Errors and сomplications in the treatment of children with anorectal malformations

Cover Page

Cite item

Full Text

Abstract

Purpose. This manuscript aims to introduce errors and complications of diagnosis and treatment in children with anorectal malformations (ARM).

Methods. A retrospective analysis of 63 children with ARM treated at a single tertiary Speransky children’s Hospital.

Results. The patients’ ages ranged from 2 mo to 17 y. o. (median, 6 y. o.). The types of ARM included: rectourethral fistula 27%, rectoperineal fistula 17.5%, rectovestibular fistula 15.9%, rectobladderneck fistula 6.3%, no fistula 7.9%, cloaca 11.1%, “cloaca” with urogenital sinus and disorder of sex development 1.6%, pouch colon 1.6%, rectal stenosis 4.8%, anal duplication 3.2%, and rectovaginal fistula 3.2%. Of these patients, 76% underwent surgery earlier at another hospital (surgical treatment completed), 14% had stomas, and 10% did not have any prior procedures. The historical analysis showed diagnostic errors in 48% of children (untimely diagnosis, incorrect interpretation of the ARM variant, prolonged delay in anorectoplasty). Errors led to emergency procedures or changes in subsequent surgical treatment (further ostomy, excess bowel resection) in 22% of cases. After anorectoplasty (stenosis, mislocated anus/rectum, rectal prolapse), complications were detected in 56% of cases, whereas ostomy complications were observed in 5% of cases. Long-term problems after the surgical treatment (constipation, incontinence, and pseudoincontinence) were evident in 98% of children. Different surgical reconstructive techniques of the sphincter formation had been performed previously in 13% of patients. Moreover, they most often had spinal pathology as the cause of functional disorders. Only half of the children’s parents had information about bowel management, 38% did not follow the recommendations and usually had fecal impaction and pseudoincontinence. 45% of children/parents performed non-effective or irregular enemas and required corrective treatment.

Conclusion. It is recommended that Russian pediatric surgeons treat children with ARM, according to Russian pediatric surgeons’ guidelines consistent with international protocols to avoid errors and complications.

About the authors

Evgeniya S. Pimenova

I.M. Sechenov First Moscow State Medical University (Sechenov University); Speransky Childrens Hospital No. 9

Author for correspondence.
Email: evgeniyapimenova@list.ru

associate Professor of the Department of pediatric surgery and urology-andrology; pediatric surgeon

Russian Federation, Moscow

Darya S. Tarasova

I.M. Sechenov First Moscow State Medical University (Sechenov University); Speransky Childrens Hospital No. 9

Email: dtarasowa@yandex.ru

assistant Professor of the Department of pediatric surgery and urology-andrology

Russian Federation, Moscow

Dmitry D. Morozov

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: mr.morozovy@mail.ru

student

Russian Federation, Moscow

Dmitry A. Morozov

I.M. Sechenov First Moscow State Medical University (Sechenov University); Speransky Childrens Hospital No. 9

