Benign intracranial hypertension in childhood (Pseudotumor Cerebri=PTC) – experiences with 13 consecutive patients

Introduction: PTC in childhood is a rare disease. item. The pathogenetical mechanism has not yet been cleared satisfyingly. Main risk of PTC is a potentially irreversible damage of the optical nerve.

Patients: We present 13 patients (8 m, 5 f; age at manifestation 2 to 14 y) with PTC, who have been treated between 07/2000 and 07/2004.

In all cases initially we did extensive anamnestic, clinical, apparative, sero- and endocrinological examinations. Leading symptoms were headache, edema of papilla N.optici and concentric restriction of visiual field.

Bacterial otogenic meningitis in previous history (n=1), obesity (n=4), inhaled steroid-therapy (n=1) were possible reasons of PTC we could individuate. In 7 cases we did not figure out any reasons. Initial lumbar CSF pressure ranged from 20,5 to 60cm H2O.

Therapy according to the following staged proceeding: 1. repeated lumbar puncture 2. Azetazolamid + Furosemid 3. Oral Steroids 4. if ineffective: lumbo-peritoneal shunting.

Results: In 2 pat. a few lumbar punctures were sufficient. 11/11 pat. were treated with Acetazolamid + Furosemid, 2/11 needed treatment with Steroids. In both cases Steroids resulted in side-effects without controlling hypertension. Furtheron this option wasn't applied in any case. 2 pat. were submitted to operative lumbo-peritoneal shunting.

In all patients we could observe almost complete resolution of clinical symptoms except persisting slight fundus-abnormalities in about a half of cases. In 3 of 9 drug-treated/non-operated pat. medical therapy could be withdrawn after 3–18 months. 6 pat. are still on drug therapy.

Discussion: The incidence of PTC among our total patients has been surprisingly high. Mainly mild symptoms and difficulties in reliable perimetric measurements could be reasons for PTC being underdiagnosed among pediatric patients. This presentation might contribute to keep in mind diagnosis of PTC. Higher case rates could be helpful to develop diagnostic procedures and therapy as well as non-invasiv methods of control of pressure levels in the course of therapy.