– radial club hand is associated with thrombocytopenia, bilaterality in at least 50%, associated thumb deficiency if the entire radius is absent, a short humerus
– the most endangered structure when doing a surgical approach to the radial clubhand is the median nerve – BEWARE!
– radial club hand is not associated with a proximal intercalary arm defect – the humerus may be short, but it is not an intercalary defect.
– the most common type of radial clubhand is the type IV – absent radius.
– congenital radioulnar synostosis usually occurs in the proximal forearm.
– for the congenital radioulnar synostosis, resection of the synostosis never restores motion – do not do this operation. If anything, you can osteotomize them to position their hand in better position, but you will not restore motion. Basically, they are stuck with a one bone forearm.
– for congenital radioulnar synostosis, males are more affected than females, the synostosis is usually in the proximal third, the forearm is usually pronated, and the disorder is often bilateral.
– for congenital radial head dislocation, it may be asymptomatic and be brought to your attention by some other incidental trauma. Look at the shape of the head to give you an idea of if it has been out for some time. It should NOT be reduced – it should be excised if symptomatic but one should wait until the patient is skeletally mature.
– thumb deformity that is passively correctable with MCP flexion, IP extension – ??? congenital deficiency of EPB?
– nail-patella syndrome – onycho-osteodystrophy.
– includes absent or hypoplastic patellas, elbow abnormalities, iliac horns, and dysplastic nails.
– transmitted as a dominant trait
– get hypoplastic or absent patella that is subluxed, knee valgus and flexion deformity, iliac horns, nails hypoplastic or absent, radial head abnormalities.
– for tibial hemimelia, if the tibia is pretty good, you can consider Symes. If it is atrophic, better off with knee disarticulation.
– for fibular hemimelia, the femur may be abit short as well. The tibia is shortened (the greater the fibular deficiency, the greater the shortening of the tibia) and anteromedially bowed. The foot is in valgus.
– arthrogryposis is caused by many things: neuropathic problem, myopathic problem, and decreased interuterine space
– for Klippel Feil, the combination of neck fusion most likely to require surgery is occipitalization of C1 and C2-3 fusion, or two long cervical fusions with a normal segment in between.
– Klippel Feil patients get shoulder stiffness (associated Sprengel’s deformity), decreased range of motion of the neck, synkinesis (an associated movement disorder) where an involuntary movement accompanies a voluntary movement. They also get facial nerve palsies, but not gaze palsy.
– the foramen magnum in achondroplastics is compressed and small – although the head is formed by intramembranous bone formation, the foramen is endochondral, and thus affected.
– the pathology behind achondroplasia is failure of calcification of the chondroid matrix and failure of cartilage cell proliferation in the physis.
– the main inheritance pattern of achondroplasia is autosomal dominant.
– in achondroplastics, the ulnar and fibular involvement is less than the radial (radial head) and tibial (genu varum) involvement. The fact that the ulnar and fibular growth plates are less involved leads to the genu varum secondary to fibular overgrowth.
– for the osteogenesis imperfecta kid with huge angular deformities of the tibia, best to do multiple osteotomies with IM fixation. Initially, you would like to brace, but when the deformity gets to be too big to brace, then you might as well do the osteotomies before the thing breaks.
– Ehlers Danlos, osteogenesis imperfecta, Marfan’s syndrome, and peyronies disease are all related to collagen disorders. Osteopetrosis is not.
– in osteogenesis imperfecta, the main defect is in collagen maturation
– melorheostosis give bones