No abstract

We reviewed the frequency of valproate-induced thrombocytopenia in children with epilepsy in our institution. Sixty-four (21%) of 306 children taking valproate developed thrombocytopenia. Thirty-two of these 64 patients had at least one platelet count lower than 100 x 10(3)/mm3. Eight patients developed signs of bleeding. Low platelet levels were typically noted in patients with serum valproate levels of over 140 micrograms/mL, and reduction of the medication dose usually resulted in a prompt increase in the number of platelets. Only one patient developed thrombocytopenia unrelated to high serum drug levels, and her platelet count did not improve until the drug was discontinued. Neither the age of the patient nor the use of additional antiepileptic medication correlated with the platelet count. However, duration of valproate use was related. These data suggest that, although valproate may cause thrombocytopenia via more than one mechanism, by far the most common factor is the presence of high valproate levels. Thus, the medication can be safely lowered in most patients with thrombocytopenia rather than discontinued altogether. Platelet counts should probably be monitored more carefully in patients known to have higher drug levels.

Volar ligament release and distal radial dome osteotomy for Madelung deformity provides lasting correction of the deformity. Long-term follow-up shows maintenance of original radiographic correction with good to excellent functional outcome. Patients with radiographic evidence of more severe disease preoperatively and the whole bone variety of Madelung deformity have poorer radiographic outcomes and trend toward poorer functional outcomes.

Although there may be specific risks associated with its use, botulinum toxin-A injection into the internal rotator muscles is a useful adjunct to the treatment of early posterior subluxation or dislocation of the shoulder in infants with neonatal brachial plexus palsy, and may help to avoid the need for open surgical procedures to restore or maintain shoulder reduction.

We propose that cases previously classified as upper-extremity phocomelia represent a spectrum of severe longitudinal dysplasia, as none of the sixty extremities that we studied demonstrated a true intercalary deficiency. These findings have both developmental and genetic implications.