Gargoyle Face

Gargoyle Face

The characteristic facies of gargoylism, now known as mucopolysaccharidoses (MPS); the classic gargoyle face is seen in MPS type I-H/Hurler syndrome and MPS type IV/Morquio syndrome and characterised by thickening and coarsening of facial features due to subcutaneous deposition of MPS, most commonly seen after the first year of age; the head is large and dolichocephalic, with frontal bossing and prominent sagittal and metopic sutures, and the face exhibits mid-face hypoplasia, depressed nasal bridge, flared nares, a prominent lower third, thickened facies, widely spaced teeth with attenuated dental enamel and gingival hyperplasia

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gargoyle face

The characteristic facies of gargoylism, formally, mucopolysaccharidoses–MPS; the classic gargoyle face is seen in MPS type
I-H/Hurler syndrome and MPS type IV/Morquio syndrome and characterized by thickening and coarsening of facial features due to subcutaneous deposition of MPSs, most commonly seen after the first yr of age; the head is large and dolichocephalic, with frontal bossing and prominent sagittal and metopic sutures, with mid-face hypoplasia, depressed nasal bridge, flared nares, and a prominent lower¹⁄₃ of face, thickened facies, widely spaced teeth and attenuated dental enamel, gingival hyperplasia