Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review

Abstract Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

Research Advances in Idiopathic Intracranial Hypertention / 中国医学科学院学报

Idiopathic intracranial hypertension,also known as pseudotumor cerebri,is a syndrome characterized by raised intracranial pressure of unknown cause.These patients present normal neuroimaging and cerebrospinal fluid analysis while increased intracranial pressure and associated symptoms and signs.Delay of treatment can cause severe visual impairment.There are some new understandings of this disease,and we will review the pathogenesis,diagnosis,and treatment of idiopathic intracranial hypertension.

Bilateral Transverse Sinus Angioplasty for the Treatment of Idiopathic Intracranial Hypertension ­ Case Report and Literature Review

Idiopathic intracranial hypertension (IIH) is a disease characterized by an increase in intracranial pressure, without presence of parenchymal lesions or hydrocephalus that justify it. Over 90% of cases there is association with stenosis of the dural venous sinuses. It is characterized by headache, tinidus, nausea, vomiting and visual disturbances. Initial treatment is clinical and when it fails there is indication of invasive procedures, among them shunts and fenestration of the optic nerve sheath. Angioplasty of dural venous sinuses, when indicated, has shown an alternative with better results and less complications. We report a case of a female patient, with 27 years old, diagnosed with IIH and bilateral transverse sinus stenosis, which was treated by bilateral stenting and total resolution of symptoms. Besides describing the case we review the literature about the subject.

Idiopathic intracranial hypertension associated with mild traumatic brain injury in a pediatric patient ? Case report / Hipertensão intracraniana idiopática associada a trauma cranioencefálico leve em paciente pediátrico ? Relato de caso

Mild head injury has been described as rare cause of idiopathic intracranial hypertension (IIH). In the presence of IIH, initial treatment is clinical and surgical treatment, such as lumboperitoneal shunt. Most cases have a good prognosis. The patient have 9-year-old male, went to the emergency room with a history of accidental fall, presenting headache, vomiting and blurred vision. Physical examination showed good overall condition. Neurological examination: normal. Fundoscopy: incipient bilateral papilledema. Normal cranial CT. The general picture suggested by exclusion of other causes IIH diagnosis. He underwent symptomatic treatment with acetazolamide, painkillers and rest. Discharged from the hospital on the eighth hospital day with no complaints being referred for outpatient treatment.

O traumatismo cranioencefálico leve tem sido uma causa rara de hipertensão intracraniana idiopática (HII). Na presença de HII, o tratamento inicial é clínico e o tratamento cirúrgico é feito por meio da derivação lumboperitoneal. A maioria dos casos cursa com bom prognóstico. Um paciente com 9 anos de idade, masculino, foi admitido na emergência com história de queda acidental, apresentava cefaleia, vômitos e visão turva. Ao exame físico apresentou bom estado geral. Exame neurológico: normal; fundoscopia: papiledema bilateral incipiente; TC do crânio normal. O quadro sugeriu o diagnóstico de HII, por exclusão de outras causas. Foi submetido a tratamento sintomático com acetazolamida, analgésicos e repouso. Recebeu alta médica hospitalar no oitavo dia, sem queixas, sendo encaminhado para acompanhamento ambulatorial.

Recent concepts in the pathophysiology and treatment of pseudotumor cerebri [PTC]

