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.