Craniofacial Severity Index for a Patient with Loeys-Dietz Syndrome

The Craniofacial Severity Index can describe the severity of craniofacial abnormalities in a patient with Loeys-Dietz Syndrome (LDS).

Parameters:

(1) ocular hypertelorism

(2) cleft palate

(3) craniosynostosis

(4) malformations of the uvula

Parameter	Finding	Points
ocular hypertelorism	none	0
	subtle (near the upper limit of normal)	1
	marked	2
cleft palate	absent	0
	present	3
craniosynostosis	absent	0
	present	3
malformation of the uvula	none	0
	broad with no cleft	1
	midline raphe	2
	bifid	3

total score =

= SUM(points for all 4 parameters)

Interpretation:

• minimum total score: 0

• maximum total score: 11

• The higher the score more severe the craniofacial abnormalities.

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