Study the role of intracranial venous system in the pathophysiology of Pseudotumor cereberi. Twenty patients diagnosed as PTC according to Dandy diagnostic criteria. All underwent general and neurological assessment. Full ophthalmologic assessment. Full Laboratory investigations were done. Radiological assessment included CT scan brain +/- MRI brain without contrast, MRV of the intracranial venous system. All underwent digital subtraction cerebral Angiography [DSA] [venous phase] to confirm the validity of filing gaps seen at the level of MRV whether they are true stenosis or not. Menstrual irregularity documented in 2 patients [10%]. Sixteen patients [80%] considered to be overweight or obese. MRV brain showed that 14 patients [70%] showed filling gaps suggestive of sinus stenosis. However, digital subtraction cerebral angiography [venous phase] gave different data that only 5 patients [25%] had stenosis in their dural sinuses. MRV showed to be a good screening tool since it had 100% sensitivity and negative predictive value. Therefore, if MRV is normal no further investigations are needed. However, since it has a moderate specificity [62%] with a positive predictive value [PPV] of only 35%, then lesions detected should be confirmed with digital subtraction cerebral angiography [venous phase] particularly those involving the transverse and sigmoid sinus. In our study most of the cases having venous sinus stenosis occurred among patients with age between 22-30 y old [p<0.05] and among those with BMI>30 [p<0.05]. Significant association was found between visual symptoms and stenosis since 80% of cases with stenosis had diplopia, TVO's, grade 3 papilledema and in grade 3 field defect [p<0.05]. By logistic regression model, diplopia and TVO's were considered as independent predictors of occurrence of stenosis among the studied cases. Studying the intracranial venous system in patients with PTC is and important step in understanding the pathophysiology of the disease. Detection of venous sinus stenosis opens the way to a novel therapeutic option for refractory patients like venous sinus stenting

Idiopathic intracranial hypertension: clinical presentation and management

Idiopathic intracranial hypertension [IIH] is an idiopathic increase in intracranial pressure [pressure around the brain] in the absence of a tumor or any other disease. To assess the clinical features and the treatment strategies for patients with IIH. We performed a prospective follow up of patients with IIH diagnosed in our surgical and medical departments. The following data were obtained: age at diagnosis, gender, symptoms of headache, vomiting, visual disturbances, neurological signs, and management. We enrolled 34 patients. A large part of them [44.1%] had an age between 30-39 years and were females [85.3%]. Headache was present in all patients; vomiting interested 23 patients [67.6%]; and papilledema and visual symptoms were seen in 26 subjects [76.5%]. In 20 patients [58.8%] there was no localized neurological symptoms apart from visual symptoms, with fits in 4 [11.8%], meningism in 3 [8.8%] and motor symptoms in 3 [8.8%] as shown in table [2]. Twenty-six cases [76.5%] had a good response to medical treatment. Surgical treatment was performed in 8 subjects [23.5%] as shown in table [3]. Early diagnosis and management are essential in preventing progression of the disease. Fortunately, there appear to be effective treatment strategies available. Headache usually becomes manageable, papilledema regresses, and vision improves in most patients

Pseudotumor cerebral secundário a trombose venosa dural: relato de caso pediátrico / Pseudotumor cerebri secondary to dural sinus thrombosis: pediatric case report

O pseudotumor cerebral é uma síndrome neurológica relativamente comum na adolescência. Na maioria dos casos, a etiologia é idiopática, mas pode haver complicações graves, como cegueira, relacionadas com a hipertensão intracraniana. O objetivo deste artigo é enfatizar o diagnóstico diferencial do pseudotumor cerebral, com atenção especial às etiologias tratáveis. Relatamos o caso de um adolescente de 12 anos que se apresentou com diplopia e cefaléia 9 dias após otite média e mastoidite à direita. A tomografia computadorizada do crânio foi normal, mas a ressonância magnética do encéfalo detectou trombose dos seios transverso e sigmóideo ipsilaterais, a qual respondeu à anticoagulação precoce. A conclusão é que a ressonância magnética do encéfalo é essencial nos pacientes com diagnóstico clínico de pseudotumor cerebral para exclusão de causas tratáveis, como a trombose venosa dural.

