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BACKGROUND: Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by abnormal and persistent peripheral blood hypereosinophilia (eosinophil count ≥ 1.5 × 109/L and ≥10% eosinophils) with duration ≥ 6 months, associated organ damage, and/or dysfunction attributable to tissue eosinophilic infiltrate of unknown cause. IHES affects different organs such as the heart, lungs, nervous system, and skin, with renal involvement being rare in this condition. CASE PRESENTATION: We present a case of a young patient with IHES and immune complex-mediated membranoproliferative glomerulonephritis with nephrotic syndrome, as a rare renal manifestation. We discuss the clinical, analytical, and histopathologic renal and hematologic features, comparing them with other reported cases in the literature.
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Prurito , Diálisis Renal , Humanos , Prurito/etiología , Prurito/tratamiento farmacológico , Masculino , Femenino , Persona de Mediana EdadRESUMEN
Phospholipidosis is a rare disorder which consists of an excessive intracellular accumulation of phospholipids and the appearance of zebra bodies or lamellar bodies when looking at them using electron microscopy. This disease is associated with certain genetic diseases or is secondary to drugs or toxins. Drug-induced phospholipidosis encompasses many types of pharmaceuticals, most notably chloroquine, amiodarone or ciprofloxacin. Clinically and histologically, renal involvement can be highly variable, with the diagnosis not being made until the zebra bodies are seen under an electron microscope. These findings may require genetic testing to discount Fabry disease, as its histological findings are indistinguishable. Most of the chemicals responsible are cationic amphiphilic drugs, and several mechanisms have been hypothesized for the formation of zebra bodies and their pathogenic significance. However, the relationship between drug toxicity and phospholipid accumulation, zebra bodies and organ dysfunction remains enigmatic, as do the renal consequences of drug withdrawal. We present, to our knowledge, the first case report of acute renal injury with a monoclonal gammopathy of renal significance, lesions, and sclerodermiform syndrome, with zebra bodies that were associated with the initiation of a hydroxychloroquine and amiodarone treatment, as an example of drug-induced-phospholipidosis.
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Amiodarona , Hidroxicloroquina , Fosfolípidos , Humanos , Lesión Renal Aguda/inducido químicamente , Amiodarona/efectos adversos , Hidroxicloroquina/efectos adversos , Hidroxicloroquina/uso terapéutico , Lipidosis/inducido químicamente , Paraproteinemias/inducido químicamente , Femenino , AncianoRESUMEN
Non-valvular atrial fibrillation (NVAF) is the most common cardiac arrhythmia in the general population, and its prevalence increases among patients with chronic kidney disease (CKD) undergoing hemodialysis. This population presents high risk of both hemorrhagic and thrombotic events, with little evidence regarding the use of oral anticoagulation treatment (OAT) and multiple complications arising from it; however, stroke prevention with percutaneous left atrial appendage closure (LAAC) is an alternative to be considered. We retrospectively describe the safety and efficacy of percutaneous LAAC in eight patients with NVAF and CKD on hemodialysis during a 12-month follow-up. The mean age was 78.8 years (range 64-86; SD ± 6.7), and seven patients were male. The mean CHA2DS2-VASC and HAS-BLED scores were high, 4.8 (SD ± 1.5) and 3.8 (SD ± 1.3), respectively. Seventy-five percent of the patients were referred for this intervention due to a history of major bleeding, with gastrointestinal bleeding being the most common type, while the remaining twenty-five percent of the patients were referred because of a high risk of bleeding. The percutaneous LAAC procedure was successfully completed in 100% of the patients, with complete exclusion of the appendage without complications or leaks exceeding 5 mm. There was one death not related to the procedure four days after the intervention. Among the other seven patients, no deaths, cardioembolic events or major bleeding were reported during the follow-up period. In our sample, percutaneous LAAC appears to be a safe and effective alternative to anticoagulation in patients with NVAF and CKD on hemodialysis.
