This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Henry, M.
Right arrow Articles by Minniti, C. P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Henry, M.
Right arrow Articles by Minniti, C. P.
Related Collections
Right arrow Blood
PEDIATRICS Vol. 113 No. 3 March 2004, pp. e265-e269

ELECTRONIC ARTICLE

Pseudotumor Cerebri in Children With Sickle Cell Disease: A Case Series

Michael Henry, MD*, M. Catherine Driscoll, MD*, Marijean Miller, MD{ddagger}, Taeun Chang, MD§ and Caterina P. Minniti, MD*

* Departments of Hematology/Oncology
{ddagger} Ophthalmology
§ Neurology, Children’s National Medical Center, Washington, DC

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.

Key Words: pseudotumor cerebri • sickle cell disease • anemia • headache

Abbreviations: SCD, sickle cell disease • MRI, magnetic resonance imaging • PC, pseudotumor cerebri • CSF, cerebrospinal fluid • SC, hemoglobin SC • TCD, transcranial Doppler ultrasonography • MRA, magnetic resonance angiography • MRV, magnetic resonance venography • SS, hemoglobin SS

Received for publication Sep 8, 2003; Accepted Nov 20, 2003.




This article has been cited by other articles:


Home page
 Arch. Dis. Child.Home page
Journal Watch
Arch. Dis. Child., September 1, 2004; 89(9): 888 - 889.
[Full Text] [PDF]

Home page
 AAP Grand RoundsHome page
J. G. Millichap
Pseudotumor Cerebri and Sickle Cell Disease
AAP Grand Rounds, June 1, 2004; 11(6): 64 - 64.
[Full Text] [PDF]

Home page
 JWatch PediatricsHome page
More Than a Simple Headache: Pseudotumor Cerebri in Patients with Sickle Cell Disease
Journal Watch Pediatrics and Adolescent Medicine, May 10, 2004; 2004(510): 3 - 3.
[Full Text]