Síndrome de hipotensión intracraneal en paciente pediátrico con síndrome de Marfan / Intracranial hypotension syndrome in a pediatric patient with Marfan syndrome

El síndrome de hipotensión intracraneal (SHI) es consecuencia del descenso de la presión de líquido cefalorraquídeo (LCR), espontáneo o secundario a antecedente traumático o enfermedad sistémica.Presentamos el caso de un niño de 11 años con cefalea ortostática y vómitos de 12 horas de evolución tras una caída sobre región sacrocoxígea; antecedente de síndrome de Marfan. La resonancia craneomedular mostró colecciones líquidas extradurales a nivel dorsal y lumbosacro compatibles con fístula de LCR. El cuadro se resolvió tras tratamiento, pero hubo dos nuevos episodios durante el seguimiento, por lo que se aplicó un parche hemático epidural a los dos años del inicio.Aunque el SHI es infrecuente en niños, debe sospecharse ante pacientes con cefalea ortostática, especialmente si existe patología favorecedora como enfermedades del tejido conectivo. Existe poca evidencia sobre el manejo en edad pediátrica, por lo que se presenta este caso y se realiza una revisión de la literatura.(AU)

Intracranial hypotension syndrome (IHS) is attributed to reduced cerebrospinal fluid (CSF) pressure. It may be spontaneous or secondary to a history of trauma or systemic disease. We present the case of an 11-year-old boy, with medical history of Marfan syndrome, with orthostatic headache and persistent vomiting (12 hours) following a fall on the sacrococcygeal region. Magnetic resonance showed extradural fluid collections at dorsal and lumbosacral levels, compatible with CSF leak. The condition was resolved with treatment, but the patient had two new episodes during the follow-up period. Thus, an epidural blood patch was performed two years after the first episode.Although HIS is uncommon in children, it should be suspected in patients with orthostatic headache, particularly if the patient presents a connectivopathy. Few studies have assessed the management of HIS in paediatric age. The case presented here and the reviewed available literature provides further data for these type of cases.(AU)

First-line steroid treatment for spontaneous intracranial hypotension.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a syndrome characterized by low cerebrospinal fluid (CSF) pressure and postural headaches, and affects 1 per 20,000 individuals every year. CASE REPORT: We report an otherwise healthy 38-year-old man admitted to the hospital with orthostatic headache that developed 48 h after a short-haul flight during which he sustained a neck injury due to turbulence. Neurological examination, blood analysis and computed tomography scan performed at the emergency service were normal. Brain and spine magnetic resonance imaging (MRI) showed diffuse pachymeningeal enhancement and contrast medium egress from the subarachnoid space into the epidural space at the level of C2. The patient was treated with bed rest, hydration and 1 mg/kg/day oral prednisone for 5 days, with a gradual withdrawal in the following 7 days. Complete symptomatic relief was observed after 16 days, with resolution of the pathological findings on brain and spinal MRI after 1 month, except for localized pachymeningeal enhancement. Clinical relief was maintained over time until last follow-up visit 9 months later. CONCLUSION: Successful conservative treatment barely exceeds one quarter of cases of SIH. The clinical benefits of steroids may result from several mechanisms of action, for example, improving brain oedema and inflammation, determining fluid retention, and facilitating reabsorption of the CSF from extradural space. Notwithstanding that epidural blood patch remains the most successful treatment for SIH, future studies should explore the effectiveness of steroids as first-line therapy in addition to the most commonly suggested measures of bed rest and hydration.

Epidural blood patch for the treatment of liquor hypotension after intrathecal chemotherapy in a 10-year-old: case report.

BACKGROUND AND OBJECTIVES: An epidural blood patch is used to treat postdural puncture and liquor hypotension headache. We report the use of an epidural blood patch in a critical pediatric patient. CASE REPORT: A 10-year-old girl with acute leukemia developed venous cerebral thrombosis with hemorrhagic transformation one month after intrathecal chemotherapy. Given the unusual clinical and imagiological evolution even after decompressive craniectomy, we suspected cerebrospinal fluid hypotension. Spine imaging revealed signs of post-lumbar puncture fistula; we hence performed a blind blood patch. CONCLUSIONS: Recognizing cerebrospinal fluid hypotension in critical pediatric patients is important. Less-conventional life-saving measures, such as a blind blood patch, may be considered in such patients.

