Nine infants with thanatophoric dysplasia (TD) and cloverleaf skull (CS) are reported. Twenty-two previously published CSTD cases are reviewed. These CSTD cases are compared to cases of TD without CS. It is concluded that there are two types of TD: type 1, with curved femora and very flat vertebral bodies; and type 2, with straight femora and taller vertebral bodies. Consistent but subtle histopathological characteristics differentiate the two types. Only a very few type 1 cases have CS, and the CS is mild. Almost all type 2 cases have severe CS.

Myocardial flow reserve is impaired in systemic RVs in survivors of the Mustard operation. This may contribute to systemic ventricular dysfunction in these patients.

We reviewed the radiographs of six patients with pseudohypoparathyroidism (PHP) manifested by variable renal and skeletal resistance to parathyroid hormone (PTH). Features compatible with hyperparathyroid bone disease, osteomalacia, and rickets were observed. Skeletal changes of hyperparathyroidism included (a) subperiosteal bone resorption, (b) radiolucent lesions, caused by either brown tumors or bone cysts, (c) slipped capital femoral epiphyses, (d) focal areas of osteosclerosis, (e) periosteal neostosis, and (f) osteopenia with reduced cortical and trabecular bone volume. Histologic bone features were compatible with osteitis fibrosa in all patients, which suggests that the skeleton responded to the bone-remodeling effects of PTH despite hormonal resistance at other target sites. Skeletal changes of rickets included metaphyseal irregularities, cupping, and widening of the growth plates. Osteomalacia was indicated by Looser zones in one patient and confirmed by histologic evidence in three patients.

We reviewed the records of 12 patients ages 9 to 16 years with knee osteochondritis dissecans. All patients had clinical histories and examinations, four radiographic views of the knee, and technetium-99m diphosphonate quantitative bone scans. Scan results (symmetric, increased, or decreased activity), clinical course, healing time, and final outcome were correlated to determine the prognostic value of the scan. We divided the patients into those with open physes (distal femoral and proximal tibial) and those with closed physes. Four of the six patients with open physes had increased activity on the bone scan. All four of these knees healed with nonsurgical treatment. The other two patients had decreased activity on bone scan, and both required surgical treatment after nonsurgical treatment failed. Of the six patients with closed physes, all had increased activity on the bone scan, but only two patients had healing of the osteochondral lesion without surgery. Quantitative bone scanning had a 100% predictive value for the prognosis in osteochondritis dissecans patients with open physes, but for those with closed physes the predictive value was less. Because the natural history in the adolescent group is less predictable, it is in this group that the quantitative scan would be most helpful. In this small group of patients, quantitative bone scanning had limited prognostic value.

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