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10-Year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeck classification
Journal of Pediatric Surgery (2009) 44, 399–403
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10-Year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeck classification Sinead Hassett, Stella Snell, Amy Hughes-Thomas, Keith Holmes ⁎ Department of Paediatric Surgery, St George's Hospital, SW17 0QT London, UK Received 6 October 2008; accepted 23 October 2008
Key words: Anorectal malformation; Continence; Long-term follow up
Abstract Background: Anorectal malformations (ARMs) affect 1 in 4000 to 5000 births. The Krickenbeck conference developed a classification based on anatomical and functional criteria to better compare treatment outcome. Aim: The aim of this study is to evaluate the functional outcome in patients 10 years following standardized surgical treatment of ARM related to the Krickenbeck classification. . Methods: Anatomical anomalies were classified as above. Children and carers were followed closely in a multidisciplinary clinic. Data were collected using a functional outcome questionnaire for a minimum of 10 years after surgical reconstruction. Outcome measurements were related to the Krickenbeck classification. Results: There were 53 children in the study group (29 male, 24 female). Krickenbeck anatomy: perineal fistula, 36%; vestibular fistula, 26%; rectourethral fistula, 36%; rectovesical fistula, 2%.All children were treated by posterior sagittal anorectoplasty. In children with perineal fistula, continence was achieved in 90%. Grade 2 constipation was noted in 21%. One child had a Malone antegrade continence enema (MACE) procedure.In children with vestibular fistula, continence was achieved in 57%. Grade 3 constipation was noted in 28%. One child had grade 1, and one child had grade 2 soiling. Two children had a MACE procedure.In children with rectourethral fistula, continence was achieved in 58%. One child had grade 3 soiling. Grade 3 constipation was found in 42% of children and grade 2 constipation in 1 child. A MACE procedure was performed in 36%. The only child with a bladder neck fistula had a MACE procedure for intractable soiling. Conclusions: The outcome for patients with ARM is related to the severity of the anomaly. The uniform application of the Krickenbeck classification should allow rational comparison of treatment outcome. © 2009 Elsevier Inc. All rights reserved.
Presented at the 55th Annual Congress of the British Association of Paediatric Surgeons, Salamanca, Spain, July 2-5, 2008. ⁎ Corresponding author. Tel.: +44 0208 725 2290; fax: +44 0208 725 0711. E-mail address: [email protected] (K. Holmes). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.10.092
Anorectal malformation (ARM) is an uncommon condition affecting 1 in 4000 to 5000 births [1,2]. The embryological pathology is related to dysmorphogensis of the cloaca and urorectum in early fetal life [3]. The anomaly may occur in isolation but is commonly associated with other anomalies, urogenital and musculoskeletal abnormalities
400 Table 1
Female
Male
S. Hassett et al. Wingspread classification High ARMs
Intermediate ARMs
Low ARMs
Cloaca
Rare malformations
Anorectal agenesis (a) with rectovaginal fistula (b) without fistula Rectal atresia Anorectal agenesis (a) with rectoprostatic urethral fistula (b) without fistula Rectal atresia
Rectovestibular fistula Recto vaginal fistula Anal agenesis without fistula
Anovestibular fistula Anocutaneous fistula Anal stenosis
Cloaca
Rare malformations
Rectobulbar urethral fistula Anal agenesis without fistula
Anocutaneous fistula Anal stenosis
being the most common [4]. Antenatal diagnosis of an isolated ARM is rare, and most are diagnosed in the early neonatal period. Consistent with incomplete separation of the cloaca, most patients have a fistula. In males and females, rectourethral fistula and rectovestibular fistula, respectively, are the most frequent variants [5]. Anorectal malformation without a fistula is uncommon and found in only 5% of cases. The earliest classification of ARM was based on the position of the terminal rectum in relation to the levator ani or pelvic floor [6]. Termination of the rectum above the levator ani was termed “high,” and termination below the levator ani termed “low.” The relationship of the terminal rectum to the levator ani formed the basis of the 1984 Wingspread classification [7,8]. Anomalies were grouped into high, intermediate. and low lesions with separate categories for male and female (Table 1). This classification was widely accepted and used for many years. A classification based on the presence and position of the fistula was described by Pena in 1995 as a result of his experience with the posterior sagittal anorectoplasty (PSARP) (Table 2) [9,10]. The position of the fistula was used to determine operative management. Pena also determined the prognosis for each group by long-term analysis of continence, constipation, and soiling. Although PSARP has become the operation of choice for many centers in the treatment of ARM, there is still variation in the follow-up criteria used. This has