Email: damorozov@list.ru

Head of the Department of pediatric surgery and urology-andrology

Russian Federation, Moscow

References

  1. Аверин В.И., Ионов А.Л., Караваева С.А., и др. Аноректальные мальформации у детей (федеральные клинические рекомендации) // Детская хирургия. — 2015. — Т. 19. — № 4. — С. 29–35. [Averin VI, Ionov AL, Karavaeva SA, et al. Anorectal malformations in children (federal clinical recommendations. Russian Journal of Pediatric Surgery. 2015;19(4):29-35. (In Russ.)]
  2. Хамраев А.Ж. Корригирующие операции последствий ятрогенных хирургических осложнений на аноректальной зоне в период новорожденности // Вопросы детской диетологии. — 2007. — Т. 5. — № 4. — С. 75. [Khamraev AZh. Korrigiruyushchie operacii posledstvij yatrogennyh hirurgicheskih oslozhnenij na anorektal’noj zone v period novorozhdennosti. Voprosy Detskoi Dietologii. 2007;5(4):75. (In Russ.)]
  3. Киргизов И.В., Шишкин И.А., Апросимова С.И., Апросимов М.Н. Современные подходы к диагностике и лечению детей с высокой атрезией ануса и прямой кишки // Кремлевская медицина. Клинический вестник. — 2017. — Т. 1. — № 4. — С. 26–31. [Kirgizov IV, Shishkin IA, Aprosimova SI, Aprosimov MN. Sovremennye podhody k diagnostike i lecheniyu detej s vysokoj atreziej anusa i pryamoj kishki. Kremlin Medical Journal. 2017;1(4):26-31. (In Russ.)]
  4. Ионов А.Л., Мызин А.В., Щербакова О.В., и др. Преимущества лапароскопически-ассистированной проктопластики при лечении атрезии ануса и прямой кишки // Материалы Всероссийской научно-практической конференции с международным участием «Российский колопроктологический форум». 10–12 октября 2019 г. Самара. Колопроктология. — 2019. — Т. 18. — № S3. — С. 83. [Ionov AL, Myzin AV, Shcherbakova OV, et al. Advantages of laparoscopically-assisted anorectoplasty in comparison with operation with pull-through fromlaparotomic access in children with anorectal malformations. Abstracts of Russian Association of Coloproctologists Annual International Meeting “Russian Coloproctology Forum”, 10-12 October 2019, Samara, Russia. Coloproctology. 2019;18(S3):83. (In Russ.)]
  5. Holbrook C, Misra D, Zaparackaite I, Cleeve S. Post-operative strictures in anorectal malformation: trends over 15 years. Pediatr Surg Int. 2017;33(8):869-873. DOI: https://doi.org/10.1007/s00383-017-4111-6.
  6. Sinha SK, Kanojia RP, Wakhlu A, et al. Delayed presentation of anorectal malformations. J Indian Assoc Pediatr Surg. 2008;13(2):64-68. DOI: https://doi.org/10.4103/0971-9261.43023.
  7. Заполянский А.В., Коростелев О.Ю., Абу-Варда И.Ф., и др. Первичная проктопластика у новорожденных с аноректальными пороками развития // Медицинские новости. — 2016. — № 7. — С. 13–16. [Zapolyanskii AV, Korostelev OY, Abu-Varda IF, et al. Primary anorectoplasty in newborns with anorectal malformations. Meditsinskie novosti. 2016;(7):13-16. (In Russ)]
  8. Щапов Н.Ф., Мокрушина О.Г., Ватолин К.В., и др. Результаты ранней одномоментной радикальной коррекции низких форм атрезии ануса // Вопросы практической педиатрии. — 2014. — Т. 9. — № 3. — С. 8–14. [Shchapov NF, Mokrushina OG, Vatolin KV, et al. Results of early single-moment radical correction of low types of anal atresia. Voprosy Prakticheskoi Pediatrii. 2014;9(3):8-14. (In Russ.)]
  9. Jumbi T, Kuria K, Osawa F, Shahbal S. The effectiveness of digital anal dilatation in preventing anal strictures after anorectal malformation repair. J Pediatr Surg. 2019;54(10):2178-2181. DOI: https://doi.org/10.1016/j.jpedsurg.2019.04.004.
  10. Alam S, Lawal TA, Peña A, et al. Acquired posterior urethral diverticulum following surgery for anorectal malformations. J Pediatr Surg. 2011;46(6):1231-1235. DOI: https://doi.org/10.1016/j.jpedsurg.2011.03.061.
  11. Bischoff A, Martinez-Leo B, Peña A. Laparoscopic approach in the management of anorectal malformations. Pediatr Surg Int. 2015;31(5):431-437. DOI: https://doi.org/10.1007/s00383-015-3687-y.
  12. Divarci E, Ergun O. General complications after surgery for anorectal malformations. Pediatr Surg Int. 2020;36(4):431-445. doi: 10.1007/s00383-020-04629-9.
  13. Bhojwani R, Ojha S, Gupta R, Doshi D, Long-term follow-up of anorectal malformation – how long is long term? Annals of Pediatric Surgery. 2018;14(3):111-115. DOI: https://doi.org/10.1097/01.XPS.0000529797.96055.cc.
  14. Peña A., Bischoff A. Surgical treatment of colorectal problems in children. Springer; 2015. 497 p.
  15. Kyrklund K, Pakarinen MP, Koivusalo A, Rintala RJ. Long-term bowel functional outcomes in rectourethral fistula treated with PSARP: controlled results after 4-29 years of follow-up: a single-institution, cross-sectional study. J Pediatr Surg. 2014;49(11):1635-1642. DOI: https://doi.org/10.1016/j.jpedsurg.2014.04.017.
  16. Van Meegdenburg MM, Heineman E, Broens PM. Dyssynergic defecation may aggravate constipation: results of mostly pediatric cases with congenital anorectal malformation. Am J Surg. 2015;210(2):357-364. DOI: https://doi.org/10.1016/j.amjsurg.2014.09.038.
  17. Kyrklund K, Pakarinen MP, Rintala RJ. Long-term bowel function, quality of life and sexual function in patients with anorectal malformations treated during the PSARP era. Semin Pediatr Surg. 2017;26(5):336-342. DOI: https://doi.org/10.1053/j.sempedsurg.2017.09.010.
  18. Minneci PC, Kabre RS, Mak GZ, et al. Can fecal continence be predicted in patients born with anorectal malformations? J Pediatr Surg. 2019;54(6):1159-1163. DOI: https://doi.org/10.1016/j.jpedsurg.2019.02.035.
  19. Schmiedeke E, Zwink N, Schwarzer N, et al. Unexpected results of a nationwide, treatment-independent assessment of fecal incontinence in patients with anorectal anomalies. Pediatr Surg Int. 2012;28(8):825-830. DOI: https://doi.org/10.1007/s00383-012-3127-1.
  20. Иванов П.В., Киргизов И.В., Баранов К.Н., Шишкин И.А. Этапное лечение аноректальных пороков у детей // Медицинский вестник Северного Кавказа. — 2010. — Т. 19. — № 3. — С. 88–89 [Ivanov PV, Kirgizov IV, Baranov KN, Shishkin IA. Step treatment of anorectal diseases in children. Medical News of North Caucasus. 2010;19(3):88-89. (In Russ.)].
  21. Totonelli G, Morini M, Catania VD, et al. Anorectal malformations associated spinal cord anomalies. Pediatr Surg Int. 2016;32(8):729-735. DOI: https://doi.org/10.1007/s00383-016-3914-1.
  22. Сенина М.С. Совершенствование организации медицинской помощи детям с атрезией прямой кишки и ануса в нижегородской области // Российский педиатрический журнал. — 2019. — Т. 22. — № 5. — С. 317. [Senina MS. Improving the organization of medical care for children with rectal and anus atresia in the Nizhny Novgorod Region. Russian Pediatric Journal. 2019;22(5):317. (In Russ.)]
  23. Bischoff A, Bealer J, Wilcox DT, Peña A. Error traps and culture of safety in anorectalmalformations. Semin Pediatr Surg. 2019;28(3):131-134. DOI: https://doi.org/ 10.1053/j.sempedsurg.2019.04.016

