Gorham's Disease of the Spine with Chylothorax: A Case Report / 대한정형외과학회잡지

We present the case of a 26-year-old girl with pleural effusion and destruction of adjacent ribs and vertebrae. An open biopsy revealed the typical histopathological changes of Gorham's disease. Pleural effusion was treated with drainage, pleurodesis and high dose radiation therapy. The disease process was arrested and she remains well with no sign of recurrence at an 18-month follow up. Now we are observing spinal deformity and considering early surgical stabilization.

Scoliosis Associated with Marfan Syndrome / 대한척추외과학회지

OBJECTIVES: To evaluate characteristics and results of treatment of scoliosis associated with Marfan syndrome METHODS: The clinical data of 57 patients diagnosed as Marfan syndrome from January 1989 to June 1999 were reviewed. Thirty one patients had major curves more than 10 degrees by Cobb's method on whole spine standing film and their curve patterns and treatment methods were analyzed. Twelve cases of Marfan scoliosis who underwent posterior instrumented fusion were compared with randomly-sampled cases with idiopathic scoliosis about flexibility, correctability and correction loss of curves. RESULTS: Scoliosis was identified in 31 of 57 patients (54.4%) and among them 15 patients had the curve more than 40 degrees. Twelve curves were double major, 10 were thoracic, and 8 were double thoracic. Seventy-five per cent of the 57 patients had congenital heart disease and 40% had lens dislocation. Twelve of 31 cases underwent posterior fusion with pedicle screw instrumentation. Marfan scoliosis (mean flexibility; 39% in thoracic, 52% in lumbar) were significantly less flexible than idiopathic scoliosis (60% in thoracic, 93% in lumbar) (P0.05). Mean correction loss in Marfan syndrome was 3.2% at the mean 4.1 year follow-up in 9 cases. CONCLUSIONS: The prevalence of scoliosis in Marfan syndrome shows positive correlation with severity of the disease. As compared to idiopathic scoliosis, the curve associated with Marfan syndrome was larger and less flexible. After posterior fusion using pedicle screw instrumentation, the correctability of Marfan curve was poorer than that of idiopathic curve, which is, however, insignificant statistically. So it was considered to be a good method to treat Marfan scoliosis.