Postoperative Treatment of Intracranial Hypotension Venous Congestion-Associated Brain Injury With Zolpidem.

ABSTRACT: A previously independent 75-yr-old man developed postoperative intracranial hypotension-associated venous congestion after an elective T10-pelvis fusion, which was complicated by durotomy. Postoperative day 0 magnetic resonance imaging noted symmetric edema of the basal ganglia, thalami, and cerebellar cortex as well as smooth diffuse pachymeningeal enhancement and dural thickening, consistent with postoperative intracranial hypotension-associated venous congestion. On postoperative day 0, patient developed tonic clonic seizures, and on postoperative day 2, patient was unable to follow commands or blink to visual threat, able to track eyes to sound only, and spontaneously moved all limbs. Patient was started on zolpidem 2.5 mg on postoperative day 2, and 12 hrs later, he had significantly improved motor function, arousal, verbalization, and followed simple commands. After three doses, patient was fully alert and oriented with improved mobility and comprehension. Six zolpidem doses were administered in total, and repeat magnetic resonance imaging on postoperative day 16 showed markedly improved regional edema. The patient was admitted to a brain injury inpatient rehabilitation unit and was discharged to home 9 days later with Functional Independence Measure gain of 17. Intracranial hypotension can adversely affect primary mesocircuit structures supporting arousal. Zolpidem, a selective α-1-subunit GABA-A agonist, supports GABAergic tone in these regions. This patient's clinical presentation and recovery paralleled selective basal ganglial-thalamic edema development and resolution.

Greater Occipital Nerve Block as a Tool to Diagnose Spontaneous Intracranial Hypotension Before Epidural Blood Patch: A Case Report.

Spontaneous intracranial hypotension (SIH) has been increasingly characterized in recent years. A definitive diagnostic algorithm remains controversial because several symptoms are often found to be nonspecific. When neuroimaging fails to identify a cerebrospinal fluid leak and symptoms are atypical, an epidural blood patch (EBP) may be performed but not without risks. Our case shows how greater occipital nerve block (GONB) can expedite SIH diagnosis in a man with atypical presentation by reducing the sensory input from the posterior cranial fossa. The relief provided by GONB allowed to diagnose SIH promptly and the patient underwent a curative EBP.

Spontaneous intracranial hypotension associated with pachymeningeal enhancement in a patient with systemic lupus erythematosus (SLE): an extremely rare presenting feature.

A 16-years-old girl presented to our institution with history of severe bilateral headache for 5 days associated with vomiting. She also had fever for 2 months without any localising symptoms and skin lesions for 1 month. Examination revealed erythematous rash over bridge of nose and ear lobes, ulcer over hard palate and tenderness of small joints of both hands. Systemic examination was unremarkable except for bilateral papilloedema. Investigations revealed anaemia, leucopenia and elevated erythrocyte sedimentation rate. Cranial imaging revealed diffuse pachymeningeal enhancement with subdural effusion. Lumbar puncture revealed no abnormal findings in cerebrospinal fluid except low opening pressure. Antinuclear antibody was 4+ with anti-Sm antibody positive. She was diagnosed to have spontaneous intracranial hypotension associated with pachymeningeal enhancement secondary to systemic lupus erythematosus. She showed dramatic improvement with steroid and azathioprine therapy. She continues to be asymptomatic after 2 years of follow-up.

Epidural Blood Patch for the Treatment of Spontaneous and Iatrogenic Orthostatic Headache.

