Q. The child presenting with below deformity of leg was provisionally diagnosed as non traumatic bowing of tibia. What important skin lesion can help rule out a particular association of the disorder?
a. Spider naevus
b. Hemangiomas
c. Melanotic patch
d. Cafe au lait spots
Answer -
d
Congenital pseudoarthrosis of tibia (CPT) is an important differential in this case.
The bowing typically is anterolateral and one common association is neurofibromatosis (up to 50%).
Neurofibromatosis has major and minor criteria for diagnosis based on associated clinical features. One important clinical feature is presence of large cafe au lait spots (CALS).
CALS present here have smooth borders thus often referred to ' coast of California' ( in contrast to CALS with irregular borders in Mc Cune Albright syndrome referred as ' coast of Maine).
P.S. - below image shows multiple CALS in the back of same patient and one of them is very large.
a. Spider naevus
b. Hemangiomas
c. Melanotic patch
d. Cafe au lait spots
Answer -
d
Congenital pseudoarthrosis of tibia (CPT) is an important differential in this case.
The bowing typically is anterolateral and one common association is neurofibromatosis (up to 50%).
Neurofibromatosis has major and minor criteria for diagnosis based on associated clinical features. One important clinical feature is presence of large cafe au lait spots (CALS).
CALS present here have smooth borders thus often referred to ' coast of California' ( in contrast to CALS with irregular borders in Mc Cune Albright syndrome referred as ' coast of Maine).
P.S. - below image shows multiple CALS in the back of same patient and one of them is very large.
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