MICROCEPHALIC OSTEODYSPLASTIC PRIMORDIAL DWARFISM TYPE-II - A CHILD WITH UNUSUAL SYMPTOMS AND CLINICAL COURSE

We report on a 13-month old boy with microcephalic osteodysplastic pri mordial dwarfism (MOPD), whose radiographic signs correspond with type II of this entity, Some of his clinical signs, such as the anomalies of the external genitalia and tile urinary tract, are common to this s ubgroup of MOPD, but he also shows unusual clinical signs including bi lateral knee dislocation and hypoplasia of the anterior corpus callosu m. His clinical course was unusual with several episodes of breathing difficulties and increased intracranial pressure secondary to craniosy nostosis at the age of 16 months. After fronto-orbital advancement for the treatment of brachycephaly, his psychomotor development improved remarkably. Conclusion MOPD type II may have a wider range of expressi on than previously delineated.