Orbital Dysmorphology in Untreated Children with Crouzon and Apert Syndromes

Abstract

A shortened bony orbit, decreased orbital and orbital soft-tissue volumes, and an increased volume of the globe were found in both conditions. Despite normal volume of the overall orbital contents, the contents are altered, and the bony orbit is shorter and holds less volume.

Rectus muscle excyclorotation and V-pattern strabismus: a quantitative appraisal of clinical relevance in syndromic craniosynostosis

“…These features narrow the approach to the orbital apex, as demonstrated in figure 3, imaging from a patient with Apert syndrome and seesaw strabismus. In addition, nasal sloping of the orbital roof and elevation of the central roof, characterising patients with Apert syndrome,22 23 may secondarily narrow the nasal superior intraconal space, forcing lateral shift of the superior rectus demonstrated in our patients with seesaw pattern.…”

“…Crouzon and Pfeiffer syndromes are associated with a shorter orbital floor and retrusion of the inferior and lateral orbital walls not seen in patients with Apert syndome 22 23 24. In contrast, patients with Apert syndrome develop anterior protrusion of the lateral orbital wall (greater wing of the sphenoid) along with prolapse of the cribriform plate and lateral ballooning of the ethmoidal sinuses 22 23. These features narrow the approach to the orbital apex, as demonstrated in figure 3, imaging from a patient with Apert syndrome and seesaw strabismus.…”

“…Previously described anatomical differences in the orbits of patients with Apert, Crouzon and Pfeiffer syndromes are consistent with the orbital features we have described and support our demonstrated association of greater V-pattern severity in the Apert cohort. Crouzon and Pfeiffer syndromes are associated with a shorter orbital floor and retrusion of the inferior and lateral orbital walls not seen in patients with Apert syndome 22 23 24. In contrast, patients with Apert syndrome develop anterior protrusion of the lateral orbital wall (greater wing of the sphenoid) along with prolapse of the cribriform plate and lateral ballooning of the ethmoidal sinuses 22 23.…”

Avaliação por imagem tridimensional das características morfológicas e do crescimento do terço médio da face de pacientes com craniossinostose sindrômica submetidos ao avanço frontofacial em monobloco associado à distração osteogênica

“…A realização de tarsorrafia é mandatória em muitos pacientes além dos cuidados clínicos para preservação do globo ocular 19,21 . A sindactilia de mãos e pés observada na SA deve ser corrigida visando ganhos funcionais e estéticos para melhor aquisição de habilidades e satisfação dos pacientes 72 .…”

“…Recentemente, mais um estudo baseado na avaliação de imagens 3D demonstrou que o exorbitismo acentuado, característico das CS, não se deve exclusivamente à configuração rasa das órbitas e sim a um aumento no volume do globo ocular associado 21 . O fechamento precoce da sincondrose esfenoccipital, último centro de crescimento da base do crânio a fusionar, identificado em imagens tomográficas e sua influência na severidade da retrusão facial nas CS também é exemplo da variabilidade de informações que podem ser extraídas desses programas de computação dedicados à análise tomográfica [86][87][88][89] .…”