Approach to a patient with Dwarfism

Young boy or a girl

height stunted

Ratio of upper and lower body height proportion normal or not (normally 1:1)

LE reaches upto middle thigh or down ?

proportion of arm and forearms looks normal

fingers shape is normal

lower extremity look shorter

alignment of LE on a photo shows multiplanar deformity of leg with anterior bowing and areas of flattening anateroposteriorly on leg

knee and hip are at flexion attitude ,

Intelligence - normal
Eye sight - normal

head ,neck examination - frontal bossing , sclera color , dentition , neck webbing ,

chest any signgs or deformity , belly protuded or not , umbilical hernia or not ,

back - kyphoscoliosis or not , dimpling of hair or not , any swellings on upper or lower extremities

gives  a clue to plan and proceed further





Impression

Rickets

Osteogenesis Imperfecta

   will have history of reccurent fractures with trivial trauma ,

    family history

    eyes color might be different , poor dentition


Polyostotic  Fibrous Dyslplasia

     dont have recurrent history of fracture ,

     leonine faces ,
 
      Mc Cune Albright Syndrome - precocious puberty and deformity


How to say deformity is on tibia ?

           does not correct with flexion of knee

 How to proceed ?

     Serum Calcium , Alkaline Phosphatase , Phosphorous Level

      Orthoscanogram

      Xray of affected parts

      Biopsy


Management ?

Coxa Vara and femoral medial bowing

 open multiple osteotomies

 William's technique of Multiple Osteotomies

   closed percutaneous osteotomy

  fixation ( rush nail / LC-DCP  in neutralization mode to prevent rotation )


same for bowing of tibia

Classification of Osteogenesis Imperfecta - very important .

 













   










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