Download PDF
Semin Neurol 2020; 40(03): 286-293
DOI: 10.1055/s-0040-1708847
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Update in Pediatric Pseudotumor Cerebri Syndrome

Shannon J. Beres
1   Department of Neurology and Ophthalmology, Stanford University, Palo Alto, California
› Author Affiliations
Further Information

Publication History

Publication Date:
18 May 2020 (online)

    Permissions and Reprints

Abstract

Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences between prepubertal and postpubertal children, further elucidating the complex pathophysiology. Management requires brain imaging, lumbar puncture, and frequent eye exams with medical and rarely surgical treatment. Visual outcomes in children are favorable if caught early and management can be prolonged over years. Pediatric PTCS is different from adult PTCS in many ways, and this review will focus on the most updated definitions of the disease, theories of pathophysiology, management, and treatment in the pediatric population.

Keywords

pseudotumor cerebri syndrome - primary intracranial hypertension - idiopathic intracranial hypertension - papilledema - headache
 

  • References

  • 1 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 (08) 715-721
    CrossrefPubMedGoogle Scholar
  • 2 Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC. Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol 2017; 73: 42-47
    CrossrefPubMedGoogle Scholar
  • 3 Bursztyn LL, Sharan S, Walsh L, LaRoche GR, Robitaille J, De Becker I. Has rising pediatric obesity increased the incidence of idiopathic intracranial hypertension in children?. Can J Ophthalmol 2014; 49 (01) 87-91
    CrossrefPubMedGoogle Scholar
  • 4 Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007; 52 (06) 597-617
    CrossrefPubMedGoogle Scholar
  • 5 Bassan H, Berkner L, Stolovitch C, Kesler A. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 2008; 118 (04) 251-255
    PubMedGoogle Scholar
  • 6 Nonne M. Ueber falle vom symptomkomplex “tumor cerebri” mit ausgang in heilung. Dtsch Z Nervenheilkd 1904; 27: 169-216
    CrossrefPubMedGoogle Scholar
  • 7 Buchheit WA, Burton C, Haag B, Shaw D. Papilledema and idiopathic intracranial hypertension. N Engl J Med 1969; 280 (17) 938-942
    CrossrefPubMedGoogle Scholar
  • 8 Johnston I, Hawke S, Halmagyi M, Teo C. The pseudotumor syndrome. Disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly. Arch Neurol 1991; 48 (07) 740-747
    CrossrefPubMedGoogle Scholar
  • 9 Aylward SC. Pediatric idiopathic intracranial hypertension: a need for clarification. Pediatr Neurol 2013; 49 (05) 303-304
    CrossrefPubMedGoogle Scholar
  • 10 Beres SJ, Digre KB, Friedman DI, Liu GT. Pseudotumor cerebri syndrome is the best term for this condition. Pediatr Neurol 2018; 87: 9-10
    CrossrefPubMedGoogle Scholar
  • 11 Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937; 106 (04) 492-513
    CrossrefPubMedGoogle Scholar
  • 12 Smith JL. Whence pseudotumor cerebri?. J Clin Neuroophthalmol 1985; 5 (01) 55-56
    PubMedGoogle Scholar
  • 13 Wall M, Kupersmith MJ, Kieburtz KD. , et al; NORDIC Idiopathic Intracranial Hypertension Study Group. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol 2014; 71 (06) 693-701
    CrossrefPubMedGoogle Scholar
  • 14 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
    CrossrefPubMedGoogle Scholar
  • 15 Avery RA, Shah SS, Licht DJ. , et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363 (09) 891-893
    CrossrefPubMedGoogle Scholar
