The Acetazolamide Challenge: A Tool for Surgical Decision Making and Predicting Surgical Outcome in Patients with Arachnoid Cysts

Abstract

Background and Study Aims Arachnoid cysts (ACs) are benign congenital malformations of the arachnoid that may present with either localizing or nonspecific symptoms. ACs with mass effect are detected frequently in asymptomatic patients. Conversely, symptomatic patients may present without imaging signs of a focal mass effect that emphasizes the difficulty of relying on imaging as the sole criteria in surgical selection. We hypothesize that symptomatic AC may be related to the elevated fluid pressure within the cyst on surrounding structures. Thus reducing the amount of fluid in the cyst or surrounding cerebrospinal fluid (CSF) by using acetazolamide (AZM), a carbonic anhydrase inhibitor known to reduce CSF production, might mimic surgical decompression and therefore could serve as a decision-making tool in patients with ACs.

Methods and Patients A total of 103 patients with radiographically proven ACs were initially identified. Twenty (19.4%) were symptomatic and underwent a trial of oral AZM. Data were collected meeting inclusion/exclusion criteria for this cohort study and analyzed retrospectively/prospectively.

Results Overall, 17 patients were able to tolerate the AZM and had at least some subjective improvement in their symptoms during the AZM challenge and underwent surgical therapy. Surgery was beneficial in 16 patients (94.1%). Following surgery, symptoms resolved in 13 patients (76.5%) and improved in 3 (17.6%).

Conclusion The AZM challenge may support the clinical decision to recommend surgery in those patients whose symptoms improve during AZM therapy.

