title: Adult long-term outcome of patients after congenital hydrocephalus shunt therapy.
abstract: BACKGROUND
  Even though shunt surgery has been an established and widely accepted treatment for congenital hydrocephalus for five decades, long-term follow-up and functional outcome data is rare.
  
  
  METHODS
  Sixty-nine patients were examined after congenital hydrocephalus had been treated between 1971 and 1987 at the Department of Pediatric Surgery (University of Leipzig) within the first 12 months of life. Median age of patients was 32 years (range 25-42 years). Patients agreed to undergo a contemporary hydrocephalus assessment.
  
  
  RESULTS
  Seven patients (10.1%) became shunt-independent before adulthood. By the age of 20, 82% of the patients had needed at least one shunt revision, 100% by the age of 30. 21.7% of the primary valves (Spitz-Holter) remained intact without revision until today up to 35 years (mean functional intactness 23 years). Shunt infections occurred in 4.3% of primary implantations. 48% of the patients had a good functional outcome (mRS = 0-1). 40.9% of the patients attended regular school. In summary, 75% patients work on a daily basis. 44.6% live an independent life, 35.4% rely on parental supervision, and 20% depend on professional care institutions.
  
  
  CONCLUSIONS
  Compared to the pre-shunt era, surgery within the first year of life is advantageous regarding visual function, educational progress, and social results. The outcome achieved throughout childhood remains stable during adult life as long as catastrophic events of shunt malfunction can be prevented. Epilepsy, motor deficits, acute shunt dysfunction, and problems of social integration as well as aging parental caregivers seem to be prominent factors of morbidity in adulthood.
authors.affiliation: Department of Neurosurgery, Pediatric Neurosurgery, University Hospital Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany, preuss@neurosurgeon.ch.
authors.forename: M
authors.initials: M
authors.lastname: Preuss
authors.forename: A
authors.initials: A
authors.lastname: Kutscher
authors.forename: R
authors.initials: R
authors.lastname: Wachowiak
authors.forename: A
authors.initials: A
authors.lastname: Merkenschlager
authors.forename: M K
authors.initials: MK
authors.lastname: Bernhard
authors.forename: M
authors.initials: M
authors.lastname: Reiss-Zimmermann
authors.forename: J
authors.initials: J
authors.lastname: Meixensberger
authors.forename: U
authors.initials: U
authors.lastname: Nestler
mesh_terms: D000293:Adolescent; D000328:Adult; D004868:Equipment Failure; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery*; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D018754:Ventricular Dysfunction / Q000209:etiology; D017287:Ventriculoperitoneal Shunt / Q000009:adverse effects / Q000379:methods*; D055815:Young Adult