Journal of Pediatric Neurology 2021; 19(01): 001-006
DOI: 10.1055/s-0040-1709510
Review Article

Reconstructive Surgery in Children with Down Syndrome: Bioethical Implications

Cristina Tornali
1   Department of Biomedical and Biotechnological Sciences, Section of Neurology, University of Catania, Catania, Italy
,
Marcello Migliore
2   Department of Surgical Sciences, Section of Thoracic Surgery, University of Catania, Catania, Italy
,
Agata Polizzi
3   Department of Educational Sciences, University of Catania, Catania, Italy
,
Nicola L. Bragazzi
4   Department of Health Sciences, University of Genoa, Genoa, Italy
,
Mariano Martini
5   Department of Health Sciences, Section of History of Medicine and Ethics, University of Genoa, Genoa, Italy
,
Martino Ruggieri
6   Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy
,
Andrea D. Praticò
6   Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy
,
Ignazio Vecchio
7   Department of Clinical and Experimental Medicine, Section of History of Medicine, University of Catania, Catania, Italy
› Author Affiliations

Abstract

Down Syndrome is a genetic disorder caused by a third copy of chromosome 21. One of the main feature of the syndrome is the facial dysmorphism, characterized by broad, flat face, oblique eyelid rhymes, skin folds at the inner corner of the eyes (epicanthus), receding chin, protrusion of the tongue. These features are often social and functional obstacles, reducible with surgical interventions. The first facial plastic/cosmetic surgery approaches on Down children were performed in 1960 in Germany, Great Britain and the United States, where plastic surgeons began to reshape the Down patients physiognomy through the surgical correction of the face, tongue, eyes, nose, of the nostrils, of the neck. Recent studies have shown that in spite of the tendency to infections and early pulmonary hypertension in children with DS, surgery is not contraindicated, as previously suggested, but can be performed with very good results. This treatment has aesthetic, functional, rehabilitative, social effects, as well as favorable outcome on the familiar behavior. However different issues emerge in cases of patients presenting intellectual disability and unable to understand the consequences or the side effects related to plastic surgery. In this aspect, different Countries have enacted distinct laws directly regulating the consensuses for such intervention, with the goal to reduce the risks for the affected patients and the respect of their willing and social inclusion.



