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Very rare syndrome of premature aging facies combined with growth retardation.

Growth Retardation, Aged Facies, Normal Development, Decreased Subcutaneous Fat Syndrome.

Seven cases have been described worldwide. Probably autosomal dominant inheritance.

Patients present with intrauterine growth retardation and postnatal growth delay with normal mental development. There is decreased subcutaneous fat and thin or absent scalp hair. Main features involve the face, which seems prematurely aged with a triangular facies, prominent forehead, deep-set eyes, midfacial hypoplasia, prominent nasal septum, hypoplastic alar nasae, prominent ears, and thin lips.

Tracheal intubation and face-mask ventilation may be difficult because of anatomical abnormalities. Nasal intubation should be avoided secondary to small nares.

Marion RW, Goldberg RB, Young RS, et al: The GRANDDAD syndrome: A disorder combining growth delay, “aged facies,” normal development, and deficiency of subcutaneous fat. Am J Hum Genet 45(suppl):A53, 1989.

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