NIVALDO ALONSO
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Cirurgia, Faculdade de Medicina - Docente
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/04 - Laboratório de Microcirurgia, Hospital das Clínicas, Faculdade de Medicina - Líder
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/04 - Laboratório de Microcirurgia, Hospital das Clínicas, Faculdade de Medicina - Líder
2 resultados
Resultados de Busca
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- Orbital and Periorbital Dysmorphology in Untreated Pfeiffer Syndrome(2022) LU, Xiaona; FORTE, Antonio Jorge; ALLAM, Omar; PARK, Kitae Eric; WILSON, Alexander; ALPEROVICH, Michael; STEINBACHER, Derek M.; TONELLO, Cristiano; ALONSO, Nivaldo; PERSING, John A.Background: Visual impairment secondary to orbital and periorbital dysmorphology is frequent in Pfeiffer syndrome patients. The etiopathogenesis of this aberrancy, however, remains unclear. Methods: Untreated Pfeiffer syndrome patients (n = 31) and normal control subjects (n = 43) were compared. Craniometric and volumetric analyses related to the orbital and periorbital anatomy were performed using Materialise (Leuven, Belgium) software. Results: Overall, orbital cavity volume of Pfeiffer patients is reduced by 28 percent (p < 0.001), compared to normal, starting before 3 months of age (p = 0.004). Globe volume was diminished by 10 percent (p = 0.041) before 3 months of age, yet tended to catch up thereafter. However, the retrobulbar soft-tissue volume remained smaller beyond 1 year of age (17 percent, p = 0.003). Globe volume projection beyond the bony orbit increased in all observed ages (82 percent, p < 0.001). The volumes of sphenoid bone, maxilla, and mandible proportionately were restricted by 24 to 25 percent (p = 0.003 to 0.035) before 3 months of age. The volume of maxilla and mandible gradually approximate normal; however, the sphenoid bone volume in Pfeiffer patients remains less than normal (p = 0.002) into childhood. The anteroposterior length of both the zygoma and the maxilla was reduced by 14 percent (p < 0.001). Anterior positioning of the zygoma is less by 23 percent (p < 0.001) in Pfeiffer patients overall, with anterior positioning of maxilla reduced similarly by 23 percent (p < 0.001). Conclusions: Pfeiffer syndrome patients develop decreased retrobulbar soft-tissue and globe volume, along with a restricted orbital cavity volume in infancy. Significant hypoplasia of the sphenoid bone is associated with more severe central facial (maxilla) retrusion, compared to lateral facial structures (zygoma).
- Orbitofacial morphology changes with different suture synostoses in Crouzon syndrome(2022) LU, Xiaona; FORTE, Antonio Jorge; JUNN, Alexandra; DINIS, Jacob; ALPEROVICH, Michael; ALONSO, Nivaldo; PERSING, John A.This study aims to investigate the influence of different cranial vault suture synostoses on orbital and periorbital morphological development in Crouzon syndrome. Computed tomography (CT) scans of Crouzon syndrome patients who had not undergone operation were subgrouped as follows: type I: bicoronal synostosis; type II: sagittal synostosis; type III: pansynostosis; type IV: perpendicular combinations of suture synostoses; and type V: bilateral squamosal synostosis. CT scans were measured using Materialise software. CT scans of 80 Crouzon syndrome patients and 72 normal controls were included. Orbital bony cavity volume was reduced in all subgroups (16-24%), including type V bilateral squamosal synostosis (16%, p = 0.003), although the reduction in type II sagittal synostosis Crouzon patients failed to reach statistical significance (p = 0.071). Globe volume was reduced only in type I bicoronal synostosis (9%, p = 0.018), while the retrobulbar soft tissue volume decreased in type III pansynostosis group by 11% (p = 0.005). Globe volume projection beyond the orbital rim was increased in all groups (p < 0.001), with the greatest increase in type IV perpendicular combination of sutures synostoses, by 100% (p < 0.001). The anteroposterior length of maxilla was significantly shortened in type I (10%, p = 0.028) and type III (9%, p = 0.022) but developed normally in other groups, although the maxilla was posteriorly displaced in all groups (all p <= 0.026). The influence of squamosal synostosis on craniofacial malformation is not equivalent to that of a major vault suture. Therefore, subtype suture fusion individualized surgical interventions, after initial occipital expansion, likely provide additional benefits in mitigating oculo-orbital disproportion.