Related Experiment Video
Updated: Aug 26, 2026
Robot-Assisted Laparoscopic Splenectomy In Children: A Case Report with Literature Review
Published on: March 27, 2026
Pseudotumor cerebri in children with sickle cell disease: a case series
Michael Henry1, M Catherine Driscoll, Marijean Miller
1Department of Hematology/Oncology, Children's National Medical Center, Washington, DC 20010, USA.
Insights
Headache in pediatric sickle cell disease (SCD) can signal pseudotumor cerebri (PC). Early diagnosis and treatment of PC in SCD patients prevent vision loss.
Area of Science:
- Neurology
- Hematology
- Ophthalmology
Background:
- Headache is a common symptom in sickle cell disease (SCD), often linked to anemia or cerebrovascular issues.
- Pseudotumor cerebri (PC), also known as idiopathic intracranial hypertension, is a condition of increased intracranial pressure.
- Previous reports have linked PC to SCD-SC, but not specifically to pediatric SCD.
Observation:
- Three pediatric patients with SCD (SCD-SC and SCD-SS) presented with headaches.
- Lumbar puncture revealed elevated opening pressures with normal cerebrospinal fluid.
- Ophthalmologic exams showed bilateral papilledema, but no retinopathy or significant visual acuity changes.
Findings:
- Magnetic resonance imaging ruled out hydrocephalus, arteriopathy, and cerebral sinus thrombosis.
- All three patients were diagnosed with PC and treated with acetazolamide.
- Two patients experienced complete symptom resolution, and one showed improvement, with no permanent visual deficits.
Implications:
- These cases highlight PC as a potential cause of headache in pediatric SCD.
- Prompt recognition and management of PC are crucial to prevent long-term ophthalmologic complications in these patients.
- This study expands the understanding of PC in the context of pediatric sickle cell disease.
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.
