| Popis: |
The authors describe a case of Moebius syndrome associated with arthrogryposis multiplex congenita, a rare presentation, with no previous reports in the South American literature. The Moebius syndrome consists of VII and VI cranial nerves paralysis, usually associated with other musculoskeletal anomalies, often situated in the extremities. Arthro- gryposis multiplex congenita is generally characterized by rigidity and contracture of multiple joints and sometimes is associated with other congenital anomalies. The patient was a 3-year-old boy, presenting with convergent strabismus, ophthalmoplegia, VII cranial nerve paralysis and tongue musculature deficiencies, alterations consistent with Moebius syndrome. Associated with these alterations, musculoskeletal abnormalities consistent with arhrogryposis were found: hands and forearms in flexion and scapular muscular dystrophy. Surgical treatment was performed in order to correct convergent strabismus, with good follow-up. The authors suggest arthrogryposis investigation in all patients that present with Moebius syndrome, because this association is possibly being underdiagnosed. |
