Abordagem cirúrgica do hiperteleorbitismo na displasia craniofrontonasal

Abstract

RESUMO Objetivo: apresentar nossa experiência no tratamento cirúrgico do hiperteleorbitismo na displasia craniofrontonasal. Métodos: análise retrospectiva dos pacientes com displasia craniofrontonasal operados por orbital box osteotomy ou por bipartição facial entre os anos de 1997 e 2015. Informações sobre as intervenções cirúrgicas foram obtidas dos prontuários médicos, exames complementares, fotografias e entrevistas clínicas. Os resultados cirúrgicos foram classificados com base na necessidade de cirurgia adicional, e a recidiva orbital foi calculada. Resultados: sete pacientes do sexo feminino foram incluídas, três submetidas à orbital box osteotomy (42,86%) e quatro (57,14%) à bipartição facial. Houve uma recidiva orbital média de 3,71±3,73mm. A média global dos resultados cirúrgicos de acordo com a necessidade de novas cirurgias foi de 2,43±0,53. Conclusão: a abordagem cirúrgica do hiperteleorbitismo na displasia craniofrontonasal deve ser individualizada, respeitando, sempre que possível, a idade e as preferências dos pacientes, seus familiares e cirurgiões.

Hypertelorbitism Corrected by Facial Bipartition Improves Exotropia

“…Syndromic patients with hypertelorbitism and midface deficiency benefit from a simultaneous facial bipartition and midface (or monobloc) advancement. 4 This technique has been shown to be particularly effective in treating the Apert facies by changing the retruded, flat face to an advanced, more round face. 5,6…”

“…10 through 13), the facial bipartition procedure is used to decrease intraorbital distance and gain orbital symmetry in asymmetrical cases. 4 Each of these rare craniofacial cleft cases are unique and, thus, require adjustments in the procedure based on the patient’s deformity. 4 With craniofacial dysostosis syndromes, only select cases with hypertelorbitism benefit from a facial bipartition procedure.…”

“…4 Each of these rare craniofacial cleft cases are unique and, thus, require adjustments in the procedure based on the patient’s deformity. 4 With craniofacial dysostosis syndromes, only select cases with hypertelorbitism benefit from a facial bipartition procedure. 6 These syndromic patients typically suffer from midface hypoplasia manifested by exorbitism and malar/maxillary deficiency.…”

“…Since that time, the facial bipartition procedure has been shown to be a powerful operation in correction orbital dystopia for both rare craniofacial clefts and craniofacial dysostosis syndromes, such as Apert, Crouzon, and Pfeiffer syndromes. [4][5][6] For patients with midline craniofacial clefts (nos. 0 through 14) and paramedian clefts (nos.…”

“…Syndromic patients with hypertelorbitism and midface deficiency benefit from a simultaneous facial bipartition and midface (or monobloc) advancement. 4 This technique has been shown to be particularly effective in treating the Apert facies by changing the retruded, flat face to an advanced, more round face. 5,6 With both these patient groups, ocular problems related to exposure problems (e.g., conjunctivitis, ulcers, subluxation) or from increased intracranial pressure (optic atrophy) have been well-known.…”