Pediatrik popülasyonda sekonder psödotümör serebri: klinik özellikler, tedavi ve prognoz

Year 2024, Volume: 17 Issue: 2, 279 - 284, 01.04.2024

Olcay Güngör , Emine Ün , Beste Kıpcak , Osman Parça

Abstract

Amaç: Psödotümör serebri sendromu (PTSS), intrakranyal kitle, hidrosefali veya beyin omurilik sıvısı (BOS) bileşiminde anormallikler olmadan yüksek kafa içi basıncı (KİB) ile karakterizedir. İkincil PTSS'de (sPTSS), BOS basıncını artıran bir neden vardır. Bu çalışmanın amacı sPTSS tanısı alan pediatrik hastaların tanı, tedavi ve takiplerinin değerlendirilmesidir.
Gereç ve yöntem: Bu çalışma tek merkezli üçüncü basamak bir pediatri hastanesinde gerçekleştirilen retrospektif bir çalışmadır. 2018 ile 2023 yılları arasında üçüncü basamak bir hastanede sPTSS tanısı konan ve PTSS için mevcut tanı kriterlerini karşılayan 1-17 yaş arası hastaları dahil ettik. Olguların şikayetleri, etiyolojisi, oftalmolojik değerlendirmeleri ve tedavi sonuçları değerlendirildi.
Bulgular: Çalışmaya PTSS tanısı alan 17 hasta dahil edildi. Ortalama yaş 9,82 (±4,6) idi. Hastaların 9'u (%56,2) erkek, 8'i (%43,7) kadındı. En sık görülen semptomlar 10 hastada (%62,5) baş ağrısı, 6 hastada (%37,5) bulantı/kusma, 5 hastada (%31,2) çift görme idi. Hastaların tamamında papilödem, 7 (%43,7) hastada ise altıncı sinir felci vardı. 3 (%16) hastada nüks görüldü. Üç hastaya ikinci basamak tedavi olarak optik sinir fenestrasyonu uygulandı.
Sonuç: Medikal tedaviye yanıt alınamayan durumlarda optik sinir kılıfı fenestrasyonu iyi bir tedavi seçeneği olabilir.

Keywords

Optik sinir, psödotümör serebri, çocuk

Project Number

yok

Secondary pseudotumor cerebri in the pediatric population: clinical features, treatment, and prognosis

Abstract

Purpose: Pseudotumor cerebri syndrome (PTCS) is characterized by elevated intracranial pressure (ICP) without intracranial mass, hydrocephalus, or abnormalities in cerebrospinal fluid (CSF) composition. In secondary PTCS (sPTCS), there is a reason that increases the CSF pressure. The aim of this study is to evaluate the diagnosis, treatment, and follow-up of pediatric patients diagnosed with sPTCS.
Materials and methods: This is a retrospective study conducted in a single-center tertiary pediatric hospital. We included patients aged 1-17 years who were diagnosed with sPTCS in a tertiary hospital between 2018 and 2023 and met the current diagnostic criteria for PTCS. We evaluated the complaints, etiology, ophthalmological evaluations, and treatment results of the cases.
Results: Seventeen patients with a diagnosis of PTCS were included in the study. The mean age was 9.82 (±4.6). Of the patients, 9 (56.2%) were male and 8 (43.7%) were female. The most common symptoms were headache in 10 patients (62.5%), nausea/vomiting in 6 patients (37.5%), and double vision in 5 patients (31.2%). All patients had papilledema and 7 (43.7%) patients had sixth nerve palsy. Recurrence was observed in 3 (16%) patients. Optic nerve fenestration was performed in three patients as a second-line treatment.
Conclusion: In cases that do not respond to medical treatment, optic nerve sheath fenestration may be a good treatment option.

Keywords

Optic nerve, pseudotumor cerebri, child

Ethical Statement

Etik kurulu onayı alındı.

Supporting Institution

yok

Project Number

yok

Thanks

.

