Orofacial function of persons having Report from questionnaires 9 questionnaires Synonym ICD-10 Estimated occurance General symptoms Oral symptoms Encephalotrigeminal Angiomatosis Q85.8B 2-3:100,000 live births. The syndrome is divided in three subgroups: Sturge Weber type I includes facial and brain angiomas, epilepsy and glaucoma; Sturge Weber type II includes only facial angiomas and glaucoma may occur; Sturge Weber type III includes only brain angiomas. Facial angiomas, a port wine colored birthmark, may be seen on the forehead, upper eyelid and on one side (or sometimes both sides) of the face. The color is light pink to dark purple and is caused by an overabundance of capillaries around the trigeminal nerve. Brain angiomas are vascular malformations of the brain surface which can cause learning disabilities, behavioral abnormality, hemiparesis/weakness and headaches/migraines. Facial angiomas can cause facial asymmetry and in some persons the front teeth of the upper jaw may be offset to the side that does not have the skin lesion. Certain medications for epilepsy can give dry mouth as a side effect, increasing the risk of cavities (tooth decay). Children with and neurological impairment may have delayed speech and language development. Oral treatment Early contact with dental services for intensified prophylactic care and oral hygiene information is essential. Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed. Speech and language difficulties should be treated by a speech therapist. Sources The MHC base Rare diseases Dokumentation-Ågrenska 1 (7)
Age distribution : 9 Ages: 4-60 (5) (4) Additional diagnoses Medical impairment Yes No Missing Inborn heart defect Other cardiovascular disease 2 7 0 Epilepsy 5 4 0 Asthma 2 7 0 Need of respiratory support 1 8 0 Allergy 1 8 0 Neuropsychiatric diagnosis Yes No Missing ADHD/ADD 0 7 2 Autism (Includes autism, Asperger syndrome and autistic traits) 0 8 1 General disability Yes No Missing Intellectual disability 1 6 2 Motoric functional impairment 2 5 2 Visual impairment 2 3 4 Hearing loss 1 8 0 Communication difficulties 3 5 1 2 (7)
About dental care and oral health Do you feel that you receive the dental care you need? Yes, very much so 6 Yes, somewhat 3 How many times per year do you normally seek dental care? Less than once per year 4 One time per year 3 Two times per year 1 Three or more times per year 1 When were your teeth last X-rayed? During the past two years 6 More than two years ago 1 Never had my teeth X-rayed 2 3 (7)
Do you look after your teeth in a good way? Yes, very much so 5 Yes, somewhat 3 No, not really 1 Who brushes your teeth? I always brush myself 7 Someone else always helps me 2 How often are your teeth brushed? Not everyday 1 Once per day 2 Two times per day 5 Three or more times per day 1 4 (7)
About dental care and oral health Yes No Missing Does your mouth hurt? Does your mouth feel dry? 3 5 1 Have you ever taken a serious hit to your permanent front teeth? 2 6 1 Do you feel that you have a divergent bite? 2 7 0 Have you had a brace? 4 4 1 Do you feel that you need orthodontics/a brace? 3 5 1 Do you grind or press your teeth at night? Never 5 Once or twice per week 3 Missing 1 Do you grind or press your teeth during the day? Never 8 Once or twice per week 1 5 (7)
About eating Do you have any problems with eating? No, not really 2 No, not at all 7 Missing 0 Yes No Missing Do you cough daily in connection with meals? 1 8 0 Do you gag daily in connection with meals? Do you get acid reflux daily? 2 7 0 Do you throw up often (at least twice per week)? Do you have a poor appetite? 1 8 0 Does it take a long time before you can swallow a mouthful? Do you press your tongue forward when you swallow so that food ends up outside the mouth? Do you find it difficult to chew, i.e. grind food using your molars? Do you find it difficult to take food from the spoon using your lips? Have you had problems with food and drink leaking out through the corners of your mouth? 1 8 0 Does food tend to remain in your mouth after meals? 1 8 0 Do you get nutrition in any other way than through your mouth? 6 (7)
About drooling Do you drool? Never drool 6 Drool sometimes not every day 2 Missing 0 How much do you drool? Slight drooling, only on the lips 1 Moderate drooling, on lip and chin 1 Total: 2 Is your drooling a problem for you? Yes, somewhat 1 No, not at all 1 Total: 2 Is your drooling a problem for your family or people around you? No, not at all 2 Total: 2 7 (7)