Comparative retrospective analysis of patients with idiopathic normal pressure hydrocephalus and aqueductal web-related aqueductal stenosis.

Year 2023, Volume: 9 Issue: 6 - November 2023, 1464 - 1473, 04.11.2023

Alper Türkkan , Pınar Eser Ocak , Oğuz Altunyuva , Buket Sönmez , Rifat Özpar , Ahmet Bekar

https://doi.org/10.18621/eurj.1347626

Abstract

Objectives: Aquaductal web (AW) is a special form of aqueductal stenosis with similar clinical presentation with idiopathic normal pressure hydrocephalus (iNPH). iNPH is indeed a communicating hydrocephalus syndrome whereas AW is a noncommunicating subtype. Here, we aimed to investigate the similarities and differences between these two different chronic hydrocephalus syndromes in terms of clinical signs and symptoms, response to shunt treatment and postoperative complications.

Methods: Forty-one patients who underwent shunt operation with the diagnosis of iNPH or AW at our clinic between January 2010-May 2019 were retrospectively analyzed. Patients were evaluated by age, gender, clinical sign and symptoms, comorbidities, intraoperative and postoperative complications, and early and late postoperative outpatient follow-up findings.

Results: Twenty-six patients were classified as iNPH group and 15 patients as AW group. Patients in the AW group were significantly younger (45.5 ± 15.6 years vs. 60.3 ± 15.4 years) than the iNPH group (p = 0.006). There was no statistical difference between the groups in terms of subdural effusion formation, need for shunt revision (p = 1.000). Chronic hydrocephalus symptoms regressed in 23 (88.5%) patients in the NPH group, and at least one of them improved. symptoms. This rate was 66.7% (n = 10) in the AW group. Both groups showed similar clinical improvement with VPS (p = 0.1169).

Conclusions: The placement of ventriculoperitoenal shunt is widely used in the treatment of iNPH. As iNPH and AW has clinical similarities despite the discrepancies between underlying pathophysiological mechanisms and both clinical entities respond similarly to shunt treatment we advocate VPS surgery in the management of AW as well.

Keywords

Normal pressure hydrocephalus, aqueductal stenosis, ventriculoperitoneal (VP) shunt.

