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Journal of Otorhinolaryngology and Facial Plastic Surgery

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Vol. 5 No. 1 (2019)

September 2019

Anesthetic Management of a Patient with Achondroplasia Undergoing Adenotonsillectomy: A Case Report

  • Parisa Sezari
  • Farhad Safari
  • Masoud Nashibi
  • Kamran Mottaghi

Journal of Otorhinolaryngology and Facial Plastic Surgery, Vol. 5 No. 1 (2019), 22 September 2019 , Page 1-4
https://doi.org/10.22037/orlfps.v5i1.27208 Published: 2019-09-24

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Abstract

Background: There are several situations that can create a challenge to every anesthesiologist; one of them is genetic disorders such as achondroplastic dwarfism. Achondroplastic dwarfism is the most common form of skeletal dysplasia, affecting about one in 20,000 newborns. It is an autosomal disorder caused by a mutation of the fibroblastic growth factor receptor-3 gene.

Case Presentation: A 7‐year‐old boy diagnosed with achondroplasia was admitted for adenotonsillectomy. He had several classical symptoms and signs of upper airway and cardiac involvement. In this case report, we describe the anesthetic management of this patient, while reviewing the difficulties encountered by the anesthesia team perioperatively.

Conclusions: Difficulties with airway management and physiology of this disease can create significant challenges to the anesthesiologist. So, we should keep in mind that achondroplasia can be a complicated situation and were should be prepared to manage it.

Keywords:
  • Achondroplasia
  • Adenotonsillectomy
  • Continuous Positive Airway Pressure
  • CPAP
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How to Cite

Sezari, P., Safari, F., Nashibi, M., & Mottaghi, K. (2019). Anesthetic Management of a Patient with Achondroplasia Undergoing Adenotonsillectomy: A Case Report. Journal of Otorhinolaryngology and Facial Plastic Surgery, 5(1), 1–4. https://doi.org/10.22037/orlfps.v5i1.27208
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References

Igwegbe AO, Eleje GU, Ugwueke IF. De novo achondroplasia causing four consecutive unsuccessful pregnancies: a case report. J Med Case Rep. 2012;6:256.

Julliand S, Boule M, Baujat G, Ramirez A, Couloigner V, Beydon N, et al. Lung function, diagnosis, and treatment of sleep-disordered breathing in children with achondroplasia. Am J Med Genet A. 2012;158A(8):1987-93.

Yao G, Wang G, Wang D, Su G. Identification of a novel mutation of FGFR3 gene in a large Chinese pedigree with hypochondroplasia by next-generation sequencing: A case report and brief literature review. Medicine (Baltimore). 2019;98(4):e14157.

Furuichi Y, Nakazato K, Suzuki N, Hongo T, Sakamoto A. [Anesthetic Management for Cervicomedullary Decompression in a Patient with Achondroplasia--A Case Report]. Masui. 2015;64(12):1254-7.

Huecker M, Harris Z, Yazel E. Occult Spinal Cord Injury after Blunt Force Trauma in a Patient with Achondroplasia: A Case Report and Review of Trauma Management Strategy. J Emerg Med. 2017;53(4):558-62.

Guilleminault C, Sullivan SS, Huang YS. Sleep-Disordered Breathing, Orofacial Growth, and Prevention of Obstructive Sleep Apnea. Sleep Med Clin. 2019;14(1):13-20.

Mofid M. Obstructive sleep apnea: the sleeping giant of the childhood obesity epidemic. JAAPA. 2014;27(10):27-30.

Waters KA, Everett F, Sillence DO, Fagan ER, Sullivan CE. Treatment of obstructive sleep apnea in achondroplasia: evaluation of sleep, breathing, and somatosensory-evoked potentials. Am J Med Genet. 1995;59(4):460-6.

Fremion AS, Garg BP, Kalsbeck J. Apnea as the sole manifestation of cord compression in achondroplasia. J Pediatr. 1984;104(3):398-401.

Pauli RM, Horton VK, Glinski LP, Reiser CA. Prospective assessment of risks for cervicomedullary-junction compression in infants with achondroplasia. Am J Hum Genet. 1995;56(3):732-44.

Berkowitz ID, Raja SN, Bender KS, Kopits SE. Dwarfs: pathophysiology and anesthetic implications. Anesthesiology. 1990;73(4):739-59.

Thomas IT, Frias JL, Williams JL, Friedman WA. Magnetic resonance imaging in the assessment of medullary compression in achondroplasia. Am J Dis Child. 1988;142(9):989-92.

Mayhew JF, Katz J, Miner M, Leiman BC, Hall ID. Anaesthesia for the achondroplastic dwarf. Can Anaesth Soc J. 1986;33(2):216-21.

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The journal of "Otorhinolaryngology and Facial Plastic Surgery" is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

Print ISSN: 2538-5224; Online ISSN: 2538-5216

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