Edit an article: Idiopathic Intracranial Hypertension (IIH) in Pregnancy

You do not have permission to edit this page, for the following reasons:

You are not allowed to execute the action you have requested.
You must confirm your email address before editing pages. Please set and validate your email address through your user preferences.

Original article author(s) EyeWiki username.

Additional contributor (who has not actually edited this page)

Select one or more categories for this page.

Articles
- Cataract/Anterior Segment
- Cornea/External Disease
- Glaucoma
- Miscellaneous
- Neuro-ophthalmology/Orbit
- Ocular Trauma
- Oculoplastics/Orbit
- Oncology/Pathology
- Pediatric Ophthalmology/Strabismus
- Refractive Management/Intervention
- Retina/Vitreous
- Uveitis

Reviewers

Enter the name of the assigned editor for this page.
Enter the name of the reviewer for this page.
Enter the date this page was reviewed.
Select the status of this page.

Local Videos

Meta Description

Enter a short description of the article. Used to generate the page's meta description tag.

Article Text

Edit article text below. You can save this page without filling in all of the sections and come back to it later.

Idiopathic intracranial hypertension (IIH) in pregnancy = Disease Entity = Heinrich Quincke in 1893 described the first case of elevated intracranial pressure and called it “meningitis serosa.” In 1904 Nonne termed this “pseudo-tumor cerebri.” The term “benign intracranial hypertension” was then coined by Foley in 1955 but subsequently the more descriptive name “idiopathic intracranial hypertension” (IIH) has gained acceptance because of the often non-benign and sometimes significant vision loss that can occur.<ref name=":0">Thirumalaikumar, L, Ramalingam, K, Heafield, T. Idiopathic intracranial hypertension in pregnancy. The Obstetrician & Gynaecologist 2014; 16: 93– 97.</ref> == Disease == IIH is a disease characterized by elevated intracranial pressure and its associated signs and symptoms in an otherwise alert and awake patient, with no apparent cause. It is seen predominantly in overweight women of childbearing age. It is a diagnosis of exclusion and defined by diagnostic clinical, lumbar puncture, and radiographic criteria. Patients with IIH in pregnancy typically have the same evaluation, course, and treatment as non-pregnant patients with IIH but with some exceptions described below. Patients with IIH in pregnancy have the same spontaneous abortion rate as the general population and it is not generally considered to be a high risk pregnancy. Additionally, visual outcomes for IIH in pregnancy are believed to be the same as for non-pregnant IIH patients.<ref name=":1">Tang RA. Management of idiopathic intracranial hypertension in pregnancy. MedGenMed. 2005;7(4):40. Published 2005 Nov 10.</ref> == Incidence and prevalence == The majority of IIH patients are child bearing aged females, with a female to male ratio of 8:1. Thus, it is not surprising that IIH often occurs in pregnancy. The incidence of IIH in women of childbearing age is 0.9/ 100,000. The incidence in obese women increases to 19.3/100,000. 61% of cases of IIH occur in the first trimester, although it can occur in any trimester<ref name=":0" />.<ref name=":1" /> Digre et al. reported the prevalence of IIH in pregnancy as 2% to 12%.<ref name=":2">Digre KB, Varner MW, Corbett JJ: Pseudotumor cerebri and pregnancy. Neurology 1984, 34:721–729.</ref> Huna-Baron and Kupersmith reported it at 5%.<ref name=":3">Huna-Baron, R. & Kupersmith, M. J Neurol (2002) 249: 1078. https://doi.org/10.1007/s00415-002-0791-4</ref> == Risk Factors == There have been several case reports and review articles suggesting pregnancy as a risk factor for IIH. However, IIH in pregnancy occurs at the same rate as the general population, therefore, pregnancy is not considered an etiologic factor and might be better explained by the weight gain that occurs normally in pregnancy and that IIH occurs predominantly in women of childbearing age. <ref name=":4">Kesler A, Kupferminc M. Idiopathic intracranial hypertension and pregnancy. Clin Obstet Gynecol. 2013;56(2):389–96.</ref> == Pathophysiology == The pathophysiology of IIH with or without pregnancy is still not fully known. Some theories include increased CSF production, decreased CSF drainage, and increased cerebral venous sinus pressure as the leading cause. Hormones may potentially play a role in the pathophysiology of IIH, however this has not been adequately studied<ref name=":4" />. <ref name=":0" /> = Diagnosis = == Physical examination == It is important to calculate body mass index (BMI) as obesity is a risk factor for IIH. It is also important to measure blood pressure to help rule out hypertensive emergency or eclampsia/pre-eclampsia. A full eye exam including dilated fundus exam is recommended to detect papilledema. Formal visual field testing is also recommended to assess baseline and follow patients for the development of vision loss which is the most feared complication of IIH.