Table 2
Rare malformations
perpetuated the difficulties in comparing reports of functional outcome. In an attempt to rationalize this problem, the Krickenbeck group published their findings in 2005 [11]. The resulting classification, devised after an international workshop attended by experts in the field, incorporated criteria from the Wingspread and Pena classification. The Krickenbeck classification system is composed of 3 distinct elements: a diagnostic category (Table 3), a surgical procedure category (Table 4), and a category documenting functional outcome criteria (Table 5). There have been no large long-term outcome studies using this classification. We present this series of patients, all treated by PSARP, and monitored for a minimum of 10 years after surgical treatment. We related long-term outcome to anatomy at presentation as defined by the Krickenbeck group.
1. Methods A database maintained by the senior author was searched, and all patients who were at least 10 years post definitive surgery for ARM were identified. Information was obtained from analysis of detailed questionnaires completed at each patient visit to a specialized multidisciplinary clinic (Fig. 1). The Krickenbeck classification was used for anatomical diagnostic classification and for functional outcome 10 years
Pena classification
Males
Females
Perineal fistula Rectourethral fistula (a) Bulbar (b) Prostatic Rectovesical fistula
Perineal fistula Vestibular fistula
Imperforate anus without fistula Rectal atresia
Persistent cloaca b3 cm common channel N3 cm common channel Imperforate anus without fistula Rectal atresia
Table 3
Krickenbeck classification for ARMs
Major clinical groups
Rare regional variants
Perineal fistula Rectourethral fistula (a) Bulbar (b) Prostatic Rectovesical fistula Vestibular fistula Cloaca No fistula Anal stenosis
Pouch Colon Rectal atresia/stenosis Rectovaginal fistula H fistula Others
ARM assessed according to the Krickenbeck classification Table 4
401 Table 5
Krickenbeck classification of surgical procedures
Operative procedures Perineal operation Anterior saggital approach Sacroperineal approach Posterior saggital anorectoplasty Abdominosacroperineal pull-through Abdominoperineal pull-through Laparoscopic-assisted pull-through
after reconstruction. All patients were treated by PSARP with a modified Nixon anoplasty to produce a skin-lined anus [12].
Krickenbeck classification for postoperative results
1. Voluntary bowel movements Yes/No Feeling of urge, capacity to verbalize, hold the bowel movement 2. Soiling Yes/No (a) Grade 1 Occasionally (once/ twice per week) (b) Grade 2 Every day, no social problem (c) Grade 3 Constant, social problem 3. Constipation Yes/No (a) Grade 1 Manageable by changes in diet (b) Grade 2 Requires laxatives (c) Grade 3 Resistant to laxatives and diet
2. Results A search of the database identified 53 children eligible for inclusion. There were 29 males and 24 females. Perineal fistula was found in 36% of children; vestibular fistula in 26% of children; rectourethral fistula in 36% of children; and rectovesical fistula in 1 male child (2%). Associated anomalies were present in 43% of children. Of these, 52% involved the renal tract, 39% involved the spine, 35% involved the heart, and 2% of the study group had a tracheooesophageal fistula or limb abnormality. All children had a PSARP reconstruction as their definitive operation. In 28%, PSARP was the primary
Fig. 1
procedure; the remainder had a preliminary colostomy. An abdominal operation was required in addition to PSARP in 4%. In 3 children, males with a rectourethral fistula, a repeat procedure was necessary. In all these patients, the original operation was performed at a different institution. Postoperative complications were noted in 6 children. Laparotomy was done for release of adhesions in 1 child and to repair colonic perforation in another. Mucosal prolapse requiring operative correction occurred in 3 children. One child required urethral dilatation for a temporary stricture.
