Pediatric Idiopathic Intracranial Hypertension Treatment & Management
- Author: Jasvinder Chawla, MD, MBA; Chief Editor: Amy Kao, MD more...
The care of patients with pseudotumor cerebri requires a multidisciplinary approach. Neurosurgical interventions are sometimes needed for diagnostic and treatment purposes. Prompt and accurate communication among specialists is necessary to ensure timely treatment and optimal outcomes.
Medical therapy appeared to be successful in treating pediatric pseudotumor cerebri in most patients. However, despite adequate treatment, children can rarely experience loss of visual field and acuity; thus, prompt diagnosis and management are important. Sometimes, the symptoms of idiopathic intracranial hypertension (IIH) resolve with the initial diagnostic lumbar puncture. If this occurs, no further medical treatment is required. When medical treatment is required, most children respond to medications such as steroids, acetazolamide, furosemide, or topiramate.
Surgical treatment such as optic nerve sheath fenestration, sinus stenting, and shunting procedures are indicated for children with severe headaches, visual loss, or both, despite maximal tolerated medical treatment.[3, 28]
The most serious potential complication is permanent visual loss and blindness. Low-salt diet and weight loss may be beneficial. The authors’ experience suggests that weight loss is difficult to achieve in the overweight adolescent.
Consultations with an optometrist, a neuro-ophthalmologist, and/or a pediatric neurologist may be indicated. Neuro-ophthalmology follow-up with frequent assessment of visual fields is indicated.
Although IIH has been recognized for over a century, the need remains for prospectively collected data to promote a better understanding of the etiology, risk factors, evaluative methods, and effective treatments for children with this syndrome.
In a study by Elder et al, data show that transverse sinus stenting, in conjunction with temporary CSF diversion, represents an appropriate treatment option in the acute setting.
Acetazolamide is administered at an initial dosage of 25 mg/kg/day, which is titrated upward until a clinical response is attained (maximum, 100 mg/kg/day). Electrolyte concentrations must be monitored to evaluate for the development of hypokalemia and acidosis. If the patient remains on treatment for more than 6 months, renal ultrasonography should be ordered to look for the presence of renal calculi. If acetazolamide is ineffective, prednisone can be given at a dosage of 2 mg/kg/day for 2 weeks, followed by a 2-week taper.
Topiramate is now being widely used in the treatment of migraine and IIH in adults. Topiramate functions as a carbonic anhydrase (CA) inhibitor and appears to be efficacious in the treatment of both conditions. This medication may prove to be useful in selected children with IIH.
Repeat lumbar puncture may help in some patients, but its invasiveness and the difficulty of performing it in children make it a less than ideal medical therapy. The reduction in pressures is often only transient.
A low-salt diet and weight reduction have been shown to be helpful in adult patients. If the child is obese, weight reduction may be beneficial.
Optic Nerve Sheath Fenestration and CSF Diversion
The main indications for surgical intervention to treat IIH are deterioration in vision and incapacitating headaches despite aggressive medical management. There are 2 main surgical approaches to the treatment of pediatric IIH: optic nerve sheath fenestration and CSF diversion (typically, lumboperitoneal shunting). The authors prefer optic nerve fenestration to lumboperitoneal shunting.
Optic nerve sheath fenestration (ONSF) has been shown to improve visual outcome. It yields better results in patients with acutely decompensating vision and papilledema. Lumboperitoneal shunting may relieve headache and reduce ICP in patients with IIH. The long-term visual outcome of patients treated with this procedure is unknown. Complications include infection and shunt obstruction. Low-pressure headaches have also been reported to develop as a result of lumboperitoneal shunting.
There are pros and cons for doing either ONSF or CSF diversion. However, the decision for ONSF versus CSF shunting is somewhat institution- and surgeon-dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache.
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].
Dave SB, Subramanian PS. Pseudotumor cerebri: an update on treatment options. Indian J Ophthalmol. 2014 Oct. 62(10):996-8. [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].