Tanner-Whitehouse bone age reference values for North American children.

On the basis of 1090 x-ray films from 225 boys and 225 girls ages 8 to 16 years-participants in the Project Heartbeat longitudinal study who were living in a generally above-average income environment near Houston, Tex.-we provide Tanner-Whitehouse Mark 2 RUS (radius, ulna, and selected metacarpals and phalanges) bone age reference values for North American children of European origin. We designate these values as US90 (for the 1990s) reference values, in contrast to the original British bone age standards, called UK60 (for the 1960s). The US90 children matured considerably earlier than those on which the UK60 standards were based, though only about 3 months earlier than contemporary Spanish children. A study of 190 x-ray films from a research longitudinal series of 23 healthy boys in Virginia yielded values very close to the Houston values, confirming that our US90 reference values should be used in North America, pending a more extensive survey.

Diastolic runoff with widened arterial pulse pressure in an adolescent with widely metastatic testicular choriocarcinoma.

Widened arterial pulse pressure is often found with conditions having exaggerated diastolic runoff flow, such as aortic insufficiency and arteriovenous malformation. We report a 17-year-old boy with metastatic testicular choriocarcinoma and a widened pulse pressure. Ultrasonography and Doppler analysis revealed significant runoff flow into multiple large hepatic metastases. After chemotherapy, there was diminution in his metastases, a narrowing of the pulse pressure, and no evidence of runoff flow in the liver. These cardiovascular findings have not been previously described in association with metastatic vascular malignancies.

Reliability and validity of computer-assisted estimates of Tanner-Whitehouse skeletal maturity (CASAS): comparison with the manual method.

Three observers rated 57 X-rays from normal healthy children in Project HeartBeat! twice each by CASAS, the computer-assisted version of the TW2 RUS bone age method. Differences between duplicates of individual bone ratings which reached or exceeded 1.0 unit (or 1 stage) were 5% within observer and 8% between observers for CASAS, and 17 and 33%, respectively, for the unassisted MANUAL method. In children followed longitudinally, CASAS scores increased much more steadily than MANUAL scores, largely because the bones were rated, in the former system, on a continuous rather than a discrete-integer scale. We conclude that CASAS is a more reliable and probably a more valid estimator of skeletal maturity than the MANUAL version of the TW2 RUS method.

Isolated bone relapse during hematologic remission in childhood acute lymphoblastic leukemia: report of a metatarsal relapse and review of the literature.

An 11-year-old boy with prior bone marrow and testicular relapses of his acute lymphoblastic leukemia (ALL) developed an isolated metatarsal bone relapse during complete hematologic remission 10 months after completion of chemotherapy. Although there was no radiographic or histologic evidence of additional occult leukemia, the polymerase chain reaction (PCR) technique detected a leukemic clone in both his bone marrow and metatarsal. A literature survey revealed only 10 reported cases of isolated bone relapse occurring during complete bone marrow remission in childhood ALL. Most of these patients had prior bone marrow or extramedullary relapses. The majority experienced subsequent relapses after their isolated bone recurrence. We report a case of isolated bone recurrence, review all previously reported cases, and suggest that PCR elucidation of clonal disease may provide a better understanding of these extremely rare extramedullary events.

Hemiparesis and ischemic changes of the white matter after intrathecal therapy for children with acute lymphocytic leukemia.

Three children with acute lymphocytic leukemia (ALL) developed delayed-onset transient hemiparesis and facial palsy after intrathecal (IT) administration of methotrexate (MTX) alone or as part of triple intrathecal chemotherapy for central nervous system (CNS) prophylaxis. The hemiparesis developed 10 to 14 days after IT therapy. Two of three children also experienced transient, profound expressive dysarthria. These episodes occurred during maintenance treatment after multiple IT administrations and without previous CNS toxicity. Two of three children received intermediate-dose MTX, 1 g/m2, not less than 5 weeks before events. These patients had not received cranial irradiation and had no evidence of CNS leukemia before or after these episodes. Ischemic changes on computerized tomographic scan or magnetic resonance imaging studies were documented in all three cases. Such changes are unusual manifestations of neurotoxicity in children after intrathecal therapy.

Tethered-cord syndrome after repair of meningomyelocele.

The occurrence of tethered-cord syndrome is one of the delayed consequences of the repair of meningo-myelocele. The existing neurological deficit worsens, or a new deficit is superimposed on the existing one. In addition, urological and orthopedic symptoms are also frequently encountered. Although radiological studies may be suggestive of tethering of the cord, not all children are symptomatic. Magnetic resonance imaging is the best radiologic study available. Ultrasonography, although economical and easy to perform, does not yield an optimal image. It appears that a careful periodic clinical evaluation is the best way to evaluate the patients for surgery.

Dominantly inherited craniodiaphyseal dysplasia: a new craniotubular dysplasia.

We describe a mother and her male infant affected with a craniotubular dysplasia characterized by severe craniofacial hyperostosis and sclerosis with obliteration of paranasal sinuses and foramina of the skull base. Subsequent severe bilateral hearing loss and facial diplegia with relative sparing of the optic nerves were noted. The long bones show extreme asymmetric hyperostosis and sclerosis of the diaphyses and evidence of a modelling defect in the metaphyses. The spine, ribs, clavicles, and pelvis all show some degree of sclerosis and defective modelling, but are less severely involved. According to the definition by Gorlin, this disorder would best be classified as craniodiaphyseal dysplasia. Distinguishing features in these two patients as contrasted to previously described cases include a greater degree of hyperostosis and sclerosis than that described for other patients with craniodiaphyseal dysplasia, apparent dominant transmission, and significant metaphyseal involvement.

Radiographic appearances following surgical correction of the partially fused epiphyseal plate.

Surgical correction of partial post-traumatic fusion of an epiphyseal plate by resection of the bony bridge across the plate and replacement of the bridge by a fat implant causes a characteristic radiographic appearance which is illustrated by two patients and an animal model. Failure to recognize this characteristic postoperative pattern may lead to a misdiagnosis of infection or other bone pathology.

Lobar collapse with respiratory syncytial virus pneumonitis.

In a study of 30 children with uncomplicated respiratory syncytial virus (RSV) pneumonias, a high incidence of lobar collapse (8/30-26%) was noted. This involved the right upper lobe in seven patients and the left upper lobe in one patient. It is probably attributable to anatomical predispositions, sloughing of necrotic epithelium, and stimulation of mucus production. Lobar collapse should be considered part of the spectrum of RSV pneumonitis.