  • 16 Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial hypertension in children: etiologies, clinical features, and outcome. J Child Neurol 2015; 30 (12) 1562-1568
    CrossrefPubMedGoogle Scholar
  • 17 Gerstl L, Schoppe N, Albers L. , et al. Pediatric idiopathic intracranial hypertension - Is the fixed threshold value of elevated LP opening pressure set too high?. Eur J Paediatr Neurol 2017; 21 (06) 833-841
    CrossrefPubMedGoogle Scholar
  • 18 Delaney AC, Velarde A, Harper MB. , et al. Predictors of primary intracranial hypertension in children using a newly suggested opening pressure cutoff of 280 mm H2O. Pediatr Neurol 2019; 91: 27-33
    CrossrefPubMedGoogle Scholar
  • 19 Avery RA. Interpretation of lumbar puncture opening pressure measurements in children. J Neuroophthalmol 2014; 34 (03) 284-287
    CrossrefPubMedGoogle Scholar
  • 20 Janes LE, Sabino J, Matthews JA, Papadimitriou JC, Strome SE, Singh DP. Surgical management of craniofacial neurofibromatosis type 1 associated tumors. J Craniofac Surg 2013; 24 (04) 1273-1277
    CrossrefPubMedGoogle Scholar
  • 21 Inger HE, Rogers DL, McGregor ML, Aylward SC, Reem RE. Diagnostic criteria in pediatric intracranial hypertension. J AAPOS 2017; 21 (06) 492-495.e2
    CrossrefPubMedGoogle Scholar
  • 22 Tibussek D, Distelmaier F, Karenfort M, Harmsen S, Klee D, Mayatepek E. Probable pseudotumor cerebri complex in 25 children. Further support of a concept. Eur J Paediatr Neurol 2017; 21 (02) 280-285
    CrossrefPubMedGoogle Scholar
  • 23 Mollan SP, Davies B, Silver NC. , et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry 2018; 89 (10) 1088-1100
    CrossrefPubMedGoogle Scholar
  • 24 Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 1999; 127 (02) 178-182
    CrossrefPubMedGoogle Scholar
  • 25 Phillips PH, Repka MX, Lambert SR. Pseudotumor cerebri in children. J AAPOS 1998; 2 (01) 33-38
    CrossrefPubMedGoogle Scholar
  • 26 Balcer LJ, Liu GT, Forman S. , et al. Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology 1999; 52 (04) 870-872
    CrossrefPubMedGoogle Scholar
  • 27 Sheldon CA, Paley GL, Xiao R. , et al. Pediatric idiopathic intracranial hypertension: age, gender, and anthropometric features at diagnosis in a large, retrospective, multisite cohort. Ophthalmology 2016; 123 (11) 2424-2431
    CrossrefPubMedGoogle Scholar
  • 28 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 (02) 344-52.e1
    CrossrefPubMedGoogle Scholar
  • 29 Chiu AM, Chuenkongkaew WL, Cornblath WT. , et al. Minocycline treatment and pseudotumor cerebri syndrome. Am J Ophthalmol 1998; 126 (01) 116-121
    CrossrefPubMedGoogle Scholar
  • 30 Reeves GD, Doyle DA. Growth hormone treatment and pseudotumor cerebri: coincidence or close relationship?. J Pediatr Endocrinol Metab 2002; 15 (Suppl. 02) 723-730
    PubMedGoogle Scholar
  • 31 Darendeliler F, Karagiannis G, Wilton P. Headache, idiopathic intracranial hypertension and slipped capital femoral epiphysis during growth hormone treatment: a safety update from the KIGS database. Horm Res 2007; 68 (Suppl. 05) 41-47
    CrossrefPubMedGoogle Scholar
  • 32 Khan MU, Khalid H, Salpietro V, Weber KT. Idiopathic intracranial hypertension associated with either primary or secondary aldosteronism. Am J Med Sci 2013; 346 (03) 194-198
    CrossrefPubMedGoogle Scholar
  • 33 Sheldon CA, Kwon YJ, Liu GT, McCormack SE. An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics. Pediatr Res 2015; 77 (02) 282-289
    CrossrefPubMedGoogle Scholar
  • 34 Sinclair AJ, Onyimba CU, Khosla P. , et al. Corticosteroids, 11beta-hydroxysteroid dehydrogenase isozymes and the rabbit choroid plexus. J Neuroendocrinol 2007; 19 (08) 614-620
    CrossrefPubMedGoogle Scholar
  • 35 Sinclair AJ, Walker EA, Burdon MA. , et al. Cerebrospinal fluid corticosteroid levels and cortisol metabolism in patients with idiopathic intracranial hypertension: a link between 11beta-HSD1 and intracranial pressure regulation?. J Clin Endocrinol Metab 2010; 95 (12) 5348-5356
    CrossrefPubMedGoogle Scholar
  • 36 Kovarik JJ, Doshi PN, Collinge JE, Plager DA. Outcome of pediatric patients referred for papilledema. J AAPOS 2015; 19 (04) 344-348
    CrossrefPubMedGoogle Scholar
  • 37 Davis PL, Jay WM. Optic nerve head drusen. Semin Ophthalmol 2003; 18 (04) 222-242
    CrossrefPubMedGoogle Scholar
  • 38 Erkkilä H. Clinical appearance of optic disc drusen in childhood. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1975; 193 (01) 1-18
    CrossrefPubMedGoogle Scholar
  • 39 Malmqvist L, Li XQ, Eckmann CL. , et al. Optic disc drusen in children: the Copenhagen Child Cohort 2000 Eye Study. J Neuroophthalmol 2018; 38 (02) 140-146
    CrossrefPubMedGoogle Scholar
  • 40 Friedman AH, Gartner S, Modi SS. Drusen of the optic disc. A retrospective study in cadaver eyes. Br J Ophthalmol 1975; 59 (08) 413-421
    CrossrefPubMedGoogle Scholar
  • 41 Kurz-Levin MM, Landau K. A comparison of imaging techniques for diagnosing drusen of the optic nerve head. Arch Ophthalmol 1999; 117 (08) 1045-1049
    CrossrefPubMedGoogle Scholar
  • 42 Malmqvist L, Bursztyn L, Costello F. , et al. The optic disc drusen studies consortium recommendations for diagnosis of optic disc drusen using optical coherence tomography. J Neuroophthalmol 2018; 38 (03) 299-307
    CrossrefPubMedGoogle Scholar
  • 43 Ramirez H, Blatt ES, Hibri NS. Computed tomographic identification of calcified optic nerve drusen. Radiology 1983; 148 (01) 137-139
    CrossrefPubMedGoogle Scholar
  • 44 Gili P, Flores-Rodríguez P, Martin-Ríos MD, Carrasco Font C. Anatomical and functional impairment of the nerve fiber layer in patients with optic nerve head drusen. Graefes Arch Clin Exp Ophthalmol 2013; 251 (10) 2421-2428
    CrossrefPubMedGoogle Scholar
  • 45 Pineles SL, Arnold AC. Fluorescein angiographic identification of optic disc drusen with and without optic disc edema. J Neuroophthalmol 2012; 32 (01) 17-22
    CrossrefPubMedGoogle Scholar
  • 46 Lee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of ocular coherence tomography in children with idiopathic intracranial hypertension-a single-center experience. Pediatr Neurol 2016; 58: 101-106.e1
    CrossrefPubMedGoogle Scholar
  • 47 El-Dairi MA, Holgado S, O'Donnell T, Buckley EG, Asrani S, Freedman SF. Optical coherence tomography as a tool for monitoring pediatric pseudotumor cerebri. J AAPOS 2007; 11 (06) 564-570
    CrossrefPubMedGoogle Scholar
  • 48 Merchant KY, Su D, Park SC. , et al. Enhanced depth imaging optical coherence tomography of optic nerve head drusen. Ophthalmology 2013; 120 (07) 1409-1414
    CrossrefPubMedGoogle Scholar
  • 49 Katz BJ, Pomeranz HD. Visual field defects and retinal nerve fiber layer defects in eyes with buried optic nerve drusen. Am J Ophthalmol 2006; 141 (02) 248-253
    CrossrefPubMedGoogle Scholar
  • 50 Kulkarni KM, Pasol J, Rosa PR, Lam BL. Differentiating mild papilledema and buried optic nerve head drusen using spectral domain optical coherence tomography. Ophthalmology 2014; 121 (04) 959-963
    CrossrefPubMedGoogle Scholar
  • 51 Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of pediatric intracranial hypertension from the Intracranial Hypertension Registry. Pediatr Neurol 2016; 61: 76-82
    CrossrefPubMedGoogle Scholar
