RESUMO

OBJECTIVES: The objective is to describe a new surgical procedure developed in the San Jose Pediatric University Hospital for the management of syndromic synostosis of the metopic suture in a patient clinically diagnosed with Saethre-Chotzen syndrome. METHODS: The diagnosis of Saethre-Chotzen syndrome, bilateral coronal sutures, and metopic suture synostoses was made through photographic, anthropometric, exophthalmometric, and computed tomography analysis. The keel-like frontal bone deformity was corrected following resection using a fusiform osteotomy, remodelling was obtained by milling the edges, and the bony fragments were repositioned and fixed on the posterior wall of the frontal bone. Additionally, a fronto-orbital advancement with a self-stabilizing bar was performed. RESULTS: The 1-year postoperative results showed improvement in the position of the fronto-orbital bar, adequate cranial expansion, satisfactory correction of the upper facial third alteration, and correction of the keel-like deformity. CONCLUSIONS: The surgical approach has not previously been described in the literature and offers an alternative management for syndromic craniosyntostosis of the metopic suture, avoiding skull irregularities.

Assuntos

Acrocefalossindactilia/cirurgia , Craniossinostoses/cirurgia , Acrocefalossindactilia/diagnóstico , Suturas Cranianas/cirurgia , Craniossinostoses/diagnóstico , Craniossinostoses/genética , Feminino , Seguimentos , Osso Frontal/anormalidades , Osso Frontal/cirurgia , História Antiga , Humanos , Lactente , Recém-Nascido , Doenças do Prematuro/diagnóstico , Doenças do Prematuro/cirurgia , Osteotomia/métodos , Fenótipo , Síndrome , Tomografia Computadorizada por Raios X

RESUMO

In medicine, classifications are designed to describe accurately and reliably all anatomic and structural components, establish a prognosis, and guide a given treatment. Classifications should be useful in a universal way to facilitate communication between health professionals and to formulate management protocols. In many situations and particularly with craniofacial microsomia, there have been many different classifications that do not achieve this goal. In fact, when there are so many classifications, one can conclude that there is not a clear one that accomplishes all these ends and defines a treatment protocol. It is our intent to present a new classification based on the Pruzansky's classification, later modified by Kaban, to determine treatment protocols based on the degree of osseous deficiency present in the body, ramus, and temporomandibular joint. Different mandibular defects are presented in two patients with craniofacial microsomia type III and IV according to our classification with the corresponding management proposed for each type and adequate functional results.