectosigmoid SCTT was prolonged in 1 (NF). The rest of the group had prolonged SCTT and TCTT only in the rectosigmoid region in 3 (VF, RUF, unknown [UN]); in the right colon or in the left colon plus rectosigmoid in 3 (PF, VF, CL) and 2 (VF, CL), respectively; both in the right and left colon in 2 (2 RUF); and only in the right colon in 2 (2 RUF). We could not obtain follow-up information from 2 patients in this group (CL, UN). One patient (CL) who had no anorectal obstruction, required a redo-PSARP procedure because of mislocation of the
Groups of the patients having soiling as to presence of VBM with or without constipation
Soiling (+) (n = 40) VBM (+) (n = 21)
VBM (−) (n = 19)
Group 1, with constipation (n = 9) PF RUF VF CL
Group 2, without constipation (n = 12) 4 2 2 1
PF RUF VF RVGF PC
3 6 1 1 1
Group 3, with constipation (n = 14)
Group 4, without constipation (n = 5)
PF RUF VF CL NF UN
RUF RVF
1 4 5 2 1 1
4 1
1842
B. Demirogullari et al.
Table 5
Changes in grades of constipation and soiling
Groups
Grade of soiling (pre-/posttreatment no. of patients) NS
1
2
3
1
2
3
1 (n = 2 (n = 3 (n = 4 (n = Total
0/3 0/6 0/7 0/2 0/18 40/37 a
3/4 1/5 1/3 0/0 5/12
0/2 5/1 0/1 1/1 6/5
6/0 6/0 13/1 4/1 29/2
2/4
6/4
1/1
0/2
9/8
5/2
9) 12) 14) 5)
Grade of constipation (pre-/posttreatment no. of patients)
2/6 15/12 23/21 a
6/3
NS indicates no soiling. a Three missing patients (2 in group 3, 1 in group 4).
rectum, and her grade 3 constipation decreased to 1 after surgery. She was the single patient whose grade of constipation was changed in this group. In group 4, after 2 patients (2 RUF) received a bowel management program including constipating diet, lowvolume enemas, and loperamide HCl twice a day, grade 3 soiling was kept under control. The same therapy resulted in failure in 1 child (rectovesical fistula [RVF]) with sacral agenesis and in another (RUF) who lost his rectosigmoid region during pull-through procedure. The transabdominal route was used for sigmoid resection. Histopathologic findings of the resected specimens were found normal under light microscope. Twelve patients (5 in group 1 and 7 in group 3) had megarectum in total. The relation of megarectum to the type of malformation, severity of the constipation, and duration of the colonic transit time was evaluated; however, there was no correlation between these factors. Colonic transit time studies were associated with falsenegative results in 8.6% and 5.8% of the patients with and without constipation, respectively. Although different sacral abnormalities were seen in 68.2% (28/41) of the patients, tethered cord was found in 24.3% (10/41) of them (3 PF, 2 RUF, 2 VF, CL, NF), including the unique patient who was completely continent. The distribution of the patients with tethered cord was 2, 2, 3, and 2 in groups 1, 2, 3, and 4, respectively. We could not find any relationship of tethered cord with VBM and colonic transit time. Only 1 patient (VF) in group 1 was operated on for tethered cord before referral to our center.
