Long-term outcome in aqueductal stenosis.
In this study, 78 patients with aqueductal stenosis were submitted to detailed neurodevelopmental assessment with a follow-up of 5-25 years. Sixty-eight percent of patients were categorized as normal; they either attended normal school courses or had regular jobs. Among these, 34% had some motor abnormalities (ataxia, mild hemiparesis, visual disturbances). Twenty-four percent (19 cases) were moderately disabled (trainable retardation) and 8% (6 cases) were severely handicapped. Epilepsy was observed in 13% of the cases. Incidence of recurrent and generalized seizures paralleled neurodevelopmental outcome (5% in normal, 16% in moderately disabled and 50% in severely disabled patients). Endocrine dysfunctions were evident in 28% of the cases and were characterized by precocious or delayed puberty, amenorrhea and somatic underdevelopment. No patient with ventricular enlargement and a cortical mantle width below 20 mm showed a good outcome. Large ventricles were compatible with normal mental development when compensated with a corresponding cranial vault enlargement. In patients with normal mental status and motor abnormalities, long-term CT scan findings revealed the presence of focal brain abnormalities (poroencephaly, brain atrophy, calcifications, extracerebral collections).
Villani
R
R
Institute of Neurosurgery, University of Milan, Ospedale Maggiore Policlinico, Italy.
Tomei
G
G
Gaini
S M
SM
Grimoldi
N
N
Spagnoli
D
D
Bello
L
L
D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology; D005500:Follow-Up Studies; D006429:Hemiplegia / Q000209:etiology; D006801:Humans; D006849:Hydrocephalus / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery*; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D010264:Paraplegia / Q000209:etiology; D010913:Pituitary-Adrenal System / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt / Q000009:adverse effects*; D014786:Vision Disorders / Q000209:etiology