Pseudotumor Cerebri Syndrome and Renal Diseases in the Pediatric Population

AbstractPseudotumor 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.

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Pseudotumor cerebri syndrome in a patient with narcolepsy type 1

European Journal of Paediatric Neurology ◽

10.1016/j.ejpn.2017.11.002 ◽

2018 ◽

Vol 22 (1) ◽

pp. 194-198 ◽

Cited By ~ 1

Author(s):

Thomas Rossor ◽

Ming Lim ◽

Kirandeep VanDenEshof ◽

Paul Gringras

Keyword(s):

Pseudotumor Cerebri ◽

Pseudotumor Cerebri Syndrome

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Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement

Journal of Neurosurgery ◽

10.3171/jns.2003.98.5.1045 ◽

2003 ◽

Vol 98 (5) ◽

pp. 1045-1055 ◽

Cited By ~ 146

Author(s):

Brian K. Owler ◽

Geoffrey Parker ◽

G. Michael Halmagyi ◽

Victoria G. Dunne ◽

Verity Grinnell ◽

...

Keyword(s):

Stent Placement ◽

Pseudotumor Cerebri ◽

Venous Sinus ◽

Stent Insertion ◽

Benign Intracranial Hypertension ◽

Content Type ◽

Venous Sinuses ◽

Pseudotumor Cerebri Syndrome ◽

New Treatment ◽

Fine Print

Object. Pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. Pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment. Methods. Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures. Conclusions. Patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions.

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