Pediatric idiopathic intracranial hypertension

Our understanding of pediatric idiopathic intracranial hypertension has been refined since Dr. Simmons Lessell's review in 1992. The use of rigorous methodologies and standard definitions in recent studies has demonstrated distinct demographic trends. Specifically, the incidence of idiopathic intracranial hypertension seems to be increasing among adolescent children, and among older children its clinical picture is similar to that of adult idiopathic intracranial hypertension (female and obese). Within younger age groups there are more boys and nonobese children who may develop idiopathic intracranial hypertension. The pathogenesis of the disease has yet to be elucidated. Idiopathic intracranial hypertension among young children has been associated with several new etiologies, including recombinant growth hormone and all-trans-retinoic acid. More modern neuroimaging techniques such as MRI and MRI-venograms are being used to exclude intracranial processes. Although most cases of pediatric idiopathic intracranial hypertension improve with medical treatment, those who have had visual progression despite medical treatment have undergone optic nerve sheath fenestration and lumboperitoneal shunting. Because idiopathic intracranial hypertension in young children appears to be a different disorder than in adolescents and adults, separate diagnostic criteria for younger children are warranted. We propose new criteria for pediatric idiopathic intracranial hypertension in which children should have signs or symptoms consistent with elevated intracranial pressure, be prepubertal, have normal sensorium, can have reversible cranial nerve palsies, and have an opening cerebrospinal fluid pressure greater than 180 mm H₂O if less than age 8 and papilledema is present, but greater than 250 mm H₂0 if age 8 or above or less than 8 without papilledema.     

Papilledema in paroxysmal nocturnal hemoglobinuria

A 26-year-old man with the diagnosis of paroxysmal nocturnal hemoglobinuria presented with papilledema due to increased intracranial pressure without localizing neurological signs. It is concluded that papilledema and benign intracranial hypertension could result from a cerebral vein thrombosis which can complicate the course of paroxysmal nocturnal hemoglobinuria. This has not been a well-recognized feature of the disease.     

Now you see it..

A patient experienced recurrent postural headache, transient visual obscurations, and papilledema. The patient initially presented with papilledema without cerebral venous sinus thrombosis (pseudotumor cerebri, idiopathic intracranial hypertension). One year later she had the similar symptoms and a recurrence of papilledema but now had obvious venous sinus thrombosis.     

IIH with normal CSF pressures?

Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space occupying lesions. ICP is usually measured by lumbar puncture and a cerebrospinal fluid (CSF) pressure above 250 mm H₂O is one of the diagnostic criteria of IIH. Recently, we have encountered two patients who complained of headaches and exhibited disc swelling without an increased ICP. We prescribed acetazolamide and followed both patients frequently; because of the definite disc swelling with IIH related symptoms. Symptoms and signs resolved in both patients after they started taking acetazolamide. It is generally known that an elevated ICP, as measured by lumbar puncture, is the most important diagnostic sign of IIH. However, these cases caution even when CSF pressure is within the normal range, that suspicion should be raised when a patient has papilledema with related symptoms, since untreated papilledema may cause progressive and irreversible visual loss.     

Visual loss in idiopathic intracranial hypertension after resolution of papilledema

PURPOSE: To demonstrate that progressive visual field loss may occur after resolution of papilledema in patients with idiopathic intracranial hypertension and persistently elevated intracranial pressure. METHODS: A patient with idiopathic intracranial hypertension was evaluated with serial Humphrey automated static perimetry after initial treatment and resolution of papilledema. RESULTS: The patient developed recurrent headache and elevated cerebrospinal fluid pressure. Optic nerve head appearance did not change. Automated perimetry demonstrated reproducible, worsening visual field loss; mean deviation decreased 11 dB in each eye. Visual field defects resolved after optic nerve sheath fenestration. CONCLUSIONS: Increased intracranial pressure caused visual field loss after resolution of papilledema. Optic nerve sheath fenestration improved visual function in this patient.     