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Apéndice Atrial , Fibrilación Atrial , Fallo Renal Crónico , Insuficiencia Renal Crónica , Accidente Cerebrovascular , Humanos , Masculino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Femenino , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/prevención & control , Accidente Cerebrovascular/epidemiología , Fibrilación Atrial/complicaciones , Fibrilación Atrial/cirugía , Cierre del Apéndice Auricular Izquierdo , Estudios Retrospectivos , Resultado del Tratamiento , Hemorragia/complicaciones , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Insuficiencia Renal Crónica/complicaciones , Anticoagulantes/uso terapéutico , Diálisis Renal/efectos adversos , Apéndice Atrial/cirugíaRESUMEN
La propagación vía hematógena de Neisseria gonorrhoeae, patógeno de transmisión sexual, resulta en una enfermedad gonocócica diseminada (EGD), también conocida como síndrome artritis-dermatitis por el desarrollo de lesiones cutáneas, tenosinovitis y artritis. La población que más frecuentemente se ve afetada es la de adultos jóvenes. Describimos el caso de una adolescente que de forma aguda desarrolló lesiones cutáneas, artritis, tenosinovitis y síntomas constitucionales por EGD. La identificación del agente causal fue por cultivo de secreción vaginal y fue tratada con ceftriaxona intravenosa durante 7 días, con recuperación clínica completa. Es importante diferenciar este cuadro clínico de otros tipos de artritis desarrollados en la adolescencia.(AU)
Hematogenous spread of Neisseria gonorrhoeae, a sexually transmitted pathogen, results in disseminated gonococcal disease (DGD), also known as arthritis-dermatitis syndrome, due to the development of skin lesions, tenosynovitis, and arthritis. The most frequently affected population is young adults. We describe the case of an adolescent female who acutely developed skin lesions, arthritis, tenosynovitis, and constitutional symptoms. The causal agent was identified by a culture of vaginal secretion and treated with ceftriaxone for 7 days with complete recovery. It is important to differentiate this clinical picture from other types of arthritis developed in adolescence.(AU)
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Humanos , Femenino , Adolescente , Tenosinovitis , Artritis Reumatoide , Gonorrea , Neisseria gonorrhoeae , Tenosinovitis/complicaciones , Pacientes Internos , Examen Físico , Reumatología , Pediatría , Evaluación de SíntomasRESUMEN
Hematogenous spread of Neisseria gonorrhoeae, a sexually transmitted pathogen, results in disseminated gonococcal disease (DGD), also known as arthritis-dermatitis syndrome, due to the development of skin lesions, tenosynovitis, and arthritis. The most frequently affected population is young adults. We describe the case of an adolescent female who acutely developed skin lesions, arthritis, tenosynovitis, and constitutional symptoms. The causal agent was identified by a culture of vaginal secretion and treated with ceftriaxone for 7 days with complete recovery. It is important to differentiate this clinical picture from other types of arthritis developed in adolescence.
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Artritis Infecciosa , Gonorrea , Tenosinovitis , Adolescente , Adulto Joven , Humanos , Femenino , Niño , Tenosinovitis/complicaciones , Antibacterianos/uso terapéutico , Gonorrea/complicaciones , Gonorrea/diagnóstico , Gonorrea/tratamiento farmacológico , Neisseria gonorrhoeae , Artritis Infecciosa/diagnósticoRESUMEN
Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström's macroglobulinemia. We present the case of a 52-year-old patient with a history of cutaneous vasculitis and hypothyroidism, who presented with generalized edema, moderate anemia, hypercholesterolemia, nephrotic range proteinuria of 12.69 g/day, microhematuria, arterial hypertension, and hypocomplementemia via the classical pathway, without acute kidney injury and with negative serological studies and positive cryoglobulins in the second determination. Serum and urine protein electrophoresis and immunofixation studies showed a monoclonal band of IgM and kappa light chain. Renal biopsy was consistent with cryoglobulinemic glomerulonephritis. In the context of dysproteinemia and cryoglobulinemic glomerulonephritis, bone-marrow aspiration and biopsy were performed, leading to the diagnosis of Waldenström's macroglobulinemia. Monoclonal gammopathies have been described in association with type I cryoglobulinemias. This described association is uncommon, which is why we present this case, along with a review of the literature.
