Shawn Aylward
In pediatrics, PIH is typically divided into pre-pubertal and pubertal groups. Pubertal patients have the same risk factors as adults, whereas obesity and gender are not prominent risk factors in pre-pubertal patients. Balcer et al. found that obesity did correlate with an increased risk of PIH in children over 11 years old.(13) Bursztyn et al. found similar results in those >12 years old.(10)

Symptoms of intracranial hypertension tend to be less evident in younger individuals. In the pre-pubertal population, optic disc edema incidentally discovered on routine ophthalmologic examination is seen. Asymptomatic cases are often younger, have a male predominance, and a lower percentage of obesity.(14) Up to 48% of cases lack papilledema, yet have other symptoms consistent with elevated intracranial pressure and documented elevated opening pressure.(7, 15, 16)
The most consistent symptom is headache that is typically constant with variable severity throughout the day. Headaches tend to be most severe in the morning following prolonged horizontal positioning and may be exacerbated by maneuvers such as Valsalva, bending over or coughing. Other symptoms include nausea, vomiting and diplopia.
Cranial nerve VI palsy is the most common nerve palsy with patients complaining of diplopia with horizontal gaze. Tinnitus is experienced by 10% of pediatric PIH patients and is often unreported until asked.(8) Patients will report a “whooshing” sound coinciding with their heartbeat.

Papilledema refers to the presence of optic disc edema in the setting of verified increased intracranial pressure. Optic disc edema in absence of increased intracranial pressure can be seen in optic neuritis, neuroretinitis, anterior ischemic optic neuropathy, and infiltration of the optic nerve head by tumor cells. An afferent pupillary defect is rare in intracranial hypertension, and if found should raise concern for the possibility of optic neuritis. Pseudo-papilledema gives the appearance of optic nerve edema to those unfamiliar with the fundus exam (and even the experienced at times). Common culprits are anomalous optic nerves and optic nerve drusen. Unilateral or marked asymmetric edema should raise concern for one of these conditions.

Secondary Intracranial Hypertension
Numerous medications have been reported to lead to SIH (table 1). The most widely known is the tetracycline class of medications, including tetracycline, minocycline and doxycycline.(17-19) Chronic corticosteroid use can result in increased pressure following weight gain from chronic use, but also with acute withdrawal or rapid wean.
Patients being treated with recombinant growth hormone can develop symptoms of intracranial hypertension including headache and optic nerve edema.(20, 21) The timing from initiation of growth hormone to presentation is often in the first 12 weeks of treatment, though it can occur years after treatment was started.(21) Treatment includes cessation of the growth hormone and often acetazolamide until symptom resolution. Once symptoms resolve, patients can be restarted on a lower dose of growth hormone with gradual titration without recurrence of symptoms.

Other medications reported in the literature include lithium, nalidixic acid, hypervitaminosis A, hypovitaminosis A, and All-Trans Retinoic Acid, a vitamin A derivative. Oral contraceptives are reported to be associated with SIH, though the association is likely due to CVST from the use of contraceptives.

Cerebral Venous Sinus Thrombosis (CVST) is a common non-medication cause of SIH. Superior sagittal sinus and the transverse sinuses are most often seen (figure 1), though thrombosis of any venous outflow can result in increased pressure. Prior to the widespread use of antibiotics to treat chronic otitis or mastoiditis, patients would develop thrombosis of the sigmoid sinus or jugular vein, formerly referred to as otitic hydrocephalus. Hypercoagulabile states such as acute post-partum period, oral contraceptive use, cancer (lymphoma/leukemia) and various inherited coagulopathies can also result in CVST.
Initiation of feeding in cases of poor nutrition from starvation or malabsorption syndromes has resulted in a transient increase in intracranial pressure.(22, 23) Often a bulging of the anterior fontanel is the only symptom in infants. Older children may develop irritability and cranial suture separation. Fortunately in most cases, it is a transient process that self resolves within a few days to weeks.

Figure 1. Superior sagittal sinus thrombosis (large arrows) and transverse sinus thrombosis (small arrow).

Table 1: Common Causes of Secondary Intracranial Hypertension
   Minocycline / Tetracycline / Doxycycline
   Corticosteroids (especially withdrawal)
   Growth hormone
   Lithium carbonate
   Cyclosporine A
   Nalidixic acid
   Retinoic acid
   Vitamin A (excess or deficiency)
   Vitamin D

Medical conditions
   Cerebral venous thrombosis
   Refeeding syndrome
   Adrenal insufficiency (often on steroids)
   Congestive heart failure
   Hypoparathyroidism (early in correction)
   Crohn’s disease
   Intracranial hemorrhage
   Lyme disease
   Demyelinating disease / multiple sclerosis
   Craniofacial syndrome
   Chiari Malformation


Contributor Information and Disclosures

Shawn Aylward
MD, Assistant professor,
Nationwide Children’s Hospital,
The Ohio State University. Columbus, OH. USA

First Created : 3/25/2016


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