Eficacia de la válvula tipo diafragma en hidrocefalia aguda / Efficacy of valve type diaphragm in acute hydrocephaly

El problema mayor del tratamiento de la hidrocefalia, es el alto indice de complicaciones asociadas a la colocación de sistemas derivativos del LCR, por disfunción de los mismos; el objetivo de este trabajo fue evaluar la eficacia de la válvula tipo diafragma, comparándola con otros dos sistemas utilizados en este Instituto, para determinar si se disminye el número de revisiones valvulares por disfunción, mejorando de esta forma la calidad de vida de los pacientes

Pseudotumor cerebri / Cerebri pseudotumor

El pseudotumor cerebri (Hipertensión intracraneal ideopática) es una entidad patológica de etiología no bien precisada, caracterizada por un aumento de la presión intracraneana, asociada a síntomas y signos como cefalea, tinnitus, obscurecimientos transitorios, diplopia pero sin hallazgos neurológicos focalizados, ni debe encontrarse evidencias de obstrucción o deformidad del sistema ventricular y los estudios neurodiagnósticos son normales, exceptuando la elevación de la presión intracraneal. Se revisa la estadística de la Unidad de Neuroftalmología del Hospital Domingo Luciani del I.V.S.S., Caracas, desde agosto de 1992 hasta mayo de 1995, encontrándose 10 casos de Pseudotumor Cerebri (P.C), estableciéndose el diagnóstico de acuerdo a los criterios modificados de Dandy. De los 10 casos evaluados, el 80 por ciento correspondieron a la tercera y cuarta década de la vida. Igualmente un 70 por ciento correspondió al sexo femenino. Las manifestaciones clínicas más frecuentemente encontradas fueron cefalea y diplopia en un 90 y 80 por ciento de los casos. la obesidad constituyó la patología sistémica más observada. El tratamiento recibido fue a base de dieta y diuréticos en el 100 por ciento de los casos. La evolución fue a mejoría en un 80 por ciento de casos y estacionario en el 20 por ciento restante. Ningún caso requirió tratamiento quirúrgico

Idiopatthic intracranial hypertension [IIH] a clinical study of diagnosis, therapeutic, and prognostic factors

In this study, forty-two patients with idiopathic intracranial hypertension were diagnosed and managed medically and surgically with emphasizing on the predisposing factors, the presenting symptoms, the clinical signs and the diagnostic procedures [ophthalmological and radiological]. Medical treatment was tried in thirty cases with improvement in twenty cases, no improvement in four cases and deterioration occurred in six cases. Surgery by lumboperitoneal shunting procedure was done in twelve cases with improvement in seven cases, no improvement in three cases and deterioration occurred in two cases

Daño neurológico por preeclampsia-eclampsia: fisiopatología, prevención y tratamiento / Neurologic damage due to preeclampsia-eclampsia: physiolopathology, prevention and treatment

La preeclampsia-eclampsia se complica a menudo con alteraciones cerebrovasculares. Las manifestaciones clínicas son variables y el vasoespasmo es un problema habitual. Se presenta edema, trombosis, hemorragias, cambios fibrinoides transmurales, se pierde la autorregulación cerebral en las regiones dañadas y los trastornos iónicos transmembrana pueden ser la causa de convulsiones. El tratamiento preventivo de la preeclamsia-eclampsia consiste en el control de la presión arterial y del volumen intravascular, protección de las lesiones edoteliales e interrupción del embarazo. Las medidas específicas incluyen el empleo de glucocorticoides, clacioantagonistas, fenobarbital, flunitrazepan, clonazepan, difenilhidantoína y sulfato de magnesio intravenoso. El seguimiento de la enfermedad se hace mediante la evaluación frecuente del estado físico de la enferma, EEG, tomografía computada y resonancia magnética. Las emergencias quirúrgicas requieren de la presencia de un neurocirujano. En este artículo revisamos la fisiopatología, prevención y tratamiento del daño neurológico debido a la preeclampsia-eclampsia

Pseudotumor cerebri: relato de um caso / Pseudotumor cerebi: a case report

Os autores descrevem um caso de pseudotumor cerebri em uma menina de 6 anos de idade, com evoluçäo favorável com 1 ano de seguimento. É aventada a possibilidade de que algumas drogas usadas no tratamento de um traumatismo prévio tenham sido os responsáveis pelo surgimento do problema, além da anemia exibida