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Glomerulonefritis , Gammopatía Monoclonal de Relevancia Indeterminada , Paraproteinemias , Macroglobulinemia de Waldenström , Humanos , Persona de Mediana Edad , Crioglobulinas , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnóstico , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Paraproteinemias/complicaciones , Paraproteinemias/diagnóstico , Macroglobulinemia de Waldenström/complicaciones , Macroglobulinemia de Waldenström/diagnósticoRESUMEN
SGLT-2i are the new standard of care for diabetic kidney disease (DKD), but previous studies have not included patients on kidney replacement therapy (KRT). Due to their high risk of cardiovascular, renal complications, and mortality, these patients would benefit the most from this therapy. Residual kidney function (RKF) conveys a survival benefit and cardiovascular health among hemodialysis (HD) patients, especially those on incremental hemodialysis (iHD). We retrospectively describe the safety and efficacy of SGLT2i regarding RKF preservation in seven diabetic patients with different clinical backgrounds who underwent iHD (one or two sessions per week) during a 12-month follow-up. All patients preserved RKF, measured as residual kidney urea clearance (KrU) in 24 h after the introduction of SGLT2i. KrU levels improved significantly from 4.91 ± 1.14 mL/min to 7.28 ± 1.68 mL/min at 12 months (p = 0.028). Pre-hemodialysis blood pressure improved 9.95% in mean systolic blood pressure (SBP) (p = 0.015) and 10.95% in mean diastolic blood pressure (DBP) (p = 0.041); as a result, antihypertensive medication was modified. Improvements in blood uric acid, hemoglobin A1c, urine albumin/creatinine ratio (UACR), and 24 h proteinuria were also significant. Regarding side effects, two patients developed uncomplicated urinary tract infections that were resolved. No other complications were reported. The use of SGLT2i in our sample of DKD patients starting iHD on a 1-2 weekly regimen appears to be safe and effective in preserving RKF.
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The present work demonstrates the application of the spray drying technique to produce microparticulates of different dietary fibers with particle sizes<10 µm. It examines their role as potential fat replacers for hazelnut spread creams. Optimization of a dietary fiber formulation containing inulin, glucomannan, psyllium husk, and chia mucilage to obtain high viscosity, water holding capacity, and oil holding capacity was conducted. Microparticles containing 46.1, 46.2, and 7.6 weight percentages of chia seed mucilage, konjac glucomannan, and psyllium husk showed a spraying yield of 83.45 %, a solubility of 84.63 %, and viscosity of 40.49 Pas. When applied to hazelnut spread creams, microparticles substituted palm oil by 100 %; they produced a product with a total unsaturated and saturated fat reduction of 41 and 77 %, respectively. An increase in dietary fibers of 4 % and a decrease in total calories of 80 % were also induced when compared with the original formulation. Hazelnut spread with dietary fiber microparticles were preferred by 73.13 % of the panelist in the sensory study due to an enhancement in brightness. The demonstrated technique could be used to increase the fiber content while decreasing the fat content in some commercial products, such as peanut butter or chocolate cream.
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Cacao , Corylus , Psyllium , Fibras de la DietaRESUMEN
This study screened for Fabry disease (FD) in patients in hemodialysis (HD) in the region of Madrid (CAM) with a cross-sectional design to evaluate HD-prevalent patients, followed by a three-year period prospective design to analyze HD-incident patients. INCLUSION CRITERIA: patients older than 18 years on HD in the CAM, excluding patients diagnosed with any other hereditary disease with renal involvement different from FD, that sign the Informed Consent (IC). EXCLUSION CRITERIA: underaged patients or not agreeing or not being capable of signing the IC. RESULTS: 3470 patients were included, 63% males and with an average age of 67.9±9.7 years. 2357 were HD-prevalent patients and 1113 HD-incident patients. For HD-prevalent patients, average time in HD was 45.2 months (SD 51.3), in HD-incident patients proteinuria was present in 28.4%. There were no statistical differences in plasmatic alpha-galactosidase A (α-GAL-A) activity or Lyso-GL-3 values when comparing HD-prevalent and HD-incident populations and neither between males and females. A genetic study was performed in 87 patients (2.5% of patients): 60 male patients with decreased enzymatic activity and 27 female patients either with a decreased GLA activity, increased Lyso-Gl3 levels or both. The genetic variants identified were: p.Asp313Tyr (4 patients), p.Arg220Gln (3 patients) and M290I (1 patient). None of the identified variants is pathogenic. CONCLUSIONS: 76% of HD Centers of the CAM participated in the study. This is the first publication to describe the prevalence of FD in the HD-population of a region of Spain as well as its average α-GAL-A-activity and plasmatic Lyso-Gl3 levels. It is also the first study that combines a cross-sectional design with a prospective follow-up design. This study has not identified any FD patient.
