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The authors describes a case of Menkes' disease, sex-linked recessive hereditary disease characterized by early progressive psychomotor deterioration, failure to gain weight, seizures, hypothermie and characteristic Kinky hair (Pilitorti). This boy is a blind child, unable to fix and follow light. Fundus examination shows optic nerve atrophy with tortuosity of the retinal blood vessels. The case is discussed in the light of similar and others reports in the literature. The disease is believed to be cause by a generalized copper deficiency in the body by defect in copper intestinal absorbtion. The pathologic changes in the retina are similar to those seen in the brain which shows diffuse neuronal degeneration lose of nerve filers and optic atrophy. Retinal changes should be reversible by short term systemic copper administration.

This case is reported because of the technical problems raised when excision of very large tumors of the liver is required. A patient was found to have a hemangioma of the right lobe of the liver. A right hepatectomy to include Spiegel's lobe was performed by a subcostal approach extended to a right thoracophrenotomy. After clamping the vascular pedicle of the right liver, the section of the liver was removed and a tumor weighing 3.420 g discovered. Postoperative recovery was uneventful. During right hepatectomies for very large tumors, excellent control of the inferior vena cava and right superior hepatic vein can be maintained by the use of thoracophrenotomy associated with laparotomy. Though the possibility of complexe vascular exclusion of the liver has to be considered during hepatic sectioning, this aves not appear to be essential, and the use of manual dissection after clamping the vascular pedicles of the right liver is a rapid and certain procedure.