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Concept: Spontaneous cerebrospinal fluid leak

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Cerebrospinal fluid leaks (CSF) result from an abnormal communication between the subarachnoid space and the extracranial space. Approximately 90% of CSF leak at the anterior skull base manifests as rhinorrhea and can become life-threatening condition. Endoscopic sinus surgery (ESS) has become a common otolaryngologist procedure. The aim of this article is to consider our experience and to evaluate the outcomes in patients who underwent a purely endoscopic repair of CSF leaks of the anterior skull base.

Concepts: Spinal cord, Cerebrospinal fluid, Meninges, Spontaneous cerebrospinal fluid leak

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We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.

Concepts: Genetics, Collagen, Marfan syndrome, Genetic disorders, Pneumothorax, Connective tissue, Ehlers-Danlos syndrome, Spontaneous cerebrospinal fluid leak

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Cerebrospinal fluid (CSF) leaks are one of the most common complications of endoscopic sinus and skull base surgery, and are associated with significant morbidity and cost. A quality improvement program can be designed for individual surgeons or groups of surgeons to monitor outcomes and implement changes to decrease the risk of a postoperative CSF leak. Analytic tools include a root cause analysis, statistical analysis of risk factors, and predictive modeling. Monitoring of outcomes is accomplished with a time series plot or run chart. A circular action loop for assessing and implementing changes facilitates incremental progress and creates a learning culture for the institution.

Concepts: Scientific method, Statistics, Quality, Quality assurance, Cerebrospinal fluid, Time series, Root cause analysis, Spontaneous cerebrospinal fluid leak

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Background: A spontaneous cerebrospinal fluid leak can sometimes only become apparent following grommet insertion and usually represents dehiscence of the tegmen tympani, which is an uncommon condition. Objectives: This report aimed to reaffirm the importance of recognising this unusual presentation and outline management options. Case report: A 63-year-old man with conductive hearing loss and type B (flat) tympanometry underwent grommet insertion into his left ear, which resulted in cerebrospinal fluid otorrhoea. A defect of the tegmen tympani was found. This was successfully repaired via a transmastoid approach using a multi-layered grafting technique. Conclusion: Dehiscence of the tegmen tympani is uncommon and may only come to light following grommet insertion, which may be problematic for the uninformed otolaryngologist. Education is important to ensure early recognition and appropriate management.

Concepts: Ear, Conductive hearing loss, Spontaneous cerebrospinal fluid leak

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Spontaneous intracranial hypotension is characterized by postural headache that is generally associated with neck and/or back pain, radicular symptoms, nausea, and vomiting, and can sometimes be accompanied by cranial nerve symptoms. Although ocular manifestations are common, visual impairment due to optic nerve involvement is not commonly reported. Here, we report a case of a 34-year-old woman with postural headache and visual loss in the left eye associated with spontaneous intracranial hypotension. The orbital magnetic resonance imaging revealed increased intensity on T2-weighted images and on the pathologic contrast enhancement of the left optic nerve. Radionuclide cisternography showed bilateral cerebrospinal fluid leakage at the top of the thoracic region. To our knowledge, this is the first report documenting an association between optic neuropathy and spontaneous intracranial hypotension.

Concepts: Brain, Intracranial pressure, Magnetic resonance imaging, Multiple sclerosis, Cranial nerves, Ophthalmology, Optic nerve, Spontaneous cerebrospinal fluid leak

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BACKGROUND: Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension. METHODS: Medical records and radiologic imaging of the two patients were reviewed. RESULTS: Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.  Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia. CONCLUSIONS: Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.

Concepts: Brain, Traumatic brain injury, Neurology, Subdural hematoma, Hematoma, Headache, Lumbar puncture, Spontaneous cerebrospinal fluid leak

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BackgroundDecreased level of consciousness is a rare neurological manifestation of spontaneous intracranial hypotension (SIH), which typically presents with orthostatic headache. The optimal management of this uncommon presentation remains uncertain.MethodsWe analyzed the presentation, management and outcome of two patients in our institution and reviewed 22 patients reported in the literature with SIH and decreased level of consciousness, defined as any decrease in the patient’s Glasgow Coma Scale score.ResultsThere were 20 male and four female patients (M:F ratio of 5:1) with an average age of 52 years (range 37 to 68 years). There was a variable time interval of up to many months between the initial presentation of SIH and changes in the level of consciousness. An epidural autologous blood patch was eventually successful in 79% of the patients, although up to three trials were necessary in seven of these patients. Intrathecal saline infusion used as a temporizing measure resulted in excellent response within hours in five out of six patients who received this treatment. Drainage of the subdural collection either did not result in any sustained improvement or resulted in clinical deterioration in 12 out of 12 patients who received this treatment.ConclusionsIn the absence of a clinical trial because of the rarity of this entity, the treatment of SIH complicated by decreased level of consciousness remained controversial in the past. However, current collective experience supports early treatment of patients with SIH and decreased level of consciousness with one or more epidural blood patches. Fibrin glue and surgical duroplasty are the next steps in the management of patients in whom epidural blood patches fail. Drainage of the subdural collections may be detrimental.