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Enfermedad de Fabry , Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Enfermedad de Fabry/epidemiología , Enfermedad de Fabry/genética , Enfermedad de Fabry/diagnóstico , Estudios Transversales , alfa-Galactosidasa/genética , Diálisis Renal , ProteinuriaRESUMEN
OBJECTIVE: Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults. As a result, a treatment concept for adult patients with a craniopharyngioma has not yet been established. The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence. METHODS: The demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery (n=50) or surgery for recurrence (n=18) were retrospectively analyzed. In addition, the patients were followed up for an average of 38.6 months (range: 1-133 months). RESULTS: The cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data, or radiological tumor features. However, patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma. The success rate of complete resection in primary surgery was 53.2%. Even after repeat surgery, a satisfactory rate of complete resection of 35.7% was achieved. Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery (postoperative bleeding P=0.560; meningitis P=1.000; CSF leak P=0.666; visual disturbance P=0.717) nor in patients with complete resection compared with those with partial resection. We found no difference in recurrence-free survival between initial surgery and repeat surgery (P=0.733). The recurrence rate was significantly lower after complete resection (6.9%) than after partial resection (47.8%; P<0.001). CONCLUSION: Attempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma. However, the surgeon must settle for less than total resection if postoperative morbidity is anticipated.
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Craneofaringioma , Neoplasias Hipofisarias , Humanos , Adulto , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico , Craneofaringioma/patología , Resultado del Tratamiento , Estudios Retrospectivos , Neoplasias Hipofisarias/cirugía , Procedimientos NeuroquirúrgicosRESUMEN
We present a case of a sizeable foramen magnum meningioma that was resected through a C1 hemilaminectomy in prone (concorde) position. The patient is a 51-year-old woman with a 3-month history of progressive paresthesia of the upper and lower extremities, followed by gait disturbance, and hand apraxia. There was no complaint of nuchal pain. On magnetic resonance imaging (MRI) a briskly enhancing extra-axial, intradural craniospinal lesion, extending from the basion of the lower clivus, over the tectorial membrane to the middle of the axis' body was discovered. There was significant transposition and compression of the medulla and corresponding focal hyperintensity on T2-weighted imaging. On physical examination, the patient was ambulatory independently, notwithstanding a pronounced spinal ataxia. There were deficits in sensation and proprioception, as well as urinary retention, but preserved function of the lower cranial nerves. In view of the profound transposition of the medulla, utilization of the corridor created by the tumor seemed feasible and we felt that a limited C1 hemilaminectomy would provide sufficient microsurgical access thus obviating a more extensive and invasive approach to the craniocervical junction. A gross-total resection was achieved; histopathology confirmed a World Health Organization (WHO) grade I angiomatous meningioma with a low-proliferation index. The patient was discharged home 3 days after surgery and her spinal ataxia resolved completely within 3 months of out-patient rehabilitation. At 3-year follow-up, there was no indication of residual or recurrence. The link to the video can be found at: https://youtu.be/WyShbfr-xi0 .
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Glial tumors in the cerebellopontine angle (CPA) are uncommon and comprise less than 1% of CPA tumors. We present four cases of pilocytic astrocytoma of the CPA (PA-CPA) that were treated in our department. Patients who received surgical treatment for PA-CPA from January 2004 to December 2019 were identified by a computer search of their files from the Department of Neurosurgery, Tübingen. Patients were evaluated for initial symptoms, pre- and postoperative facial nerve function and cochlear function, complications, and recurrence rate by reviewing surgical reports, patient documents, neuroradiological data, and follow-up data. We identified four patients with PA-CPA out of about 1500 CPA lesions (~ 0.2%), which were surgically treated in our department in the last 16 years. Of the four patients, three were male, and one was a female patient. Two were adults, and two were children (mean age 35 years). A gross total resection was achieved in three cases, and a subtotal resection was attained in one case. Two patients experienced a moderate facial palsy immediately after surgery (House-Brackmann grade III). In all cases, the facial function was intact or good (House-Brackmann grades I-II) at the long-term follow-up (mean follow-up 4.5 years). No mortality occurred during follow-up. Three of the patients had no recurrence at the latest follow-up (mean latest follow-up 4.5 years), while one patient had a slight recurrence. PA-CPA can be safely removed, and most complications immediately after surgery resolve in the long-term follow-up.