<ref name=":1" /> == Signs and symptoms == The signs and symptoms of IIH in pregnancy are similar to the signs and symptoms in the non-pregnant population. Headache is the most common presenting symptom usually described as a daily, throbbing, positional headache with nausea and with or without vomiting. Other symptoms such as pulsatile tinnitus, dizziness, neck pain, back pain, and radicular pain may also be present. Visual symptoms include transient visual obscurations, diplopia, and visual loss. Visual loss is the most serious and feared outcome of IIH in both the general population and pregnancy.<ref>AG. Lee, Andrew & Sinclair, Alexandra & Sadaka, Ama & Berry, Shauna & Mollan, Susan. (2019). Neuro-Ophthalmology Global Trends in Diagnosis, Treatment and Management: Global Trends in Diagnosis, Treatment and Management. 10.1007/978-3-319-98455-1.</ref> Headache in pregnancy is a particularly important symptom to pay attention to as it may be a sign of a serious and life- threatening diseases such as eclampsia, meningitis, or cerebral venous thrombosis. These diseases may present similarly to IIH with headache, nausea and vomiting, neck stiffness, or visual disturbances and therefore need a proper workup<ref name=":0" />. == Clinical diagnosis == The diagnosis of IIH in pregnancy (as well as in non-pregnant patients) is made by using the modified Dandy Criteria: (1) increased intracranial pressure and associated symptoms, (2) without localizing neurological findings, (3) patient is alert and oriented, (4) normal neurodiagnostic studies except for increased CSF pressure and (5) no other cause of increased ICP is present.<ref>Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol. 1985;5:55–6.</ref> IIH is a diagnosis of exclusion, therefore when doing a proper workup for suspected IIH, an ophthalmologist should make efforts to exclude other more serious causes of increased intracranial pressure. Questions concerning past history of headache, associated symptoms, history of drug use during or before pregnancy, past psychiatric history, and a thorough review of symptoms are necessary. == Diagnostic procedures == Magnetic resonance imaging (MRI) and MR venography (MRV) of the brain with and without contrast are the neuroimaging procedure of choice in IIH but gadolinium is not recommended in pregnancy. There is no data to demonstrate any adverse effects of MRI in pregnancy, but there are also few studies to prove it is safe. This risk needs to be communicated with the patient. <ref name=":1" /> The current recommendation is to defer contrast agents in pregnancy as it has been shown experimentally to cause developmental delay without congenital anomalies when given in 2.5 times the human dose to rats and 7.5 times the human dose to rabbits<ref>Chung SM: Safety issues in magnetic resonance imaging. J Neuro-ophthalmol 2002, 22:35–39.</ref>. A diagnostic lumbar puncture measuring the opening CSF pressures has no contraindications in pregnancy and may be done safely to aid in the diagnosis of IIH. Computed tomography (CT) could have deleterious effects on the fetus due to ionizing radiation and therefore is not the first choice when evaluating a possible IIH. In addition, CT is not as sensitive or specific as MRI and MRV in diagnosing intracranial tumors, meningeal disease, and cerebral venous and dural sinus thromboses. <ref name=":1" /> == Laboratory test == There are no laboratory tests used in the diagnosis of IIH in pregnancy. It may be important, however, for obstetrics to check the blood pressure as well as routine monitoring of urine protein, complete blood counts (CBC), serum creatinine, and liver function testing (LFT). == Differential diagnosis == # Cerebral venous and dural sinus thrombosis # Pre-eclampsia with or without severe features # Eclampsia # Intracranial space occupying lesion # Meningitis # Hypertensive emergency = Management = == General treatment == Diet and Weight control: A low fat diet and pregnancy appropriate weight gain are recommended for pregnant patients. Weight gain is physiologically normal in pregnancy, but too much weight gain can be detrimental. There is little literature that defines a “healthy” amount of weight gain in pregnant patients with IIH. Friedman recommended controlling weight gain during pregnancy to no more than 20lbs.<ref>Evans RW, Friedman DI. Expert opinion: the management of pseudotumor cerebri during pregnancy. Headache 2000;40:495–7.</ref> == Medical therapy == Analgesics: The use of simple nonsteroidal anti-inflammatory drugs (NSAIDs) are generally safe during pregnancy with the exception of the third trimester due to the risk of premature closure of the fetal ductus arteriosus and oligohydramnios. Opioids are not typically used to treat the headaches in IIH due to risk of fetal dependence and withdrawal symptoms.