Unilateral papilledema after trabeculectomy in a patient with intracranial hypertension

BACKGROUND: Increased intracranial pressure usually leads to bilateral disc swelling. HISTORY AND SIGNS: A patient presented with recurrent visual disturbances following trabeculectomy in the right eye. Intraocular pressure in the right and left eye were 11 and 24 mmHg, respectively. The optic nerve head was swollen in the right, but not in the left eye. Lumbar puncture showed an opening pressure of 32 cmH (2)O. Magnetic resonance imaging, neurological examination and composition of cerebrospinal fluid were normal. According to the modified Dandy criteria, an idiopathic intracranial hypertension was diagnosed. THERAPY AND OUTCOME: Treatment with acetazolamide led to resolution of papilledema in the right eye within six months. CONCLUSION: The intracranial-intraocular pressure gradient in the right eye was markedly higher as compared to that of the left eye. We suggest that this pressure gradient induced the collapse of axoplasmatic transport at the lamina cribrosa with subsequent disc swelling. As no significant pressure gradient was present in the left eye, the optic disc remained normal. Based on analogous calculations in three additional published cases of unilateral papilledema we thus suggest that intraocular pressure should be taken into account when evaluating patients with papilledema.     

Recurrence of idiopathic intracranial hypertension after weight loss: the carrot craver

PURPOSE: To describe a patient with stable idiopathic intracranial hypertension whose papilledema worsened. METHOD: Case report. RESULTS: A patient with documented idiopathic intracranial hypertension had resolution of disc edema with weight loss. Recurrence of papilledema led to the discovery that she consumed large quantities of raw carrots to help maintain her weight. Her increased vitamin A levels normalized, and the disc edema resolved when she stopped eating carrots. CONCLUSION: Patients with idiopathic intracranial hypertension should be counseled regarding carrot intake.     

Papilledema and pseudotumor cerebri

There is perhaps no neuro-ophthalmic sign that is as ominous as papilledema. True papilledema from increased intracranial pressure may be a harbinger of serious neurological disease. There, however, are other conditions that may mimic papilledema, contributing to the diagnostic dilemma. This article concerns the detection and differential diagnosis of papilledema, focusing on increased intracranial pressure without a mass lesion (pseudotumor cerebri).     

Medication-Related Pseudotumor Cerebri Syndrome

ABSTRACT

Pseudotumor cerebri syndrome refers to elevated intracranial pressure associated with papilledema without an identified etiology for intracranial hypertension. Over the past few decades, several medications have been described to be associated with this syndrome. We searched the literature for those case reports and series and evaluated the evidence for the association of such medications with pseudotumor cerebri syndrome.     

Detection of nerve fiber atrophy in apparently effectively treated papilledema in idiopathic intracranial hypertension

Background  

Since papilledema in idiopathic intracranial hypertension is a passive event not primarily affecting the visual tract, resolution with restitution ad integrum is expected if intracranial pressure is rebalanced. Retinal nerve fiber swelling due to papilledema in the acute phase and possible axon loss after long-lasting elevated intracranial pressure was investigated in a controlled cross-sectional study by scanning laser polarimetry.     

Papilledema: A review of etiology,pathophysiology, diagnosis,and management

Papilledema is optic nerve head edema secondary to raised intracranial pressure (ICP). It is distinct from other causes of optic disk edema in that visual function is usually normal in the acute phase. Papilledema is caused by transmission of elevated ICP to the subarachnoid space surrounding the optic nerve that hinders axoplasmic transport within ganglion cell axons. There is ongoing controversy as to whether axoplasmic flow stasis is produced by physical compression of axons or microvascular ischemia. The most common cause of papilledema, especially in patients under the age of 50, is idiopathic intracranial hypertension (IIH); however, conditions that decrease cerebrospinal fluid (CSF) outflow by either causing CSF derangements or mechanically blocking CSF outflow channels, and rarely conditions that increase CSF production, can be the culprit. When papilledema is suspected clinically, blood pressure should be measured, and pseudopapilledema should be ruled out. Magnetic resonance imaging of the brain and orbits with venography sequences is the preferred neuroimaging modality that should be performed next to look for indirect imaging signs of increased ICP and to rule out nonidiopathic causes. Lumbar puncture with measurement of opening pressure and evaluation of CSF composition should then be performed. In patients not in a typical demographic group for IIH, further investigations should be conducted to assess for underlying causes of increased ICP. Magnetic resonance imaging of the neck and spine, magnetic resonance angiography of the brain, computed tomography of the chest, complete blood count, and creatinine testing should be able to identify most secondary causes of intracranial hypertension. Treatment for patients with papilledema should be targeted toward the underlying etiology. Most patients with IIH respond to weight loss and oral acetazolamide. For patients with decreased central acuity and constricted visual fields at presentation, as well as patients who do not respond to treatment with acetazolamide, surgical treatments should be considered, with ventriculoperitoneal shunting being the typical procedure of choice.     

