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.

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.

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.

We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence.

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Measurement of atlanto-occipital translation by any of these methods is not reproducible. Although the technique by Wiesel and Rothman is the easiest to apply, confirmation of instability with magnetic resonance imaging should guide management.

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