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Reports from the MHC data base

Report from the Observation Chart (PDF)


ICD-10: Q03.0
ORPHA: 2185

Estimated occurrence

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. Hydrocephaus in fullterm births may be caused by events during pregnancy or in connection with delivery, after birth or occur for unknown reasons.

General symptoms

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.

Orofacial/odontological symptoms

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.

Orofacial/odontological treatment

  • Training in oral motor skills in cases of eating difficulties, speech difficulties and drooling.
  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • Training in oral motor skills in cases of eating difficulties, speech difficulties and drooling.
  • Speech and language difficulties should be investigated and treated by a speechlanguage pathologist.
  • When treating medically compromised patients always contact their doctor for medical advice.


  • The rare disease database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-12 14:25