There is a Need to Request Cervical Spine Routine Radiographs for Patients with Down's Syndrome Before Carrying out Otorhinolaryngologic Surgery?

Summary

Introduction: The Down's Syndrome (DS) is a genetic syndrome characterized by several changes and the Atlanto-axial Instability is critical for the otorhinolaryngologist.

Objective: Check the prevalence of atlanto-axial instability in patients with Down's Syndrome who undergo service follow-up in the Clinical Hospital of the UFPR [Federal University of Paraná] and review the need to carry out routine cervical radiography in the patients with prescription to otorhinolaryngologic surgery.

Method: Prospective study of patients with the syndrome who undergo CH/UFPR's follow up, through questionnaire and cervical X-ray.

Results: No case of IAA was found in the population studied. Discussion: we consider there's a high frequency of AAI in patients with DS, and for all patients who will take part in sports activities that involve motion of the region, or who are submitted to surgeries, an investigation with clinical and radiological exam is recommended. However, as the incidence has many variable findings we question the real validity of this research for all patients, even the asymptomatic ones.

Conclusion: In spite of a DS's peculiar change, there are no evidences of the need to research the AAI as a routine in asymptomatic patients and the symptomatology should guide the investigation. But more studies are required to evaluate the importance of the radiological exams in such cases.

Resumo

Introdução: A Síndrome de Down é uma síndrome genética caracterizada por varias alterações, sendo que a Instabilidade Atlanto-axial é de grande importância para o Otorrinolaringologista.

Objetivo: Verificar a prevalência de instabilidade atlanto-axial em pacientes portadores de Síndrome de Down que fazem acompanhamento ambulatorial no Hospital de Clínicas da UFPR e analisar se há necessidade de se realizar de rotina radiografia cervical nos pacientes com indicação de realizar cirurgia otorrinolaringológica.

Método: Estudo prospectivo com paciente portadores da síndrome que tem acompanhamento no HC/UFPR, através de questionário e raios-X cervical.

Resultados: Não foi encontrado nenhum caso de IAA na população estudada.

Discussão: Considera-se que há uma alta frequência de IAA em pacientes com Síndrome de Down, sendo recomendado para todos os pacientes que vão participar de atividades esportivas que envolvam movimentação da região ou que sejam submetidos a cirurgias uma investigação com exame clinico e radiológico. No entanto, devido à incidência ter achados muito variados questiona-se a real validade desta investigação para todos os pacientes, mesmo que assintomáticos.

Conclusão: Apesar de ser uma alteração peculiar da Síndrome de Down, não ha evidencias da necessidade de investigar a IAA rotineiramente em pacientes assintomáticos, sendo que a sintomatologia deve guiar a investigação. Porém, mais estudos são necessários para avaliar a importância dos exames radiológicos nesses casos.

Keywords

Down's syndrome - articular instability - atlanto-axial joint - radiography

Palavras-chave

síndrome de Down - instabilidade articular - articulação atlanto-axial - radiografia

• Bibliographical References

• 1 Nahas MV, Rosário AV, Nahas AB, Luza GR. Instabilidade atlanto-axial em crianças com Síndrome de Down na Grande Florianópolis: um estudo piloto. ACM Arq Catarin Méd 1991; 20 (4) 149-54
• 2 Barros Filho TE, Oliveira RP, Rodrigues NR, Galvão PE, Souza MP. Instabilidade atlanto-axial na Síndrome de Down. Relato de 10 casos tratados cirurgicamente. Rev Bras Ortop 1998; 33 (2) 91-4
• 3 Siegfried MP. Should Children With Sown Syndrome Be Screened for Atlantoaxial Instability?. Arch Pediatr Adolesc Med 1998; 152: 123-125
• 4 Ali FE, Al-Bustan AM, Al-Busairi WA, Al-Mulla FA, Esbaita EY. Cervical spine abnormalities associated with Down syndrome. Int Orthopaedics 2006; 30 (4) 284-289
• 5 Pueschel SM, Scola FH, Tupper TB, Pezzullo JC. Skeletal anomalies of the upper cervical spine in children with Down syndrome. J Pediatr Orthop 1990; 10 (5) 607-611
• 6 Hata T, Todd MM. Cervical Spine Considerations when Anesthetizing patients with Down Syndrome. Anesthesiology 2005; 102 (3) 680-685
• 7 Barros Filho TEP, Oliveira RP, Rodrigues NR, Galvão PE, Souza MP. Instabilidade Atlanto-Axial na Síndrome de Down. Relato de dez casos tratados cirurgicamente. Rev Bras Ortop 1998; 33 (2) 91-4
• 8 Singer SJ, Rubin IL, Strauss KJ. Atlantoaxial Distance in Patients, Standardization of Measurement. Radiology 1987; 164: 871-872
• 9 Molin ED, Molin ECD, Chueire A. Instabilidade de C1-C2 na coluna cervical na Síndrome de Down. Semina 1988; 9 (3) 156-8
• 10 Minatel E, Ortiz J, Campos RC, Rupp AC. Coluna Cervical na Síndrome de Down. Rev Bras Ortop 1991; 26 (5) 131-6
• 11 Cros T, Linares R, Castro A, Mansilla F. Estúdio radiológico de lãs alteraciones cervicales em el síndrome de Down. Nuevos hallzgos mediante tomografia computarizada y reconstrucciones tridimensionales. Rev Neurol 2000; 30 (12) 1101-7
• 12 Morton RE, Khan MA, Murray-Leslie C, Elliott S. Atlantoaxial instability in Down's syndrome: a five year follow up study. Arch Dis Child 1995; 72 (2) 115-8 ; discussion 118–9
• 13 Cremers MJ, Bol E, de Roos F, van Gijn J. Risk of sports activities in children with Down's sundrome and atlantoaxial instability. Lancet 1993; 342 (8870): 511-4
• 14 Taylor TK, Walter WL. Screening of children with Down syndrome for atlantoacial (C1-2) instability: another contentious health question. Med J Aust 1996; 165 (8) 448-50
• 15 Atlantoaxial instability in Down syndrome: subject review. American Academy of Pediatrics Committee on Sports Medicine and Fitness. Pediatrics 1995; 96: 151-4
• 16 Committee on Sports Medicine: Atlantoaxial instability in Down Syndrome. Pediatrics. 1984; 74 (1) 152-154
• 17 Harley EH, Collins MD. Neurologic sequelae secondary to atlantoaxial instability in Down syndrome. Arch Otolaryngol Head Neck Surg 1994; 120: 159-165
• 18 Brockmeyer D. Down Syndrome and Craniovertebral Instability. Pediatr Neurosurg 1999; 31: 71-77
• 19 Cohen WI. Current dilemmas in Down syndrome clinical care: celiac disease, thyroid disorders, and atlanto-axial instability. Am J Med Genet C Semin Med Genet Aug 15 2006; 142C (3) 141-8
• 20 Birrer RB. The Special Olympics athlete: evaluation and clearance for participation. Clin Pediatr (Phila) 2004; 43 (9) 777-82
• 21 Pizzutillo PD, Herman MJ. Cervical spine issues in Down syndrome. J Pediatr Orthop 2005; 25 (2) 253-9