[Macrocephaly and multiple hamartoma: a very variable entity].
The Bannayan-Zonana is a dominant autosomal polymalformation syndrome.


METHODS
We report a case of Bannayan-Zonana syndrome in a 3-year-old girl, who presented with macrocephaly and a cervical lipoma. The patient's mother had neurofibromatosis I. No mutation in the PTEN gene was found. The slowly progressive lipoma increased with age and the surgical reduction was necessary.


CONCLUSIONS
Macrocephaly is constant in different syndromes characterized with multiple hamartomas. The multiple hamartomas syndrome represents an entity with a high variability of expression.
Mcheik
J N
JN
Service de chirurgie pédiatrique, centre hospitalo-universitaire de Poitiers, 350, avenue Jacques-Coeur, BP 577, 86021 Poitiers, France. j.mcheik@chu-poitiers.fr
Cordier
M P
MP
Longy
M
M
Levard
G
G
D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006222:Hamartoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008067:Lipoma / Q000473:pathology* / Q000601:surgery; D009333:Neck / Q000473:pathology; D013577:Syndrome