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Dear colleagues!

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1. Now we all know that the posterior sagittal anorectoplasty (PSARP) is far from the ideal. For example, the long-term outcome after PSARP is reported to depend especially on the ARM, fecal incontinence is reported by 40-67% and lack of voluntary bowel control by 15-30% [1]. At the low type, there is always a chronic constipation [2].

 

2. Ethical problems

2.1 For several generations, until 1982, the scientific works of the histologists, embryologists, surgeons and radiologists kept proving that the gut located caudal to pubococcygeal line in patient with the ARM functions as the anal canal.

2.2 In 1982, deVries PA, who studied the anatomy of the perineum, proposed the posterior sagittal approach, which facilitates the work of pediatric surgeons, but proved not to have a significant effect on the treatment results [3] (authorship is determined by the order of the co-authors).

2.3 In 2 months, an article was published where the first author showed a lack of knowledge on anatomy and physiology of the anorectal area and lack of understanding of the importance of scientific evidence [4]. Since then a new generation of pediatric surgeons has grown on misconceptions about the anatomy and physiology of the anorectal area in norm and ARM [5].

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3. Pathophysiology of the anorectal malformations with visible fistulas

3.1 Recently there has been collected enough evidence that the caudal intestinal segment at the ARM is the anal ectopy [6,7].

3.2 The internal anal sphincter (IAS) responsible for the anorectal inhibitory reflex in all patients with the rectoperineal and rectovestibular fistulas was discovered [6-8].

3.2 Fecal retention and defecation were normal in all patients, where a strong anal stenosis was absent.

3.3 The normal function of the levator ani muscle (LAM - ileococcygeus and pubococcygeus)  and puborectalis muscle (PRM) was found.

3.4 Below is the anterior anal ectopy with stenotic ring at the skin level, which located outside the subcutaneous portion of the EAS (Figure 1). Consequently, the ARM with visible fistulas are a low type anomaly.

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Figure 1. Lateral radiographs of the anorectal area. Studies with Foley catheter. The distance between the button located in the anal dimple and the balloon is 2 mm. Comorbidity of the sacrum does not affect the anorectal function.

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4. The PSARP – anatomical and physiological effects

4.1 The IAS, responsible for the prolonged fecal retention in rest, is removed. This is one of the reasons for fecal incontinence.

4.2 The PRM, responsible for the fecal retention during the rectal pressure increase, is damaged. This is another reason for fecal incontinence.

4.3 All parts of the EAS, responsible for emergency fecal retention, are damaged. This is one more reason for fecal incontinence.

4.4. The connection of the superficial portion of the EAS with the coccyx is intersected during posterior dissection, which leads to a lack of the disclosure of the lower part of the anal canal during a bowel movement. This is the reason of chronic constipation.

4.5. The connection of the LAM to the anal canal is intersected, which leads to a lack of the disclosure of the upper part of the anal canal during a bowel movement. This is the reason of chronic constipation too.

4.6. The PSARP converts the functional anal ectopy into the poorly functioning fistula, where the weakness of the fecal retention is partially compensated by the chronic fecal congestion and vice versa.

 

5. The purpose, rationales and limitations

5.1. It is known, that the use of a fistula (which is actually the IAS) improves functional results.

5.2. After the operation of "anal formation" in the majority of patients under one year with visible fistulas the functions of the fecal retention and defecation were not different from the norm [7,8,9].

5.3. After the operation of «perineum perforation» all 3 newborns without fistula, who didn’t have stapling of the IAS with the skin, were completely healthy [7,8].

5.4. It is known, that the compression anastomosis is not complicated by the stenosis.

5.5. The goal of each of us is to achieve the best functional outcome. This is possible if you preserve all of the existing elements of the anal canal.

5.6. Chronic constipation is caused by the anal stenosis, leading to secondary damage of all structures of the anal canal. Therefore, the treatment should begin before the occurrence of the secondary damage of the anal canal or after dissection of the ectopic anal stenosis.

5.7. Is it fair to continue performing the PSARP if there is at least a little chance that the patients can be completely healthy having had the operation proposed by me?

