Extremely rare form of cerebral gigantism that combines:

-         cerebral gigantism with macrocephaly, frontal and temporal bumps, hydrocephalus or malformation of the ventricles, intracerebral calcifications and a cerebellar syndrome; sometimes hypotonia or even a muscular atrophy.

-         odontogenic maxillary cysts   

-         a basocellular nevomatosis.

Sometimes: cleft lip palate, scoliosis due to vertebral malformations, kyphosis.

Associated abnormal metabolism of calcium? Decreased sensitivity to pain?

Anesthetic implications: 

check calcemia; hydrocephalus; risk of difficult intubation; imaging of the spine if a neuraxial block is planned.

References : 

• Cramer H, Niederdellmann H. 
  Cerebral gigantism associated with jaw cyst basal naevoid syndrome in two families. 
  Arch Psychiatr Nervenkr 1983; 233: 111-24.

Updated: September 2019