Nuestra experiencia en el monitoreo de presión endocraneana en pediatria / Our experience on monitoring intracraneal pressure in pediatrics

Los objetivos de este trabajo fueron el monitoreo de presión endocraneana y el tratamiento de la hipertensión endocraneana en: traumatismo encefalocraneano, encefalopatía hipóxica isquémica, síndrome de REye, accidentes cerebrovasculares, meningoencefalitis, coma tóxico. La metodología empleada fue la selección de pacientes en base a las escalas de Glasgow, Levejoy, Conn y tomografía computada; monitoreo de PIC con sistema de fibra óptica y tornillo de Richmond: medidas generales y terapéuticas específicas: hiperventilación mecánica, coma barbitúrico, corticoides, diureticos, hipotermia, entre otros. Se monitorizaron y trataron 100 pacientes; 66 fueron traumatismos encefalocraneanos, 7 asfixias por inmersión, 8 síndromes de Reyes, un accidente cerebro vascular, 3 comas hepáticos fallecidos por hepatitis fulminante, 2 intoxicados por órgano-fosforados y monóxido de carbono, 4 electrocuciones con Conn de coma tres, 8 paradas cardiorespiratorias, 5 con heridas de bala craneoencefálicas. Una craneosintosis se utilizó como método de diagnóstico. Los signos clínicos de hipertensión endocraneana son tardíos. la precocidad en la utilización del método disminuye la morbimortalidad. La derivación precoz de los pacientes y en forma correcta favorece la supervivencia de los mismos. En los niños con encefalopatías agudas graves una terapéutica intensiva adecuada puede mejorar las elevadas cifras de morbimortalidad, como lo demuestran serias estadísticas mundiales