<ref name=":0" /> Diuretics: Acetazolamide is the most common diuretic used to treat IIH. It is a carbonic anhydrase inhibitor that reportedly decreases CSF production. It is a Food and Drug Administration (FDA) category C drug. The FDA categorizes class C fetal risk factor as: “Animal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.”<ref name=":5">Content and Format of Labeling for Human Prescription Drug and Biological Products; Requirements for Pregnancy and Lactation Labeling. Federal Register/Vol. 73, No. 104/Thursday, May 29, 2008.</ref> There are no well controlled studies detailing the effects of acetazolamide in pregnant women or fetuses. Animal studies in rodents has shown a possible relation to limb abnormalities but these have not been reproducible in primates or observed in humans<ref name=":1" />. There has been a single report by Worsham and colleagues in 1978, of sacrococcygeal teratoma, in an infant born to a mother treated with acetazolamide throughout her pregnancy. Because of this, the use of acetazolamide in pregnancy is often limited to after 20 weeks of gestation after consultation with obstetrics. Steroids: Corticosteroids are considered a category B in terms of fetal risk factor. The FDA describes a class B as: “Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.”<ref name=":5" /> Because of the adverse effects of corticosteroids, they are generally reserved for acute settings of IIH in pregnancy. == Serial Lumbar Punctures == Serial Lumbar Punctures: Performed 10 days to 2 weeks apart, serial lumbar punctures can help improve headaches. The limiting factor in many is the technical difficulty of performing a lumbar puncture in a pregnant woman, especially if she is obese. Additionally, serial lumbar punctures are not well received by many patients. Serial lumbar punctures may however be an option in the pregnant patient who fails or refuses medical therapy and wishes to delay or avoid surgery. <ref name=":1" /><ref name=":3" /> == Surgery == Optic nerve sheath fenestration (ONSF): ONSF is a procedure that involves making a small window in the optic nerve sheath to relieve elevated intracranial pressure. It is considered safer than other surgical options in IIH but has yet to be extensively studied in pregnancy.<ref>Satti SR, Leishangthem L, Spiotta A, Chaudry MI. Dural venous sinus stenting for medically and surgically refractory idiopathic intracranial hypertension. Interv Neuroradiol. 2017;23(2):186–193. doi:10.1177/1591019916680110</ref> ONSF is typically reserved for patients with moderate to severe or progressive visual loss in a patient who has failed or is unable to receive medical therapy. It is considered by most experts to be safe in pregnancy. <ref name=":0" /> CSF Diversion Procedure: This includes both lumboperitoneal shunts (LPS) and ventriculoperitoneal shunts (VPS). These procedures make a connection from the CSF to the peritoneum to relieve elevated intracranial pressure and therefore treat headaches or visual problems. There are limited data to support the safety and effectiveness of CSF diversion procedures in pregnancy, however, the data that is available suggests lumboperitoneal shunts are not contraindicated in pregnancy and can be safe for both mother and fetus.<ref name=":1" /> <ref>Landwehr JB, Isada NB, Pryde PG, et al.: Maternal neurosurgical shunts and pregnancy outcome. Obstet Gynecol 1994, 83:134–137.</ref> == Modes of Delivery == IIH in pregnancy is not a specific indication for a cesarean delivery and active labor is not contraindicated. Active labor does not place the pregnant woman at an increased risk for vision loss. During delivery no special analgesic requirements are necessary except for epidural anesthesia in patients with a lumboperitoneal shunt, as the anesthesia may move into the peritoneum and not be as effective. The increased ICP during labor is not harmful to the mother or the baby because it is transient<ref name=":4" /><ref name=":1" />. == Prognosis == The prognosis for pregnant women with IIH is excellent for both mother and child. Visual outcomes in women with IIH in pregnancy are the same as non-pregnant women. There is no greater risk of recurrence of IIH for subsequent pregnancies when compared to the general population. The spontaneous abortion rate for women with IIH in pregnancy is the same as women without IIH in pregnancy. <ref name=":2" /> Therefore, IIH is generally not an indication for medical termination of pregnancy<ref name=":4" />. = References = <references />
Please note that all contributions to EyeWiki may be edited, altered, or removed by other contributors. If you do not want your writing to be edited mercilessly, then do not submit it.
You are also promising us that you wrote this yourself, or copied it from a public domain or similar free resource (see EyeWiki:Copyrights for details). Do not submit copyrighted work without permission!

Cancel
Watch this page