Follow-up questionnaire.
402 Table 6
S. Hassett et al. Results Perineal Vestibular Rectourethral Rectovesical fistula fistula fistula fistula (n = 19) (n = 14) (n = 19) (n = 1)
Continence a Soiling Constipation MACE
17 (90) 0 4 (21) 1 (5)
8 (57) 2 (14) 4 (28) 2 (14)
11 (58) 1 (5) 8 (42) 7 (36)
N/A N/A N/A 1 (100)
Values are expressed as number (percentage). a Defined as voluntary bowel movements with no soiling.
In children with a perineal fistula, 90% were continent and 21% had grade 2 constipation. One child in this group underwent a MACE procedure for resistant constipation. In the vestibular fistula group, 57% of children were continent and grade 3 constipation was noted in 28%. One child had grade 1 soiling, and 1 child had grade 2 soiling. In this group, 2 children had a MACE stoma fashioned because of intractable constipation. In the rectourethral fistula group, 58% of children had achieved full continence at 10 year follow-up. Grade 3 constipation was noted in 42% of children, and grade 2 constipation was recorded in 1 child. One child in this group had grade 3 soiling. A MACE procedure was performed 36%. The one child with a rectovesical fistula had a MACE procedure [13]. These data are summarized in Table 6.
3. Discussion The purpose of this analysis was to adopt the Krickenbeck classification for both diagnosis of the presenting anomaly and for assessment of long-term outcome. The classification was developed after an international workshop in 2005 [11]. Categories relating to diagnosis, surgical procedure, and outcome criteria were proposed. The drive to create a new classification arose from the persistent variation in reports of long-term outcome in patients born with ARM [10,14,15]. The diagnostic criteria described are a modification of the classification system previously described by Pena (Table 3). The Krickenbeck group undertook a review of the Wingspread and Pena classification systems, and a novel classification was devised.. The group made emphasis of the presence and position of the fistula. The Krickenbeck classification also included separate categories for rare variations such as pouch colon, rectal atresia, rectal stenosis, rectovaginal fistula, and H fistula. Although the PSARP procedure is seen by many as the definitive surgical treatment of ARM, the participants of the Krickenbeck workshop recognized the use of alternative techniques and proposed a separate category for classification of the operative procedure. This important addition was to allow direct comparison of functional outcome following differing surgical techniques.
A major contribution of the Krickenbeck group was the definition of precise criteria to evaluate functional outcome. The need for standardization of these outcome measures is apparent on review of the recent literature on functional outcome after PSARP. Pena reported “total continence” rates of 100% in patients with perineal fistula, 66% in patients with vestibular fistula, 34% in patients with bulbar fistula, and 26% in patients with prostatic fistula [10]. Rintala et al [14] reported 35% of children had “normal bowel scores” compared with matched controls, 35% of children were classified as having a “good” outcome, 22% of children had “fair” scores, and 8% of children had a “poor” outcome. Langemeijer and Molenaar [15] reported “full continence” in only 7% of their patients, whereas 56% had soiling and 5% were affected by constipation. In all of these reports, the functional outcome of PSARP was assessed, but the follow-up criteria were different. Yet, more clinical scoring criteria for outcome assessment have been described by Kelly [7], Templeton and Ditesheim [16], and Holschneider [17]. The ability to pass a voluntary bowel movement implies the presence of adequate innervation and function of the pelvic floor, rectum, and internal and external anal sphincter. This is an important finding in the patients after surgical correction of ARM and forms the basis for the first of 3 outcome criteria in the Krickenbeck classification (Table 5). In the patient born with ARM, anatomical and functional elements of these structures may be disrupted because of the anatomy of the ARM itself or as a consequence of operation. Constipation commonly occurs as a consequence of chronic dilatation of the rectal pouch, leading to poor peristaltic action and failure to evacuate stool adequately. It is often seen in association with vestibular and perineal fistulae [10,18-20]. Soiling occurs because of defects in the sphincteric mechanism or as a consequence of overflow from chronic constipation [10,21]. These common postoperative problems are addressed in the Krickenbeck classification, where the categories of constipation and soiling form the remaining 2 outcome criteria. An important feature of the Krickenbeck classification is the precise description of functional outcome criteria. These criteria are based on a simple grading classification with strictly defined variables for constipation and soiling. The Krickenbeck classification gives equal emphasis to both constipation and soiling. Previous scoring systems placed minimal emphasis on constipation despite its frequency in this group of patients [7,17,22]. The Krickenbeck group further introduced simplicity to a complicated subject. This simplicity enables the Krickenbeck classification to be used easily in a number of different situations: multidisciplinary clinics, nurse practioner clinics, and telephone review. The uncomplicated questions mean that the classification is relevant to the younger child when parents are largely responsible toileting and also to the older child who is usually able to answer the questions in person.
