RESUMO
Weill-Marchesani syndrome is a rare, generalized disorder of connective tissue manifested by short stature, brachymorphia, and spherophakia. Inheritance is autosomal recessive. In the less than 50 reported cases, joint stiffness in the hands and thenar atrophy have been noted in adults. A kindred is reported here in which release of multiple trigger fingers and bilateral carpal tunnel syndrome in childhood has improved hand function in a brother and sister.
Assuntos
Síndrome do Túnel Carpal/genética , Doenças do Tecido Conjuntivo/genética , Dedos/anormalidades , Estatura , Síndrome do Túnel Carpal/fisiopatologia , Pré-Escolar , Doenças do Tecido Conjuntivo/fisiopatologia , Oftalmopatias/genética , Feminino , Dedos/fisiopatologia , Humanos , Masculino , Movimento , SíndromeRESUMO
Experience with 240 midface (Le Fort and zygoma) fractures in multiple trauma patients has emphasized that superior aesthetic results are obtained by immediate extended open reduction with primary bone grafting. Internal fixation of 110 zygomatic and 130 Le Fort fractures was performed in the lower midface (zygomaticomaxillary and nasomaxillary buttresses). Open reduction of the condyle was employed in five concomitant Le Fort and subcondylar fractures with a loss of ramus height to prevent superior and posterior displacement of the middle and lower face. Bone grafts were utilized in 74 patients. They were most frequently employed in the orbit and less frequently in the lower midface. Bone graft survival paralleled that observed under elective conditions, and a slightly higher infection rate was observed. Extended open reduction and immediate bone grafting adds a new dimension to the aesthetic results obtained from facial fracture treatment. Structural bony integrity and pre-injury facial architecture may be restored in the absence of soft-tissue contracture. Restoration of the pre-injury facial architecture (the essence of facial fracture treatment) is more accurately accomplished when these techniques are utilized.