Anesthesia Management in an Acondroplasic Patient Developing Cardiac Tamponade Due to Myxedema

Abstract

Achondroplasia is the most common cause of dwarfism with short stature, short body and disproportionate development. This rare genetic disease, which is 0.5-1.5 in 10000 live births, is autosomal dominant inheritance and is common in women. Deficiency of endochondral bone formation and normal periostal bone formation disorder are characteristic; patients have bone deformities and systemic anomalies. 1 Atlantoaxial dislocation (AAD) may concurrently exist in achondroplastic patients either de novo, following surgery (foramen magnum decompression) or due to odontoid abnormalities (os odontoideum). Anesthetic management of achondroplastic patients with coexisting AAD offers a complex proposition for anesthesiologists in view of the anatomical and physiological constraints and the possible multisystem involvement. 2 In this case report, we aimed to present general anesthesia management in the context of the literature in an achondroplasic patient who was operated due to cardiac tamponade developed after pericardial effusion. CASE REPORT Pericardiocentesis was planned due to cardiac tamponade for a 24-year-old female patient, who is 92 cm tall and weighing 28 kg, diagnosed with achondroplasia in the ASA IIIE physiological class according to the American Society of Anesthesiologists (ASA). She was being treated at the endocrinology clinic due to myxedema and her T4: 0.9, TSH>100, with normal complete blood and renal functions. As she experienced respiratory distress, her endocrinologist demanded a cardiology consultation. Her echocardiography showed an ejection fraction of 60%, central arterial pressure 50 mmHg, tricuspid insufficiency, and a pericardial fluid surrounding the whole hearth. Her computed tomography revealed a malignant pericardial effusion entirely surrounding the heart, which reached 28-30 mm. She had distention due to abdominal ascites and her Glasgow Coma Score (GKS) was 12 (E:3M:5V:4). She had no limited neck extension, her mentohyoid and mentothy