Airway Management in Pediatric MPS: A Case Report
Abstract
Mucopolysaccharidoses (MPS) refer to a group of disorders classified as congenital metabolic defects, characterized by airway management challenges because of facial deformities, macroglossia, short neck, hypertrophic tonsils and adenoids, kyphoscoliosis, an immobile jaw, narrowed nasal passages, and atlantoaxial instability. In this report, we present a case of successful management of a difficult airway in a pediatric patient with MPS.
[1] Muenzer J. Overview of the mucopolysaccharidoses. Rheumatology. 2011;50(suppl_5):v4-v12.
[2] Al-Maawali AA, Joshi SN, Koul RL, Al-Maawali AA, Al-Sedari HS, Al-Amri BM, Al-Futaisi AM. Spectrum of paediatric lysosomal storage disorders in oman. Sultan Qaboos Univ Med J. 2012;12(3):295-9.
[3] Uribe A, Giugliani R. Selective screening for lysosomal storage diseases with dried blood spots collected on filter paper in 4,700 high-risk colombian subjects. JIMD Rep. 2013;11:107-16.
[4] Suarez-Guerrero JL, Gómez Higuera PJ, Arias Flórez JS, Contreras-García GA. Mucopolisacaridosis: características clínicas, diagnóstico y de manejo [Mucopolysaccharidosis: clinical features, diagnosis and management]. Rev Chil Pediatr. 2016;87(4):295-304.
[5] González-Meneses A, Barcia A, Diáz J. Protocolo de actuación en las mucopolisacaridosis. Protoc diagn ter pediatr. 2010;1:24-36.
[6] de Ru MH, Boelens JJ, Das AM, Jones SA, van der Lee JH, Mahlaoui N, et al. Enzyme replacement therapy and/or hematopoietic stem cell transplantation at diagnosis in patients with mucopolysaccharidosis type I: results of a European consensus procedure. Orphanet J Rare Dis. 2011;6(1):55.
[7] Giugliani R, Federhen A, Muñoz Rojas MV, Vieira T, Artigalás O, Lapagesse Pinto L, et al. Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment. Genet Mol Biol. 2010;33:589-604.
[8] Clark BM, Sprung J, Weingarten TN, Warner ME. Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management. Bosn J Basic Med Sci. 2018;18(1):1.
[9] Arn P, Wraith JE, Underhill L. Characterization of surgical procedures in patients with mucopolysaccharidosis type I: findings from the MPS I Registry. J Pediatr. 2009;154(6):859-64. e3.
[10] Moretto A, Bosatra MG, Marchesini L, Tesoro S. Anesthesiological risks in mucopolysaccharidoses. Ital J Pediatr. 2018;44(Suppl 2):116.
[11] Gönüldaş B, Yılmaz T, Sivri HS, Güçer KŞ, Kılınç K, Genç GA, et al. Mucopolysaccharidosis: Otolaryngologic findings, obstructive sleep apnea and accumulation of glucosaminoglycans in lymphatic tissue of the upper airway. Int J Pediatr Otorhinolaryngol. 2014;78(6):944-9.
[12] Ballıkaya E, Eymirli PS, Yıldız Y, Avcu N, Sivri HS, Uzamış-Tekçiçek M. Oral health status in patients with mucopolysaccharidoses. Turk J Pediatr. 2018;60(4):400-6.
[13] James A, Hendriksz CJ, Addison O. The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder. JIMD Rep. 2012;2:51-8.
[14] Walker R, Belani KG, Braunlin EA, Bruce IA, Hack H, Harmatz PR, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36(2):211-9.
[15] Arn P, Whitley C, Wraith JE, Webb HW, Underhill L, Rangachari L, et al. High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry. J Pediatr Surg. 2012;47(3):477-84.
[16] Walker R, Darowski M, Morris P, Wraith J. Anaesthesia and mucopolysaccharidoses: a review of airway problems in children. Anaesthesia. 1994;49(12):1078-84.
[17] Frawley G, Fuenzalida D, Donath S, Yaplito‐Lee J, Peters H. A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses. Paediatr Anaesth. 2012;22(8):737-44.
[18] Kurdi MS, Deshpande S. Mucopolysaccharidoses-An adventurous anaesthetic encounter. Indian J Anaesth. 2008;52(4):453-8.
[19] Madoff LU, Kordun A, Cravero JP. Airway management in patients with mucopolysaccharidoses: The progression toward difficult intubation. Paediatr Anaesth. 2019;29(6):620-7.
[20] Scaravilli V, Zanella A, Ciceri V, Bosatra M, Flandoli C, La Bruna A, et al. Safety of anesthesia for children with mucopolysaccharidoses: A retrospective analysis of 54 patients. Paediatr Anaesth. 2018;28(5):436-42.
[21] Tümer M, Yılbaş AA, Debbağ S, Sarıcaoğlu F, Canbay Ö. Airway management in mucopolysaccharidosis: a retrospective case series review. Braz J Anesthesiol. (English Edition). 2023;73(5):680-2.