ARM assessed according to the Krickenbeck classification The inclusion of an operative procedure category in the Krickenbeck classification allows for comparison of the different techniques. The relation of functional outcome to operative technique should identify any differences. In our patients, the Pena classification for outcome criteria, in the form of a questionnaire, has been used to assess functional outcome in follow-up visits to the multidisciplinary specialist clinic. The similarity of the Krickenbeck outcome criteria to those of Pena allowed retrospective categorization of patients using the Krickenbeck classification. Analysis of these questionnaires enabled the assessment of long-term functional outcome. Continence, defined as the ability to initiate a voluntary bowel movement with no soiling, was achieved in 68% of patients. In the rectourethral fistula group, continence was noted in 58% of our patients. The constipation rate in children with perineal fistula and vestibular fistula was 26%, which is lower than the 53% reported by others [10,14,15]. Problems with soiling were noted in 14% of children with a vestibular fistula and 5% of patients with a rectourethral fistula. The one child in our study with a rectovesical fistula failed to achieve continence and required a MACE procedure. None of the 19 patients with a rectovesical fistula reported by Pena were fully continent at 3 year follow-up [10]. These results have been published for 2 reasons: firstly, to demonstrate that good functional outcome can be achieved in patients with ARM particularly in the previously poor rectourethral fistula category; secondly, to demonstrate application of the Krickenbeck classification for both diagnosis and functional outcome. Adoption of this classification should allow uniform assessment and reporting of functional outcome and comparison of different or similar management strategies internationally. The information obtained using these criteria will be invaluable in predicting realistic outcome in patients born with ARM.
References [1] Santulli TV. The treatment of imperforate anus and associated fistulas. Surg Gynecol Obstet 1952;95:601-14. [2] Trusler GA, Wilkinson RH. Imperforate anus: a review of 147 cases. Can J Surg 1962;5:269-77.
403 [3] Qi BQ, Williams A, Beasley S, et al. Clarification of the process of separation of the cloaca into rectum and urogenital sinus in the rat embryo. J Pediatr Surg 2000;35:1810-6. [4] Cho S, Moore SP, Fangman T. One hundred three consecutive patients with anorectal malformations and their associated anomalies. Arch Pediatr Adolesc Med 2001;155:587-91. [5] Pena A. Posterior sagittal anorectoplasty: results in the management of 332 cases of anorectal, malformations. Pediatr Surg Int 1988;3: 94-104. [6] Stephens FD. Imperforate rectum; a new surgical technique. Med J Aust 1953;1:202-3. [7] Kelly JH. Cine radiography in anorectal malformations. J Pediatr Surg 1969;4:538-46. [8] Douglas Stephens F, Durham Smith E, Paul N. Anorectal malformations in children: update 1988 March of Dimes Foundation. New York: Alan R. Liss Inc; 1988. [9] deVries PA, Pena A. Posterior sagittal anorectoplasty. J Pediatr Surg 1982;17:638-43. [10] Pena A. Anorectal malformations. Semin Pediatr Surg 1995;4:35-47. [11] 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. [12] Nixon HH. Use of lateral flap anoplasty. J Pediatr Surg 1985;20:563. [13] Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegrade continence enema. Lancet 1990;336:1217-8. [14] Rintala RJ, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations? J Pediatr Surg 1995; 30:491-4. [15] Langemeijer RA, Molenaar JC. Continence after posterior sagittal anorectoplasty. J Pediatr Surg 1991;26:587-90. [16] Templeton Jr JM, Ditesheim JA. High imperforate anus—quantitative results of long-term fecal continence. J Pediatr Surg 1985;20: 645-52. [17] Holschneider AM. Treatment and functional results of anorectal continence in children with imperforate anus. Acta Chir Belg 1983;82: 191-204. [18] Rintala RJ, Lindahl HG, Rasanen M. Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg 1997;32:823-6. [19] Pakarinen MP, Koivusalo A, Lindahl H, et al. Prospective controlled long-term follow-up for functional outcome after anoplasty in boys with perineal fistula. J Pediatr Gastroenterol Nutr 2007;44:436-9. [20] Yeung CK, Kiely EM. Low anorectal anomalies: a critical appraisal. Pediatr Surg Int 1991;6:333-5. [21] Cheu HW, Grosfeld JL. The atonic baggy rectum: a cause of intractable obstipation after imperforate anus repair. J Pediatr Surg 1992;27: 1071-3 [discussion 1073-4]. [22] Rintala RJ, Lindahl HG. Posterior sagittal anorectoplasty is superior to sacroperineal-sacroabdominoperineal pull-through: a long-term follow-up study in boys with high anorectal anomalies. J Pediatr Surg 1999;34:334-7.
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10-Year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeck classification Sinead Hassett, Stella Snell, Amy Hughes-Thomas, Keith Holmes ⁎ Department of Paediatric Surgery, St George's Hospital, SW17 0QT London, UK Received 6 October 2008; accepted 23 October 2008
Key words: Anorectal malformation; Continence; Long-term follow up
Abstract Background: Anorectal malformations (ARMs) affect 1 in 4000 to 5000 births. The Krickenbeck conference developed a classification based on anatomical and functional criteria to better compare treatment outcome. Aim: The aim of this study is to evaluate the functional outcome in patients 10 years following standardized surgical treatment of ARM related to the Krickenbeck classification. . Methods: Anatomical anomalies were classified as above. Children and carers were followed closely in a multidisciplinary clinic. Data were collected using a functional outcome questionnaire for a minimum of 10 years after surgical reconstruction. Outcome measurements were related to the Krickenbeck classification. Results: There were 53 children in the study group (29 male, 24 female). Krickenbeck anatomy: perineal fistula, 36%; vestibular fistula, 26%; rectourethral fistula, 36%; rectovesical fistula, 2%.All children were treated by posterior sagittal anorectoplasty. In children with perineal fistula, continence was achieved in 90%. Grade 2 constipation was noted in 21%. One child had a Malone antegrade continence enema (MACE) procedure.In children with vestibular fistula, continence was achieved in 57%. Grade 3 constipation was noted in 28%. One child had grade 1, and one child had grade 2 soiling. Two children had a MACE procedure.In children with rectourethral fistula, continence was achieved in 58%. One child had grade 3 soiling. Grade 3 constipation was found in 42% of children and grade 2 constipation in 1 child. A MACE procedure was performed in 36%. The only child with a bladder neck fistula had a MACE procedure for intractable soiling. Conclusions: The outcome for patients with ARM is related to the severity of the anomaly. The uniform application of the Krickenbeck classification should allow rational comparison of treatment outcome. © 2009 Elsevier Inc. All rights reserved.
