Escobar syndrome, difficult airway management in pediatrics: A case report

Angelina Lippi Quiñones; Nathalie López Jaque; Verónica Maureira Moreno

doi:10.5554/22562087.e1057

Angelina Lippi Quiñones Anesthesia and Resuscitation Department, School of Medicine. Universidad de Valparaíso, Valparaíso, Chile. https://orcid.org/0000-0002-0634-7692
Nathalie López Jaque a. Anesthesia and Resuscitation Department, School of Medicine. Universidad de Valparaíso, Valparaíso, Chile. b. Anesthesiology Service, Carlos Van Buren Hospital. Valparaíso, Chile. https://orcid.org/0000-0002-4668-4335
Verónica Maureira Moreno a. Anesthesia and Resuscitation Department, School of Medicine. Universidad de Valparaíso, Valparaíso, Chile. b. Anesthesiology Service, Carlos Van Buren Hospital. Valparaíso, Chile. https://orcid.org/0000-0001-7539-6087

DOI: https://doi.org/10.5554/22562087.e1057

Keywords: Síndrome de Escobar, Vía aérea difícil, Pediatría, Anestesia, Síndrome de pterigium múltiple, Reporte de caso

Abstract References How to Cite Downloads

Abstract

Escobar syndrome is a rare, autosomal recessive disease of unknown incidence. It is characterized by multiple skeletal, genitourinary and orofacial abnormalities. The multiple malformations (mainly orofacial) and restricted mobility of these patients pose a challenge to the anesthesia team, especially as regards airway management. We describe the clinical case of a pediatric patient diagnosed with Escobar syndrome who underwent two consecutive anesthesia interventions, with evidence of progressive airway anomalies that characterize this syndrome. The case required adaptation, according to the clinical stage of the disease, of the current algorithms used to approach an anticipated difficult airway in pediatrics, and the incorporation of new devices, not described so far in patients with this pathology, as part of the planning and execution phases.

References

Arpaci AH, Bozkirli F, Konuk O. Anesthesic management for escobar syndrome: Case report. Case Reports Med. 2011:515719. doi: https://doi.org/10.1155/2011/515719.

Sethi P, Bhatia PK, Gupta N, Singh K. Multiple pterygium syndrome: Challenge for anesthesiologist. Saudi J Anaesthesia. 2016;10:350-2. doi: https://doi.org/10.4103/1658-354X.174901.

Mathew S, Chaudhuri S, Arun Kumar HD, Joseph TT. Airway management in Escobar syndrome: A formidable challenge. Indian J Anaesthesia. 2013;57:603-5. doi: https://doi.org/10.4103/0019-5049.123336.

Echeverry Marín PC, Engelhardt T. Algoritmo para el manejo de la vía aérea difícil en pediatría. Colombian Journal of Anesthesiology. 2014;42:325-34. doi: https://doi.org/10.1016/j.rca.2014.05.008.

Schmucker Agudelo E, Farré Pinilla M, Andreu Riobello E, Franco Castanys T, Villaverde Castillo I, Monclus Diaz E, et al. Actualización en el manejo de la vía aérea difícil en pediatría. Rev Esp Anest Reanim. 2021;69(8):472-86. doi: https://doi.org/10.1016/j.redar.2021.05.012.

Daga V, Mendonca C, Choksey F, Elton J, Radhakrishna S. Anaesthetic management of a patient with multiple pterygium syndrome for elective caesarean section. Int J Obstetric Anesthesia. 2017;31:96-100. doi: https://doi.org/10.1016/j.ijoa.2017.04.005.

Escobar V, Bixler D, Gleiser S, Weaver DD, Gibbs T. Multiple Pterygium syndrome. American J Diseases Children. 1978;132:609-11. doi: https://doi.org/10.1001/archpedi.1978.02120310073016.

Brooke J. Multiple pterygium syndrome, Escobar variant. Anasthesiologie Und Intensivmedizin. 2021;62. doi: https://doi.org/10.19224/ai2021.s183.

Weiss M, Engelhardt T. Proposal for the management of the unexpected difficult pediatric airway. Paediatric Anaesthesia. 2010;20(5):454-64. doi: https://doi.org/10.1111/j.1460-9592.2010.03284.x.

Kuzma PJ, Calkins MD, Kline MD, Karan SM, Matson MD. The anesthetic management of patients with multiple pterygium syndrome. Anesthesia Analgesia. 1996;83(2):430-2. doi: https://doi.org/10.1097/00000539-199608000-00041.

Stoll WD, Hebbar L, Marica LS. Anesthetic management of a pregnant patient with multiple pterygium syndrome (Escobar type). Int J Obstetric Anesthesia. 2012;21:197-99. doi: https://doi.org/10.1016/j.ijoa.2011.12.002.

Robinson LK, O’Brien NC, Puckett MC, Cox MA. Multiple pterygium syndrome: a case complicated by malignant hyperthermia. Clinical Genetics. 1987;32(1):5-9. doi: https://doi.org/10.1111/j.1399-0004.1987.tb03315.x.

Brooke J, Stoll D W, Kariminejad Ariana. Anaesthesia recommendations for patients suffering from Multiple pterygium syndrome, Escobar variant [internet]. [citado 2022 mar 13]. Disponible en: https://www.Orphananesthesia.Eu/En/Rare-Diseases/Published-Guidelines/Escobar-Syndrome/272-Escobar-Syndrome/FileHtml.

Mayhew JF, Mychaskiw G. Escobar syndrome: Is this child prone to malignant hyperthermia? Paediatric Anaesthesia 2009;19(1):69-70. doi: https://doi.org/10.1111/j.1460-9592.2008.02856.x.

Ghafffar W, Haq IU, Shahid A, Ismail S. Anaesthetic challenges in a paediatric patient with Escobar syndrome-difficult airway and postoperative pneumothorax. Turkish J Anesthesiology Reanimation. 2021;49:486-9. doi: https://doi:10.5152/TJAR.2021.955.

How to Cite

1.

Lippi Quiñones A, López Jaque N, Maureira Moreno V. Escobar syndrome, difficult airway management in pediatrics: A case report. Colomb. J. Anesthesiol. [Internet]. 2022 Dec. 15 [cited 2024 Apr. 26];51(1). Available from: https://www.revcolanest.com.co/index.php/rca/article/view/1057