References

• 1. Chiu HH, Reginald YA, Moharir M, Wan MJ. Secondary pseudotumour cerebri syndrome in children: clinical characteristics and long-term outcomes. Can J Ophthalmol 2022;57:216-218. https://doi.org/10.1016/j.jcjo.2022.02.021
• 2. Kilic K, Korsbæk JJ, Jensen RH, Cvetkovic VVJC. Diagnosis of idiopathic intracranial hypertension-the importance of excluding secondary causes: a systematic review. Cephalalgia 2022;42:524-541. https://doi.org/10.1177/03331024211056580
• 3. Paley GL, Sheldon CA, Burrows EK, Chilutti MR, Liu GT, McCormack SE. Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome. Am J Ophthalmol 2015;159:344-352.e1. https://doi.org/10.1016/j.ajo.2014.11.003
• 4. Matthews YY, Dean F, Lim MJ, et al. Pseudotumor cerebri syndrome in childhood: incidence, clinical profile and risk factors in a national prospective population-based cohort study. Arch Dis Child 2017;102:715-721. https://doi.org/10.1136/archdischild-2016-312238
• 5. Sinclair AJ, Walker EA, Burdon MA, et al. Cerebrospinal fluid corticosteroid levels and cortisol metabolism in patients with idiopathic intracranial hypertension: a link between 11β-HSD1 and intracranial pressure regulation? J Clin Endocrinol Metab 2010;95:5348-5356. https://doi.org/10.1210/jc.2010-0729
• 6. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1159-1165. https://doi.org/10.1212/WNL.0b013e3182a55f17
• 7. Kalyvas AV, Hughes M, Koutsarnakis C, et al. Efficacy, complications and cost of surgical interventions for idiopathic intracranial hypertension: a systematic review of the literature. Acta Neurochir 2017;159:33-49. https://doi.org/10.1007/s00701-016-3010-2
• 8. Gilbert AL, Chwalisz B, Mallery R, editors. Complications of optic nerve sheath fenestration as a treatment for idiopathic intracranial hypertension. Semin Ophthalmol 2018;33:36-41. https://doi.org/10.1080/08820538.2017.1353810
• 9. Moreau A, Lao KC, Farris B. Optic nerve sheath decompression: a surgical technique with minimal operative complications. J Neuroophthalmol 2014;34:34-38. https://doi.org/10.1097/WNO.0000000000000065
• 10. Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology 2000;107:1907-1912. https://doi.org/10.1016/s0161-6420(00)00340-7
• 11. Spoor TC, Ramocki JM, Madion MP, Wilkinson MJ. Treatment of pseudotumor cerebri by primary and secondary optic nerve sheath decompression. Am J Ophthalmol 1991;112:177-185. https://doi.org/10.1016/s0002-9394(14)76698-x
• 12. Per H, Canpolat M, Gümüş H, et al. Clinical spectrum of the pseudotumor cerebri in children: etiological, clinical features, treatment and prognosis. Brain Dev 2013;35:561-568. https://doi.org/10.1016/j.braindev.2012.08.008
• 13. Değerliyurt A, Teber S, Karakaya G, et al. Pseudotumor cerebri/idiopathic intracranial hypertension in children: an experience of a tertiary care hospital. Brain Dev 2014;36:690-699. https://doi.org/10.1016/j.braindev.2013.09.007
• 14. Distelmaier F, Göbel U, Vandemeulebroecke N, Mayatepek E, Rosenbaum T, Laws HJ. Secondary pseudotumor cerebri in pediatric oncology and hematology: an unpredictable condition of varying etiology. Pediatr Blood Cancer 2007;49:1029-1033. https://doi.org/10.1002/pbc.20783
• 15. Walker AE, Adamkiewicz JJ. Pseudotumor cerebri associated with prolonged corticosteroid therapy: reports of four cases. JAMA 1964;188:779-784. https://doi.org/10.1001/jama.1964.03060350005001
• 16. Levine A, Watemberg N, Hager H, Bujanover Y, Ballin A, Lerman Sagie TL. Benign intracranial hypertension associated with budesonide treatment in children with Crohn's disease. J Child Neurol 2001;16:458-461. https://doi.org/10.1177/088307380101600617
• 17. Katsuyama E, Sada Ke, Tatebe N, et al. Bilateral abducens nerve palsy due to idiopathic intracranial hypertension as an initial manifestation of systemic lupus erythematosus. Intern Med 2016;55:991-994. https://doi.org/10.2169/internalmedicine.55.5990
• 18. Mollan SP, Ball AK, Sinclair AJ, et al. Idiopathic intracranial hypertension associated with iron deficiency anaemia: a lesson for management. Eur Neurol 2009;62:105-108. https://doi.org/10.1159/000222781
• 19. Sébire G, Tabarki B, Saunders D, et al. Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. Brain 2005;128:477-489. https://doi.org/10.1093/brain/awh412