Presented at the 55th Annual Congress of the British Association of Paediatric Surgeons, Salamanca, Spain, July 2-5, 2008. ⁎ Corresponding author. Tel.: +44 0208 725 2290; fax: +44 0208 725 0711. E-mail address: [email protected] (K. Holmes). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.10.092
Anorectal malformation (ARM) is an uncommon condition affecting 1 in 4000 to 5000 births [1,2]. The embryological pathology is related to dysmorphogensis of the cloaca and urorectum in early fetal life [3]. The anomaly may occur in isolation but is commonly associated with other anomalies, urogenital and musculoskeletal abnormalities
400 Table 1
Female
Male
S. Hassett et al. Wingspread classification High ARMs
Intermediate ARMs
Low ARMs
Cloaca
Rare malformations
Anorectal agenesis (a) with rectovaginal fistula (b) without fistula Rectal atresia Anorectal agenesis (a) with rectoprostatic urethral fistula (b) without fistula Rectal atresia
Rectovestibular fistula Recto vaginal fistula Anal agenesis without fistula
Anovestibular fistula Anocutaneous fistula Anal stenosis
Cloaca
Rare malformations
Rectobulbar urethral fistula Anal agenesis without fistula
Anocutaneous fistula Anal stenosis
being the most common [4]. Antenatal diagnosis of an isolated ARM is rare, and most are diagnosed in the early neonatal period. Consistent with incomplete separation of the cloaca, most patients have a fistula. In males and females, rectourethral fistula and rectovestibular fistula, respectively, are the most frequent variants [5]. Anorectal malformation without a fistula is uncommon and found in only 5% of cases. The earliest classification of ARM was based on the position of the terminal rectum in relation to the levator ani or pelvic floor [6]. Termination of the rectum above the levator ani was termed “high,” and termination below the levator ani termed “low.” The relationship of the terminal rectum to the levator ani formed the basis of the 1984 Wingspread classification [7,8]. Anomalies were grouped into high, intermediate. and low lesions with separate categories for male and female (Table 1). This classification was widely accepted and used for many years. A classification based on the presence and position of the fistula was described by Pena in 1995 as a result of his experience with the posterior sagittal anorectoplasty (PSARP) (Table 2) [9,10]. The position of the fistula was used to determine operative management. Pena also determined the prognosis for each group by long-term analysis of continence, constipation, and soiling. Although PSARP has become the operation of choice for many centers in the treatment of ARM, there is still variation in the follow-up criteria used. This has
Table 2
Rare malformations
perpetuated the difficulties in comparing reports of functional outcome. In an attempt to rationalize this problem, the Krickenbeck group published their findings in 2005 [11]. The resulting classification, devised after an international workshop attended by experts in the field, incorporated criteria from the Wingspread and Pena classification. The Krickenbeck classification system is composed of 3 distinct elements: a diagnostic category (Table 3), a surgical procedure category (Table 4), and a category documenting functional outcome criteria (Table 5). There have been no large long-term outcome studies using this classification. We present this series of patients, all treated by PSARP, and monitored for a minimum of 10 years after surgical treatment. We related long-term outcome to anatomy at presentation as defined by the Krickenbeck group.
1. Methods A database maintained by the senior author was searched, and all patients who were at least 10 years post definitive surgery for ARM were identified. Information was obtained from analysis of detailed questionnaires completed at each patient visit to a specialized multidisciplinary clinic (Fig. 1). The Krickenbeck classification was used for anatomical diagnostic classification and for functional outcome 10 years
Pena classification
Males
Females
Perineal fistula Rectourethral fistula (a) Bulbar (b) Prostatic Rectovesical fistula
Perineal fistula Vestibular fistula
Imperforate anus without fistula Rectal atresia
Persistent cloaca b3 cm common channel N3 cm common channel Imperforate anus without fistula Rectal atresia
Table 3
Krickenbeck classification for ARMs
Major clinical groups
Rare regional variants
Perineal fistula Rectourethral fistula (a) Bulbar (b) Prostatic Rectovesical fistula Vestibular fistula Cloaca No fistula Anal stenosis
Pouch Colon Rectal atresia/stenosis Rectovaginal fistula H fistula Others
ARM assessed according to the Krickenbeck classification Table 4
401 Table 5
Krickenbeck classification of surgical procedures
Operative procedures Perineal operation Anterior saggital approach Sacroperineal approach Posterior saggital anorectoplasty Abdominosacroperineal pull-through Abdominoperineal pull-through Laparoscopic-assisted pull-through
after reconstruction. All patients were treated by PSARP with a modified Nixon anoplasty to produce a skin-lined anus [12].
