Sturge-Weber syndrome Case report

Sturge-Weber Syndrome (SWS – encephalotrigeminal

angiomatosis) is a rare non-hereditary congenital condition.

It develops in the early foetal period, at the stage when the

embryo head is being formed, as a result of a random somatic

mutation of the GNAQ gene due to unknown reasons. The

main symptom is the formation of congenital angiomas,

especially in the area of capillaries, in the leptomeninges in

the brain, and along the course of the trigeminal nerve. Such

angiomas may also be present on lips, and in the whole oral

cavity: on its bottom, palate and gingivae. It may lead to

serious malocclusions and breathing problems. Aim. This

article aims to present problems regarding orthodontic

treatment of patients with Sturge-Weber syndrome, using

an example of a 7-year-old female patient. Case report.

A 7-year-old girl with all signs of this syndrome, namely

glaucoma, epilepsy, neurological disorders, cutis marmorata

telangiectatica congenita and after sanitation of the oral

cavity, had hygiene procedures and exercises with a vestibular plate introduced. Two attempts to take impressions for

appliances failed due to patient’s anxiety reactions.

Conclusions. This case shows difficulties that may be faced

by an orthodontist when treating patients with Sturge-Weber

syndrome. In this case, namely type I in the Roach scale, due

to neurological disorders it was necessary to take special

precautions in relation to procedures introduced and to

avoid stress in a patient. It makes orthodontic treatment

difficult. (Witanowska J, Laskowska M, Zadurska M.

Sturge-Weber syndrome. Case report. Orthod Forum

2018; 14: 143-9)