3. Discussion Long-term outcomes of ARM have been reported by many authors. Different scoring methods have been used to assess clinical status in these series in which low and intermediate-high malformations have been inspected and compared [4]. To standardize the assessment of outcome
after ARM repair, the Krickenbeck consensus was achieved. In this study, the patients were evaluated regardless of the type of the anomalies. Soiling and VBM were the determinant factor to group the patients. The number of the patients was a drawback of this study. When they were divided into 4 groups, interpretation of the results according to the types of ARM was very difficult. However, it was not a problem related to this study because there was no anatomical consideration in Krickenbeck. Importance of VBM was emphasized by Peña [5] in 1995. Recently, Peña and Hong [6] reported that 75% of 1192 patients had VBM, with half of them still occasionally soiling. In the present study, almost all patients with VBM had soiling. Approximately half of the patients were referred from different centers because of their complaints, and that is why the surgical procedures (Table 1) are so varied, and this could be a reason for the high soiling ratio observed in this series. However, ratio of the patients with VBM increased to 68.2%, and soiling disappeared in 45% of the patients after specific treatment for each. Assessment of VBM before the treatment may be misleading, especially in patients with constipation, which has been previously described as pseudoincontinence [7]. Laxative therapy guided the decision about the grade of constipation, which is another difficulty at the beginning of our study. Most of the patients had grade 2 constipation (Table 5). Because of low response to diet and laxative therapy, the patients with grade 3 constipation may be ideal candidates for a bowel management program [8]. Rintala et al [3] did not find a correlation between rectal index and severity of constipation. However, they reported that there was a positive correlation between rectal index measured from the loopogram (which was performed before the closure of the protecting colostomy) and the severity of later symptoms of constipation. The patients with megarectum in this study did not differ from the others in terms of ARM type, constipation grade, and colonic transit time. Motility disturbances after repair of ARM have resulted in chronic constipation or fecal incontinence, and the former should be distinguished from the latter [1]. Both can cause soiling, and additional studies and clinical assessment may be required. The barium contrast enema may have a valuable place in demonstrating the anatomical appearance of the colon. However, the results of this and a previous study [3] showed that the barium enema was insufficient to assess severity of constipation; hence, we needed additional functional studies. Different methods for measuring colonic transit time have been used [9]. Today, ingestion of multiple radiopaque markers is the most preferred technique in children and adults [2,10]. Although there is a sparsity of transit time studies for ARM patients in the literature, Rintala et al [11] published their results in 1997. They noted that the patients with low anomalies had hypomotility in the rectosigmoid, but patients with high anomalies had more generalized motility disturbances, especially in the right colon. Colonic transit studies in this study were generally consistent with the symptoms. Although 2 patients with
Patients with anorectal malformations constipation had normal transit time, 1 patient without constipation had slow transit. The slowest region in patients with constipation was the rectosigmoid colon. Unfortunately, because of ethical problems, we could not collect data from our own control group. This study showed that one was not able to get much accurate information about VBM at the first examination. After the appropriate treatment, however, the grade of soiling significantly decreased, but improvement in the grade of constipation was not so apparent. In addition, the patients with constipation mostly had rectal stasis regardless of ARM type, and colonic transit time may be used as a complementary study to the clinical assessment.
References [1] Holschneider A, Hutson J, Pena A, et al. Preliminary report of the International Conference for the development of standards for the treatment of anorectal malformations. J Pediatr Surg 2005;40:1521-6.
1843 [2] Bautista CA, Varela CA, Villanueva J, et al. Measurement of colonic transit time in children. J Pediatr Gastroenterol Nutr 1991;13:42-5. [3] 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 1993;28:1054-8. [4] Davies MC, Creighton SM, Wilcox DT. Long-term outcomes of anorectal malformations. Pediatr Surg Int 2004;20:567-72. [5] Pena A. Anorectal malformations. Semin Pediatr Surg 1995;4:35-47. [6] Pena A, Hong A. Advances in the management of anorectal malformations. Am J Surg 2000;180:370-6. [7] Pena A, el Behery M. Megasigmoid: a source of pseudoincontinence in children with repaired anorectal malformations. J Pediatr Surg 1993; 28:199-203. [8] Pena A, Guardino K, Tovilla JM, et al. Bowel management for fecal incontinence in patients with anorectal malformations. J Pediatr Surg 1998;33:133-7. [9] Mulinos MG. The value of selective drugs in the treatment of constipation. Rev Gastroenterol 1935;2:292-301. [10] Bouchoucha M, Devroede G, Arhan P, et al. What is the meaning of colorectal transit time measurement? Dis Col Rectum 1992;35: 773-82. [11] Rintala RJ, Marttinen E, Virkola K, et al. Segmental colonic motility in patients with anorectal malformations. J Pediatr Surg 1997;32:453-6.