Operative Management of OSAS in a Complex Case of Proteus Syndrome

Table 1

Revised Proteus syndrome diagnostic criteria [4].

General criteria	Mosaic distribution
	Progressive course
	Sporadic occurrence
Specific criteria	Subgroup A
	(1) Cerebriform connective tissue nevus
	Subgroup B
	(1) Linear epidermal nevus
	(2) Asymmetric, disproportionate overgrowth of limbs, skull, external auditory canal, vertebrae, or viscera (spleen/thymus)
	(3) Specific tumors before 2nd decade of life, as bilateral ovarian cystadenomas or monomorphic parotid adenomas
	Subgroup C
	(1) Dysregulation of adipose tissue: lipomas, regional lipohypoplasia
	(2) Vascular malformations (capillary, venous, and/or lymphatic)
	(3) Bullous pulmonary disease
	(4) Facial phenotype with long face, dolichocephaly, downslanted palpebral fissures, low nasal bridge, wide or anteverted nares, and open mouth at rest

For the diagnosis of Proteus syndrome all general criteria and one feature of subgroup A or two features of subgroup B or three features of subgroup C must be satisfied.