The Role of Ultrasonography for Assessment of Pseudotumor Cerebri Syndrome in the Emergency Department

Abstract

Pseudotumor cerebri syndrome (PTCS) is a condition of still obscure etiology, characterized by elevated cerebrospinal fluid (CSF) pressure but no detectable intracranial CSF compositional anomalies or abnormalities of the brain parenchyma. This syndrome generally affects adult obese individuals, especially females, but it has been frequently described also in the pediatric population. Pediatric PTCS has been reported in association with secondary factors (e.g., medical illness, infections, endocrine disturbances, drug intoxication) that seem to augment intracranial pressure in a still unexplained way. Clinical signs and symptoms of PTCS in children include headache and papilledema at the funduscopic examination. The diagnosis of PTCS in children and adults usually involves brain imaging to exclude intracranial mass and/or vascular anomalies; the lumbar puncture is also crucial to confirming elevated CSF pressure (>250 mm H₂O) and, lately, normal CSF contents at the chemical-physical examination. In recent years, ultrasonography has acquired an emerging role in the initial assessment of patient suspected to have PTCS. In fact, ocular ultrasounds allow the detection of optic disc swelling, suggestive of papilledema; additionally, ultrasonography allows the measurement of the retrobulbar optic nerve sheath diameter, which has been proposed as a noninvasive (indirect) method to assess the intracranial pressure. Ultrasound examination of the optic disc may therefore represent an important adjunct to funduscopic examination when assessing children with headache and/or visual disturbances, especially in the setting of the emergency department. We briefly discuss the application of ultrasonography in the assessment of pediatric patients with PTCS.

Keywords

idiopathic intracranial hypertension - pseudotumor cerebri - ultrasonography - optical coherence tomography - children - emergency department

• References

• 1 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
• 2 Radhakrishnan K, Ahlskog JE, Cross SA, Kurland LT, O'Fallon WM. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol 1993; 50 (1) 78-80
• 3 Salpietro V, Chimenz R, Arrigo T, Ruggieri M. Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a role for weight gain. J Pediatr 2013; 162 (5) 1084
• 4 Standridge SM. Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol 2010; 43 (6) 377-390
• 5 Salpietro V, Polizzi A, Bertè LF , et al. Idiopathic intracranial hypertension: a unifying neuroendocrine hypothesis through the adrenal-brain axis. Neuroendocrinol Lett 2012; 33 (6) 569-573
• 6 Khan MU, Khalid H, Salpietro V, Weber KT. Idiopathic intracranial hypertension associated with either primary or secondary aldosteronism. Am J Med Sci 2013; 346 (3) 194-198
• 7 Salpietro V, Polizzi A, Di Rosa G , et al. Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications. Int J Endocrinol 2014; 2014: 282489
• 8 Salpietro V, Ruggieri M. Pseudotumor cerebri pathophysiology: the likely role of aldosterone. Headache 2014; 54 (7) 1229
• 9 Salpietro V, Mankad K, Kinali M , et al. Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study. J Pediatr Endocrinol Metab 2014; 27 (1–2) 107-115
• 10 Salpietro V, Ruggieri M, Sancetta F , et al. New insights on the relationship between pseudotumor cerebri and secondary hyperaldosteronism in children. J Hypertens 2012; 30 (3) 629-630
• 11 Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105 (9) 1686-1693
• 12 Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32 (11) 1986-1993
• 13 Stone MB. Ultrasound diagnosis of papilledema and increased intracranial pressure in pseudotumor cerebri. Am J Emerg Med 2009; 27 (3) 376.e1-376.e2
• 14 Daulaire S, Fine L, Salmon M , et al. Ultrasound assessment of optic disc edema in patients with headache. Am J Emerg Med 2012; 30 (8) 1654.e1-1654.e4
• 15 Kimberly HH, Shah S, Marill K, Noble V. Correlation of optic nerve sheath diameter with direct measurement of intracranial pressure. Acad Emerg Med 2008; 15 (2) 201-204
• 16 Wu EH, Fagan MJ, Reinert SE, Diaz JA. Self-confidence in and perceived utility of the physical examination: a comparison of medical students, residents, and faculty internists. J Gen Intern Med 2007; 22 (12) 1725-1730
• 17 Tayal VS, Neulander M, Norton HJ, Foster T, Saunders T, Blaivas M. Emergency department sonographic measurement of optic nerve sheath diameter to detect findings of increased intracranial pressure in adult head injury patients. Ann Emerg Med 2007; 49 (4) 508-514
• 18 Newman WD, Hollman AS, Dutton GN, Carachi R. Measurement of optic nerve sheath diameter by ultrasound: a means of detecting acute raised intracranial pressure in hydrocephalus. Br J Ophthalmol 2002; 86 (10) 1109-1113
• 19 Geeraerts T, Launey Y, Martin L , et al. Ultrasonography of the optic nerve sheath may be useful for detecting raised intracranial pressure after severe brain injury. Intensive Care Med 2007; 33 (10) 1704-1711
• 20 Fujimoto JG, Pitris C, Boppart SA, Brezinski ME. Optical coherence tomography: an emerging technology for biomedical imaging and optical biopsy. Neoplasia 2000; 2 (1-2) 9-25
• 21 Krishnakumar D, Pickard JD, Czosnyka Z, Allen L, Parker A. Idiopathic intracranial hypertension in childhood: pitfalls in diagnosis. Dev Med Child Neurol 2014; 56 (8) 749-755