Concepts: Patient, Neurology, Orthostatic hypotension, Glasgow Coma Scale, Coma, Result, Spontaneous cerebrospinal fluid leak, Orthostatic headache

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Headache occurs after dural puncture in about 1%-25% of children who undergo the procedure-a rate similar to that seen in adults. Persistence of post-dural puncture headache in spite of bed rest, increased fluid intake, and epidural blood patch treatment, however, is rare. The authors reviewed the medical records and imaging studies of all patients 19 years of age or younger who they evaluated between 2001 and 2010 for intracranial hypotension, and they identified 8 children who had persistent post-dural puncture headache despite maximal medical treatment and placement of epidural blood patches. A CSF leak could be demonstrated radiologically and treated surgically in 3 of these patients, and the authors report these 3 cases. The patients were 2 girls (ages 14 and 16 years) who had undergone lumbar puncture for evaluation of headache and fever and 1 boy (age 13 years) who had undergone placement of a lumboperitoneal shunt using a Tuohy needle for treatment of pseudotumor cerebri. The boy also had undergone a laminectomy and exploration of the posterior dural sac, but no CSF leak could be identified. All 3 patients presented with new-onset orthostatic headaches, and in all 3 cases MRI demonstrated a large ventral lumbar or thoracolumbar CSF collection. Conventional myelography or digital subtraction myelography revealed a ventral dural defect at L2-3 requiring surgical repair. Through a posterior transdural approach, the dural defect was repaired using 6-0 Prolene sutures and a dural substitute. Postoperative recovery was uneventful, with complete resolution of orthostatic headache and of the ventral cerebrospinal fluid leak on MRI. The authors conclude that persistent postdural puncture headache requiring surgical repair is rare in children. They note that the CSF leak may be located ventrally and may require conventional or digital subtraction myelography for exact localization and that transdural repair is safe and effective in eliminating the headaches.

Concepts: Spinal cord, Intracranial pressure, Neurology, Cerebrospinal fluid, Lumbar puncture, Spontaneous cerebrospinal fluid leak, Post dural puncture headache, Epidural blood patch

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Spontaneous intracranial hypotension (SIH) is a rare syndrome characterized by postural headache associated with a low cerebrospinal fluid pressure in the absence of dural puncture or penetrating trauma. Cranial magnetic resonance imaging (MRI) typically shows diffuse pachymeningeal gadolinium enhancement, subdural fluid collections, prominence of cerebral venous sinuses and brain descent. Visual signs and symptoms have been described infrequently in patients with SIH. These include third or sixth nerve palsy, superior nasal quadrantanopia and temporal hemianopia. We report a 34 year-old woman who presented with a two-year history of orthostatic headache, dizziness and transient visual obscurations. Campimetry showed a bilateral concentric visual field defect. She also described that intermittently a transparent fluid leaked out of her nose. She had no past history of trauma, sinus surgery or intracranial surgery. Cranial MRI was normal. Neuro-ophthalmological examination ruled out any other causes of concentric visual field defects. Lumbar puncture showed a cerebrospinal fluid (CSF) opening pressure of 9 cm H(2)O. Radioisotope cisternography suggested a dural leak at cribiform plate. The cribiform plate region was repaired endoscopically with improvement of all symptoms. One year later she remains asymptomatic and the visual field defects have improved.

Concepts: Spinal cord, Brain, Magnetic resonance imaging, Multiple sclerosis, Neurology, Cerebrospinal fluid, Visual field, Spontaneous cerebrospinal fluid leak

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Rathke’s cleft cysts (RCC) are benign lesions that originate from remnants of Rathke’s pouch. They can compress adjacent structures causing visual loss and endocrine dysfunction. The endoscopic endonasal transsphenoidal approach (EETA) has gained popularity in the surgical management of pituitary and parasellar tumors. However, postoperative cyst recurrence and endocrine dysfunction are still major concerns. A retrospective chart review was performed on 11 patients who underwent a purely EETA. Subtotal resection of the cyst wall with drainage of the intracystic contents followed by obliteration of the cyst with a fat graft was performed in all patients. Two patients underwent repeated surgeries for symptomatic cyst recurrence. One patient ultimately underwent extracapsular removal of the entire cyst wall because of multiple recurrences after simple drainage. There were no incidences of new permanent hypopituitarism, visual deficits, or postoperative cerebrospinal fluid leaks. All patients reported an improvement of initial preoperative symptoms. A non-aggressive strategy of partial cyst wall removal and simple drainage of cyst contents via EETA is a viable approach for surgical treatment of RCC with a low rate of postoperative endocrine and visual complications. A more aggressive strategy of extracapsular removal of the cyst wall may be indicated in patients with repeated recurrence.

Concepts: Neuroendocrinology, Sebaceous cyst, Anterior pituitary, Spontaneous cerebrospinal fluid leak, Rathke's pouch, Rathke's cleft cyst