We devised a direct percutaneous transhepatic cholangial drainage under fluoroscopic control. The principle is as follows. After percutaneous transhepatic cholangiography, the three dimensional structure of intrahepatic bile ducts is projected to a two dimensional plane under fluoroscopy; the needle can then be introduced into the selected bile duct with accuracy. The technique can be used as a preoperative management of operations of patients with jaundice and also as a palliative management of advanced cancer without much complication.

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Clincopathological features of Fukuyama type congenital muscular dystrophy (FCMD), a combination of brain malformation and muscular dystrophy with facial muscle and CNS involvement and high prevalence in Japan, are reviewed. Evidence of progressive dystrophy, negative correlations between muscle enzyme levels and age and CT numbers of muscle and age, are presented. Skeletal muscle histopathology is reviewed. Febrile illness‐induced transient exacerbation of muscle weakness is reported. Characteristic brain malformations, e.g. micropolygyria, other dysgenesis, are reviewed. Their severity correlated with maximal mental and motor function. The etiology and significance of low density areas (LDA) in white matter on CT, possibly reflecting delayed or abnormal myelination, and ventricular dilatation are discussed. Spontaneous LDA improvement makes hydrocephaly unlikely. Ophthalmological differential diagnosis from Santavouri disease and Walker‐Warburg syndrome, characterized by visual disturbance/glaucoma and microphthalmia/anterior chamber defects, respectively, is discussed. A single defective gene, manifesting as a metabolic error, may produce CNS and ocular defects as well as muscle degeneration in FCMD.

Conversion of percutaneous transhepatic cholangiodrainage to an internal drainage is safe and effective in rerouting bile back to the intestinal tract in obstructive jaundice without major operative procedure. The method consists of insertion of a drainage tube with several side holes beyond the bile duct stricture. Usually, a single lumen tube is used. However, double lumen tube is preferred when the amount of bile discharge is excessive. This simple technique is found to be useful in the management of obstructive jaundice not only for preoperative decompression of biliary tree but also for the inoperable lesions.

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