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Astrocitoma/cirugía , Ángulo Pontocerebeloso/cirugía , Manejo de la Enfermedad , Neuroma Acústico/cirugía , Procedimientos Neuroquirúrgicos/métodos , Anciano , Astrocitoma/complicaciones , Astrocitoma/diagnóstico por imagen , Ángulo Pontocerebeloso/diagnóstico por imagen , Niño , Parálisis Facial/diagnóstico por imagen , Parálisis Facial/etiología , Parálisis Facial/cirugía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico por imagen , Recurrencia Local de Neoplasia/cirugía , Neuroma Acústico/complicaciones , Neuroma Acústico/diagnóstico por imagenRESUMEN
CONTEXT: Craniopharyngioma is a rare neoplastic entity of the central nervous system. Childhood-onset craniopharyngioma is the subject of frequent research whereas the information on adult-onset craniopharyngioma is scarce. OBJECTIVE: The objective of this study was to examine the level of daily impairment in adult patients suffering from craniopharyngioma. DESIGN: Noninterventional patient registry indexed as PV4842 with the local ethics committee. SETTING: The study is set in a hospitalized and ambulatory setting. PATIENTS: 148 patients with adult-onset craniopharyngioma were recruited from 8 centers, 22 prospectively and 126 retrospectively. Mean follow-up was 31 months. INTERVENTIONS: No interventions performed. MAIN OUTCOME MEASURES: Complications, symptoms, body mass index (BMI), and quality of life (QoL; EORTC QLQ C30 and BN20) were recorded preoperatively and at follow-up. The hypotheses tested were generated after data collection. RESULTS: Complications were more frequent after transcranial than transsphenoidal approaches (31 % vs. 11%; P < 0.01). Preoperative obesity was present in 0% papillary and in 38% of all adamantinomatous craniopharyngiomas (P = 0.05), and diabetes insipidus was more frequent for papillary craniopharyngioma (36.8% vs. 16,7%; P < 0.05). Hormone deficits at follow-up were reduced in 16.9%, equal in 31.4%, and increased in 63.6% (P < 0.001). BMI increased from 28.7 ± 7.4 kg/m2 before surgery to 30.2 ± 7.4 kg/m2 at follow-up (P < 0.001). In QoL, a decrease of future uncertainty (62.5 vs. 36.8; P = 0.02) and visual disorders (38.9 vs. 12.0; P = 0.01) were observed in the prospective collective after surgery. CONCLUSIONS: Adult craniopharyngioma is associated with a complex sociological and psychological burden and hypothalamic dysfunction, warranting further investigation and emphasizing the need for a wider treatment approach.
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Craneofaringioma , Neoplasias Hipofisarias , Adolescente , Adulto , Edad de Inicio , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Craneofaringioma/diagnóstico , Craneofaringioma/epidemiología , Craneofaringioma/terapia , Femenino , Estudios de Seguimiento , Alemania/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos/efectos adversos , Procedimientos Neuroquirúrgicos/estadística & datos numéricos , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/epidemiología , Neoplasias Hipofisarias/terapia , Complicaciones Posoperatorias/epidemiología , Calidad de Vida , Estudios Retrospectivos , Hueso Esfenoides/cirugía , Adulto JovenRESUMEN
We present a case of a medium-sized foramen magnum meningioma that was resected through a midline suboccipital subtonsillar approach with C1 laminectomy in prone (Concorde) position. The patient is a 77-year-old woman with a 6-month history of intermittent vertigo, moderate gait instability, and slight decline of memory. On magnetic resonance imaging (MRI) an extra-axial intradural lesion was discovered that originated from the right epicondylar region just inferior to the jugular tubercle and occupied the anterolateral aspect of the foramen magnum. There was moderate transposition and compression of the medulla at the level of the cerebellar tonsils. On physical examination the patient was ambulatory independently without motor weakness but exhibited some gait instability. The function of the lower cranial nerves was preserved. A gross-total resection was achieved, histopathology confirmed a WHO grade-I meningothelial meningioma with a low-proliferation index. The patient was discharged home 5 days after surgery, her gait instability improved significantly immediately after surgery and had resolved completely after 2 weeks of inpatient rehabilitation. There was no other neurological deficit. At 3-month follow-up MRI, there was no indication of meningioma residual or recurrence. In summary, the midline suboccipital subtonsillar approach is a powerful tool with limited morbidity in the armamentarium for the microsurgical management of a variety of pathologies residing in the posterior cranial fossa and the craniocervical junction. Oftentimes the space created by the pathology opens up corridors that can be exploited for microsurgical access to avoid more extensive surgical approaches. The link to the video can be found at: https://youtu.be/0uUxs13ze7w .
