De Barsy Syndrome: Orthopedic Case report and Literature Review

Abstract

MOJ Orthop Rheumatol 2017, 7(5): 00284OGJA, male patient of 5 years and 6 months sent for firsttime orthopedic evaluation from a telethon child rehabilitation center in the city of Irapuato, Mexico due to the antecedent of psychomotor retardation, asymmetry of the pelvic extremities and inability to stand and walk. It does not have a hereditary family history of importance, mother at the time of pregnancy 30 years of age, second pregnancy, no drug addiction during pregnancy, history of intake of folic acid, iron and vitamins, pregnancy at term 40 weeks gestation, Natural birth, weight 3000 gr, size 51 cm, do not report hypoxia at birth (does not remember APGAR scale), both graduates of the hospital at 24 hours. During development and birth it negates hyperbilirubinemia; Cephalic support at 1 year 5 months, sedestation 1 year and 8 months, currently does not achieve standing, ambulation not achieved, sporadic babbling, history of previous hospitalizations for bilateral congenital cataract surgery operated at 4 months of age, history of pneumonia Repetition, has been diagnosed with Barsy syndrome since the beginning of 2015. Physical ExaminationAthetoid movements, Macrocephaly, cephalic support, adequate hair implantation, asymmetric eyes (hypertelorism), eruptive asymmetry of the upper and lower canines, cylindrical mobile neck, short, chest thorax, with limitation to the amplexation of the Respiratory movements, unstructured kyphosis, normal cervical lordosis, no spinal dysrhaphy, soft abdomen, hepatic dullness 5 cm below the costal border, 2 cm umbilical hernia not protruded. Without ambulation, Thoracic integral extremities, normal glenohumeral joint, elbow with hyperextension, hand with integral fingers, thumb in proper position, can perform fine and thick clamp. Asymmetric pelvic extremities with bilateral proximal femoral angular deformity, barlow and ortolani negative bilateral positive piston, bilateral positive yawning sign, positive drainage test for anterior and posterior of both knees, and ankle without alterations, preserved distal mobility. Brighton criteria of 8 points (touching the ground with the palms was not possible) . In all radiographic projections, metaphyseal rarefaction, proximal femur, distal, proximal tibia, as well as proximal and distal humerus, radius and cubit are observed. Radiographic FindingsPulmonary perfusion scan: low uptake of right lower lobe and bilateral apices.