Publication History

Received: 01 August 2019

Accepted: 03 August 2020

Article published online:
25 April 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Parsons C, Naqvi S, Wheeler R. Intra-thoracic appendicitis in a child with Down's syndrome. J Pediatr Surg 2013; 48 (06) E29-E31
  • 2 Saffirio C, Marino B, Formigari R. Better surgical prognosis for patients with Down syndrome. J Thorac Cardiovasc Surg 2008; 135 (01) 230
  • 3 Migliore M. Efficacy and safety of single-trocar technique for minimally invasive surgery of the chest in the treatment of noncomplex pleural disease. J Thorac Cardiovasc Surg 2003; 126 (05) 1618-1623
  • 4 Migliore M, Calvo D, Criscione A, Borrata F. Uniportal video assisted thoracic surgery: summary of experience, mini-review and perspectives. J Thorac Dis 2015; 7 (09) E378-E380
  • 5 Cunningham C, Turner S, Sloper P, Knussen C. Is the appearance of children with Down syndrome associated with their development and social functioning?. Dev Med Child Neurol 1991; 33 (04) 285-295
  • 6 Goeke J, Kassow D, May D, Kundert D. Parental opinions about facial plastic surgery for individuals with down syndrome. Ment Retard 2003; 41 (01) 29-34
  • 7 Michael AI, Jarrett OO. Parental views on plastic surgery for Down syndrome: an African perspective. Pan Afr Med J 2019; 32: 207
  • 8 Cielo CM, Montalva FM, Taylor JA. Craniofacial disorders associated with airway obstruction in the neonate. Semin Fetal Neonatal Med 2016; 21 (04) 254-262
  • 9 Ruggieri M, Praticò AD, Scuderi A, Sorge G, Polizzi A. The multiple faces of artwork diagnoses. Lancet Neurol 2017; 16 (06) 417-418
  • 10 Ruggieri M, Praticò AD, Caltabiano R, Polizzi A. Early history of the different forms of neurofibromatosis from ancient Egypt to the British Empire and beyond: first descriptions, medical curiosities, misconceptions, landmarks, and the persons behind the syndromes. Am J Med Genet A 2018; 176 (03) 515-550
  • 11 Ruggieri M, Praticò AD, Serra A. et al. Early history of neurofibromatosis type 2 and related forms: earliest descriptions of acoustic neuromas, medical curiosities, misconceptions, landmarks and the pioneers behind the eponyms. Childs Nerv Syst 2017; 33 (04) 549-560
  • 12 Suziedelis AK. Adding burden to burden: cosmetic surgery for children with down syndrome. Virtual Mentor 2006; 8 (08) 538-540
  • 13 Suziedelis AK. Cosmetic surgery for children with Down syndrome: the cruelest cut of all?. Health Care Ethics USA 2003; 11 (01) E1
  • 14 Roizen NJ. Complementary and alternative therapies for Down syndrome. Ment Retard Dev Disabil Res Rev 2005; 11 (02) 149-155
  • 15 Parsons CL, Iacono TA, Rozner L. Effect of tongue reduction on articulation in children with Down syndrome. Am J Ment Defic 1987; 91 (04) 328-332
  • 16 Pierce M, Ramsey K, Pinter J. Trends in obesity and overweight in Oregon children with Down syndrome. Glob Pediatr Health 2019; 6: X19835640
  • 17 Bagattoni S, D'Alessandro G, Gatto MR, Piana G. Applicability of Demirjian's method for age estimation in a sample of Italian children with Down syndrome: a case-control retrospective study. Forensic Sci Int 2019; 298: 336-340
  • 18 Milojevich HM, Slonecker EM, Lukowski AF. Participation in social skills therapy is associated with enhanced recall memory by children with Down syndrome: an exploratory study. Behav Modif 2019; (e-pub ahead of print). DOI: 10.1177/145445519841051.
  • 19 Barisnikov K, Straccia C. Social adaptive skills and psychopathology in adults with intellectual disabilities of non-specific origin and those with Down syndrome. Res Dev Disabil 2019; 87: 31-42
  • 20 Neocleous AC, Syngelaki A, Nicolaides KH, Schizas CN. Two-stage approach for risk estimation of fetal trisomy 21 and other aneuploidies using computational intelligence systems. Ultrasound Obstet Gynecol 2018; 51 (04) 503-508
  • 21 Saviolo-Negrin N, Cristante F. Teachers' attitudes towards plastic surgery in children with Down's syndrome. J Intellect Disabil Res 1992; 36 (Pt 2): 143-155
  • 22 Abbate B. [Plastic and esthetic surgery on children with Down's syndrome: ethical and technical aspects] (in Italian). Minerva Pediatr 2010; 62 (06) 585-590
  • 23 Del Rio A, Rinaldi R, Napoletano S, di Luca NM. Cosmetic surgery for children and adolescents. Deontological and bioethical remarks. Clin Ter 2017; 168 (06) e415-e420
  • 24 Jones RB. Parental consent to cosmetic facial surgery in Down's syndrome. J Med Ethics 2000; 26 (02) 101-102
  • 25 Beauchamp TL, Childress JF. eds. Principles of Biomedical Ethics. New York, NY: Oxford University Press; 1994
  • 26 Farkas LG, Katic MJ, Forrest CR. Age-related changes in anthropometric measurements in the craniofacial regions and in height in Down's syndrome. J Craniofac Surg 2002; 13 (05) 614-622
  • 27 Farkas LG, Katic MJ, Forrest CR. Surface anatomy of the face in Down's syndrome: anthropometric proportion indices in the craniofacial regions. J Craniofac Surg 2001; 12 (06) 519-524
  • 28 Glasper E, Powell C. Facial surgery and children with Down's syndrome. Br J Nurs 1999; 8 (01) 6