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We present a case of a petroclival meningioma that was resected through an endoscope-assisted retrosigmoid approach via corridors above and below the facial-vestibulocochlear nerve complex. The patient is a 61-year-old female with complaints of left-sided hypesthesia and neuralgia of the infraorbital and zygomatic region, intermittent periorbital myokymia, and a slight facial palsy (HB II). This 2D video demonstrates the operative technique, anatomical and surgical nuances of the skull base approach and microdissection of the tumor from the critical neurovascular structures. A gross total resection was achieved. The patient's facial and trigeminal symptoms resolved completely within a few weeks. At 2 year follow up there was no indication of residual or recurrence. In summary, the retrosigmoid approach with endoscopic assistance is an important and powerful tool in the armamentarium for the microsurgical management of meningiomas of the lateroventral skullbase of the posterior fossa. The link to the video can be found at: https://youtu.be/Px4XIRDoALc .
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We present a case of a mid-sized vestibular schwannoma (T3b according to the Hannover classification) that was resected through a retrosigmoid transmeatal approach in semi-sitting position under endoscopic assistance. The patient is a 52-year-old male with acute loss of functional hearing on the right side. Audiometry confirmed a loss of up to 60 dB and lost speech discrimination, there were no associated symptoms such as tinnitus or vertigo. This 2D video demonstrates positioning, OR set-up, anatomical and surgical nuances of the skull base approach and the operative technique for microdissection of the tumor from the critical neurovascular structures, especially the facial and cochlear nerves. A gross total resection was achieved and the patient discharged home after four days with unaltered function of the facial nerve (HB I). At one year follow up there was no indication of residual or recurrence. In summary, the retrosigmoid transmeatal approach is an important and powerful tool in the armamentarium for the microsurgical management of all kinds of vestibular schwannomas. Provided the necessary anesthesiological precautions and intraoperative procedures the semi-sitting position is safe and effective. If needed, the approach can be complemented by the use of an endoscope for visualization of the distal internal auditory canal. The link to the video can be found at: https://youtu.be/pPKT4_5nIn0 .
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The anterior inferior cerebellar artery (AICA) usually runs loosely within the cerebellopontine cistern; in rare cases, however, it is firmly adherent to the petrous dura mater.1,2 Recognizing this variation is particularly important in vestibular schwannoma surgery via the retrosigmoid transmeatal approach to prevent the high morbidity associated with vascular injury. This video demonstrates a surgical technique to effectively mobilize the AICA when firmly adherent to the petrous dura mater. A 39-year-old man presented with a history of progressive right-sided hearing loss without facial weakness or other associated symptoms3. Magnetic resonance imaging (MRI) demonstrated an intracanalicular lesion, suggestive of vestibular schwannoma. During follow-up, audiometry confirmed a further slight deterioration of hearing and repeated MRI demonstrated tumor growth (T2 according to Hannover classification). Since the patient opted against radiosurgery, a retrosigmoid transmeatal approach under continuous intraoperative monitoring was performed in supine position. Following drainage of cerebrospinal fluid and exposure of the cerebellopontine cistern, the AICA was found to be firmly adherent to the petrous dura mater. Both structures were elevated conjointly and displaced medially for safe drilling of the inner auditory canal, sufficient exposure, and complete excision of the vestibular schwannoma. The patient had an excellent recovery, hearing and facial function were preserved, and no secondary neurological deficits noted.The patient consented to publication of this anonymized video.
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BACKGROUND: Complete surgical removal of intracanalicular vestibular schwannomas with nerve VII and VIII sparing and without worsening patient's status is challenging. Also the choice of an optimal surgical technique, which is usually limited to selection between retrosigmoid transmeatal (RT) and middle fossa (MF) approach, can be a challenge. Although many previous studies documented superiority of RT to MF approach and vice versa, still no consensus has been reached regarding an optimal approach to intracanalicular vestibular schwannomas. In this technical note, we present RT approach with an endoscopic assistance and highlight its advantages over MF approach in surgical management of pure intracanalicular vestibular schwannomas. METHOD: RT approach with an endoscopic assistance is presented as an optimal surgical treatment for intracanalicular vestibular schwannomas, and its advantages are compared to those offered by MF approach. RESULTS: Under an endoscopic guidance, we found a residual tumor in the fundus of the inner acoustic canal and performed its gross total resection. CONCLUSIONS: RT approach is an excellent technique suitable for safe radical surgical treatment of T1 vestibular schwannomas; this technique is associated with lower morbidity risk than MF approach.