[22] Walker R, Colovic V, Robinson D, Dearlove O. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth. 2003;13(5):441-7.
[2] Al-Maawali AA, Joshi SN, Koul RL, Al-Maawali AA, Al-Sedari HS, Al-Amri BM, Al-Futaisi AM. Spectrum of paediatric lysosomal storage disorders in oman. Sultan Qaboos Univ Med J. 2012;12(3):295-9.
[3] Uribe A, Giugliani R. Selective screening for lysosomal storage diseases with dried blood spots collected on filter paper in 4,700 high-risk colombian subjects. JIMD Rep. 2013;11:107-16.
[4] Suarez-Guerrero JL, Gómez Higuera PJ, Arias Flórez JS, Contreras-García GA. Mucopolisacaridosis: características clínicas, diagnóstico y de manejo [Mucopolysaccharidosis: clinical features, diagnosis and management]. Rev Chil Pediatr. 2016;87(4):295-304.
[5] González-Meneses A, Barcia A, Diáz J. Protocolo de actuación en las mucopolisacaridosis. Protoc diagn ter pediatr. 2010;1:24-36.
[6] de Ru MH, Boelens JJ, Das AM, Jones SA, van der Lee JH, Mahlaoui N, et al. Enzyme replacement therapy and/or hematopoietic stem cell transplantation at diagnosis in patients with mucopolysaccharidosis type I: results of a European consensus procedure. Orphanet J Rare Dis. 2011;6(1):55.
[7] Giugliani R, Federhen A, Muñoz Rojas MV, Vieira T, Artigalás O, Lapagesse Pinto L, et al. Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment. Genet Mol Biol. 2010;33:589-604.
[8] Clark BM, Sprung J, Weingarten TN, Warner ME. Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management. Bosn J Basic Med Sci. 2018;18(1):1.
[9] Arn P, Wraith JE, Underhill L. Characterization of surgical procedures in patients with mucopolysaccharidosis type I: findings from the MPS I Registry. J Pediatr. 2009;154(6):859-64. e3.
[10] Moretto A, Bosatra MG, Marchesini L, Tesoro S. Anesthesiological risks in mucopolysaccharidoses. Ital J Pediatr. 2018;44(Suppl 2):116.
[11] Gönüldaş B, Yılmaz T, Sivri HS, Güçer KŞ, Kılınç K, Genç GA, et al. Mucopolysaccharidosis: Otolaryngologic findings, obstructive sleep apnea and accumulation of glucosaminoglycans in lymphatic tissue of the upper airway. Int J Pediatr Otorhinolaryngol. 2014;78(6):944-9.
[12] Ballıkaya E, Eymirli PS, Yıldız Y, Avcu N, Sivri HS, Uzamış-Tekçiçek M. Oral health status in patients with mucopolysaccharidoses. Turk J Pediatr. 2018;60(4):400-6.
[13] James A, Hendriksz CJ, Addison O. The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder. JIMD Rep. 2012;2:51-8.
[14] Walker R, Belani KG, Braunlin EA, Bruce IA, Hack H, Harmatz PR, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36(2):211-9.
[15] Arn P, Whitley C, Wraith JE, Webb HW, Underhill L, Rangachari L, et al. High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry. J Pediatr Surg. 2012;47(3):477-84.
[16] Walker R, Darowski M, Morris P, Wraith J. Anaesthesia and mucopolysaccharidoses: a review of airway problems in children. Anaesthesia. 1994;49(12):1078-84.
[17] Frawley G, Fuenzalida D, Donath S, Yaplito‐Lee J, Peters H. A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses. Paediatr Anaesth. 2012;22(8):737-44.
[18] Kurdi MS, Deshpande S. Mucopolysaccharidoses-An adventurous anaesthetic encounter. Indian J Anaesth. 2008;52(4):453-8.
[19] Madoff LU, Kordun A, Cravero JP. Airway management in patients with mucopolysaccharidoses: The progression toward difficult intubation. Paediatr Anaesth. 2019;29(6):620-7.
[20] Scaravilli V, Zanella A, Ciceri V, Bosatra M, Flandoli C, La Bruna A, et al. Safety of anesthesia for children with mucopolysaccharidoses: A retrospective analysis of 54 patients. Paediatr Anaesth. 2018;28(5):436-42.
[21] Tümer M, Yılbaş AA, Debbağ S, Sarıcaoğlu F, Canbay Ö. Airway management in mucopolysaccharidosis: a retrospective case series review. Braz J Anesthesiol. (English Edition). 2023;73(5):680-2.
[22] Walker R, Colovic V, Robinson D, Dearlove O. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth. 2003;13(5):441-7.
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How to Cite
1.
Ahmadi H, Shahabi Shojaei F, Moradi E, Mahdavi N. Airway Management in Pediatric MPS: A Case Report. Arch Anesth & Crit Care. 2026;.
