Abstract
A 30-year-old female patient presented in a comatose state with clinical and radiographic signs of aspiration pneumonia 16 hours following elective surgery. Subsequent linical assessment and investigations revealed the characteristic facies, proximal muscle weakness, lenticular opacities, pulmonary function defects, arterial desaturation and abnormal breathing during rapid eye movement (REM) sleep often associated with myotonia dystrophica. Although these characteristic features were evident on clinical examination postoperatively they were not noted in the preoperative assessment. The aspiration pneumonia and coma were unusual presenting features of this disease.
Unsuspected myotonia dystrophica should be considered in the differential diagnosis of unexplained respiratory depression, aspiration or comatose state following surgery. Recognition of the disorder during the preoperative assessment is the key to avoiding complications during the perioperative management of such patients.
Résumé
Une patiente âgée de 30 ans a présenté un état comateux avec des signes cliniques et radiologiques d’aspiration bronchique seize heures après la chirurgie élective. Une évaluation clinique subséquente ainsi que des investigations ont révélé un faciès caractéristique, une faiblesse des muscles proximaux, une opacification du cristallin, une altération des fonctions pulmonaires, une désaturation artérielle ainsi qu’une respiration anormale durant la période du“rapid eye movement” (REM) souvent associés à la myotonie dystrophique. Même si ces caractéristiques étaient évidentes à l’examen clinique postopératoire elles n’étaient pas notées à l’évaluation préopératoire. L’aspiration bronchique et le coma étaient des signes de présentation peu communs de cette maladie.
La dystrophie myotonique non suspectée doit être considérée dans le diagnostic différentiel d’une dépression respiratoire non expliquée, l’aspiration bronchique, ou un état comateux après la chirurgie. La détection du problème en période préopératoire demeure la clé afin de prévenir les complications durant la période périopératoire.
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Hannon, V.M., Cunningham, A.J., Hutchinson, M. et al. Aspiration pneumonia and coma — an unusual presentation of dystrophica myotonia. Can Anaesth Soc J 33, 803–806 (1986). https://doi.org/10.1007/BF03027135
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DOI: https://doi.org/10.1007/BF03027135