Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2016.07.015
Brazilian Journal of Anesthesiology
Clinical Information

Anesthetic management of two patients with alkaptonuric ochronosis for total knee arthroplasty

Manejo anestésico de dois pacientes com ocronose alcaptonúrica para artroplastia total do joelho

Betul Kozanhan

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Abstract

Abstract The current case report describes two cases of alkaptonuric ochronosis for anesthetic management. Alkaptonuria is a rare genetic orphan disease of tyrosine metabolism characterized by an accumulation of homogentisic acid in cartilage and connective tissues. Patients present most commonly for orthopedic joint surgery due to progressive arthropathy that can be misdiagnosed many a times. However respiratory, airway, cardiovascular and genitourinary systems complications can occur with age progressing. Restricted range of motion of cervical spine may lead to difficulty with airway management. In addition, degenerative changes and stiffness of lumbar spine due to ochronosis would make neuraxial blockade challenging. Although this inherited condition is extremely rare, anesthesiologists should be aware of its existence and prepare for management of potential challenging problems. This report highlights special care and precautions that need to be taken during anesthetic management.

Keywords

Alkaptonuria, Ochronosis, Anesthesia

Resumo

Resumo Este relato descreve o manejo anestésico em dois casos de ocronose alcaptonúrica. Alcaptonúria é uma doença genética rara do metabolismo de tirosina caracterizada por acúmulo de ácido homogentísico em cartilagem e tecidos conjuntivos. Os pacientes geralmente recorrem à cirurgia ortopédica devido à artropatia progressiva, que, muitas vezes, pode ser diagnosticada incorretamente. No entanto, complicações das vias respiratórias, cardiovasculares e geniturinárias podem ocorrer com o avanço da idade. A restrição de mobilidade da coluna cervical pode levar ao manejo difícil das vias aéreas. Além disso, as alterações degenerativas e a rigidez da coluna lombar devido à ocronose podem tornar o bloqueio neuroaxial um desafio. Embora essa condição hereditária seja extremamente rara, os anestesiologistas devem estar cientes de sua existência e se preparar para o manejo de potenciais problemas desafiadores. Este relato destaca os cuidados e as precauções especiais que devem ser tomadas durante o manejo anestésico.

Palavras-chave

Alcaptonúria, Ocronose, Anestesia

References

Phornphutkul C, Introne WJ, Perry MB. Natural history of alkaptonuria. N Engl J Med. 2002;347:2111-21.

Butany JW, Naseemuddin A, Moshkowitz Y. Ochronosis and aortic valve stenosis. J Card Surg. 2006;21:182-4.

Collins E, Hand R. Alkaptonuric ochronosis: a case report. AANA J. 2005;73:41.

Hannoush H, Introne WJ, Chen MY. Aortic stenosis and vascular calcifications in alkaptonuria. Mol Genet Metab. 2012;105:198-202.

Leslie D, Stacey M. Awake intubation. Continuing education in anesthesia. Crit Care Pain. 2015;15:64-7.

Chin KJ, Chan V. Ultrasonography as a preoperative assessment tool: predicting the feasibility of central neuraxial blockade. Anesth Anal. 2010;110:252-3.

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Links & Downloads

Braz J Anesthesiol

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