Hypertension intra-cranienne benigne

Benign intracranial hypertension [BICH]. We have accepted 3 criteries to define the BICH - Existence of an ICH clinical syndrome.- Existence of a high I.C.P. - Absence of clinical ICH etiologies that would wate known physiopthological mechanisms such as a neovolume, brain oedema or hydrocephalus. A study of 35 observations meeting this definition has allowed us to conclude that the BICH hes become more and more frequent in neurosurgical practice [6% of the total ICH in general and 7% of secondary ICH with an expansive intracranial development]. It is a disease that is to be found more frequently within young and obese women [4 women for 3 men, 22 years old, 71 kg for 1 m 61 in average for women]. Reasons for this disease are multiple and variable. They can be divided in 2 groups: * A group of patients with antecedents of which the cause-effect relationship seems to be arguable Among these: * 17 patients of our study, had in their antecedents a cranio-facial pathology with 6 quiet important head injuries, 8 O.R.L. diseases with 2 M.C.O.; 2 ophtalmological fits and a meningitis going back to childhood. * 4 patients had obstetrical and endocrinal antecedents, a thyroidal goitre, a dysmenorrhoea, a repeating gravidic toximia and recurring abortions. - Within 27 patients of our study we have noticed antecedents due to use of drugs, among them solycil [4 times], cyclines [Twin], penicillin [5 times], corticoids [6 times], contraceptive pills [twin] and A vitamin [once] without nevertheless being able to incriminate one of these factors in the BICH genesis. * 11 patients of our study have directly accused some decompensating factors which were immediately followed by the appearance of BICH symptoms that is how we have noticed amomg the triggering causes, 5 BEHCET diseases, 4 pregnancies, a head injury, tonsilitis treated with G penicillin, and a salpingitis treated with the Bactrim. The BICH symptomatology is one of an ICH without any localization signs associating a visual symptomatology of which the papilledema is one of the constant elements. Subjective visual troubles have been reported by the majority of our patients in the form of a decline in the visual acuity, a diplopia [11 cases], unsharp vision episodes [9 cases], transitory amaurosis [3 times]; and photophobia [3 times]. Four patients have reported having ear-humming and 8 others troubles in standing up and walking. The ophthalmological attack is the most important element in the objective symptomatology. This needs a particular attention as it determines the pronosis. The drop in the visual acuity from the very first test has been detected 24 times out of 33; 12 patients were unfortunately blind, seven had a very altered visual acuity [1/10 to 4/10], 5 an altered visual acuity [5/10 to 7/10] and only 9 had a normal visual acuity on their admission; out of the 14 indicating a carried out visual fields; 9 had campimetric defects extending from the simple expansion of the blind sport [6 times], to the global contraction of the isopters [1 case], to a systematized amputation evoking a bitemporal hemianopsia [1 case] or even a reduction of the visual field to a simple islet of central vision. The C.S.F. study appears mostly normal. Albumin rate was superior to 0,60 g/l in 6 cases but has never exceeded 1 g/l, Cytology has been in all cases inferior to 10 BE/mm3. Always systematically carried out and analysed, simple brain X-rays have shown ICH signs in 10 cases [28%] such as sutures disjunctions [7 times], finger-like impressions [5 times] and small modifications of the sella turcica [3 times]. The angiography have been carried out 32 times out of 35. The phlebographical times have clearly indicated a pathology of the intracranial veinous sinuses within 12 patients. It was either a size reduction, or a bad visualization, or an absence of injection of one or several of the sinuses. The pneumencephalography, ventriculography and the T.A.C. have been carried out in 31 cases. To measure the ventricular volume we have used the EVANS ratio whose normal volumes are in between 0,25 and 0,34 [24 cases]. 3 patients have shown a slight increase of the ventricular size and in 4 others, ventricles were inferior to the normal. In two cases, we have noticed the aspect of an empty sella on the T.A.C. The continuous recording of the ICP has allowed us to confirm the ICH in all cases. The average pressure in our study varied from 15 to 61 cm H20. 23 patients had an average ICP between 25 and 50 cm H20. We have had recourse to 3 symptomatic and therapeutic methods: - The brain anti-edema treatment [used in 27 cases] with in order of frequency in the use of corticoides [in 77%], the osmotherapy [17%] and the diuretics [14%]. - The repeated substractions of the C.S.F. have been carried out in all cases with an average number of 4 substractions per patient. - The external derivations of C.S.F. have been carried out with 14 patients of our study among which 2 have become complicated by a thrombosis of the superior vena cava on atrial catheter, in two cases, it was ICH on BEHCET diseases. The total follow up of the B.I.C.H. usually happens towards the clinical and manometric stabilisation in a delay going from 1 month to two years and a half after one or several attacks, The fonctional signs disappear first, after a general 4 months evolution, that is about 1 month before the normalization of the ICP [average time of evolution = 5 months]. The ophtalmological signs are the last to disapear 6 months and a half in general after the manometric remission. However few observations are to be stated as far as the stabilization of the BICH is concerned. 1 - From the manometric point of view, 4 patients in our study have remained with a quite high I.C.P. [20 cm H20] in spite of the clinical and ophthalmological remissin. These 4 patients are in fact still in danger of falling into hypertensive fits on the smallest triggering factor. 2 - 6 patients of our study [18%] have had a relapse from their BICH and this in a delay of evolution which didn't exceed one year. 3 - If the majority of our patients have positively followed up, in particular on the ophthalmological level, thanks to an adequate and timely treatment; this has not unfortunately prevented an after - effect atrophy within 11 patients among whom 7 [20%] have kept their initial blindness. This leads.us to wonder about the qualificative " benign " associated with this type of ICH specially that 2 other patients of our study have been hospitalized and treated in emergency in a pre-engagement state. In the last chapter we have reported 4 physiopathogenic hypothesis classicaly accepted: a trouble in the resorption of the C.S.F., increase of the blood brain volume, cerebral edema, increase in the secretion of C.S.F. It seems that these 4 factors have an inter relationship with each other to give birth to the B.I.C.H. but the trouble in the resorption of C.S.F. appears to be the most important factor. A better knowledge of the physiopathogenic phenomena in the genesis of this disease should allow for a better knowledge of this disease and thus to foresee a better codified therapeutic approach