Krickenbeck classification for postoperative results
1. Voluntary bowel movements Yes/No Feeling of urge, capacity to verbalize, hold the bowel movement 2. Soiling Yes/No (a) Grade 1 Occasionally (once/ twice per week) (b) Grade 2 Every day, no social problem (c) Grade 3 Constant, social problem 3. Constipation Yes/No (a) Grade 1 Manageable by changes in diet (b) Grade 2 Requires laxatives (c) Grade 3 Resistant to laxatives and diet
2. Results A search of the database identified 53 children eligible for inclusion. There were 29 males and 24 females. Perineal fistula was found in 36% of children; vestibular fistula in 26% of children; rectourethral fistula in 36% of children; and rectovesical fistula in 1 male child (2%). Associated anomalies were present in 43% of children. Of these, 52% involved the renal tract, 39% involved the spine, 35% involved the heart, and 2% of the study group had a tracheooesophageal fistula or limb abnormality. All children had a PSARP reconstruction as their definitive operation. In 28%, PSARP was the primary
Fig. 1
procedure; the remainder had a preliminary colostomy. An abdominal operation was required in addition to PSARP in 4%. In 3 children, males with a rectourethral fistula, a repeat procedure was necessary. In all these patients, the original operation was performed at a different institution. Postoperative complications were noted in 6 children. Laparotomy was done for release of adhesions in 1 child and to repair colonic perforation in another. Mucosal prolapse requiring operative correction occurred in 3 children. One child required urethral dilatation for a temporary stricture.
Follow-up questionnaire.
402 Table 6
S. Hassett et al. Results Perineal Vestibular Rectourethral Rectovesical fistula fistula fistula fistula (n = 19) (n = 14) (n = 19) (n = 1)
Continence a Soiling Constipation MACE
17 (90) 0 4 (21) 1 (5)
8 (57) 2 (14) 4 (28) 2 (14)
11 (58) 1 (5) 8 (42) 7 (36)
N/A N/A N/A 1 (100)
Values are expressed as number (percentage). a Defined as voluntary bowel movements with no soiling.
In children with a perineal fistula, 90% were continent and 21% had grade 2 constipation. One child in this group underwent a MACE procedure for resistant constipation. In the vestibular fistula group, 57% of children were continent and grade 3 constipation was noted in 28%. One child had grade 1 soiling, and 1 child had grade 2 soiling. In this group, 2 children had a MACE stoma fashioned because of intractable constipation. In the rectourethral fistula group, 58% of children had achieved full continence at 10 year follow-up. Grade 3 constipation was noted in 42% of children, and grade 2 constipation was recorded in 1 child. One child in this group had grade 3 soiling. A MACE procedure was performed 36%. The one child with a rectovesical fistula had a MACE procedure [13]. These data are summarized in Table 6.
3. Discussion The purpose of this analysis was to adopt the Krickenbeck classification for both diagnosis of the presenting anomaly and for assessment of long-term outcome. The classification was developed after an international workshop in 2005 [11]. Categories relating to diagnosis, surgical procedure, and outcome criteria were proposed. The drive to create a new classification arose from the persistent variation in reports of long-term outcome in patients born with ARM [10,14,15]. The diagnostic criteria described are a modification of the classification system previously described by Pena (Table 3). The Krickenbeck group undertook a review of the Wingspread and Pena classification systems, and a novel classification was devised.. The group made emphasis of the presence and position of the fistula. The Krickenbeck classification also included separate categories for rare variations such as pouch colon, rectal atresia, rectal stenosis, rectovaginal fistula, and H fistula. Although the PSARP procedure is seen by many as the definitive surgical treatment of ARM, the participants of the Krickenbeck workshop recognized the use of alternative techniques and proposed a separate category for classification of the operative procedure. This important addition was to allow direct comparison of functional outcome following differing surgical techniques.
