Article Text
Abstract
Background Pseudotumor Cerebri syndrome (PTCS) in children prior to onset of puberty has unique phenotypic features and risk factors compared to the older adolescent and adult forms. Reports on endovascular treatment for PTCS secondary to dural venous sinus stenosis in the prepubescent age group are sparse. We sought to understand current concepts and endovascular practices related to prepubescent PTCS through literature review, highlight the role of cranial lymphatic and glymphatic systems through an illustrative case, and provide recommendations for future research directions.
Methods A literature review was conducted using PubMed database for all accounts of prepubescent PTCS treated with venous sinus stenting (VSS) up to August 2023. A total of 217 studies were screened with 7 studies meeting inclusion criteria. All cases age 11 years of age or younger, with Dandy criteria diagnostic of PTCS, all of whom underwent VSS were included for analysis; cohort demographics, endovascular procedure details, and VSS outcomes are summarized. We also present a 3-year old patient with PTCS who was found to have a right temporal bone lymphatic malformation with ipsilateral severe transverse sinus stenosis successfully treated with VSS.
Results A total of 9 cases were identified from the literature and 1 included from our own institution. Patient were aged from 2–11 years old, with 7 males, and a median BMI of 16.1 kg/m2. The majority underwent unilateral VSS with self-expanding stents through a femoral approach under general anesthesia, with mean trans-stenotic gradients (TSG) of 15.7 mmHg pre-stenting reducing to 2.8 mmHg post-stenting (ΔTSG 13.6 mmHg, range 2–31 mmHg). All patients were maintained on dual antithrombotic therapies (DATT) post procedure for 1–6 months. At follow up, 90% of patients reported improvement in symptoms, two developed stent adjacent stenosis requiring retreatment, and two required additional shunting procedures. Complications were infrequent and involved minor bleeding related to DATT use.
Conclusion Prepubescent PTCS is a distinct disease driven by craniocervical processes, with endovascular VSS a safe and effective treatment option for this increasingly recognized condition. There remain unmet clinical and research needs for this patient population.
Disclosures M. Gaub: None. M. Lafuente: None. K. Russell: None. A. Stallings: None. M. Webb: None. R. Morton: None. F. Al Saiegh: None. J. Mascitelli: 2; C; Stryker. B. Faux: None. I. Tarasiewicz: None. L. Birnbaum: 2; C; Imperative Care, Rapid AI.