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Vol. 13. Issue 6.
Pages 437-445 (January 2002)
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Vol. 13. Issue 6.
Pages 437-445 (January 2002)
Surgical treatment of trigonocephalies and associated hypoteleorbitism
Tratamiento quirúrgico de las trigonocefalias
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J. Hinojosa
, J. Esparza, M.J. Muñoz, R. Salvan, A. Romance, J.F. Alen
Servicio de Neurocirugía Pediátrica y Unidad de Cirugía Craneofacial. Hospital Universitario 12 de Octubre. Madrid
A. Muñoz*
* Servicio de Radiodiagnóstico Infantil. Hospital Universitario 12 de Octubre. Madrid
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Article information
Abstract

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 fronto-orbitary 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.

Key words:
Trigonocephaly
Metopic synostosis
Hypotelorism
Hypoteleorbitism
Craniofacial surgery
Resumen

El cierre prematuro de la sutura metópica es una forma relativamente infrecuente de craneosinostosis con una incidencia estimada en torno al 0,3 por 1000 nacidos vivos y que comprende aproximadamente el 7% de todas las craneosinostosis referidas a una unidad de cirugía craneofacial. El espectro fenotípico es muy amplio y varía desde pequeñas crestas metópicas hasta formas severas de trigonocefalia con indentación pterional, retrusión de la barra supraorbitaria, prominencias temporales y parietales compensadoras e hipotelorismo. En la mayoría de los casos surge espontáneamente aunque están descritos casos de herencia autosómica dominante y la asociación con anomalías cromosómicas y diferentes formas sindrómicas. El tratamiento consiste habitualmente en la corrección quirúrgica empleando diferentes formas de avance fronto-orbitario. Los resultados cosméticos y funcionales son muy favorables. Existen sin embargo aún algunas cuestiones por resolver en relación con esta patología, principalmente sobre su patogenia, historia natural y tratamiento. El abordaje quirúrgico dirigido del hipotelorismo es un tema de discusión permanente al considerar el restablecimiento de las distancias interorbitarias normales.

Hemos desarrollado un análisis retrospectivo de una serie quirúrgica de veintiocho (28) casos de trigonocefalia. En once (11) casos se realizó tratamiento dirigido del hipotelorismo, mientras que en los restantes diecisiete (17) casos éste permaneció “sin tratamiento”. El objetivo de este estudio es revisar la evolución desde el punto de vista estético y funcional comparando las distintas técnicas aplicadas al hueso frontal y observar la evolución del hipotelorismo tras el tratamiento con o sin osteotomías e injertos del área naso-etmoidal.

Palabras clave:
Trigonocefalia
Sinostosis metópica
Hipotelorismo
Hipoteleorbitismo
Cirugía craneofacial

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