A major contribution of the Krickenbeck group was the definition of precise criteria to evaluate functional outcome. The need for standardization of these outcome measures is apparent on review of the recent literature on functional outcome after PSARP. Pena reported “total continence” rates of 100% in patients with perineal fistula, 66% in patients with vestibular fistula, 34% in patients with bulbar fistula, and 26% in patients with prostatic fistula [10]. Rintala et al [14] reported 35% of children had “normal bowel scores” compared with matched controls, 35% of children were classified as having a “good” outcome, 22% of children had “fair” scores, and 8% of children had a “poor” outcome. Langemeijer and Molenaar [15] reported “full continence” in only 7% of their patients, whereas 56% had soiling and 5% were affected by constipation. In all of these reports, the functional outcome of PSARP was assessed, but the follow-up criteria were different. Yet, more clinical scoring criteria for outcome assessment have been described by Kelly [7], Templeton and Ditesheim [16], and Holschneider [17]. The ability to pass a voluntary bowel movement implies the presence of adequate innervation and function of the pelvic floor, rectum, and internal and external anal sphincter. This is an important finding in the patients after surgical correction of ARM and forms the basis for the first of 3 outcome criteria in the Krickenbeck classification (Table 5). In the patient born with ARM, anatomical and functional elements of these structures may be disrupted because of the anatomy of the ARM itself or as a consequence of operation. Constipation commonly occurs as a consequence of chronic dilatation of the rectal pouch, leading to poor peristaltic action and failure to evacuate stool adequately. It is often seen in association with vestibular and perineal fistulae [10,18-20]. Soiling occurs because of defects in the sphincteric mechanism or as a consequence of overflow from chronic constipation [10,21]. These common postoperative problems are addressed in the Krickenbeck classification, where the categories of constipation and soiling form the remaining 2 outcome criteria. An important feature of the Krickenbeck classification is the precise description of functional outcome criteria. These criteria are based on a simple grading classification with strictly defined variables for constipation and soiling. The Krickenbeck classification gives equal emphasis to both constipation and soiling. Previous scoring systems placed minimal emphasis on constipation despite its frequency in this group of patients [7,17,22]. The Krickenbeck group further introduced simplicity to a complicated subject. This simplicity enables the Krickenbeck classification to be used easily in a number of different situations: multidisciplinary clinics, nurse practioner clinics, and telephone review. The uncomplicated questions mean that the classification is relevant to the younger child when parents are largely responsible toileting and also to the older child who is usually able to answer the questions in person.
ARM assessed according to the Krickenbeck classification The inclusion of an operative procedure category in the Krickenbeck classification allows for comparison of the different techniques. The relation of functional outcome to operative technique should identify any differences. In our patients, the Pena classification for outcome criteria, in the form of a questionnaire, has been used to assess functional outcome in follow-up visits to the multidisciplinary specialist clinic. The similarity of the Krickenbeck outcome criteria to those of Pena allowed retrospective categorization of patients using the Krickenbeck classification. Analysis of these questionnaires enabled the assessment of long-term functional outcome. Continence, defined as the ability to initiate a voluntary bowel movement with no soiling, was achieved in 68% of patients. In the rectourethral fistula group, continence was noted in 58% of our patients. The constipation rate in children with perineal fistula and vestibular fistula was 26%, which is lower than the 53% reported by others [10,14,15]. Problems with soiling were noted in 14% of children with a vestibular fistula and 5% of patients with a rectourethral fistula. The one child in our study with a rectovesical fistula failed to achieve continence and required a MACE procedure. None of the 19 patients with a rectovesical fistula reported by Pena were fully continent at 3 year follow-up [10]. These results have been published for 2 reasons: firstly, to demonstrate that good functional outcome can be achieved in patients with ARM particularly in the previously poor rectourethral fistula category; secondly, to demonstrate application of the Krickenbeck classification for both diagnosis and functional outcome. Adoption of this classification should allow uniform assessment and reporting of functional outcome and comparison of different or similar management strategies internationally. The information obtained using these criteria will be invaluable in predicting realistic outcome in patients born with ARM.
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