References

• 1. Bådagård H, Braun M, Nilsson D, Stridh L, Virhammar J. Negative predictors of shunt surgery outcome in normal pressure hydrocephalus. Acta Neurol Scand 2020;141:219-25.
• 2. Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 1965;2:307-27.
• 3. Skalický P, Mládek A, Vlasák A, De Lacy P, Beneš V, Bradáč O. Normal pressure hydrocephalus-an overview of pathophysiological mechanisms and diagnostic procedures. Neurosurg Rev 2020;43:1451-64.
• 4. Nakajima M, Yamada S, Miyajima M, Ishii K, Kuriyama N, Kazui H, et al.; Research Committee of Idiopathic Normal Pressure Hydrocephalus. Guidelines for management of idiopathic normal pressure hydrocephalus (third edition): endorsed by the Japanese Society of Normal Pressure Hydrocephalus. Neurol Med Chir (Tokyo) 2021;61:63-97.
• 5. Spina S, Laws SM. Insights into the pathogenesis of normal-pressure hydrocephalus. Neurology 2019;92:933-4.
• 6. Jaraj D, Rabiei K, Marlow T, Jensen C, Skoog I, Wikkelsø C. Prevalence of idiopathic normal-pressure hydrocephalus. Neurology 2014;82:1449-54.
• 7. Kitagaki H, Mori E, Ishii K, Yamaji S, Hirono N, Imamura T. CSF spaces in idiopathic normal pressure hydrocephalus: morphology and volumetry. AJNR Am J Neuroradiol 1998;19:1277-84.
• 8. Ryska P, Slezak O, Eklund A, Malm J, Salzer J, Zizka J. Radiological markers of idiopathic normal pressure hydrocephalus: Relative comparison of their diagnostic performance. J Neurol Sci 2020;408:116581.
• 9. Capone PM, Bertelson JA, Ajtai B. Neuroimaging of normal pressure hydrocephalus and hydrocephalus. Neurol Clin 2020;38:171-83.
• 10. Kuriyama N, Tokuda T, Miyamoto J, Takayasu N, Kondo M, Nakagawa M. Retrograde jugular flow associated with idiopathic normal pressure hydrocephalus. Ann Neurol 2008;64:217-21.
• 11. Jeppsson A, Wikkelsö C, Blennow K, Zetterberg H, Constantinescu R, Remes AM, et al. CSF biomarkers distinguish idiopathic normal pressure hydrocephalus from its mimics. J Neurol Neurosurg Psychiatry 2019;90:1117-23.
• 12. Krahulik D, Vaverka M, Hrabalek L, Hampl M, Halaj M, Jablonsky J, et al. Ventriculoperitoneal shunt in treating of idiopathic normal pressure hydrocephalus-single-center study. Acta Neurochir (Wien) 2020;162:1-7.
• 13. Parekh ND, Prabhu SP. Aqueductal web causing obstructive hydrocephalus demonstrated on sagittal FIESTA sequence. Pediatr Radiol 2010;40 Suppl 1:154.
• 14. Ciraolo L, Mascalchi M, Bucciolini M, Dal Pozzo G. Fast multiphase MR imaging of aqueductal CSF flow: 1. Study of healthy subjects. AJNR Am J Neuroradiol 1990;11:589-96.
• 15. Fritsch MJ, Kienke S, Mehdorn HM. Endoscopic aqueductoplasty: stent or not to stent? Childs Nerv Syst 2004;20:137-42.
• 16. Giordan E, Palandri G, Lanzino G, Murad MH, Elder BD. Outcomes and complications of different surgical treatments for idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis. J Neurosurg 2018;131:1024-36.
• 17. Alkan Ö, Çekinmez M, Tokmak N, Demir Ş, Yıldırım T. [Obstructive hydrocephallus due to aqueductal web]. J Nervous Sys Surgery 2009;2:37-41. [Article in Turkish]
• 18. Chen G, Zheng J, Xiao Q, Liu Y. Application of phase-contrast cine magnetic resonance imaging in endoscopic aqueductoplasty. Exp Ther Med 2013;5:1643-8.
• 19. Matsuda M, Shibuya S, Oikawa T, Murakami K, Mochizuki H. [A case of late-onset aqueductal membranous occlusion and a successful treatment with neuro-endoscopic surgery]. Rinsho Shinkeigaku 2011;51:590-4. [Article in Japanese]
• 20. Terada Y, Yamamoto M, Motoie R, Matsui Y, Katsuki T, Mori N, et al. Hydrocephalus resulting from late-onset aqueductal membranous occlusion: a case report and review of the literature. World Neurosurg 2020;137:345-9.
• 21. Woodworth GF, McGirt MJ, Williams MA, Rigamonti D. Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery 2009;64:919-25.
• 22. Nikaido Y, Urakami H, Akisue T, Okada Y, Katsuta N, Kawami Y, et al. Associations among falls, gait variability, and balance function in idiopathic normal pressure hydrocephalus. Clin Neurol Neurosurg 2019;183:105385.
• 23. Wang Z, Zhang Y, Hu F, Ding J, Wang X. Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus. CNS Neurosci Ther 2020;26:1230-40.
• 24. Murphy C, Vassallo G, Burkitt-Wright E, Hupton E, Eelloo J, Lewis L, et al. A retrospective regional study of aqueduct stenosis and fourth ventricle outflow obstruction in the paediatric complex neurofibromatosis type 1 population; Aetiology, clinical presentation and management. Clin Neurol Neurosurg 2020;193:105791.
• 25. Kazui H, Miyajima M, Mori E, Ishikawa M, Hirai O, Kuwana N, et al. Lumboperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (SINPHONI-2): an open-label randomised trial. Lancet Neurol 2015;14:585-94.
• 26. Hashimoto M, Ishikawa M, Mori E, Kuwana N. Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: a prospective cohort study. Cerebrospinal Fluid Res 2010;7:18.
• 27. Park JE, Ju H, Im K, Kwon KY. Revisiting the diagnostic value of Evans’ index: lessons from an unusual case of normal pressure hydrocephalus with Evans’ index less than 0.3. Neurol Sci 2019;40:2637-9.
• 28. Luikku AJ, Hall A, Nerg O, Koivisto AM, Hiltunen M, Helisalmi S, et al. Predicting development of Alzheimer’s disease in patients with shunted idiopathic normal pressure hydrocephalus. J Alzheimers Dis 2019;71:1233-43.
• 29. Scholz R, Lemcke J, Meier U, Stengel D. Efficacy and safety of programmable compared with fixed anti-siphon devices for treating idiopathic normal-pressure hydrocephalus (iNPH) in adults - SYGRAVA: study protocol for a randomized trial. Trials 2018;19:566.
• 30. Fukuhara T, Vorster SJ, Ruggieri P, Luciano MG. Third ventriculostomy patency: comparison of findings at cine phase-contrast MR imaging and at direct exploration. AJNR Am J Neuroradiol 1999;20:1560-6.