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 1. Descriptive statistics of a group of patients with anorectal malformations

Download (244KB)
Indexing metadata
3. Fig. 2. Status localis of patients with anorectal malformations: a — patient A., 1.9 y. o., anorectal malformations with rectourethral fistula, view after anorectoplasty in the other hospital (concomitant diagnosis—tethered cord, terminal lipoma). Rectal prolapse immediately after anorectoplasty (a technical feature of the performed procedure); b — patient M., 8 y. o., anorectal malformations with rectobladderneck fistula, condition after two anorectoplasty (concomitant diagnosis—caudal regression syndrome, dysfunction of the pelvic organs). Stenosis of the anus and prolapse of the rectal mucosa six months after anorectoplasty; degeneration of the mucosa into a fibroepithelial polyp, probably due to trauma during enemas

Download (130KB)
Indexing metadata
4. Fig. 3. Diagnostic and preoperative planning errors in children with anorectal malformations after birth

Download (891KB)
Indexing metadata
5. Fig. 4. Complications after anorectoplasty: a — descriptive statistics (n = 38); b — distal sigmoidostomography of a patient with a cloaca (a catheter is inserted into the bladder). П — the rectum, which flows into the uterus; В — doubled vagina; the brace marks the distance from the rectum to the anal place; c — mislocatsd anus and rectum (anterior postoperative ectopia of the anus). The white arrow indicates the place of the intended anus, considering the location of the fibers of the external sphincter during electroidentification; d — cystourethroscopy of the patient after surgical correction of the arm with a rectourethral fistula. White arrow shows the entrance to the bladder, black arrow — a wide posterior diverticulum of the urethra (rectal stump)

Download (343KB)
Indexing metadata
6. Fig. 5. Clinical case. Patient B., 6.5 y. o. Meningomyeloradiculocele and anorectal malformations with rectovestibular fistula (absent of rectal sensitivity and voluntary sphincters contraction): a — anorectal water-perfusion profilometry (blue oval — resting pressure in the anal canal, red oval — an attempt at volitional contraction), no increase in anal pressure (impossibility of contraction of the sphincters); b — anorectal manometry with a balloon (oval — markers of gradient air insufflation (from 10 to 80 ml), rectangle — lack of reaction of the rectal ampulla in response to stretching (combined with a subjective lack of rectal sensitivity, urge)); c — status localis after surgical correction of the herniated disc; d — status localis after anterosagittal anorectoplasty at the age of one year and “reconstruction of the obturator apparatus of the striated muscles” at the age of five years

Download (377KB)
Indexing metadata

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies