Pediatric Idiopathic Intracranial Hypertension Clinical Presentation
- Author: Jasvinder Chawla, MD, MBA; Chief Editor: Amy Kao, MD more...
Common signs and symptoms of idiopathic intracranial hypertension (IIH) in the young include headache, vomiting, blurred vision, and diplopia.[16, 17]
Headaches are intermittent, diffuse, and worse at night; they may awaken the child and are often aggravated by sudden movement. Visual disturbances include transient visual obscurations, blurred vision, double vision, and photophobia. Diplopia is almost always horizontal (side by side) and is secondary to paresis of cranial nerve (CN) VI (the abducens nerve). It has been estimated that up to 50% of children with IIH have CN VI dysfunction.
Other symptoms of increased intracranial pressure (ICP) include lethargy, irritability, and vomiting. Nonspecific associated symptoms include neck stiffness, tinnitus, dizziness, clumsiness, and paresthesias.
The possible association of pseudotumor cerebri and signs and symptoms suggestive of varicella infection has previously been mentioned in a few case reports. Ravid S et al have described 3 immunocompetent children with pseudotumor cerebri as the only manifestation of varicella zoster virus reactivation. They have suggested considering varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection.
The general medical examination may reveal signs of otitis media or mastoiditis, which raise the possibility of venous sinus thrombosis. The presence of acne vulgaris should prompt an inquiry about the possible use of retinoic acid or tetracyclines. Physical findings of adrenal or thyroid dysfunction may also be present.
The neurologic examination typically yields normal results, with the exception of papilledema and weakness of one or both of the abducens nerves. Obeid M et al reported 10-week-old monozygotic twins with cystic fibrosis, facial palsy, and increased intracranial pressure. Low levels of vitamin A are associated with facial nerve paralysis and are at least partly implicated in the development of increased intracranial pressure in infants with cystic fibrosis. Other CN palsies have been reported on rare occasions.
Ophthalmoscopic (funduscopic) examination reveals optic disk nerve swelling (papilledema). The diagnosis of IIH should not be made in the absence of papilledema unless the patient has optic atrophy. Papilledema is typically bilateral but may be asymmetrical or unilateral. Initially, visual acuity is usually preserved, which helps the clinician to distinguish acute papilledema from optic neuritis.
Visual field testing is useful for both examination and monitoring. Perimetry can be used to evaluate response to therapy. Common field defects include enlargement of the blind spot, loss of the inferonasal portion and generalized constriction. Other defects include a variety of scotomas and altitudinal patterns of visual loss. Visual acuity assessment is helpful. Most patients will have some measurable visual loss that is often asymptomatic, usually occurs gradually, and improves with therapy. Komur et al have described coexisting optic disc drusen and idiopathic intracranial hypertension.
Serial photographs of the fundus may be taken for follow-up. A related article is Anterior Segment and Fundus Photography.
Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS. Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol. 2010 Apr. 34 Suppl 2:217-21. [Medline].
Jindal M, Hiam L, Raman A, Rejali D. Idiopathic intracranial hypertension in otolaryngology. Eur Arch Otorhinolaryngol. 2009 Jun. 266(6):803-6. [Medline].
Phillips PH. Pediatric pseudotumor cerebri. Int Ophthalmol Clin. 2012 Summer. 52(3):51-9, xii. [Medline].
Standridge SM. Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol. 2010 Dec. 43(6):377-90. [Medline].
Malm J, Kristensen B, Markgren P, Ekstedt J. CSF hydrodynamics in idiopathic intracranial hypertension: a long-term study. Neurology. 1992 Apr. 42(4):851-8. [Medline].
Farb RI, Vanek I, Scott JN, Mikulis DJ, Willinsky RA, Tomlinson G, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. 2003 May 13. 60(9):1418-24. [Medline].
Szewka AJ, Bruce BB, Newman NJ, Biousse V. Pediatric idiopathic intracranial hypertension and extreme childhood obesity: A comment on visual outcomes. J Pediatr. 2012 Nov. 161(5):972. [Medline].
Brara SM, Koebnick C, Porter AH, Langer-Gould A. Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr. 2012 Oct. 161(4):602-7. [Medline].
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 Feb. 49(1):87-91. [Medline].
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 Feb. 159(2):344-352.e1. [Medline].
Ertekin V, Selimoglu MA, Tan H. Pseudotumor Cerebri Due to Hypervitaminosis A or Hypervitaminosis D or Both in Alagille Syndrome. Headache. 2009 Jul 8. [Medline].
Tibussek D, Schneider DT, Vandemeulebroecke N, et al. Clinical spectrum of the pseudotumor cerebri complex in children. Childs Nerv Syst. 2009 Nov 10. [Medline].
Kelly SJ, O'Donnell T, Fleming JC, Einhaus S. Pseudotumor cerebri associated with lithium use in an 11-year-old boy. J AAPOS. 2009 Apr. 13(2):204-6. [Medline].
