Special Report

Preliminary Analysis of the Surgical Treatment of Anorectal Malformations in Russia Dmitry Morozov1,2

Evgeniya Pimenova1,2

Evgeniy Oculov1

1 Research Institute of Pediatric Surgery, Scientific Centre of Children

Health, Moscow, Russian Federation 2 Department of Pediatric Surgery, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation

Alexey Gusev1

Kseniya Utkina1,2

Address for correspondence Dmitry Morozov, MD, Research Institute of Pediatric Surgery, Scientific Centre of Children Health, Lomonosovsky prospekt, 2, b.1 Moscow 119991, Russian Federation (e-mail: [email protected]).

Abstract

Keywords

► anorectal malformations ► Russian consensus ► pediatric coloproctology

The article provides the analysis of a survey of the professional community of Russian pediatric surgeons, dedicated to the treatment of anorectal malformations (ARM). The authors evaluated the differences and similarities in classification, surgical procedures, time of definitive repair, and postoperative management of ARM in different hospitals and centers. This was done by a survey upon specialists and experts in Russia followed by a symposium with live surgery, open discussion, and vote. Overall, 85% of the delegates supported the idea to create several regional centers of pediatric coloproctology as the way to improve the treatment of ARM in Russia. Moreover, 80% of delegates agreed to create a universal database of ARM information. The development of neonatal surgery and videoendoscopic surgical methods in the treatment of patients with ARM requires creation of a national guideline by the Russian Association of Pediatric Surgeons. Next step will concern standardization of the diagnosis and surgical treatment of children with ARM. This study is a collaborative effort to provide Russian Consensus on treatment of ARM.

Introduction Development of standards in the treatment of patients with anorectal malformations (ARM) is a problem of current importance in Russia,1 as in other countries2 there are several reasons for that. The variability of ARM clinically and technically requires surgeon to be skilled, but in most cases, the number of newborns, admitted to some department of pediatric surgery, are not sufficient to gain experience in this field. Up-to-date trends in pediatric coloproctology include a large variety of surgical approaches and management strategies.3,4 Personal search activity in special literature and internet resources may lead to unfounded usage of innovations.5,6 On the contrary, modern medical jurisprudence requires the restriction of such baseless approaches and the development of new standards for pediatric surgeons.7 The medical organizational measures that regulate the relations of differ-

received June 2, 2014 accepted July 3, 2014

ent children’s hospitals and other medical institutions are important factors that influence the quality of rendered surgical care to patients with congenital anomalies.8–10 It should be in our country a professional consensus made by the Russian Association of Pediatric Surgeons (RAPS).11 The development of such consensus has the following two main objects: to improve the surgical treatment of ARM and to provide legal defense for surgeons who deal with patients with ARM. We perform a survey upon specialists and experts all over Russia to analyze the up-to-date situation concerning management and surgical treatment of children with ARM.

Methods and Material Three groups of respondents were systematically analyzed. Two surveys were performed on the basis of 28 clinics in Russia. Total amount of cases requiring anorectoplasty in those clinics was around 300 annually. The survey includes

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DOI http://dx.doi.org/ 10.1055/s-0034-1387948. ISSN 0939-7248.

