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Clinical Course and Visual Outcomes of Papilledema in Pediatric Cerebral Venous Sinus Thrombosis
Jessica A Sun1, Tais Estrela1, Ryan Gise1
1From the Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Insights
Cerebral venous sinus thrombosis (CVST) in children often causes papilledema, which can worsen despite treatment. Regular ophthalmologic follow-up is crucial for preventing permanent vision loss in pediatric CVST patients.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Cerebral venous sinus thrombosis (CVST) is a rare but serious condition.
- CVST can lead to significant neurological and visual impairments, particularly in children.
- Papilledema is a key ophthalmic manifestation of increased intracranial pressure in CVST.
Purpose of the Study:
- To investigate the natural history and visual outcomes of papilledema in pediatric patients diagnosed with CVST.
- To identify risk factors and predictors of visual sequelae in children with CVST-related papilledema.
Main Methods:
- A retrospective case series design was employed.
- Records of pediatric patients with CVST evaluated between 2000 and 2023 were reviewed.
- Data collected included papilledema presence, course, treatment, and visual outcomes, with analysis of Frisén grade and associated factors.
Main Results:
- 35 pediatric patients with CVST were analyzed; 89% presented with papilledema.
- Papilledema progression occurred in 29% of cases despite treatment. Initial Frisén grade ≥3 was significantly associated with optic atrophy (OR 7.54, P<0.001).
- Papilledema resolved in a mean of 107 days, but 54% experienced permanent ophthalmic sequelae.
Conclusions:
- Children with CVST face a high risk of ophthalmologic complications, including progressive papilledema.
- Effective management requires vigilant ophthalmologic monitoring throughout the treatment course.
- Early identification and management of papilledema are essential to mitigate irreversible vision loss in pediatric CVST.
Purpose:
Cerebral venous sinus thrombosis (CVST) is a rare but life-threatening event with significant neurologic and visual morbidity. In this study, we report on the natural history and visual outcomes of papilledema in children with CVST.
Design:
Retrospective case series.
Methods:
Patients with CVST evaluated by the Department of Ophthalmology between 2000 and 2023 were included. Records were reviewed for presence and course of papilledema, treatment, and final visual outcomes following papilledema resolution.
Results:
The study included 35 patients with a mean age of 9 ± 5 years and 40% were female. The most common risk factors for CVST were infection (69%), dehydration (26%), and hypercoagulability (23%). 31 patients (89%) had papilledema. Of these patients, 9 (29%) had progression of papilledema despite treatment, 17 patients (55%) did not have progression, and 5 patients (16%) lacked follow-up records. Initial Frisén grade among all cases was 2 ± 1, and cases with progression reached a grade of 4 ± 1 between 10 and 32 days following initial identification. Most patients (97%) were treated with anticoagulation and 100% required acetazolamide and/or lumbar puncture. Among 26 patients with follow-up, papilledema resolved in 107 ± 128 days. Fifty-four percent of patients had permanent ophthalmic sequelae. An initial Frisén grade ≥3 (odds ratio 7.54, 95% confidence interval 6.53-8.70, P< .001) was significantly associated with eventual optic atrophy.
Conclusions:
Children with CVST are at high risk for ophthalmologic sequelae. Papilledema can progress despite appropriate therapy. Our results highlight the importance of ophthalmologic follow-up during treatment course to prevent irreversible vision loss.
