PFEIFFER SYNDROME - A CLINICAL REVIEW

The combination of bicoronal craniosynostosis, broad thumbs and great toes, and partial variable soft tissue syndactyly of the hands and fee t (i.e., Pfeiffer syndrome) classically followed a benign clinical cou rse. A review of the clinical features of those Pfeiffer syndrome pati ents presenting to our unit confirm another subgroup in whom the crani ofacial and associated manifestations are more extreme, with a signifi cant risk of early demise. The early aggressive surgical management of craniostenosis, hydrocephalus, exorbitism, faciostenosis, and upper a irway obstruction has provided the potential for prolonged useful surv ival in these cases.