The Management of Spontaneous Otogenic CSF Leaks: A Presentation of Cases and Review of Literature.
Ontology highlight
ABSTRACT: Objective The types of otogenic cerebrospinal fluid (CSF) fistulae were previously classified into defects through, adjacent to, or distal to the otic capsule. This article presents cases of the three different types of spontaneous CSF fistulae and reviews pertinent literature. We examine the management of the different types of otogenic CSF leaks with modern audiovestibular testing, imaging, and surgical techniques. Design Case series and review of the literature. Setting Academic tertiary neurotologic referral practice. Participants Four patients identified through a retrospective search. Main outcome measures Resolution of CSF leak and absence of meningitis. Results Surgical intervention was performed on the four cases described in this series; none had a return of CSF otorrhea in the postoperative period or meningitis. Conclusions Otogenic CSF fistulae may lead to life-threatening infection and in congenital forms are typically not diagnosed unless meningitis has occurred. Rapid and proper recognition, work-up, and treatment of such leaks decrease the risk of permanent neurologic sequelae as well as recurrent meningitis.
Project description:BackgroundIdiopathic intracranial hypertension (IIH) is strongly associated with spontaneous skull-base cerebrospinal fluid (CSF) leaks. Venous sinus stenting (VSS) has proven effective for the treatment of IIH. Hence, its role in spontaneous skull-base CSF leaks is being explored actively.MethodsWe performed a systematic literature search across EMBASE, MEDLINE, Scopus, The Cochrane Library, and Google Scholar to identify studies reporting the use of VSS for spontaneous skull-base CSF leaks. Studies with pediatric patients, non-English articles, and nonspontaneous leaks were excluded. Failure of treatment (persistence / recurrence of CSF leak) was regarded as the primary outcome.ResultsEight studies with 62 patients undergoing VSS for spontaneous skull-base CSF leaks were included. Mean age of the patients was 51.9 years; 87.5% were females. Obesity was highly prevalent, with a mean body mass index of 33.9 kg/m2 (4 studies). IIH was noted in 74.6% patients (7 studies). Twenty-six patients (41.9%) underwent VSS alone whereas 36 patients (58.1%) underwent surgical repair + VSS. Seven patients (11.3%) had a failure of treatment. Three failures from one study could not be definitively ascribed to either of the groups. Hence, the estimated failure rate for VSS alone ranged from 18.6% (95% CI [0.02 - 0.46]) to 26.4% (95% CI [0.11 - 0.46]), whereas that for surgical repair + VSS ranged from 5.5% (95% CI [0.00 - 0.16]) to 12.2% (95% CI [0.01 - 0.32]). Furthermore, the estimated rate for resolution of concomitant IIH-related symptoms was 88.7% (95% CI [0.75 - 0.98%]). Majority of the studies did not report any serious complications or mortality related to VSS.ConclusionVSS has a potential role in the management of spontaneous skull-base CSF leaks. Its exact indications as a standalone treatment versus as an adjuvant to surgical repair, and the predictors for successful treatment remain to be defined.
Project description:Spontaneous intracranial hypotension (SIH) is a debilitating condition caused by spinal CSF leaks or CSF-venous fistulas (CVFs). Localizing the causative CSF leak or CVF is critical for definitive treatment but can be difficult using conventional myelographic techniques because these lesions are often low contrast compared to background, diminutive, and in some cases may be mistaken for calcified structures. Dual energy CT (DECT) can increase the conspicuity of iodinated contrast compared to background and can provide the ability to distinguish materials based on differing anatomic properties, making it well suited to address the shortcomings of conventional myelography in SIH. The purpose of this report is to illustrate the potential benefits of using DECT as an adjunct to traditional myelographic techniques in order to increase the conspicuity of these often-subtle CVFs and CSF leaks. This retrospective case series included 4 adult patients with SIH who demonstrated findings equivocal for either CVF or CSF leak using our institution's standard initial CT myelogram and in whom subsequent evaluation with DECT ultimately helped to identify the CVF or CSF leak. DECT demonstrated utility by increasing the conspicuity of two subtle CVFs compared to background and also helped to differentiate between calcified osteophytes and extradural contrast in 2 CSF leaks, confirming their presence and identifying the causative pathology. Our observations demonstrate the benefit of DECT as a problem-solving tool in the accurate diagnosis and localization of CVFs and CSF leaks.