 

6. Description of the surgical intervention in patients with visible fistulas

It consists of two stages:

6.1. The first stage - formation of the anus in the anal dimple with the help of the device. It can be applied, if the blind contour of the anal canal is located from 0.2 to 0.5 cm from the marker in the anal dimple. To determine this distance, a Foley catheter is inserted into the rectum through the fistula. After introduction of 5 - 7 cm³ of air or contrast medium into its balloon, the catheter is pulled down until it stops (Figure 1).  

The operation begins with allocation of the subcutaneous portion of the external anal sphincter and stretching it to rings 1 cm in diameter. Operation continues with the device, the movable part of which, 0.8 cm in diameter, is introduced into the anal canal through the fistula (see Fig. 2, A).

A

B

C

Figure 2. A device for creation of the anus. Stages of the application.

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Then the "knee" is rectified and fixed by the mobile tube (Fig. 2, B). The lower cylinder 0.8 cm in diameter with a needle is inserted into the center of the ring of the external anal sphincter and is screwed to the upper cylinder of the same diameter with a hole for the needle (Figure 2, C). After 7-10 days the device is removed. There remains an orifice of the anal canal with fused edges without scarring. After some time begins bougienage of the new anus to the size required for the free defecation.

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6.2. At the second stage the fistula is closed. In order to prevent its re-opening, it is necessary to prevent the insertion of the anal canal mucosa in the wound channel.

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This method allows to fully preserve the anal canal which always has the internal anal sphincter. It is responsible for the pressure drop after the stretching of the rectum (anorectal inhibitory reflex). The connection between the superficial portion of the external anal sphincter and the coccyx is not destroyed, which results in retaining the ability of this portion to disclose the lower part of the anal canal during defecation. The connection of the levator ani muscle with the upper part of the anal canal is not intersected, which results in retaining the ability of the levanor ani muscle to disclose the upper part of the anal canal during defecation. The puborectalis muscle, urethra and vagina are not damaged. 

Dear colleagues! You face a dilemma:

  1) Continue doing the operations, which result in ...

«The functional outcome and quality of life in adults with anorectal malformations are closely related to the type of malformation. A large proportion of the patients have persistent fecal incontinence, constipation and sexual problems that have a negative influence on their quality of life” [10].

  2) Or check out the possibility of full recovery of the anorectal area function, because in most cases of the ARM there is a functioning anal canal, i.e. a low ectopic anus.

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I propose trying this method experimentally and clinically. I'll send this device if you send your mailing address to my e-mail (nivel70@hotmail.com). I would be very grateful if you send feedback and comments on the device and on the method of its application.  

 

Yours faithfully,

 

Michael Levin

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References:

  1. Stenström P1, Hambraeus M2, Arnbjörnsson E2, Örnö AK3.Pelvic floor in females with anorectal malformations--findings on perineal ultrasonography and aspects of delivery mode. J Pediatr Surg. 2015 Apr;50(4):622-9. doi: 10.1016/j.jpedsurg.2014.08.004. Epub 2014 Oct 1.

  2. Levitt MA1, Kant A, Peña A. The morbidity of constipation in patients with anorectal malformations. J Pediatr Surg. 2010 Jun;45(6):1228-33. doi: 10.1016/j.jpedsurg.2010.02.096.

  3. deVries PA, Peña A.Posterior sagittal anorectoplasty. J Pediatr Surg. 1982 Oct;17(5):638-43.

  4. Peña A, Devries PA. Posterior sagittal anorectoplasty: important technical considerations and new applications. J Pediatr Surg. 1982 Dec;17(6):796-811.

  5. Ruttenstock EM, Zani A, Huber-Zeyringer A, Höllwarth ME. Pre- and postoperative rectal manometric assessment of patients with anorectal malformations: should we preserve the fistula? Dis Colon Rectum. 2013 Apr;56(4):499-504.

  6. http://www.anorectalmalformations.com/Publications/. Posterior sagittal anorectoplasty. Myths and scientific facts.

  7. Misharev OS, Levin MD, Nikifonov AN, Soroka AA, Drozdovskii VN. Theoretical basis of surgical tactics in rectal atresia with fistulas in the perineum and vagina in children.  Vestn Khir Im I I Grek. 1983 Apr;130(4):92-7.

  8. http://www.anorectalmalformations.com/Publications/ The pathophysiology  of anorectal malformations. From a new concept to a new treatment.

