Priority areas for the formation of specialized care for children with congenital cleft of the maxillofacial region, complicated by anomalies of other organs and systems

   Topicality. The specialized literature does not sufficiently present the systematization of concomitant malformations additional to congenital cleft of the maxillofacial region, the frequency of their occurrence and the species correlativity of relationships. There is no program for concretizing the interaction of specialists in different fields that ensure the unity of the processes of diagnosis and rehabilitation of patients with complex disorders of embryogenesis.

   Purpose. To present the main directions of specialized care for children with congenital cleft of the maxillofacial region in combination with malformations of other organs and systems.

   Materials and methods. A group of patients (823 people) who received treatment at the Center «Bonum» (Yekaterinburg) for a period of 25 years (1998–2022) with cleft maxillofacial region and concomitant malformations was studied. In the course of the study, the data of malformations were systematized, and correlations between congenital concomitant defects were identified. Clinical and special methods of patient examination were used in the study.

   Results. An innovative methodology for systematic monitoring and treatment of patients with maxillofacial cleft in combination with concomitant malformations has been developed based on a staged algorithm of therapeutic and corrective interactions of rehabilitation specialists of various profiles. The optimal tactics of the interacting advantages of the treatment process of concomitant congenital malformations are presented, taking into account the sequence of rehabilitation measures, the volume and duration of surgical elimination of the cleft against the background of concomitant
anomalies.

   Conclusions. The proposed methodological algorithm for the dynamic observation of 823 patients with cleft of the maxillofacial region, complicated by malformations of other organs and systems, has absorbed the fundamental directions of therapeutic and corrective interactions of specialists in various fields, concentrating knowledge and experience on this issue for a period of 25 years (1998–2022) and made it possible to minimize postoperative complications (the indicator of residual deformities of the upper lip and nose after the primary cheilorhinoplasty amounted to 16,16 %, and after velourinoplasty – 2,46 %), to successfully complete (in 73,63 % of cases) the main stages of surgical treatment of the maxillofacial region, as well as the main processes of restoring the health of this group of patients in accordance with the age periods of rehabilitation.

1. Gaivoronskaya T.V., Badeeva E.A., Vasil’ev Yu.A., et al. An interdisciplinary approach to the comprehensive diagnosis and rehabilitation of children with congenital facial malformations and dentoalveolar anomalies. Clinical Practice in Pediatrics. 2021;16(2):80–85. (In Russ.) doi: 10.20953/1817-7646-2021-2-80-85

2. Durnovo E.A., Glyavina I.A., Monakova N.E. Diagnostic and therapeutic aspects of rehabilitation of children with congenital malformations of the face in the Nizhny Novgorod region. Russian Dental Journal. 2013;5:46–48. (In Russ.) Available from: https://cyberleninka.ru/article/n/diagnosticheskie-i-lechebnye-aspekty-reabilitatsii-detey-s-vrozhdennymi-porokami-razvitiya-litsa-v-nizhegorodskoy-oblasti/viewer.

3. Egorova M.V., Ivanova E.S., Amkhadova M.A. Implementation of early comprehensive rehabilitation of children with cleft lip and palate using the example of the Moscow region. Stomatology. 2020;99(6 2):10–14. (In Russ.) doi: 10.17116/stomat20209906210.

4. Mamedov A.A., Volkov Yu.O., Korsunsky А.А., et al. Interdisciplinary care for newborns with cleft lip and palate in a children’s multidisciplinary hospital. Lechaschy Vrach. 2021;8(24):39-44. (In Russ.) doi: 10.51793/OS.2021.24.8.00

5. Mamedov A.A., Geppe N.A., Skakodub A.A., et al. Interdisciplinary approach to early rehabilitation of newborns with cleft lip and cleft palate. Ros Vestn Perinatol i Pediatr. 2022;67(1):71–75. (In Russ.) doi: 10.21508/1027–4065–2022–67–1–71–75.

6. Pulatova B.Zh., Abdullajonova Sh.Zh., Shirinov M.K., et al. Diagnostic value of the relationship between congenital cleft lip and palate in children with psychoneurological disorders. Academic Research in Educational Sciences. 2023;4(1):139–150. (In Russ.). Available from: https://cyberleninka.ru/article/n/diagnosticheskoe-znachenie-vzaimosvyazi-vrozhdennoy-rasscheliny-verhney-guby-i-nyoba-u-detey-s-psihonevrologicheskimi-narusheniyami.