Project description:ObjectiveTo review the literature and our institutional experience regarding the risk of meningitis in patients with spontaneous lateral skull base cerebrospinal fluid (sCSF) leaks awaiting surgical repair, and the roles of antibiotic prophylaxis and pneumococcal vaccination, if known.MethodsA retrospective chart review and systematic review of the literature was undertaken to identify the incidence of meningitis in patients with sCSF leaks awaiting surgical repair. Adults managed surgically for sCSF leaks at an academic tertiary care center over a 10-year period were included. Data was collected on receipt of prophylactic antibiotics and/or pneumococcal vaccines during the timeframe between diagnosis and surgical repair.ResultsInstitutional review identified 87 patients who underwent surgical repair of spontaneous leaks, with a 0% incidence of meningitis over a median duration of 2 months while awaiting surgery (mean 5.5 months, range 0.5-118 months). Eighty-eight percent of patients did not receive prophylactic antibiotics. No studies in the published literature demonstrated the impact of prophylactic antibiotics or pneumococcal vaccine on meningitis risk.ConclusionsThere appears to be a low risk of meningitis among patients with lateral skull base sCSF leaks awaiting surgery for short durations (≤2 months), even in the absence of prophylactic antibiotics. There is a substantial gap in the published literature assessing the risk of meningitis and roles of antibiotics and vaccination in this patient population, indicating the need for large-scale study to conclusively elucidate the nature of this risk.
Project description:Spontaneous urinoma is a urological entity that can be complicated by a retroperitoneal abscess. Urinoma can be iatrogenic, traumatic, or can be caused by obstructive uropathy. We report two cases of spontaneous urinomas, describing a not previously published cause of urinoma: pyeloureteritis. (I) A 55-year-old Caucasian female started with intense left-sided flank pain for 3 days. CT scan revealed a voluminous retroperitoneal abscess, which extended through the posterior pararenal space and the left lumbar paravertebral musculature. (II) A 48-year-old Caucasian male presented with constitutional symptoms over the previous 2 months and investigations showed a voluminous urinoma and marked pyeloureteritis findings on CT scan. Both patients were managed by endoscopic placement of a double-J stent and drainage of the collection (open and percutaneous approach, respectively). Urinoma was confirmed by fluid biochemical analysis, which demonstrated that fluid creatinine was markedly raised as compared to the serum creatinine. Urinoma in the absence of obstruction or trauma is rare. These two case reports highlight one of the conditions leading to urinoma and the management of retroperitoneal abscess. Pyeloureteritis is a cause of spontaneous urinoma and it should be considered in the lack of another obstructive cause.
Project description:Introduction Cerebrospinal fluid (CSF) rhinorrhea is the leakage of CSF through nasal cavity, due to abnormal communication between the arachnoid membrane and nasal mucosa. Middle-age (fourth to fifth decade) group, female gender, and obesity (body mass index > 40) are the most commonly reported risk-factors for this rare entity. In this study, we present our single center experience of spontaneous CSF rhinorrhea discussing important clinicoradiological aspects in preoperative evaluation and nuances in the endoscopic repair technique. Material and Methods A retrospective study conducted for 43 spontaneous CSF rhinorrhea patients admitted between Jan 2011 to Jan 2018 at our tertiary care center. All patients underwent endoscopic repair of the defect depending upon their site of leak. Results Mean age in our study was 36.7 ± 12.3 years (range: 9-62 years). Average BMI in males was found lower (28.7) as compared with females (32). Most common site of CSF leak was cribriform plate ( n = 32, 74.4%) and Planum was found to be the least common site ( n = 1, 2.3%) of CSF leak. Intraoperatively, 23 (53.5%) patients showed high-flow leak. Intrathecal injection of fluorescein dye was used to identify the site of CSF leakage in 15 cases (34.8%). The overall success rate of primary endoscopic repair in our study was 95.3%. Conclusion Spontaneous CSF rhinorrhea occurs secondary to elevated intracranial pressure, with a predilection for obese females in fourth to fifth decade. Individualized tailored surgical approach depending upon the site, size, and flow-variety of the defect forms the cornerstone of management.
