Renal involvement in patients with tuberous sclerosis complex begins in infancy, and angiomyolipoma is the most common lesion (75%). Angiomyolipomas are more likely to grow than remain stable, although the rate of growth varies. Simple renal cysts may appear or disappear with time but angiomyolipomas do not disappear. An initially normal renal ultrasound does not rule out future development of lesions. Periodic surveillance is indicated in children with tuberous sclerosis complex.
Renal involvement in patients with tuberous sclerosis complex begins in infancy, and angiomyolipoma is the most common lesion (75%). Angiomyolipomas are more likely to grow than remain stable, although the rate of growth varies. Simple renal cysts may appear or disappear with time but angiomyolipomas do not disappear. An initially normal renal ultrasound does not rule out future development of lesions. Periodic surveillance is indicated in children with tuberous sclerosis complex.
Portal vein stenosis complicating orthotopic liver transplantation in children is uncommon. The authors report their early experience with transhepatic portal vein angioplasty in the treatment of portal vein stenosis in this setting. The technique was used in two children (aged 2 and 6 years) who had undergone liver transplantation for biliary atresia 15 and 42 months earlier, respectively. Both patients presented with chronic anemia and intermittent gastrointestinal bleeding. The diagnosis of portal vein stenosis was confirmed at fine-needle splenoportography. Access to the portal venous system was gained by means of a transhepatic approach. Low-profile angioplasty balloon catheters were used to dilate anastomotic strictures of the portal vein in both children. At the end of the procedure, the tract within the liver was occluded with gelatin sponge pledgets. Both procedures were technically successful and caused no complications, and in both patients gastrointestinal bleeding ceased.
Glenoid dysplasia is an anomaly of the scapula characterized by underdevelopment of the bony glenoid and adjacent part of the scapular neck which may be seen as a primary isolated condition, in patients with multiple anomalies or ill-defined syndromes, in well-described syndromes, in mucopolysaccharidoses and related conditions, and in certain skeletal dysplasias. The general subject of glenoid dysplasia is reviewed with reference to descriptions in the literature and personal observations.
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