Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)
Online ISSN : 1882-5133
Print ISSN : 1345-2843
ISSN-L : 1345-2843
Case Reports
A Case of Morquio Syndrome Treated with Laparoscopic High Anterior Resection of the Rectum and Cholecystectomy
Yoshihiro MIYAZAKIKenji YUZAWASatoshi YONEYAMAKoichi KOZAKIMasanori KOIZUMIHamaichi UEKI
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2015 Volume 76 Issue 5 Pages 1093-1098

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Abstract
Morquio syndrome is an autosomal recessive genetic disorder characterized by kyphosis or scoliosis, low stature, and other such skeletal lesions. This very rare pathology has a frequency of about 1 in 500,000 people in Japan. No reports have described laparoscopic surgery for a patient with this disease in Japan. We report a case in which laparoscopic high anterior resection of the rectum and cholecystectomy was performed for a patient with Morquio syndrome. A 71-year-old man with short-trunk dwarfism, short stature (127 cm), advanced scoliosis and thoracic deformation was preoperatively diagnosed with rectal cancer (RS, T3, N0, M0, cStage II) and cholecystolithiasis. His short stature and thoracic deformation meant that laparotomy would be difficult, so we decided that laparoscopic surgery would be preferable and performed laparoscopic high anterior resection and cholecystectomy. The surgery was performed safely and without complications, and the patient was discharged on postoperative day 12. However, in patients with this disease, vertebral and thoracic deformation can make intubation difficult and low lung capacity can cause intraoperative ventilation failure. Such surgery thus needs to be planned preoperatively in conjunction with the anesthesiology department.
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© 2015 Japan Surgical Association
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