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Pseudotumor cerebri pediatric rates are rising


 

Pseudotumor cerebri, benign intracranial hypertension, and idiopathic intracranial hypertension are all terms to describe a syndrome of increased intracranial pressure, headaches, vision loss, or changes without an associated mass lesion.1 The condition was considered relatively rare, presenting most commonly in obese women in childbearing years. Surprisingly, with the obesity rates increasing among children and adolescents, rates of pseudotumor cerebri also are rising sharply in these populations.2

Dr. Francine Pearce, a pediatrician in Frankfort, Ill.

Dr. Francine Pearce

Obesity is the fastest growing morbidity among adolescents. The Centers for Disease Control and Prevention reported 32% of children 2-19 years were obese.1 This reality is impacting many areas of an adolescent’s health, but it also is changing the landscape of diseases that present in this age group. Although pediatric and adult pseudotumor cerebri always have had slightly varied features, many features were similar such as the papilledema, vision loss, headaches, and sixth nerve palsy. Obesity and female predominance tended to present more in the adult population, as many pediatric patients were not obese,2 and had fewer associated symptoms at the time of diagnosis, and the cause was thought to idiopathic.

Now, with the increase in obesity, more adolescents and more male patients are presenting with pseudotumor cerebri as a cause for their headache, and 57%-100% are obese, making it a compounding factor.3

Pediatric populations also are at risk of secondary pseudotumor cerebri, which is an increase in intracranial pressure from the use of medication, or other disease states such as anemia, kidney disease, or Down syndrome. Minocycline use is the most common medication cause and usually presents 1-2 months after normal use.4 Discontinuing the drug does lead to resolution. Retinoids, vitamin A products, growth hormone, and steroids also have been implicated. Given that acne is a common complaint amongst teens, knowledge of these side effects is important.4

In 2013, the criteria for diagnosis of pseudotumor cerebri was revised. Currently, the presence of papilledema, normal neurologic exam except for abnormal sixth cranial nerve, normal cerebral spinal fluid, elevated lumbar opening pressure, and normal imaging are needed for a definitive diagnosis. A probable diagnosis can be made if papilledema is not present but there abducens nerve palsy.2

In a routine physical exam, when I questioned a patient on any medication that was used daily, she replied she took ibuprofen daily for headaches and that she had been doing this for several months. Headaches were not in her chief complaints as she had learned to live with and ignore this symptom. Upon further evaluation, she was slightly overweight and has a questionable fundoscopic exam. After further evaluation by an ophthalmologist and a neurologist, pseudotumor cerebri was diagnosed.

Index of suspicion is key in correctly diagnosing patients, and understanding the changing landscape of medicine will lead to more thoughtful questioning during routine health exams and better outcomes for your patients.

Dr. Pearce is a pediatrician in Frankfort, Ill. She said she had no relevant financial disclosures. Email her at pdnews@mdedge.com.

References

1. Am J Ophthalmol. 2015 Feb;159(2):344-52.e1.

2. Horm Res Paediatr. 2014;81(4):217-25.

3. Clin Imaging. 2018 May 24. doi: 10.1016/j.clinimag.2018.05.020.

4. Am J Ophthalmol. 1998 Jul;126(1):116-21.

5. Glob Pediatr Health. 2018. doi:10.1177/2333794X18785550.

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