Project description:The pathophysiology of giant cerebral aneurysms renders them difficult to treat. Advances in technology have attempted to address any shortcomings associated with open surgery or endovascular therapies. Since the introduction of the flow diversion technique, the endovascular approach with flow diversion has become the first-line modality chosen to treat giant aneurysms. A subset of these giant aneurysms may persistent despite any treatment modality. Perhaps the best option for these recurrent and/or persistent giant aneurysms is to employ a multimodal approach-both surgical and endovascular-rather than any single technique to provide a curative result with favourable patient outcomes. This paper provides a review of the histopathology and treatment options for giant cerebral aneurysms. Additionally, an illustrative case is presented to highlight the unique challenges of a curative solution for giant cerebral aneurysms that persist despite initial treatment.
Project description:Lemmel syndrome is a rare clinical entity characterized by the presence of a periampullary duodenal diverticulum resulting in compression and dilatation of the pancreatic and common bile ducts, accompanied by obstructive jaundice. Gastric outlet obstruction is not a known complication of this syndrome, and there are no standardized approaches to its treatment. We present the first case of Lemmel syndrome presenting as gastric outlet obstruction and provide the results of a systematic literature review.
Project description:Gray platelet syndrome (GPS) is a rare hereditary hemorrhagic disorder characterized by macrothrombocytopenia and the absence of alpha-granules in platelets. Clinically, mild-to-moderate bleeding is the main manifestation, often accompanied by thrombocytopenia, splenomegaly, and myelofibrosis. Here, we present a case of a 15-year-old male patient with a history of hepatosplenomegaly, and thrombocytopenia for 8 years, who presented with sudden generalized abdominal pain. Despite initial suspicion of gastroenteritis, diagnostic imaging revealed an extensive hemoperitoneum. Subsequent genetic testing confirmed the diagnosis of GPS, which had not been previously identified. This case highlights the importance of considering inherited platelet disorders should be considered in adolescents with long-standing thrombocytopenia, and emphasizes the need for thorough evaluation in patients with suggestive symptoms.
Project description:Chromoblastomycosis and leprosy are chronic diseases with high prevalence in tropical and subtropical regions. Brazil is one of the countries with the highest incidence and prevalence for both diseases, however, reports of co-infections are scarce. The aim of this study was to describe three cases of chromoblastomycosis-leprosy co-infection in patients from Mato Grosso state, Brazil. A review of chromoblastomycosis-leprosy co-infection was performed of English, Portuguese and Spanish publications in LILACS, SciELO, PubMed and Web of Science databases using the descriptors (chromoblastomycosis OR cromoblastomicose OR cromoblastomicosis) AND (leprosy OR hanseníase OR lepra), without time period delimitation. Nineteen cases were included, 16 cases were published in 11 articles, plus the three cases reported in the current study. Most reported coninfection cases came from Brazil. Majority of the patients were male with a mean age of 52.2 years. Farmer was the main occupational activity reported. In 12 patients, the clinical signs and symptoms of leprosy started first. No contacts with patients affected by leprosy, armadillos or history of injuries at the anatomical site of chromoblastomycosis lesions were reported. Five leprosy patients who received steroid treatment for leprosy reactions or neuropathies, were diagnosed with chromoblastomycosis during immunosuppressive therapy. Four cases (21.1%) were reported among the elderly patients. Co-infections in patients with chromoblastomycosis or leprosy are uncommon, but the possibility should always be considered, especially if the patient is undergoing immunosuppressive treatment or is elder.
Project description:In patients with occult cerebrospinal fluid (CSF) leaks or CSF leak syndrome, orthostatic headaches are a common presenting symptom. Although computed tomography (CT) myelography has historically been the gold standard for diagnosis with radioisotope cisternography as a diagnostic alternative, magnetic resonance imaging (MRI) myelography using intrathecal gadolinium has reported sensitivity of 80%-87%. Two patients with spontaneous orthostatic headaches lasting for several days were diagnosed with CSF leaks at multiple thoracic segments using MRI myelogram with intrathecal gadolinium (Gadavist, Bayer, Whippany, NJ). This allowed for subsequent targeted treatment with CT fluoroscopy guidance, resulting in therapeutic responses within 1-2 treatment with targeted blood patching. Although intrathecal gadolinium is an off-label use, the superior contrast resolution and lack of radiation exposure makes MRI myelography an excellent imaging modality for diagnosing CSF leak, targeting treatment, and monitoring outcomes compared to CT myelography and radioisotope cisternography.