Surgical treatment of trigonocephalies and associated hypoteleorbitism

Premature closure of metopic suture is a relatively uncommon form of craniosyostosis with an estimated incidence of 0,3 per 1000 live births, comprising about 7% of surgical craniosynostosis referred to craniofacial centers. A broad phenotypical spectrum spreads from minor metopic ridges to severe trigonocephaly with pterional indentation supraorbital bar retrusion, temporal and parietal compensating bossings and hypotelorism. Most of the cases arise spontaneously although autosomal dominant inheritance has been described and association with cromosomal abnormalities and different syndromes has been widely reported. Surgical correction has been attempted with good cosmetic results using several variations of the standard frontoorbitary advance. However there is still a number of questions to be solved in relation to this entity, mainly on its pathogenesis, but also on its development, natural history and treatment. Direct surgical approach to associated hypotelorism is a matter of argument when considering the reestablishment of normal interorbitary distances. We have conducted a retrospective analysis of our serie consisting of twenty-eight cases of trigonocephalies. Surgical correction of hypotelorism was attempted in eleven cases while the resting seven children remained "not treated". The objective was to review the functional outcome and cosmetic results comparing the different techniques applied to the frontal bone and to observe evolution of the hypoteleorbitism after the treatment with or without osteotomies and grafting of the nasoethmoidal area.