7. Chuykin S.V., Akat’yeva G.G., Kuchuk K.N., et al. Concomitant diseases in children with congenital cleft lip and palate residing in a region with industrial pollution. Clinical Practice in Pediatrics. 2021;16(5):44–48. (In Russ.) doi: 10.20953/1817-7646-2021-5-44-48.

8. Bartzela T., Theuerkauf B., Reichardt E., et al. Clinical characterization of 266 patients and family members with cleft lip and/or palate with associated malformations and syndromes. Clin Oral Invest. 2021;25(9):5531–5540. doi: 10.1007/s00784-021-03863-2.

9. Fell M., Fitzsimons K.J., Hamilton M.J., et al. Cleft lip Sidedness and the Association with Additional Congenital Malformations. The Cleft Palate Craniofacial Journal. 2024;0(0). doi: 10.1177/10556656241261918.

10. Nagalo K., Ouédraogo I., Laberge J.M., et al. Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso. BMC Pediatr. 2017;17(1):72. doi: 10.1186/s12887-017-0833-9.

11. Stoll C., Alembik Y., Roth M.P. Co-occurring anomalies in congenital oral clefts. Am J Med Genet A. 2022;188(6):1700–1715. doi: 10.1002/ajmg.a.62689.

12. Rogozhina Yu.S., Blokhina S.I., Bimbas E.S., Galeeva K.V. Modern tactics of follow-up care and treatment of patients with congenital maxillofacial defects combined with other comorbid conditions. Pediatric dentistry and dental prophylaxis. 2023;23(2):92-103. (In Russ.) doi: 10.33925/1683-3031-2023-606

13. Chukanov A.N. Improving the efficiency of prenatal diagnosis of cleft lip and palate. Health and Ecology Issues. 2020;(3):36–42. (In Russ.) doi: 10.51523/2708-6011.2020-17-3-5.

14. Altunhan H., Annagür A., Konak M., et al. The incidence of congenital anomalies associated with cleft palate/cleft lip and palate in neonates in the Konya region, Turkey. Br J Oral Maxillofac Surg. 2012;50(6):541-544. doi: 10.1016/j.bjoms.2011.08.001.

15. Pereira A.V., Fradinho N., Carmo S., et al. Associated Malformations in Children with Orofacial Clefts in Portugal: A 31-Year Study. Plast Reconstr Surg Glob Open. 2018.9;6(2):e1635. doi: 10.1097/GOX.0000000000001635.

16. Sanchez M.L.N., Benjamin R.H., Mitchell L.E., et al. Birth Defect Co-Occurrence Patterns Among Infants With Cleft Lip and/or Palate. Cleft Palate Craniofac J. 2022;59(4):417–426. doi: 10.1177/10556656211010060

17. Rogozhina Yu.S., Blokhina S.I., Bimbas E.S. On the issue of surgical treatment of patients with congenital orofacial clefts combined with other organ and system comorbidities. Pediatric dentistry and dental prophylaxis. 2023;23(3):281-294. (In Russ.) doi: 10.33925/1683-3031-2023-658.

18. Saidova G.B., Prityko A.G., Suleymanov A.B., Molodtsova N.A. Surgical treatment of congenital cleft lip and palate in children with multiple malformations. Quantum Satis. 2021;4(1-4):130–132. (In Russ.) Available from: https://elibrary.ru/item.asp?id=47387013.

19. Gritsyuk E.M., Dugina E.A., Goldstein S. L., Blokhina S. I. About the resource and productive potential of a medical institution. Health and Education Millennium. 2017;19(4):69-73.(In Russ.) Available from: https://www.elibrary.ru/item.asp?id=28278715.

20. Rogozhina Yu.S., Blokhina S.I., Bimbas E.S. Surgical tactics for eliminating complex variants of congenital cleft palate. Problems in dentistry.2020;16(1):121–126. (In Russ.) doi: 10.18481/2077-7566-20-16-1-121-126.

21. Lin J., Fang X., Ha P., Fu M., Wang H. Oral Health-Related Quality of Life in Chinese Children With Orofacial Cleft. Cleft Palate Craniofac J. 2020;57(8):931–937. doi: 10.1177/1055665620908426.