  9. Levin MD. The pathological physiology of the anorectal defects, from the new concept to the new treatment. Eksp Klin Gastroenterol. 2013;(11):38-48.  

  10. Danielson J1, Karlbom U2, Graf W2, Wester T3. Outcome in adults with anorectal malformations in relation to modern classification - Which patients do we need to follow beyond childhood? J Pediatr Surg. 2016 Nov 15. pii: S0022-3468(16)30563-2. Doi:10.1016/j.jpedsurg.2016.10.051.

 

 

 

I sent a letter (Dear colleagues!) To 33 pediatric surgeons with a proposal to test in an experiment or in a clinic a device for surgery of the patients with ARM with visible fistula (attached). The proposed operation allows (theoretically) to preserve all elements of the ectopic anal canal and dramatically improve the functional results of the treatment. I was impressed by the reaction of the leading surgeons:

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- «I cannot use this in clinical practice unless it is FDA approved».

- «I don't have a lot of thoughts about the physiology, sorry».

-  «I have now left the operations of anorectal malformations to my younger colleagues. I will show them the piece of instrument and discuss the method…»

 

 

-  “Dear Michael

 

I have been receiving many review papers from you the past year or so and would like to thank you for your continued interest and contribution to this field. 

 

I do however in every your review papers experience a very aggressive undertone towards people like de Vries and Pena which scientific work I value very much.

 

I accept that there are differences in opinion. I also accept that facts change with every  new scientific evidence that emerges. But new research should not be presented in such a way that other authors are denied there scientific value of their work.

 

I respect your work and other scientist alike and as a scientist myself i believe we should all follow this principle.

 

Thank you

 

Jose alves”

 

 

Open letter

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Dear Dr. Jose Alves!

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Your letter made me wonder if I crossed the red line. Of course, everything depends on priorities. Unfortunately, not always the science, interests of the patient and collegiality are in an equivalent ratio, in a single bundle.

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The de Vries article in collaboration with Peña (1982) is a fair scientific research, in which the posterior sagittal approach was proposed. It wasn’t a new type of operation – it was a different approach. The authorship of de Vries is beyond doubt not only due to that he’s the first in the order but also due to the fact, that he studied the anatomy of the anorectal zone. In 2 months Dr. Peña published an article where he put himself as the first author. All the novelty of this article is just a fantasy of Dr. Peña [1]. The article shows, first of all that the author had no idea about the scientific research on the anatomy and physiology of the anorectal zone in general and the pathophysiology of anorectal defects in particular. Secondly, it was an evident attempt to associate himself as an author of the posterior sagittal approach. Though him not having even basic knowledge in the theme contradicts such possibility. It is no surprise that in his memoir (Russian translation) Dr. Peña didn’t mention Dr. de Vries even once.

 

Since then, it is assumed that ARM with visible fistulas are intermediate types, and with fistulas to the urethra are of a high type [1]. Based on the assumption that at the ARM there’s no anal canal, all the earlier approaches had been changed: there had been adopted Krickenbeck classification, rejection of diagnostic methods of research and statement that if the anal canal is absent at birth, there cannot be an absolute recovery after the allegedly perfect procedure (posterior sagittal anorectoplasty -PSARP). This led to creation of Kelly's clinical score, where the bad results of the treatment are called the good ones. When there appeared reports that in the patients during the radiologic examination the gas was descending, almost reaching the skin, Dr. Peña explained this by the descending perineum as a result of anxiety. Meanwhile it is known that the pelvic floor rises and doesn’t descend during the anxiety.

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But let’s look at the facts:

  1. There is no scientific evidence that the anal canal is missing or damaged at the ARM with visible fistulas. If you think that there is such evidence, please, send it to me and we will discuss it.

  2. Ruttenstock EM, et al. discovered the anorectal inhibitory reflex in all patients with visible fistula [2]. It is a scientific fact. I do not doubt it, because we have found a positive reflex in all patients with visible fistula too [3]. This article is in Russian and I can send it. It means that in the so-called fistula there is at least an internal anal sphincter.

  3. In this "fistula" we found a normal function of puborectalis muscle and levator ani muscle [4,5]. From this it follows that all patients with visible fistulas have a low ARM with the functioning anal canal, i.e. anal ectopy.

  4. As was shown in your study, most patients with urethral fistula also have a functioning anal canal [4,5,6].

  5. As a scientist, you cannot but agree with me that there are two mutually exclusive views on the pathological physiology of anorectal malformations. As a scientist, you cannot but agree with me that the views are of no importance in the scientific world - scientific evidence is important.

  6. I insist that the works of Dr. Peña are a dramatic deviation from the truth. The destruction of the anal canal during PSARP is an irreversible catastrophe for each patient. There is a need in the international conference of the pediatric surgeons working in the field of anorectal malformations.

  7. I do hope that your scientific studies will contribute to improvement of the treatment of the patients with the ARM.

 

Science is the battle of ideas. This is an uncompromising battle, because two mutually exclusive ideas cannot both be true. One of them is wrong, and therefore harmful. The emergence of erroneous ideas can be either the product of the unconscious delusion, for example, due to misinterpretation of the research results, or the result of a deception, in order to achieve career ambitions. It is easy and psychologically necessary for a scientist to correct an error, because correcting an error is also a scientific achievement. Those who use deception are forced to cover the deceit with a cascade of lies. And lie and science are two incompatible things.

 

Personalization of scientifaic battle is inevitable in cases of struggle against the unscientific fiction, which became a system of "religious" beliefs of a whole generation of pediatric surgeons.

 

It is striking that there is no scientific discussion in the corporation of pediatric surgeons. A striking example:  Kyrklund and co-authors found that IAS-saving anatomical repairs leads to a better functional results [8].  Lombardi and co-authors, in contrast, suggest removing distal 3 cm [9]. Why is this not discussed?

Where there is no discussion, there is no science. Absence of scientific discussion is due to the lack of basic knowledge of the normal anatomy and physiology of the anorectal zone, as well as the pathological physiology of anorectal malformations. Nothing remains except the posterior sagittal approach.

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References

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  1.  Levin MD. Posterior sagittal anorectoplasty. Myths and Scientific facts. http://www.anorectalmalformations.com/Publications/

  2. Han Y1, Xia Z1, Guo S1, Yu X1, Li Z1. Laparoscopically Assisted Anorectal Pull-Through versus Posterior Sagittal Anorectoplasty for High and Intermediate Anorectal Malformations: A Systematic Review and Meta-Analysis. PLoS One. 2017 Jan 18;12(1):e0170421. doi: 10.1371/journal.pone.0170421. eCollection 2017.

  3. Ruttenstock EM, Zani A, Huber-Zeyringer A, Höllwarth ME. Pre- and postoperative rectal manometric assessment of patients with anorectal malformations: should we preserve the fistula? Dis Colon Rectum. 2013 Apr;56(4):499-504.

  4.  Misharev OS, Levin MD, Nikifonov AN, et al.  [Theoretical basis of surgical tactics in rectal atresia with fistulas in the perineum and vagina in children]. Vestn Khir Im I I Grek. 1983 Apr;130(4):92-7. [Article in Russian]

  5.  Alves JC1, Sidler D, Lotz JW, Pitcher RD. Comparison of MR and fluoroscopic mucous fistulography in the pre-operative evaluation of infants with anorectal malformation: a pilot study. Pediatr Radiol. 2013 Aug;43(8):958-63. doi: 10.1007/s00247-013-2653-x. Epub 2013 Mar 5.

  6. http://www.anorectalmalformations.com/Publications/ The pathophysiology of anorectal malformations. From a new concept to a new treatment.

  7. Levin MD. The pathological physiology of the anorectal defects, from the new concept to the new treatment. Eksp Klin Gastroenterol. 2013;(11):38-48. 

  8. Kyrklund K1, Pakarinen MP2, Rintala RJ2. Manometric findings in relation to functional outcomes in different types of anorectal malformations. J Pediatr Surg. 2016 Sep 2. pii: S0022-3468(16)30301-3. doi: 10.1016/j.jpedsurg.2016.08.025.  

  9. Lombardi L1, Bruder E, Caravaggi F, et al. Abnormalities in "low" anorectal malformations (ARMs) and functional results resecting the distal 3 cm. J Pediatr Surg. 2013 Jun;48(6):1294-300. doi: 10.1016/j.jpedsurg.2013.03.026.

 

 

Yours faithfully                                    Michael Levin            10/3/2017

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