Wardly DE. Intracranial hypertension associated with obstructive sleep apnea: A discussion of potential etiologic factors. Med Hypotheses. 2014 Oct 19. 83(6):792-797. [Medline].
Soiberman U, Stolovitch C, Balcer LJ, Regenbogen M, Constantini S, Kesler A. Idiopathic intracranial hypertension in children: visual outcome and risk of recurrence. Childs Nerv Syst. 2011 Nov. 27(11):1913-8. [Medline].
Hacifazlioglu Eldes N, Yilmaz Y. Pseudotumour cerebri in children: Etiological, clinical features and treatment modalities. Eur J Paediatr Neurol. 2011 Nov 1. [Medline].
Incecik F, Hergüner MO, Altunbasak S. Evaluation of sixteen children with pseudotumor cerebri. Turk J Pediatr. 2011 Jan-Feb. 53(1):55-8. [Medline].
Ravid S, Shachor-Meyouhas Y, Shahar E, Kra-Oz Z, Kassis I. Reactivation of varicella presenting as pseudotumor cerebri: three cases and a review of the literature. Pediatr Neurol. 2012 Feb. 46(2):124-6. [Medline].
Obeid M, Price J, Sun L, et al. Facial palsy and idiopathic intracranial hypertension in twins with cystic fibrosis and hypovitaminosis A. Pediatr Neurol. 2011 Feb. 44(2):150-2. [Medline].
Wolf A, Hutcheson KA. Advances in evaluation and management of pediatric idiopathic intracranial hypertension. Curr Opin Ophthalmol. 2008 Sep. 19(5):391-7. [Medline].
Komur M, Sari A, Okuyaz C. Simultaneous papilledema and optic disc drusen in a child. Pediatr Neurol. 2012 Mar. 46(3):187-8. [Medline].
Horev A, Hallevy H, Plakht Y, Shorer Z, Wirguin I, Shelef I. Changes in Cerebral Venous Sinuses Diameter After Lumbar Puncture in Idiopathic Intracranial Hypertension: A Prospective MRI Study. J Neuroimaging. 2012 Aug 22. [Medline].
Shofty B, Ben-Sira L, Constantini S, Freedman S, Kesler A. Optic nerve sheath diameter on MR imaging: establishment of norms and comparison of pediatric patients with idiopathic intracranial hypertension with healthy controls. AJNR Am J Neuroradiol. 2012 Feb. 33(2):366-9. [Medline].
Stone MB. Ultrasound diagnosis of papilledema and increased intracranial pressure in pseudotumor cerebri. Am J Emerg Med. 2009 Mar. 27(3):376.e1-376.e2. [Medline].
Soler D, Cox T, Bullock P. Diagnosis and management of benign intracranial hypertension. Arch Dis Child. 1998 Jan. 78(1):89-94. [Medline].
Chern JJ, Tubbs RS, Gordon AS, Donnithorne KJ, Oakes WJ. Management of pediatric patients with pseudotumor cerebri. Childs Nerv Syst. 2012 Jan 19. [Medline].
Per H, Canpolat M, Gümüs H, et al. Clinical spectrum of the pseudotumor cerebri in children: Etiological, clinical features, treatment and prognosis. Brain Dev. 2012 Sep 13. [Medline].
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. 2013 Nov 28. [Medline].
Elder BD, Rory Goodwin C, Kosztowski TA, Radvany MG, Gailloud P, Moghekar A, et al. Venous sinus stenting is a valuable treatment for fulminant idiopathic intracranial hypertension. J Clin Neurosci. 2015 Jan 8. [Medline].
Spitze A, Lam P, Al-Zubidi N, Yalamanchili S, Lee AG. Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension. Indian J Ophthalmol. 2014 Oct. 62(10):1015-21. [Medline]. [Full Text].
Jion YI, Raff A, Grosberg BM, Evans RW. The Risk and Management of Kidney Stones From the Use of Topiramate and Zonisamide in Migraine and Idiopathic Intracranial Hypertension. Headache. 2014 Dec 9. [Medline].
Naarden MT, Schuitemaker A, Braakman HM, van Doormaal TP, Porro GL, Straver JS. [Idiopathic intracranial hypertension and obesity]. Ned Tijdschr Geneeskd. 2015. 159(0):A7980. [Medline].
Sinclair AJ, Woolley R, Mollan SP. Idiopathic intracranial hypertension. JAMA. 2014 Sep 10. 312(10):1059-60. [Medline].
Singleton J, Dagan A, Edlow JA, Hoffmann B. Real-time optic nerve sheath diameter reduction measured with bedside ultrasound after therapeutic lumbar puncture in a patient with idiopathic intracranial hypertension. Am J Emerg Med. 2014 Dec 19. [Medline].