Keywords

surgery - endoscopic - shunt - acetazolamide - arachnoid cyst

• References

• 1 Wester K. Peculiarities of intracranial arachnoid cysts: location, sidedness, and sex distribution in 126 consecutive patients. Neurosurgery 1999; 45 (4) 775-779
• 2 Clavel M, Taborga FG, Onzain I. Arachnoid cysts as a cause of dementia in the elderly. Acta Neurochir (Wien) 1985; 78 01/02 28-32
• 3 Colameco S, DiTomasso RA. Arachnoid cyst associated with psychological disturbance. J Med Soc N J 1982; 79 (3) 209-210
• 4 De Volder AG, Michel C, Thauvoy C, Willems G, Ferrière G. Brain glucose utilisation in acquired childhood aphasia associated with a sylvian arachnoid cyst: recovery after shunting as demonstrated by PET. J Neurol Neurosurg Psychiatry 1994; 57 (3) 296-300
• 5 Lang W, Lang M, Kornhuber A, Gallwitz A, Kriebel J. Neuropsychological and neuroendocrinological disturbances associated with extracerebral cysts of the anterior and middle cranial fossa. Eur Arch Psychiatry Neurol Sci 1985; 235 (1) 38-41
• 6 Raeder MB, Helland CA, Hugdahl K, Wester K. Arachnoid cysts cause cognitive deficits that improve after surgery. Neurology 2005; 64 (1) 160-162
• 7 Sgouros S, Chapman S. Congenital middle fossa arachnoid cysts may cause global brain ischaemia: a study with 99Tc-hexamethylpropyleneamineoxime single photon emission computerised tomography scans. Pediatr Neurosurg 2001; 35 (4) 188-194
• 8 Soukup VM, Patterson J, Trier TT, Chen JW. Cognitive improvement despite minimal arachnoid cyst decompression. Brain Dev 1998; 20 (8) 589-593
• 9 Wester K, Hugdahl K. Arachnoid cysts of the left temporal fossa: impaired preoperative cognition and postoperative improvement. J Neurol Neurosurg Psychiatry 1995; 59 (3) 293-298
• 10 Wester K, Hugdahl K. Verbal laterality and handedness in patients with intracranial arachnoid cysts. J Neurol 2003; 250 (1) 36-41
• 11 Al-Holou WN, Yew AY, Boomsaad ZE, Garton HJ, Muraszko KM, Maher CO. Prevalence and natural history of arachnoid cysts in children. J Neurosurg Pediatr 2010; 5 (6) 578-585
• 12 Erman T, Göçer AI, Tuna M , et al. Intracranial arachnoid cysts: clinical features and management of 35 cases and review of the literature. Neurosurg Q 2004; 14: 84-89
• 13 Katzman GL, Dagher AP, Patronas NJ. Incidental findings on brain magnetic resonance imaging from 1000 asymptomatic volunteers. JAMA 1999; 282 (1) 36-39
• 14 Kim BS, Illes J, Kaplan RT, Reiss A, Atlas SW. Incidental findings on pediatric MR images of the brain. AJNR Am J Neuroradiol 2002; 23 (10) 1674-1677
• 15 Weber F, Knopf H. Incidental findings in magnetic resonance imaging of the brains of healthy young men. J Neurol Sci 2006; 240 01/02 81-84
• 16 Daneyemez M, Gezen F, Akbörü M, Sirin S, Ocal E. Presentation and management of supratentorial and infratentorial arachnoid cysts. Review of 25 cases. J Neurosurg Sci 1999; 43 (2) 115-121 ; discussion 122–123
• 17 Koch CA, Voth D, Kraemer G, Schwarz M. Arachnoid cysts: does surgery improve epileptic seizures and headaches?. Neurosurg Rev 1995; 18 (3) 173-181
• 18 Mayr U, Aichner F, Bauer G, Mohsenipour I, Pallua A. Supratentorial extracerebral cysts of the middle cranial fossa. A report of 23 consecutive cases of the so-called temporal lobe agenesis syndrome. Neurochirurgia (Stuttg) 1982; 25 (2) 51-56
• 19 Oberbauer RW, Haase J, Pucher R. Arachnoid cysts in children: a European co-operative study. Childs Nerv Syst 1992; 8 (5) 281-286
• 20 Sommer IE, Smit LM. Congenital supratentorial arachnoidal and giant cysts in children: a clinical study with arguments for a conservative approach. Childs Nerv Syst 1997; 13 (1) 8-12
• 21 Elhammady MS, Bhatia S, Ragheb J. Endoscopic fenestration of middle fossa arachnoid cysts: a technical description and case series. Pediatr Neurosurg 2007; 43 (3) 209-215
• 22 Galassi E, Tognetti F, Frank F, Fagioli L, Nasi MT, Gaist G. Infratentorial arachnoid cysts. J Neurosurg 1985; 63 (2) 210-217
• 23 Helland CA, Wester K. A population based study of intracranial arachnoid cysts: clinical and neuroimaging outcomes following surgical cyst decompression in adults. J Neurol Neurosurg Psychiatry 2007; a 78 (10) 1129-1135
• 24 Hoffman HJ, Hendrick EB, Humphreys RP, Armstrong EA. Investigation and management of suprasellar arachnoid cysts. J Neurosurg 1982; 57 (5) 597-602
• 25 Kang JK, Lee KS, Lee IW , et al. Shunt-independent surgical treatment of middle cranial fossa arachnoid cysts in children. Childs Nerv Syst 2000; 16 (2) 111-116
• 26 Levy ML, Wang M, Aryan HE, Yoo K, Meltzer H. Microsurgical keyhole approach for middle fossa arachnoid cyst fenestration. Neurosurgery 2003; 53 (5) 1138-1144 ; discussion 1144–1145
• 27 Marin-Sanabria EA, Yamamoto H, Nagashima T, Kohmura E. Evaluation of the management of arachnoid cyst of the posterior fossa in pediatric population: experience over 27 years. Childs Nerv Syst 2007; 23 (5) 535-542
• 28 Pradilla G, Jallo G. Arachnoid cysts: case series and review of the literature. Neurosurg Focus 2007; 22 (2) E7
• 29 Shim KW, Lee YH, Park EK, Park YS, Choi JU, Kim DS. Treatment option for arachnoid cysts. Childs Nerv Syst 2009; 25 (11) 1459-1466
• 30 Spacca B, Kandasamy J, Mallucci CL, Genitori L. Endoscopic treatment of middle fossa arachnoid cysts: a series of 40 patients treated endoscopically in two centres. Childs Nerv Syst 2010; 26 (2) 163-172
• 31 Yang SH, Lee KS, Sung JH, Son BC, Jeun SS, Kang JK. Surgical decompression of supratentorial arachnoid cysts in pediatric patients younger than one year. Pediatr Neurosurg 2008; 44 (6) 465-470
• 32 Tamburrini G, Dal Fabbro M, Di Rocco C. Sylvian fissure arachnoid cysts: a survey on their diagnostic workout and practical management. Childs Nerv Syst 2008; 24 (5) 593-604
• 33 Di Rocco C, Tamburrini G, Caldarelli M, Velardi F, Santini P. Prolonged ICP monitoring in Sylvian arachnoid cysts. Surg Neurol 2003; 60 (3) 211-218
• 34 Carrion E, Hertzog JH, Medlock MD, Hauser GJ, Dalton HJ. Use of acetazolamide to decrease cerebrospinal fluid production in chronically ventilated patients with ventriculopleural shunts. Arch Dis Child 2001; 84 (1) 68-71
• 35 Aarhus M, Helland CA, Lund-Johansen M, Wester K, Knappskog PM. Microarray-based gene expression profiling and DNA copy number variation analysis of temporal fossa arachnoid cysts. Cerebrospinal Fluid Res 2010; 7: 6
• 36 Helland CA, Wester K. Monozygotic twins with mirror image cysts: indication of a genetic mechanism in arachnoid cysts?. Neurology 2007; b 69 (1) 110-111
• 37 Go KG, Houthoff HJ, Blaauw EH, Havinga P, Hartsuiker J. Arachnoid cysts of the sylvian fissure. Evidence of fluid secretion. J Neurosurg 1984; 60 (4) 803-813
• 38 Helland CA, Aarhus M, Knappskog P , et al. Increased NKCC1 expression in arachnoid cysts supports secretory basis for cyst formation. Exp Neurol 2010; 224 (2) 424-428
• 39 World Health Organization. Headache disorders. Fact sheet No277. 2012. Available at: http://www.who.int/mediacentre/factsheets/fs277/en/ . Accessed: May 5, 2015
• 40 Dyck P, Gruskin P. Supratentorial arachnoid cysts in adults. A discussion of two cases from a pathophysiologic and surgical perspective. Arch Neurol 1977; 34 (5) 276-279
• 41 Smith RA, Smith WA. Arachnoid cysts of the middle cranial fossa. Surg Neurol 1976; 5 (4) 246-252
• 42 Sandberg DI, McComb JG, Krieger MD. Chemical analysis of fluid obtained from intracranial arachnoid cysts in pediatric patients. J Neurosurg 2005; 103 (5, Suppl): 427-432
• 43 Berle M, Wester KG, Ulvik RJ , et al. Arachnoid cysts do not contain cerebrospinal fluid: a comparative chemical analysis of arachnoid cyst fluid and cerebrospinal fluid in adults. Cerebrospinal Fluid Res 2010; 7: 8
• 44 Friedman A, Levinson A. Cerebrospinal fluid inorganic phosphorus in normal and pathologic conditions. AMA Arch Neurol Psychiatry 1955; 74 (4) 424-440
• 45 Wall M, McDermott MP, Kieburtz KD , et al; NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA 2014; 311 (16) 1641-1651