Anesthetic Management of a Patient with Coffin–Siris Syndrome

Deepa Divakar; Pravin Virappa Ubale; Pinakin Gujjar

doi:10.5005/jp-journals-10049-0061

SEARCH WITHIN CONTENT

Online First

Anesthetic Management of a Patient with Coffin–Siris Syndrome

Deepa Divakar, Pravin Virappa Ubale, Pinakin Gujjar

Keywords : Coarse facial features, Coffin–Siris syndrome, Difficult airway

Citation Information : Divakar D, Ubale PV, Gujjar P. Anesthetic Management of a Patient with Coffin–Siris Syndrome. Res Inno in Anesth 2019; 4 (1):16-18.

DOI: 10.5005/jp-journals-10049-0061

License: CC BY-NC 4.0

Published Online: 01-06-2019

ARTICLE
REFERENCES

Abstract

Coffin–Siris syndrome (CSS) is a rare genetic condition with characteristic musculoskeletal abnormalities and growth restrictions, coarse facial features, feeding abnormalities, systemic malformation, and developmental and cognitive delay.¹^–3 Main anesthetic concerns should be difficult airway that tends to get worse with age, risk of aspiration, and periop pulmonary complications in uncooperative and mentally retarded patients. We report a case of 9-years-old male patient with CSS for dental extraction and restoration under general anesthesia.

HTML PDF Share

Fleck BJ, Pandya A, Vanner L, et al. Coffin-Siris syndrome: review and presentation of new cases from a questionnaire study. Am J Med Genet 2001;99(1):1–7. DOI: 10.1002/1096-8628(20010215)99:1<1::aid-ajmg1127>3.0.co;2-a.
Schrier SA, Bodurtha JN, Burton B, et al. The Coffin-Siris syndrome: a proposed diagnostic approach and assessment of 15 overlapping cases. Am J Med Genet A 2012;158A(8):1865–1876. DOI: 10.1002/ajmg.a.35415.
Bodurtha J, Kessel A, Berman W, et al. Distinctive gastrointestinal anomaly associated with Coffin-Siris syndrome. J Pediatr 1986;109(6):1015–1017. DOI: 10.1016/s0022-3476(86)80288-8.
Nemani L, Barik R, Patnaik AN, et al. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: a case report with review of literature. Ann Pediatr Cardiol 2014;7(3):221–226. DOI: 10.4103/0974-2069.140859.
Carey JC, Hall BD. The Coffin-Siris syndrome: five cases including two siblings. Am J Dis Child 1978;132:667–671. DOI: 10.1001/archpedi.1978.02120320027005.
Dongari-Baqtzoglou A, Research, Science and Therapy Committee, American Academy of Periodontology. Drug-associated gingival enlargement. J Periodontol 2004;75(10):1424–1431. DOI: 10.1902/jop.2004.75.10.1424.
Newman MG, Takei H, Klokkevold PR, et al. Carranza's Clinical Periodontology, 10th ed., St. Louis: Saunders, Elsevier; 2006. pp. 375–376.

PDF Share