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Anestesia en México
On-line version ISSN 2448-8771Print version ISSN 1405-0056
Abstract
ROSA MOYA, Griselda De la; AVILA-LOPEZ, Armando and ANDRADE FOUCHET, Rafael. Regional anesthesia guided by fluoroscopy in a patient with Morquio Syndrome: Case report. Anest. Méx. [online]. 2018, vol.30, n.1, pp.48-54. ISSN 2448-8771.
The mucopolysaccharidoses are rare genetic diseases. Morquio syndrome (MPS type IV) is an autosomal recessive inheritance disorder, with a prevalence of 200,000 - 250,000 live births. Characterized by normal intelectual development, several skeletal involvement included short stature, shortened trunk and neck, precuts carinatum, joint laxity, odontoid dysplasia, kyphoscoliosis, cervical cord compression, prognathism, valvular heart disease, macroglossia, restricted mouth opening, considering them difficult airway patients.
Case presentation:
31 year old female with Morquio syndrome and right supracondylar femur fracture. History of asthma and ortopedic surgery. Cardiological assessment with left ventricle ejection fraction 72%, tricuspid and aortic mild regurgitation. Physical exam: Short stature, BMI 49.4, characteristic facies, pectus carinatum and scoliosis. Spinal lumbar anesthesia with fluoroscopic guidance was performed on sitting position under standard technique, L1-L2 level, subarachnoid ropivacaine and fentanyl was administrated.
Discussion:
Morquio syndrome patients represent complex airway management, regional anesthesia techniques offers advantages in set of hemodynamic control and postoperative pain management with favorable recovery features without catastrophic complications. Regional anesthesia with fluoroscopic guidance is not a common technique but it’s an alternative for patients with complex anatomic abnormalities.
Keywords : Morquio syndrome; mucopolysaccharidosis; regional anaesthesia.
