Pseudotumor cerebri in children with sickle cell disease: a case series.

Michael Henry, M. Catherine Driscoll, Marijean Miller, Taeun Chang, Caterina P. Minniti

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Headache is a frequent symptom in sickle cell disease (SCD) that usually is attributable to anemia or cerebrovascular disease. We report 3 pediatric patients with SCD (1 patient with SCD-SC and 2 patients with SCD-SS) who presented with headache and were diagnosed with pseudotumor cerebri (PC). All 3 patients had elevated opening pressures during a lumbar puncture with normal cerebrospinal fluid studies. Magnetic resonance imaging revealed no evidence of hydrocephalus or arteriopathy in all 3 cases. Magnetic resonance venograms performed in 2 of the patients at diagnosis revealed no evidence of cerebral sinus thrombosis. Each patient received a thorough ophthalmologic examination. A diagnostic funduscopic examination revealed bilateral papilledema without signs of retinopathy in all 3 patients. There were no clinically significant changes in visual acuity or abnormalities of color vision in any patient. Goldmann or Humphrey visual-field assessment was abnormal only in patient 1, who demonstrated bilaterally enlarged blind spots at diagnosis and later developed reduced sensitivity in the inferomedial quadrant of the left eye in an arcuate pattern (which later resolved). The diagnosis of PC was made in all 3 patients, and acetazolamide treatment was started. Two of the patients' symptoms resolved completely with medical treatment, whereas the third patient's symptoms improved. None of these patients had permanent visual-field deficits as a result of their syndrome. PC has been reported in several other types of anemia including SCD-SC, but these cases are the first reported in conjunction with pediatric SCD. Early recognition of the signs and symptoms of PC in patients with SCD who present with headache can expedite proper diagnosis and treatment and prevent long-term ophthalmologic sequelae.

Original languageEnglish (US)
JournalPediatrics
Volume113
Issue number3 Pt 1
StatePublished - Mar 2004
Externally publishedYes

Fingerprint

Pseudotumor Cerebri
Sickle Cell Anemia
Headache
Visual Fields
Anemia
Pediatrics
Intracranial Sinus Thrombosis
Cerebrovascular Disorders
Intracranial Thrombosis
Color Vision
Papilledema
Acetazolamide
Spinal Puncture
Delayed Diagnosis
Optic Disk
Hydrocephalus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Henry, M., Driscoll, M. C., Miller, M., Chang, T., & Minniti, C. P. (2004). Pseudotumor cerebri in children with sickle cell disease: a case series. Pediatrics, 113(3 Pt 1).

Pseudotumor cerebri in children with sickle cell disease : a case series. / Henry, Michael; Driscoll, M. Catherine; Miller, Marijean; Chang, Taeun; Minniti, Caterina P.

In: Pediatrics, Vol. 113, No. 3 Pt 1, 03.2004.

Research output: Contribution to journalArticle

Henry, M, Driscoll, MC, Miller, M, Chang, T & Minniti, CP 2004, 'Pseudotumor cerebri in children with sickle cell disease: a case series.', Pediatrics, vol. 113, no. 3 Pt 1.
Henry M, Driscoll MC, Miller M, Chang T, Minniti CP. Pseudotumor cerebri in children with sickle cell disease: a case series. Pediatrics. 2004 Mar;113(3 Pt 1).
Henry, Michael ; Driscoll, M. Catherine ; Miller, Marijean ; Chang, Taeun ; Minniti, Caterina P. / Pseudotumor cerebri in children with sickle cell disease : a case series. In: Pediatrics. 2004 ; Vol. 113, No. 3 Pt 1.
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