気管切開術にて大出血を来した Klippel-Trenaunay 症候群の1例

概要

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Klippel-Trenaunay syndrome (KTS) is defined as a clinical triad of capillary malformations (port wine stain), soft tissue and bone hypertrophy, and varicose veins. The case in this report is a 39-year-old male who had suffered from severe obstructive sleep apnea due to extensive hemangiomas in the nasopharyngeal lesion. Hemangiomas also presented in other lesions of the neck, lateral abdomen and the limbs. A tracheostomy under local anesthesia was performed for maintain the upper air way. When an incision on the anterior wall of the trachea was done, unexpected bleeding from the intra-tracheal space occurred. This bleeding originated from the capillary malformations in the tracheal wall. Hypo-oxygenation due to hypoventilation continued for a few minutes until an intubation was performed. After the treatment, patient was moved to the ICU for general recovery. During his stay in the ICU, there were recurrent incidents of active, frequent bleeding from the paratracheal space with further complications due to disseminated intravascular coagulation (DIC). Strict management for recurrent bleeding was required for a month. About two months after surgery, he was discharged from the hospital with cessation of bleeding. In the event that bleeding occurs with KTS, treatment for local hemangioma may be difficult. It should be considered that unexpected and recurrent bleeding may occur in the management of KTS.
日本喉頭科学会の論文
2010-06-01