An Anesthetic Experience in a Patient with Menkes Disease: A case report / 대한마취과학회지

Menkes disease, so-called kinky hair disease or steely hair disease, is a rare X-linked recessive disorder of intracellular copper transport protein ATP7A defect, due to mutation of ATP7A gene, resulting in copper deficiency. It is characterized by seizure, retarded neurological development, kinky hair, skeletal abnormality, recurrent infection and subnormal body temperature. In addition, gastroesophageal reflux with the risk of aspiration is another important feature. This article is the first report of anesthetic management in a patient with Menkes disease who underwent gastrostomy and bladder diverticulectomy in Korea.

Anesthetic Management for Liver Segmentectomy and Thrombectomy of Inferior Vena Cava and Right Atrium without Cardiopulmonary Bypass : A case report

The tumor thrombus of hepatocellular carcinoma extending into the right atrium (RA) via the inferior vena cava (IVC) may cause fatal complications such as heart failure and pulmonary embolism. Surgery for removal of thrombus in the RA usually needs aid of cardiopulmonary bypass (CPB). We report a case of successful anesthetic management for simultaneous liver segmentectomy and IVC and RA thrombectomy without CPB.