Abstract
BackgroundAchondroplasia is an inherited disorder and the most common type of short-limbed dwarfism in human beings, affecting more than 250,000 individuals worldwide. To the best of our knowledge, no study has reported a correlation between achondroplasia and aortic dissection. Here, we report a rare case of acute type A aortic dissection repair in a patient with achondroplasia.Case presentationAn 82-year-old Japanese female with achondroplasia was admitted to our hospital because of acute-onset severe chest pain migration to her back accompanied by numbness and pain in the right lower limb. A computed tomography scan revealed acute type A aortic dissection with right leg ischemia because of an occlusion of the right common iliac artery. We successfully performed hemiarch repair.ConclusionsThis report presents the first case of a patient at such an advanced age with dwarfism and cardiac surgery and the second case to illustrate successful acute aortic dissection repair in achondroplasia. Of note, all procedures were performed without specialized equipment. Overall, this report adds to the experience of successful cardiac surgery in this unique patient population.
Highlights
Achondroplasia is an inherited disorder and the most common type of short-limbed dwarfism in human beings, affecting more than 250,000 individuals worldwide
This report presents the first case of a patient at such an advanced age with dwarfism and cardiac surgery and the second case to illustrate successful acute aortic dissection repair in achondroplasia
To the best of our knowledge, no correlation exists between achondroplasia and aortic dissection
Summary
Achondroplasia is a rare genetic bone growth disorder that results in disproportionate dwarfism and is characterized by good general health and average life expectancy. Only nine cases of cardiac surgeries and dwarfism have been reported [3,4,5,6,7,8,9,10,11]. Our case is the most advanced-age case of cardiac surgery among patients with achondroplasia and the second case to report dealing with an acute aortic dissection repair. Studies have reported some problems while providing general anesthesia to patients with achondroplasia requiring surgery. Four case reports have described the size of the cannula in adult patients with achondroplasia [6,7,8,9]. We reported the first case of a patient at such an advanced age with dwarfism and cardiac surgery and the second case of a successful acute aortic dissection repair in a patient with achondroplasia. We believe that this report adds to the experience of successful cardiac surgery in this unique patient population
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