Maffucci
Syndrome
The disease is
sporadic in nature. It is characterized
by the presence of multiple endochromas
which are associated with multiple haemangioma
and lymphangiomas.
SYMPTOMS:
The disease shows itself at
childhood and adolescence. Extremities are affected by the endochromas most commonly at the metacarpal bones and phalanges of
the hands.
It is associated with a higher risk of pancreatic, ovarian and CNS
malignancies. It should be differentiated from Ollier syndrome.
TREATMENT:
The treatment
is symptomatic. Surgical corrections may be required. Radiotherapy may be used
to treat malignancies.
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