BACKGROUND: The cerebrospinal fluid (CSF) leakage could be happened spontaneously or related to the procedures such as spinal anesthesia, epidural anesthesia, CSF tapping, intrathecal chemotherapy or other spinal procedures. The leakage of CSF leads to intracranial hypotension of which distinguishing clinical feature is orthostatic headache. The epidural blood patch is a gold-standard treatment for intracranial hypotension-related orthostatic headaches. OBJECTIVE: We conducted this study to compare the efficacy and number of epidural blood patches for spontaneous and iatrogenic orthostatic headaches. STUDY DESIGN: Retrospective study. SETTING: University hospital inpatient and outpatient referred to our pain clinic. METHODS: Sex, weight, height, cause of orthostatic headache, leakage site evaluation test, epidural blood patch injection level, number of administered epidural blood patches, and pain intensity data were collected. We classified patients into two groups according to the cause of orthostatic headache: spontaneous (Group S) and iatrogenic (Group I). Patients with myelograms were also divided into 2 groups: multiple cerebrospinal fluid (CSF) leakages and no multiple leakages. RESULTS: Overall, 133 patients (162 procedures) were managed using epidural blood patches. Groups S and I included 34 and 99 patients, respectively. In Group I, 90.9% of the patients achieved complete recovery following a single procedure, whereas 44.1% of Group S patients required repeated procedures. The average number of administered epidural blood patches was significantly higher in Group S (1.48 ± 0.64) than in Group I (1.11 ± 0.35; P = 0.007). Among 23 patients evaluated via myelography, 12 had multiple CSF leakages. Patients with multiple leakages required a significantly higher number of epidural blood patches, compared to patients without multiple leakages (P = 0.023). LIMITATIONS: This retrospective study reveals several limitations including insufficient evaluation of CSF leakage site by myelogram and the retrospective nature of the study itself. CONCLUSIONS: Most patients with iatrogenic orthostatic headache required a single epidural blood patch, although most did not undergo a myelogram or similar test. Patients with spontaneous orthostatic headache or multiple CSF leakages were more likely to require a repeated epidural blood patch.Key words: CSF leakage, dural puncture, epidural blood patch, intracranial hypotension, orthostatic headache, spinal headache.

Treatment of spontaneous intracranial hypotension with intravenous Factor XIII administration: initial clinical experience.

AIM: Coagulation Factor XIII plays an important role in wound healing by stabilizing the fibrin clot. We hypothesized that Factor XIII administration might promote the repair of cerebrospinal fluid leak sites and lead to resolution of the orthostatic headache in patients with spontaneous intracranial hypotension (SIH). The aim of this study was to investigate the efficacy of intravenous Factor XIII administration in SIH patients. MATERIAL AND METHODS: A retrospective review of nine patients (four men, five women; mean age 42.3 yr) with SIH resistant to conservative treatment (bed rest, hydration and analgesics) was performed. All patients had an orthostatic headache. Intravenous administration of Factor XIII (1200 units per day for at least five days) was additionally performed on all patients. RESULTS: The orthostatic headache completely resolved and never reoccurred in six patients (67%), and partially resolved in two patients (22%). One patient (11%) had no change in headache activity. No complications occurred in any patients treated with Factor XIII. CONCLUSION: This study may suggest that intravenous administration of Factor XIII is useful for treating SIH, even if the patients are resistant to conservative treatment.

Spontaneous intracranial hypotension syndrome treated with fludrocortisone.

Spontaneous intracranial hypotension is a rare syndrome characterized by orthostatic headache not associated with trauma or dural puncture. In most cases, it is caused by a spontaneous spinal cerebrospinal fluid leakage as demonstrated by neuroradiological studies. The standard of care consists of conservative treatment including bed rest, hydration, and administration of caffeine or glucocorticoids. When such conservative therapy fails, an epidural blood patch is recommended. In this report, we describe the treatment of 2 patients with spontaneous intracranial hypotension who failed conservative treatment and went on to have complete and sustained resolution of their symptoms after the administration of oral fludrocortisone.

[Headache can be caused by spontaneous intracranial hypotension].

Spontaneous intracranial hypotension (SIH) is an uncommon condition associated with postural headache, nausea and dizziness. It is believed to be secondary to a dural tear with resultant cerebrospinal fluid (CSF) leak. This is a case report of a pregnant woman (gestational age 31 weeks) who contacted an obstetric department because of severe headache. Pre-eclamp-sia was suspected, but not found. An MRI showed a CSF leak at C1-C2 level and intracranial signs of SIH. The woman was treated with an autologous blood patch and recovered quickly. Focus on subjective symptoms and MRI findings seem to be important in the diagnostic procedure of SIH.

Spontaneous intracranial hypotension and epidural blood patch: a report involving seven cases.

Spontaneous intracranial hypotension is a rare condition caused by spontaneous cerebrospinal fluid leak. It is characterised by orthostatic headache, diffuse pachymeningeal enhancement on brain imaging and low cerebrospinal fluid pressure. Seven patients with spontaneous intracranial hypotension were treated conservatively: of these, four responded to drug treatment and three underwent a lumbar autologous epidural blood patch (EBP). A complete response was obtained in two patients after a single EBP; one patient underwent a second EBP and then became asymptomatic. Clinical improvement coincided with a dramatic reduction of pachymeningeal enhancement. The aetiology and brain imaging findings, and the technique and effectiveness of EBP are discussed.

Continued statin therapy could improve the outcome after spontaneous intracerebral hemorrhage.

Spontaneous intracerebral hemorrhage (ICH) often represents a devastating event despite maximal therapeutic efforts. Statins are drugs primarily used as cholesterol reducers with several pleiotropic effects that may result in neuroprotection. In this study, we assessed the continued use of statins after acute ICH. From January 2008 to October 2010, we analyzed a retrospective cohort of 178 patients with acute ICH. Patients with head injury, cerebral tumors, hemorrhage after ischemic stroke, and having a National Institute Health Stroke Scale (NIHSS) score of greater than 30 points on admission were excluded. In 29 patients, statins were continued within the first 24 h after onset of ICH and, subsequently, given daily until discharge, whereas 149 nonusers were used as controls. Inpatient mortality, NIHSS, and Glasgow Outcome Score (GOS) at discharge as well as mortality after 10 days, 3 months, and 6 months were recorded as outcomes. Additionally, changes of C-reactive protein (CRP) and white blood cell (WBC) counts, as well as aspartate transaminase and alanine transaminase levels were assessed. Except for the number of hypertensive and diabetic patients, characteristics on admission were similar between both groups. No mortality was observed in statin users, whereas 19 controls (12.7 %) died (p = 0.04) until discharge; after 10 days, 3 months, and 6 months, a similar trend was found. After 6 months, statin use was associated to lower mortality in regression models (OR = 0.32, 95 % CI = 0.11-0.95, p = 0.04). In the same way, statin use was related to NIHSS reduction (-3.53, 95 % CI = -7.59 to 0.42, p = 0.07). In mixed models, changes of WBC counts and CRP levels were associated with statin use. The hepatic enzymes were similar between groups. The continued use of statins after ICH could be associated to early neurological improvement and may reduce mortality within 6 months. Immunomodulation as a pleiotropic effect of statins may represent one of the underlying mechanisms.

Antioxidant carbon particles improve cerebrovascular dysfunction following traumatic brain injury.

Injury to the neurovasculature is a feature of brain injury and must be addressed to maximize opportunity for improvement. Cerebrovascular dysfunction, manifested by reduction in cerebral blood flow (CBF), is a key factor that worsens outcome after traumatic brain injury (TBI), most notably under conditions of hypotension. We report here that a new class of antioxidants, poly(ethylene glycol)-functionalized hydrophilic carbon clusters (PEG-HCCs), which are nontoxic carbon particles, rapidly restore CBF in a mild TBI/hypotension/resuscitation rat model when administered during resuscitation--a clinically relevant time point. Along with restoration of CBF, there is a concomitant normalization of superoxide and nitric oxide levels. Given the role of poor CBF in determining outcome, this finding is of major importance for improving patient health under clinically relevant conditions during resuscitative care, and it has direct implications for the current TBI/hypotension war-fighter victims in the Afghanistan and Middle East theaters. The results also have relevancy in other related acute circumstances such as stroke and organ transplantation.

Doble parche hemático epidural como tratamiento de un síndrome de hipotensión intracraneal espontánea / No disponible

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