Hydrocephalus

1/1000 live births, of which 1/3 with meningomyelocele.

A blockage in the cerebrospinal fluid system of the brain produces elevated pressure and enlarged ventricular cavities, which in turn cause the head to grow disproportionately large. Hydrocephalus in preterm births is most often caused by bleeding in the cavities of the brain. Hydrocephalus in fullterm births may be caused by events during pregnancy or in connection with delivery, after birth or occur for unknown reasons.

Hydrocephalus is usually treated with the surgical placement of a shunt (flexible tube) that diverts cerebrospinal fluid from the ventricles to the abdominal cavity or to the atrium of the heart. Hydrocephalus does not necessarily cause any functional impairment but may be associated with cerebral palsy, balance problems, delayed development, learning disabilities, epilepsy, or eye and vision problems. Autism spectrum disorders, e.g. ADHD, may also occur.

Oral motor function is affected in some children, resulting in speech impairment, eating difficulties and drooling. Weak muscles in the mouth and face may also impact on oral health and occlusal development.

• Antibiotic treatment may be required with certain types of shunts in connection with tooth extraction or other oral interventions associated with bleeding.
• Training in oral motor skills in cases of eating difficulties, speech difficulties and drooling.
• Speech and language difficulties should be treated by a speech-lanuage pathologist.
• An orthodontist should be consulted when the child is between the ages of 7 and 9 in order to determine whether there are any dental or bite anomalies and whether corrective treatment is necessary.
• Problems in managing oral hygiene and tooth brushing, and eating difficulties justify extra preventive dental care.

The rare disease database of the Swedish National Board of Health and Welfare.
MHC-basen - Mun-H-Center's database of orofacial manifestations in rare disorders.
Ågrenska's Newsletter (Swedish)