Ollier’s disease

Ollier’s disease/Maffuci’syndrome/Beckwith- Widerman’s syndrome /Klippel-trenaunay-Weber  syndrome

Q1.Enchondrma and heamangioma is found in

a.Ollier’s disease

b.Maffuci’syndrome

c.Beckwith- Widerman’s syndrome

d.Klippel-trenaunay –Weber  syndrome

ANS. b===MAFFUSI SYNDROME------ Maffucci syndrome is a congenital non hereditary mesodermal dysplasia  characterised by multiple enchondromas with soft  tissue cavernous haemangiomas. 

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• Ollier disease ( enchondromatosis) is a non-hereditary, sporadic, skeletal disorder characterised by multiple enchondromas that are principally located in the metaphyseal regions.

• Beckwith–Wiedemann syndrome  is an overgrowth disorder usually (but not always) present at birth characterized by an increased risk of childhood cancer and certain congenital features.
•  Five common features used to define the syndrome are: macroglossia, macrosomia (birth weight and length greater than the 90th percentile), midline abdominal wall defects (omphalocele/exomphalos, umbilical hernia, diastasis recti), ear creases or ear pits, and neonatalhypoglycemia (low blood sugar after birth).

• Klippel–Trénaunay–Weber syndrome is a rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly. 
• The three main features are nevus flammeus (port-wine   stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb.