Trombose de seio sigmoide secundária a fístula liquórica espontânea para osso temporal: um relato de caso / Sigmoid sinus thrombosis secondary to spontaneous temporal bone cerebrospinal fluid fistula: a case report

A fístula liquórica para o osso temporal constitui um evento raro que decorre da comunicação anormal entre o espaço subaracnóideo e as células da mastoide, permitindo que o líquido cefalorraquidiano flua para as porções pneumatizadas do osso temporal. Tem como consequência a hipotensão intracraniana espontânea, caracterizada por perda de líquor e pela manifestação clínica de cefaleia ortostática. Acredita-se que a hipotensão intracraniana espontânea crie condições hemodinâmicas favoráveis à ocorrência de trombose venosa cerebral, uma desordem potencialmente fatal e de difícil diagnóstico, visto a inespecificidade de sinais clínicos e sintomas. Dessa forma, é pertinente atentar para a possibilidade de trombose venosa cerebral em pacientes com fístulas liquóricas, especialmente quando houver mudança do padrão da cefaleia, que passa de ortostática a intensa e contínua.

Temporal bone cerebrospinal fluid fistula is a rare event that results from abnormal communication between the subarachnoid space and the mastoid cells, allowing the cerebrospinal fluid to flow into the pneumatized portions of the temporal bone. It leads to spontaneous intracranial hypotension, characterized by loss of cerebrospinal fluid and orthostatic headache as a clinical manifestation. Spontaneous intracranial hypotension is believed to create favorable hemodynamic conditions to the occurrence of cerebral venous thrombosis, a potentially fatal disorder of difficult diagnosis given the nonspecific clinical signs and symptoms. Therefore, it is pertinent to consider the possibility of cerebral venous thrombosis in patients with cerebrospinal fluid fistulas, especially when there is a modification in the headache pattern from orthostatic to intense and continuous pain.

Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Neurological Symptoms of Intracranial Hypotension

Intracranial hypotension usually arises in the context of known or suspected leak of cerebrospinal fluid (CSF). This leakage leads to a fall in intracranial CSF pressure and CSF volume. The most common clinical manifestation of intracranial hypotension is orthostatic headache. Post-dural puncture headache and CSF fistula headache are classified along with headache attributed to spontaneous intracranial hypotension as headache attributed to low CSF pressure by the International Classification of Headache Disorders. Headache attributed to low CSF pressure is usually but not always orthostatic. The orthostatic features at its onset can become less prominent over time. Other manifestations of intracranial hypotension are nausea, spine pain, neck stiffness, photophobia, hearing abnormalities, tinnitus, dizziness, gait unsteadiness, cognitive and mental status changes, movement disorders and upper extremity radicular symptoms. There are two presumed pathophysiologic mechanisms behind the development of various manifestations of intracranial hypotension. Firstly, CSF loss leads to downward shift of the brain causing traction on the anchoring and supporting structures of the brain. Secondly, CSF loss results in compensatory meningeal venodilation. Headaches presenting acutely after an intervention or trauma that is known to cause CSF leakage are easy to diagnose. However, a high degree of suspicion is required to make the diagnosis of spontaneous intracranial hypotension and understanding various neurological symptoms of intracranial hypotension may help clinicians.

Spontaneous Intracranial Hypotension Complicated by Cerebral Venous Thrombosis Relieved by Epidural Blood Patch

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse dural thickening, and enhancement in magnetic resonance imaging. Cerebral venous thrombosis (CVT) has been reported to be a rare complication of SIH. There is no consensus in anticoagulation treatment of CVT secondarily caused by SIH. We report a female patient with SIH complicated by CVT and spontaneously regressed CVT not by anticoagulation but by epidural blood patch.

Cervical Cerebrospinal Fluid Leakage Concomitant with a Thoracic Spinal Intradural Arachnoid Cyst / 대한신경손상학회지

We encountered a very rare case of spontaneous spinal cerebrospinal fluid (CSF) leakage and a spinal intradural arachnoid cyst (AC) that were diagnosed at different sites in the same patient. These two lesions were thought to have interfered with the disease onset and deterioration. A 30-year-old man presented with sudden neck pain and orthostatic headache. Diplopia, ophthalmic pain, and headache deteriorated. CSF leakage was confirmed in C2 by radioisotope cisternography, and an epidural blood patch was performed. While his symptoms improved gradually, paraparesis suddenly progressed. Thoracolumbar magnetic resonance imaging (MRI) revealed an upper thoracic spinal intradural AC, which was compressing the spinal cord. We removed the outer membrane of the AC and performed fenestration of the inner membrane after T3-4 laminectomy. Postoperative MRI showed complete removal of the AC and normalized lumbar subarachnoid space. All neurological deficits including motor weakness, sensory impairment, and voiding function improved to normal. We present a case of spontaneous spinal CSF leakage and consecutive intracranial hypotension in a patient with a spinal AC. Our report suggests that if spinal CSF leakage and a spinal AC are diagnosed in one patient, even if they are located at different sites, they may affect disease progression and aggravation.

Multiple epidural fibrin glue patches in a patient with spontaneous intracranial hypotension:A case report

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a condition caused by spontaneous leakage of cerebrospinal fluid, with postural headache as the primary symptom. Orthostatic headache caused by SIH is often not resolved by conservative management. CASE: We performed 15 epidural blood patch treatments in a 43-year-old female patient; however, they were only transiently effective. To improve the patient's SIH and orthostatic headache, epidural fibrin glue patch treatment was attempted. Fibrin glue is a substance that can act as a bio-friendly adhesive by facilitating the coagulation cascade. In our case, 3 epidural fibrin glue patch treatments were performed and the symptoms completely resolved. CONCLUSIONS: The epidural fibrin glue patch may be beneficial for the treatment of refractory postural headaches caused by SIH.

Susceptibility-Weighted MR Imaging for the Detection of Isolated Cortical Vein Thrombosis in a Patient with Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) can be a rare risk factor of cerebral venous thrombosis. We describe a case of isolated cortical vein thrombosis (CVT) secondary to SIH and discuss the value of susceptibility-weighted imaging for the detection of isolated CVT.

A Case of Intracranial Hypotension after Horse Riding

No abstract available.

Spontaneous intracranial hypotension and its complications / Hipotensão intracraniana espontânea e complicações

ABSTRACT Spontaneous intracranial hypotension (SIH) is a syndrome that was unknown until the advent of magnetic resonance imaging (MRI). It is a cause of orthostatic headache, which remains underdiagnosed and, rarely, can result in several complications including dural venous sinus thrombosis, subdural hematoma and subarachnoid hemorrhage. Some of these complications are potentially life-threatening and should be recognized promptly, mainly by imaging studies. We reviewed the MRI of nine patients with SIH and describe the complications observed in three of these patients. Two of them had subdural hematoma and one had a dural venous sinus thrombosis detected by computed tomography and MRI. We concluded that MRI findings are of great importance in the diagnosis of SIH and its complications, which often influence the clinical-surgical treatment of the patient.

RESUMO Hipotensão Intracraniana Espontânea (HIE) é uma síndrome desconhecida até o advento das imagens de Ressonância Magnética (RM). É uma causa de cefaleia ortostática que permanece subdiagnosticada e raramente resulta em complicações, como trombose de seios venosos durais, hematoma subdural e hemorragia subaracnoidea. Algumas dessas complicações são potencialmente ameaçadoras à vida e devem ser prontamente reconhecidas pelos estudos de imagem. Nós revisamos as RM de 9 pacientes com HIE e descrevemos as complicações observadas em 3 casos. Dois deles tiveram hematoma subdural e um teve trombose de seio venoso dural detectados por tomografia computadorizada e RM. Concluímos que achados de RM são de grande importância no diagnóstico de HIE e suas complicações, frequentemente influenciando o tratamento clínico-cirúrgico do paciente.

Symptomatic Relief of Idiopathic Infratentorial Superficial Siderosis with Maintaining Supine Position

Superficial siderosis results from the deposition of hemosiderin in subpial layers of the central nervous system following hemorrhage in subarachnoid spaces. Infratentorial superficial siderosis (ISS) presents with unique clinical features including progressive hearing loss, ataxia, and myelopathy, and the most common cause of idiopathic ISS is dural abnormality. Here we report a case of idiopathic ISS with radiological findings of spontaneous intracranial hypotension, whose clinical symptoms of ISS including cerebellar dysfunction improved after supine position was maintained for 2 months.

Spontaneous Intracranial Hypotension Followed by Subdural Hemorrhage

No abstract available.

Epidural blood patch treatment of diplopia that developed after headache resolution in a patient with spontaneous intracranial hypotension

Sudden headache onset may rarely be caused by spontaneous intracranial hypotension (SIH). Other associated symptoms in patients with SIH are nausea, vomiting, vertigo, hearing alteration, and visual disturbance. This case report describes a 43-year-old female diagnosed with SIH who developed diplopia after resolution of an abrupt-onset headache, which was managed with conservative treatments, including bed rest and hydration. She was also diagnosed with secondary right sixth cranial nerve palsy. Although conservative management relieved her headache, the diplopia was not fully relieved. Application of an autologous epidural blood patch successfully relieved her diplopia, even after 14 days from the onset of visual impairment.

Iatrogenic Development of Cerebrospinal Fluid Leakage in Diagnosing Spontaneous Intracranial Hypotension / 가정의학회지

A 34-year-old woman came to the emergency room complaining of a severe orthostatic headache. Results of a cerebrospinal fluid tap and brain computed tomography were normal. Based on her history and symptoms, she was found to have spontaneous intracranial hypotension. She was hospitalized and her symptoms improved with conservative treatment. On the next day, her headache suddenly worsened. Cisternography was performed to confirm the diagnosis and determine the spinal level of her cerebrospinal fluid leak. It revealed multiple cerebrospinal fluid leaks in the lumbar and upper thoracic regions. It was strongly believed that she had an iatrogenic cerebrospinal fluid leak in the lumbar region. An epidural blood patch was performed level by level on the lumbar and upper thoracic regions. Her symptoms resolved after the epidural blood patch and she was later discharged without any complications. In this case, an iatrogenic cerebrospinal fluid leak was caused by a dural puncture made while diagnosing spontaneous intracranial hypotension, which is always a risk and hampers the patient's progress. Therefore, in cases of spontaneous intracranial hypotension, an effort to minimize dural punctures is needed and a non-invasive test such as magnetic resonance imaging should be considered first.

A Case of Spontaneous Intracranial Hypotension Accompanied by Acute Kidney Injury in a Child / 대한소아신경학회지

Spontaneous intracranial hypotension in childhood is rare, and a few cases have been reported as a cause of headache in children. A 9-year-old boy was admitted to our hospital with a 3-day history of new-onset headache that worsened upon standing or walking, and aggravating low back pain. No medical history of injury, connective tissue disorder or migraine was detected. A neurological examination revealed neck stiffness. His initial blood tests suggested acute kidney injury by increased blood urea nitrogen (BUN) and creatinine. Brain computed tomography (CT) and cerebral spinal fluid (CSF) analysis were normal: however, opening pressure was low (< 60 mm H₂O). Magnetic resonance imaging (MRI) of the spine showed a collection of cerebral spinal fluid in the dorsal extradural space throughout the entire thoracic and lumbar spine level. The patient was diagnosed as having spontaneous intracranial hypotension accompanied by acute kidney injury. Magnetic resonance myelography and spinal MRI performed 14 days later did not show any cerebrospinal fluid leak. The headache and back pain were alleviated with strict bed rest and hydration. He remained free of headache and back pain at the 2-month follow-up. Here, we report a case of a 9-year-old boy with spontaneous intracranial hypotension.

Extended Pneumocephalus after Drainage of Chronic Subdural Hematoma Associated with Intracranial Hypotension : Case Report with Pathophysiologic Consideration

Chronic subdural hematoma (SDH) is a well-known disease entity and is traditionally managed with surgery. However, when associated with spontaneous intracranial hypotension (SIH), the treatment strategy ought to be modified, as classical treatment could lead to unwanted consequences. A 59-year-old man presented with a case of SIH that manifested as a bilateral chronic SDH. He developed fatal extensive pneumocephalus and SDH re-accumulation as a complication of burr-hole drainage. Despite application of an epidural blood patch, the spinal cerebrospinal fluid leak continued, which required open spinal surgery. Chronic SDH management should not be overlooked, especially if the exact cause has not been determined. When chronic SDH assumed to be associated with SIH, the neurosurgeon should determine the exact cause of SIH in order to effectively correct the cause.

Cardiac arrest due to intracranial hypotension following pseudohypoxic brain swelling induced by negative suction drainage in a cranioplasty patient: a case report / 대한마취과학회지

Pseudohypoxic brain swelling (PHBS) is known to be an uncommon event that may occur during and following an uneventful brain surgery, when negative suction drainage is used. The cerebrospinal fluid loss related to suction drainage can evoke intracranial hypotension that progress to PHBS. The main presentations of PHBS are sudden unexpected circulatory collapses, such as severe bradycardia, hypotension, cardiac arrest, consciousness deterioration and diffuse brain swelling as seen with brain computerized tomography (CT). We present a stuporous 22-year-old patient who underwent cranioplasty under general anesthesia. The entire course of the general anesthesia and operation progressed favorably. However, the time of scalp suture completion, sudden bradycardia and hypotension occurred, followed by cardiac arrest immediately after initiation of subgaleal and epidural suction drainage. After successful resuscitation, the comatose patient was transferred to the neurosurgical intensive care unit and PHBS was confirmed using brain CT.

Intracranial pressure and skull remodeling

In this article we review bony changes resulting from alterations in intracranial pressure [ICP] and the implications for ophthalmologists and the patients for whom we care. Before addressing ophthalmic implications, we will begin with a brief overview of bone remodeling. Bony changes seen with chronic intracranial hypotension and hypertension will be discussed. The primary objective of this review was to bring attention to bony changes seen with chronic intracranial hypotension. Intracranial hypotension skull remodeling can result in enophthalmos. In advanced disease enophthalmos develops to a degree that is truly disfiguring. The most common finding for which subjects are referred is ocular surface disease, related to loss of contact between the eyelids and the cornea. Other abnormalities seen include abnormal ocular motility and optic atrophy. Recognition of such changes is important to allow for diagnosis and treatment prior to advanced clinical deterioration. Routine radiographic assessment of bony changes may allow for the identification of patient with abnormal ICP prior to the development of clinically significant disease

Epidural Blood Patch Performed for Severe Intracranial Hypotension Following Lumbar Cerebrospinal Fluid Drainage for Intracranial Aneurysm Surgery. Retrospective Series and Literature Review

Intracranial hypotension (IH) can occur following lumbar drainage for clipping of an intracranial aneurysm. We observed 3 cases of IH, which were all successfully treated by epidural blood patch (EBP). Herein, the authors report our cases.

Intracranial Chronic Subdural Hematoma Presenting with Intractable Headache after Cervical Epidural Steroid Injection

Postdural punctural headache (PDPH) following spinal anesthesia is due to intracranial hypotension caused by cerebrospinal fluid (CSF) leakage, and it is occasionally accompanied by an intracranial hematoma. To the best of our knowledge, an intracranial chronic subdural hematoma (CSDH) presenting with an intractable headache after a cervical epidural steroid injection (ESI) has not been reported. A 39-year-old woman without any history of trauma underwent a cervical ESI for a herniated nucleus pulposus at the C5-6 level. One month later, she presented with a severe headache that was not relieved by analgesic medication, which changed in character from being positional to non-positional during the preceding month. Brain magnetic resonance imaging revealed a CSDH along the left convexity. Emergency burr-hole drainage was performed and the headache abated. This report indicates that an intracranial CSDH should be considered a possible complication after ESI. In addition, the event of an intractable and changing PDPH after ESI suggests further evaluation for diagnosis of an intracranial hematoma.

Infrequent Hemorrhagic Complications Following Surgical Drainage of Chronic Subdural Hematomas

Chronic subdural hematomas mainly occur amongst elderly people and usually develop after minor head injuries. In younger patients, subdural collections may be related to hypertension, coagulopathies, vascular abnormalities, and substance abuse. Different techniques can be used for the surgical treatment of symptomatic chronic subdural hematomas : single or double burr-hole evacuation, with or without subdural drainage, twist-drill craniostomies and classical craniotomies. Failure of the brain to re-expand, pneumocephalus, incomplete evacuation, and recurrence of the fluid collection are common complications following these procedures. Acute subdural hematomas may also occur. Rarely reported hemorrhagic complications include subarachnoid, intracerebral, intraventricular, and remote cerebellar hemorrhages. The causes of such uncommon complications are difficult to explain and remain poorly understood. Overdrainage and intracranial hypotension, rapid brain decompression and shift of the intracranial contents, cerebrospinal fluid loss, vascular dysregulation and impairment of venous outflow are the main mechanisms discussed in the literature. In this article we report three cases of different post-operative intracranial bleeding and review the related literature.