  • 52 Hamedani AG, Witonsky KFR, Cosico M. , et al. Headache characteristics in children with pseudotumor cerebri syndrome, elevated opening pressure without papilledema, and normal opening pressure: a retrospective cohort study. Headache 2018; 58 (09) 1339-1346
    CrossrefPubMedGoogle Scholar
  • 53 Mallery RM, Friedman DI, Liu GT. Headache and the pseudotumor cerebri syndrome. Curr Pain Headache Rep 2014; 18 (09) 446
    CrossrefPubMedGoogle Scholar
  • 54 Friedman DI, Quiros PA, Subramanian PS. , et al; and the NORDIC IIHTT Study Group. Headache in idiopathic intracranial hypertension: findings from the Idiopathic Intracranial Hypertension Treatment Trial. Headache 2017; 57 (08) 1195-1205
    CrossrefPubMedGoogle Scholar
  • 55 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
    CrossrefPubMedGoogle Scholar
  • 56 Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol 1994; 118 (01) 88-96
    CrossrefPubMedGoogle Scholar
  • 57 Johnson LN, Krohel GB, Madsen RW, March Jr GA. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology 1998; 105 (12) 2313-2317
    CrossrefPubMedGoogle Scholar
  • 58 Inger HE, McGregor ML, Jordan CO. , et al. Surgical intervention in pediatric intracranial hypertension: incidence, risk factors, and visual outcomes. J AAPOS 2019; 23 (02) 96.e1-96.e7
    CrossrefPubMedGoogle Scholar
  • 59 Gospe III SM, Bhatti MT, El-Dairi MA. Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension. Br J Ophthalmol 2016; 100 (04) 505-509
    CrossrefPubMedGoogle Scholar
  • 60 Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology 2000; 107 (10) 1907-1912
    CrossrefPubMedGoogle Scholar
  • 61 Alsuhaibani AH, Carter KD, Nerad JA, Lee AG. Effect of optic nerve sheath fenestration on papilledema of the operated and the contralateral nonoperated eyes in idiopathic intracranial hypertension. Ophthalmology 2011; 118 (02) 412-414
    CrossrefPubMedGoogle Scholar
  • 62 Plotnik JL, Kosmorsky GS. Operative complications of optic nerve sheath decompression. Ophthalmology 1993; 100 (05) 683-690
    CrossrefPubMedGoogle Scholar
  • 63 Anderson IA, Saukila LF, Robins JMW. , et al. Factors associated with 30-day ventriculoperitoneal shunt failure in pediatric and adult patients. J Neurosurg 2018; 130 (01) 145-153
    PubMedGoogle Scholar
  • 64 Ahmed RM, Wilkinson M, Parker GD. , et al. Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol 2011; 32 (08) 1408-1414
    CrossrefPubMedGoogle Scholar
  • 65 Radvany MG, Solomon D, Nijjar S. , et al. Visual and neurological outcomes following endovascular stenting for pseudotumor cerebri associated with transverse sinus stenosis. J Neuroophthalmol 2013; 33 (02) 117-122
    CrossrefPubMedGoogle Scholar
  • 66 Kumpe DA, Bennett JL, Seinfeld J, Pelak VS, Chawla A, Tierney M. Dural sinus stent placement for idiopathic intracranial hypertension. J Neurosurg 2012; 116 (03) 538-548
    CrossrefPubMedGoogle Scholar
  • 67 Ahmed RM, Zmudzki F, Parker GD, Owler BK, Halmagyi GM. Transverse sinus stenting for pseudotumor cerebri: a cost comparison with CSF shunting. AJNR Am J Neuroradiol 2014; 35 (05) 952-958
    CrossrefPubMedGoogle Scholar
  • 68 Ravid S, Shahar E, Schif A, Yehudian S. Visual outcome and recurrence rate in children with idiopathic intracranial hypertension. J Child Neurol 2015; 30 (11) 1448-1452
    CrossrefPubMedGoogle Scholar
  • 69 Agarwal A, Vibha D, Prasad K. , et al. Predictors of poor visual outcome in patients with Idiopathic Intracranial Hypertension (IIH): an ambispective cohort study. Clin Neurol Neurosurg 2017; 159: 13-18
    CrossrefPubMedGoogle Scholar