Bilateral peripapillary subretinal neovessel membrane associated with chronic papilledema: report of two cases

BACKGROUND: Increased intracranial pressure (ICP) usually results in headaches and papilledema with infrequent loss of visual acuity. Peripapillary subretinal neovessel membranes (PSNVM) rarely complicate chronic papilledema. HISTORY AND SIGNS: Case 1: A headache-free 9-year-old boy presented with a bilateral loss of vision due to chronic papilledema. Bilateral PSNVM was present. Cerebral imaging revealed a pilocytic astrocytoma and hydrocephaly. Case 2: A headache-free 39-year-old man presented with severe visual loss of the left eye due to an exudative macular star. Severe papilledema and PSNVM were present in both eyes due to idiopathic intracranial hypertension. THERAPY AND OUTCOME: Lowering of ICP (tumor resection - Case 1 and lumboperitoneal shunt - Case 2) resulted in involution of the PSNVMs in both patients. No improvement of visual acuity occurred for Case 1 (optic atrophy) but Case 2 benefited from gradual visual recovery. CONCLUSIONS: PSNVMs are rare complications of chronic papilledema, as only 10 patients have been reported so far in the literature. Absence of headaches in both of our patients was probably responsible for the late diagnosis, hence chronicity of papilledema. Laser therapy of PSNVM might not be necessary once the ICP is lowered.     

Optical coherence tomography of the swollen optic nerve head: deformation of the peripapillary retinal pigment epithelium layer in papilledema

PURPOSE. To examine the biomechanical deformation of load bearing structures of the optic nerve head (ONH) resulting from raised intracranial pressure, using high definition optical coherence tomography (HD-OCT). The authors postulate that elevated intracranial pressure induces forces in the retrolaminar subarachnoid space that can deform ONH structures, particularly the peripapillary Bruch's membrane (BM) and RPE layers. METHODS. The authors compared HD-OCT optic nerve and peripapillary retinal nerve fiber layer (RNFL) findings in eyes with papilledema caused by raised intracranial pressure to findings in eyes with optic disc swelling caused by optic neuritis and nonarteritic anterior ischemic optic neuropathy (NAION), conditions without intracranial hypertension. The authors measured average thickness of the RNFL and the angle of the RPE/BM at the temporal and nasal borders of the neural canal opening. The angle was measured as positive with inward (toward the vitreous) angulation and as negative with outward angulation. RESULTS. Of 30 eyes with papilledema, 20 eyes (67%) had positive RPE/BM rim angles. One of eight optic neuritis (12%) eyes and 1 of 12 NAION (8%) eyes had positive angulation. In five eyes with papilledema, RNFL thickening increased, three of which developed positive RPE/BM angles. On follow-up, 22 papilledema eyes had a reduction of RNFL swelling, and 17 of these eyes had less positive RPE/BM angulation. CONCLUSIONS. In papilledema, the RPE/BM is commonly deflected inward, in contrast to eyes with NAION or optic neuritis. The RPE/BM angulation is presumed to be caused by elevated pressure in the subarachnoid space, does not correlate with the amount of RNFL swelling, and resolves as papilledema subsides.     

Pseudotumor cerebri in idiopathic aplastic anemia.

Idiopathic intracranial hypertension, also referred to as pseudotumor cerebri (PTC), is characterized by high intracranial pressure, normal cerebral ventricles,and cerebrospinal fluid. Although papilledema is described in various anemias, only one case of PTC with idiopathic aplastic anemia has been reported in the English literature. We report a case of papilledema in an adolescent with aplastic anemia and propose its mechanism.     

Evaluation of the underlying causes of papilledema in children

ObjectiveThe purpose of this retrospective study was to identify the types and relative frequencies of intracranial disorders in pediatric patients who present with papilledema.DesignRetrospective case series.Participants and MethodsThis study was conducted in 2 pediatric ophthalmology clinics, both providing community-based care in a large inner-city urban center in the U.S. Pediatric patients aged between 0 and 16 years diagnosed with papilledema and who had an underlying etiology identified were included in the study. Patient demographic data, ophthalmologic examination findings, and diagnostic work-up results were identified from clinical records.ResultsThe mean age of 38 study patients (19 female, 19 male) was 8.6 ± 4.8 years. Of the 38 patients, 16 (42.1%) had idiopathic intracranial hypertension (IIH) as the underlying cause of the papilledema, 7 (18.4%) had a craniosynostosis disorder, 6 (15.8%) had intracranial tumours, 2 (5.3%) had primary hydrocephalus, and 1 (2.6%) patient each had transverse sinus thrombosis related to sinusitis, hypertensive crisis, subdural hematoma, intracranial abscess, Lyme disease, presumed neurosarcoidosis, and acute disseminated encephalomyelitis. Of the 6 intracranial tumours, 2 (33.3%) presented in the sellar/parasellar region, 2 (33.3%) in the posterior fossa, and 2 (33.3%) were in cortical locations.ConclusionClinicians should have a high index of suspicion for IIH and brain tumours in children presenting with papilledema. Patients with craniosynostosis should have routine eye examinations to monitor for asymptomatic papilledema. Understanding the relative incidence of etiologies for papilledema highlights the urgency of appropriate work-up and the need to consider low-frequency etiologies.     

Oculomotor palsy and papilledema with pial-dural arteriovenous malformation

A 62-year-old man presented with papilledema, a cranial bruit, and a partial left oculomotor nerve palsy. Arteriography revealed a large mixed pial-dural arteriovenous malformation involving the superior sagittal and both transverse sinuses. After the superior part of the malformation was embolized, the patient's papilledema and ocular motility disturbance resolved. The oculomotor disturbance may have been a nonspecific sign of increased intracranial pressure. Cranial auscultation should be performed in all cases of papilledema and cranial nerve palsy.     

Disc swelling: a tall tail?

A 30-year-old man presented with monocular visual loss secondary to chronic papilledema, due to an ependymoma involving the spinal cord. No other neurological symptoms were present at the time. Initial neuroradiologic tests as well as laboratory investigations were negative, except for elevated pressure and protein concentration of his cerebrospinal fluid. In spite of intensive investigation, the diagnosis of a spinal cord tumor was delayed for approximately 12 months until he presented with neurologic symptoms attributable to a spinal cord lesion. This is only the fourth case reported of a spinal cord tumor associated with papilledema presenting with visual loss, without any other manifestations of either elevated intracranial pressure, or spinal disease. Possible mechanisms for elevated intracranial pressure in cases of spinal cord tumors are reviewed.     

Benign intracranial hypertension and thyreostimulin suppression hormonotherapy

PURPOSE: To report a case of benign intracranial hypertension occurring during thyreostimulin suppression hormonotherapy after thyroidectomy for papillary cancer. DESIGN: Interventional case report. METHODS: A 45-year-old woman underwent total thyroidectomy for a 7-mm papillary cancer. Seventeen years later, she experienced headache, dizziness, irritability, sensation of full head, and blurred vision of the right eye while being treated with a mix of L thyroxin (LT4) and liothyronin (LT3). Ophthalmological data (including the presence of papilledema), cerebroorbital magnetic resonance imaging, and lumbar pressure evaluation confirmed benign intracranial hypertension. RESULTS: Substitutive hormonotherapy was decreased under specialized surveillance, permitting remission of benign intracranial hypertension symptoms and papilledema. CONCLUSIONS: Benign intracranial hypertension is usually difficult to cure, and its association with thyroid hormonotherapy is rare. In our patient, tapering LT4 and withdrawal of LT3 until a euthyroidal state was obtained resulted in successful treatment of benign intracranial hypertension.     

Severe Optic Disc Edema Without Hydrocephalus in Neurofibromatosis 2

A 26-year-old man who had neurofibromatosis type-2 with symptoms of unexplained optic disc edema is reported. Magnetic resonance imaging (MRI) revealed bilateral acoustic schwannomas. Obstructive hydrocephalus, however, was not evident in spite of his severe disc edema and visual loss. After partial removal of the right acoustic schwannoma, symptoms of intracranial hypertension, such as vomiting and headache, developed and MRI demonstrated evidence of obstructive hydrocephalus. Placement of a ventricular-peritoneal shunt relieved the symptoms of intracranial hypertension, but visual acuity in his left eye was reduced to hand motion due to secondary optic atrophy. In patients with similar symptoms it is suggested that, in addition to tumor removal, early treatment to decrease intracranial pressure should be considered when visual function is progressively impaired by the symptoms of prolonged papilledema.