[Kleeblattschädel syndrome--a case report].

The Kleeblattschädel syndrome is a type of craniofacial dysostosis having a trilobar deformity of the skull associated with congenital hydrocephalus. We present here a case of the anomaly which we operated on a patient for cranial correction and orbital decompression. The case is a 2-month-old female who was born after 40 week-gestation by spontaneous vaginal delivery with cephalic presentation and observed no abnormality in her vital signs. She was transferred to a neonatal intensive care unit because of her trilobar deformity of the head and severe proptosis. Plain X-ray films of the skull indicated a number of digital markings at the calvarium. The triobar configuration was formed by symmetrical outward protrusion of the bilateral temporal squama and bulging the vertex which made a high valt. The coronal and lambdoid sutures which were prematurely closed together could not be distinguished individually. Both sides of temporal squama were found to be quite thin. She had severe shallow orbit due to the maxillary hypoplasia. A ventriculomegaly was seen in CT scan. Cranial corrections were took place in two stages. A V-P shunt was also done. The first surgery was conducted at three months old for advancing the bifrontal part and decompression of the orbits. Bilateral frontal bone was removed by craniectomy cutting along with both sides of the prematurely closed coronal suture in the back and to the supraorbital ridge in the front after making bicoronal scalp incision on supine position.(ABSTRACT TRUNCATED AT 250 WORDS)