MPS II, Hunter syndrome or iduronate sulfatase deficiency, is caused by lack of the enzyme iduronate sulfatase. Hunter syndrome has two clinical subtypes and as it is inherited in X-linked recessive fashion, it is the only one of the mucopolysaccharidoses in which the mother alone can pass the defective gene to a son.
Genu valgum: Family history is usually important because certain heritable conditions, such as hereditary multiple exostoses, Marfan syndrome, osteogenesis imperfecta, or vitamin D–resistant rickets may predispose a patient to this condition. .
If valgus malalignment of the extremity is significant, corrective osteotomy may be required. Hemiepiphysiodesis may be required in the skeletally immature patient. Methods of Hemiepiphysiodesis …show more content…
Anatomy: Genu varum is a normal physiologic process in children. Genu varum (bowed legs) is normal in children less than 2 years. Genu varum migrates to a neutral at about 14 months.
It continues on to a peak genu valgum (knocked knees) at about 3 years of age. Genu valgum then migrates back to normal physiologic valgus at about the age of 7 years. No treatment is requires for physiological genu varum. In rare instances, physiologic genu varum in the toddler will not completely resolve and during adolescence, the bowing may cause the child and family to have cosmetic concerns. If the deformity is severe enough, then surgery to correct the remaining bowing may be needed.
Rickets.
Surgery may also be needed for children with rickets whose deformities persist despite proper management with …show more content…
Blount’s disease. Blount's disease is pathologic genu varum which is progressive and centered at the tibia. It is divided into two distinct disease entities:
1. Infantile Blount's : This is the more common pathologic genu varum in children aged 0-3 years.
2. Adolescent Blount's: This is the less common pathologic genu varum in children older than 10 years. It is usually less severe and more likely to be unilateral.
Etiology: This is likely multifactorial but related to mechanical overload in genetically susceptible individuals. The overload leads to excessive medial pressure which produces an osteochondrosis of the medial proximal tibial physis and epiphysis which can progress to a physeal bar. A common risk factor is overweight in children that are early walkers (less than one year).
Classification: Langenskiold Classification provides both descriptive and prognostic guidelines. -Type I through IV consist of increasing medial metaphyseal beaking and sloping
-Type V and VI have an epiphyseal-metaphyseal bony bridge /bar across physis).
Radiographic findings suggestive of Blounts disease are: varus deformity localized at proximal tibia, severe deformity, asymmetric bowing, progressing deformity, sharp angular deformity, metaphyseal beaking and lateral thrust during
Genu valgum: Family history is usually important because certain heritable conditions, such as hereditary multiple exostoses, Marfan syndrome, osteogenesis imperfecta, or vitamin D–resistant rickets may predispose a patient to this condition. .
If valgus malalignment of the extremity is significant, corrective osteotomy may be required. Hemiepiphysiodesis may be required in the skeletally immature patient. Methods of Hemiepiphysiodesis …show more content…
Anatomy: Genu varum is a normal physiologic process in children. Genu varum (bowed legs) is normal in children less than 2 years. Genu varum migrates to a neutral at about 14 months.
It continues on to a peak genu valgum (knocked knees) at about 3 years of age. Genu valgum then migrates back to normal physiologic valgus at about the age of 7 years. No treatment is requires for physiological genu varum. In rare instances, physiologic genu varum in the toddler will not completely resolve and during adolescence, the bowing may cause the child and family to have cosmetic concerns. If the deformity is severe enough, then surgery to correct the remaining bowing may be needed.
Rickets.
Surgery may also be needed for children with rickets whose deformities persist despite proper management with …show more content…
Blount’s disease. Blount's disease is pathologic genu varum which is progressive and centered at the tibia. It is divided into two distinct disease entities:
1. Infantile Blount's : This is the more common pathologic genu varum in children aged 0-3 years.
2. Adolescent Blount's: This is the less common pathologic genu varum in children older than 10 years. It is usually less severe and more likely to be unilateral.
Etiology: This is likely multifactorial but related to mechanical overload in genetically susceptible individuals. The overload leads to excessive medial pressure which produces an osteochondrosis of the medial proximal tibial physis and epiphysis which can progress to a physeal bar. A common risk factor is overweight in children that are early walkers (less than one year).
Classification: Langenskiold Classification provides both descriptive and prognostic guidelines. -Type I through IV consist of increasing medial metaphyseal beaking and sloping
-Type V and VI have an epiphyseal-metaphyseal bony bridge /bar across physis).
Radiographic findings suggestive of Blounts disease are: varus deformity localized at proximal tibia, severe deformity, asymmetric bowing, progressing deformity, sharp angular deformity, metaphyseal beaking and lateral thrust during