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Eur J Pediatr Surg

Preliminary Analysis of the Surgical Treatment of ARM in Russia

Table 1 Surgical approaches to colostomy Operative procedure

Surgeons

Chief experts

Total

Sigmostomy on the first sigmoid loop

9

2

11

Descendostomy

3

3

6

Transversostomy

1

Results and Discussion

Ascendostomy

We sent questionnaires to 50 hospitals in the country of which we received a reply from 28 clinics (56%). The main problem in the management of ARM in Russia is less number of newborns with such malformation in some pediatric department. More than 50% of all the clinics have not more than five to six new patients with ARM annually. Approximately 10 clinics (36%) have 10 patients, and only 3 major centers (11%) have an experience of 20 and more newborns every year. Concerning the cloacas, in most clinics, there were no such clinical cases or not more than one to two operations during the decade. Analyses of performed primary anorectoplasties, repeated and reconstructive operations show that a half of Russian pediatric department had not more than 10 such operations annually, whereas four centers performed 30 to 50 operations every year. In Russia, pediatric surgeons historically have two different approaches. Most clinics use the Lyonyushkin classification, several clinics use Pena, Stephens, Smith classifications, and only two clinics use the International Krickenbeck Classification.12 As a result of the vote during the symposium, 45% of respondents continue to support the Lyonyushkin classification, 20% the Pena, Stephens, and Smith classifications, and 22% the International Krickenbeck classification, which was made as a consensus of 25 leading European coloproctologist.12 Opinion of the experts (five over seven) support the Krickenbeck classification and that there is no necessity to include such speculative embryological terms in clinical diagnosis of anatomical types of malformations. Preoperative Colostomy. Our survey showed that only 25% of surgeons in Russia perform radical surgical correction of ARM (except imperforate anal membrane) with previous colostomy. About 25% use previous colostomy even in case of rectoperineal and rectovestibular fistulas, 50% perform colostomy only in patients with so-called high ARM and cloacas. All experts consider previous colostomy to be the correct decision. The technique of initial colostomy plays a great role, and our respondents had no agreement of opinion (►Table 1). Most of surgeons and experts prefer to use first sigmoid loop and to make double-barrel colostomy with separated stomas. Voting showed that two-thirds of respondents agree with such technique; 90% of respondents use colostomy bag in newborns. Which age is optimal for ARM repair? There is no consensus on this issue. In 2 clinics, surgical repair was usually performed in newborns; in 3 clinics, at the age of 2 to 3 months (in accordance to Pena conception); in 18 clinics

End colostomy

1

Double-barreled loop colostomy Double-barreled separated colostomy

European Journal of Pediatric Surgery

1 1

1

5

2

7

6

5

11

the optimal age was 6 to 12 months; and in 5 clinics such operations were performed on patients older than 1 year. Altogether, it seems like decision making depends on the following two key points: possibilities of anesthesiology and reanimation department. Also, many surgeons believe that optimally to do procedures for children older than 1 year, because they have a more developed muscular complex. A clinical case of newborn with rectovestibular fistula was demonstrated during the workshop, and we asked the delegates to give their opinion. Of all respondents, 53% decided to perform bougienage of fistula and to plan the repair at 2 to 3 months of age; 24% preferred to wait for the repair until the child was 1 year old; 8% decided to wait for several years. Only 15% thought it is possible to make an operative correction of this type of AMR during the neonatal period (►Table 2). It is important for our standardization to analyze the choice of surgical technique in children with “low” variants of the anorectal malformations (ARM). Of all, 50% of specialists prefer to repair such malformations using an anterior perineal approach whereas the other 50% preferred posterior sagittal anorectoplasty. Some surgeons noticed the differences in approaches for male- and female-newborns (anterior perineal anorectoplasty for females and posterior sagittal anorectoplasty for males); but most of the specialists told about individual choice for each patient. Chief experts had no consensus on preferable approach, but all of them supported

Table 2 Results of voting Answer variants

% of respondents

At neonatal period

21

At the age of 2–3 mo

27

At the age of 6 mo

18

Upon the attainment of the age of 1 y

23

Upon the attainment of proper physical development

10

Note: When radical surgical repair of anorectal malformations should be performed?

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65 variants of answers on 14 main positions (Supplementary Appendices 1 and 2, online-only). Forms that had been filled by seven experts in coloproctology (20–40 operations annually). We organized a workshop entitled “Anorectal Malformations Treatment. The Consensus.” During the entire symposium several key points of ARM treatment has been submitted to a vote: 56 workshop participants participated.

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Preliminary Analysis of the Surgical Treatment of ARM in Russia

1. Preventive colostomy (exclude vestibular and perineal fistula). 2. The surgical repair of ARM must be done according to RAPS recommendations, based on international experience. 3. Surgical repair of ARM can be performed if surgeons’ experience and technical environment are sufficient to do it; if not, patient should be transferred to specialized colorectal center.

4. The specialized center can perform even the most difficult kinds of surgical treatment and repair even the most complicated variants of ARM.

Conclusions Our analysis showed that pediatric surgeons in Russia had accumulated a great experience of ARM surgical repair, but there are a lot of differences in management of such patients in different centers. The rapid evolution of neonatal surgery and videoendoscopic surgical methods of ARM treatment requires creation of a national guideline by the Russian Association of Pediatric Surgeons. Next step will concern standardization of the diagnosis and surgical treatment of children with ARM. To control the efficacy in the management of patients with ARM, it is necessary to perform a national monitoring of the patients, to develop special centers for education and scientific researches, and to accredit pediatric surgical departments.

Conflict of Interest None.

References 1 Lenyushkin AI. Surgical Coloproctology in children [Khirurgiche-

skaya koloproktologiya u detey]. Moscow: Meditsina; 1999 2 Abdur-Rahman LO, Shawyer A, Vizcarra R, Bailey K, Cameron BH.

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7 8

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Do geography and resources influence the need for colostomy in Hirschsprung’s disease and anorectal malformations? A Canadian association of paediatric surgeons: association of paediatric surgeons of Nigeria survey. Afr J Paediatr Surg 2014;11(2): 150–157 Clermidi P, Podevin G, Crétolle C, Sarnacki S, Hardouin JB. The challenge of measuring quality of life in children with Hirschsprung’s disease or anorectal malformation. J Pediatr Surg 2013; 48(10):2118–2127 Wijers CH, de Blaauw I, Marcelis CL, et al. Research perspectives in the etiology of congenital anorectal malformations using data of the International Consortium on Anorectal Malformations: evidence for risk factors across different populations. Pediatr Surg Int 2010;26(11):1093–1099 Bischoff A, Levitt MA, Peña A. Update on the management of anorectal malformations. Pediatr Surg Int 2013;29(9): 899–904 Bischoff A, Peña A, Levitt MA. Laparoscopic-assisted PSARP - the advantages of combining both techniques for the treatment of anorectal malformations with recto-bladderneck or high prostatic fistulas. J Pediatr Surg 2013;48(2):367–371 Rintala RJ. Congenital anorectal malformations: anything new? J Pediatr Gastroenterol Nutr 2009;48(Suppl 2):S79–S82 Baranov AA, Namazova-Baranova LS, Al’bitskiy VYu. Preventive paediatrics — new challenges [in Russian]. Voprosy Sovremennoy Pediatrii 2012;11(Suppl 2):7–10 Geras’kin AV, Mokrushina OG, Morozov DA, Akhunzyanov AA, Gumerov AA. State and prospects of improvement of surgical newborns with congenital defects [in Russian]. Rossiyskiy vestnik perinatologii i pediatrii 2009;54(Suppl 6):7–12 Khamatkhanova EM, Kucherov YuI, Frolova OG, et al. Ways to improve the quality of medical care for congenital malformations[in Russian]. Akusherstvo i ginekologiya 2011; 4:79–84

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sphincter-protecting operations with diagnostic electrostimulation of the sphincter muscle complex. We can see more difficulties in decision-making for “high” malformations (without fistula or with rectourethral, rectobladder, rectovaginal fistulas, and cloacas also). Most (39%) of surgeons and three experts prefer Pena posterior sagittal anorectoplasty in combination with abdominal approach, if needed. In eight clinics, it is usual to repair such malformation by a combination of posterior sagittal anorectoplasty and laparoscopic operations. One-third of respondents thought the Romualdi operation to be the most preferable in such cases, whereas in three clinics the laparoscopic-assisted anorectoplasty is the best technique. The voting results were as follows: 56% decided to perform colostomy at first and then to repair the malformation in 2 to 3 months; 30% of respondents decided to perform colostomy and then operation at 1 year of age; 14% decided to perform radical repair in the neonatal period. During the discussion, most of the specialists underlined again the necessity of muscle structure control in all kinds of operations. Analysis of clinical cases of newborn with persistent cloaca, which was also demonstrated during the workshop, resulted in such decisions: 40% of respondents were ready to perform all steps of treatment in their department, and 56% decided to perform the preventive colostomy by themselves, and then transfer such patients to the special center where treatment should be ended. The ultimate question debated concerned the delay to close the colostomy and the post-operative management. Overall, 50% usually close the colostomy 1 to 2 months after operative repair of ARM and the other 50% think it should be guided by neoanus bougienage effectiveness. Most surgeons waited until they can introduce easily a bougie proper for the patient’s age. In some clinics, it is usual practice to close the colostomy at 5 to 6 months postrepair. Concerning postoperative management, most of the clinics perform neoanus bougienage in all patients, but four respondents noticed that they do it only in some cases, and in five clinics specialists do it only in cases of stenosis; so this question remains controversial too. At the end of the symposium, it was gratifying to note that 85% of the delegates supported the idea to create several regional centers of pediatric coloproctology as the way to improve the treatment of ARM in Russia. Moreover, 80% of respondents agreed to create a universal database of ARM information. As a result of our study, the board of the Russian Association of Pediatric Surgeons developed an algorithm of decision making for patients with ARM. The main steps are as follows:

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Preliminary Analysis of the Surgical Treatment of ARM in Russia

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12 Holschneider A, Hutson J, Peña A, et al. Preliminary report on the

surgical treatment of children with anorectal malformations. The first steps. Rossiyskiy vestnik detskoy khirurgii, anesteziologii i reanimatologii 2013;4:8–13

International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 2005; 40(10):1521–1526

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11 Morozov DA, Okulov EA, Pimenova ES. Russian consensus on the

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