Armando De Virgilio

Feasiblity of transoral robotic hypopharyngectomy for early-stage hypopharyngeal carcinoma.

Conventional surgical approaches for hypopharyngeal carcinomas have a great risk for developing treatment-related morbidity. To minimize this morbidity, hypopharyngectomy by transoral robotic surgery (TORS) was performed, and the efficacy and feasibility of this procedure were evaluated. TORS was performed using da Vinci Surgical Robot (Intuitive Surgical Inc., Sunnyvale, CA) in 10 patients with T1 or T2 pyriform sinus cancer and posterior pharyngeal wall cancer. FK retractor (Gyrus Medical Inc., Maple Grove, MN) was used for transoral exposure of the lesion. A face-up 30-degree endoscope was inserted through the oral cavity and two instrument arms were located in both sides of the endoscope. Pyriform sinus was totally resected as a cone-shape from the vallecular to apex region, and ipsilateral arytenoid cartilage was saved for function preservation. The aryepiglottic fold was resected medially. Laterally, the inner perichondrium of the thyroid cartilage was peeled off after perichondrium was incised horizontally to make sure of the safe margin of antero-lateral portion. The posterior margin is an inferior constrictor muscle of the posterior pharyngeal wall. We evaluated the robotic set up time, robotic operation time, blood loss, surgical margins, swallowing time, decannulation time, and surgery related complications. Transoral robotic hypopharyngectomy was performed successfully in all 10 patients. The mean robotic operation time was 62.4min, and an average of 17.5min was required for the setting of the robotic system. There was no significant perioperative complication in the cases. Swallowing function returned to all patients within 8.3days average. Decannulation was carried out within an average of 6.3days after surgery. Transoral robotic hypopharyngectomy was feasible and ontologically safe technique for the treatment of early hypopharyngeal cancer.

Churg–Strauss syndrome

Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative.

Transoral robotic surgery for hypopharyngeal squamous cell carcinoma: 3-year oncologic and functional analysis

The recent trend in treatment of hypopharyngeal cancer is organ preservation in order to maintain swallowing and speech function as well as improve quality of life. Transoral robotic surgery (TORS) can remove hypopharyngeal lesions successfully without an external incision, preserving physiologic functions of affected organs. However, studies have yet to assess the oncologic and functional results of TORS for the treatment of hypopharyngeal cancer. This prospective study evaluated the oncologic and functional results of TORS for the treatment of hypopharyngeal cancer obtained at our institution over a period of 3 years and confirmed the validity of TORS as a surgical organ-preserving strategy. Between April 2008 and September 2011, 23 patients who were diagnosed with hypopharyngeal cancer underwent TORS for removal of a primary lesion. The da Vinci Robotic system (Intuitive Surgical Inc., Sunnyvale, California) was used to remove the lesion. The Kaplan-Meier method was used to analyze overall survival and disease-free survival. Videopharyngogram study (VEF) was performed and functional outcome swallowing scale (FOSS) was utilized to measure and evaluate swallowing function. Acoustic wave form analysis was conducted to evaluate voice status. Overall survival at 3 years was 89% and disease-free survival was 84%. On the VEF study, serious aspiration or delay of swallowing was not observed during the pharyngeal stage of the swallowing process. Overall, 96% of the patients showed favorable swallowing abilities with an FOSS score ranging from 0 to 2. The fundamental frequency variation (vF0) and jitter were increased upon acoustic waveform analysis (vF0=2.71 ± 0.063, Jitter=2.01 ± 0.034), but the harmonic-to-noise ratio (HNR) and shimmer were maintained close to the normal range (HNR=1.28 ± 0.001, Shim=1.74 ± 0.036). The oncologic and functional results of TORS were quite acceptable for the treatment of hypopharyngeal cancer. TORS is a valid treatment option as a surgical, organ-preserving strategy for select patients with hypopharyngeal cancer.

Parkinson's disease: Autoimmunity and neuroinflammation

Parkinsons disease is a neurodegenerative disease that causes the death of dopaminergic neurons in the substantia nigra. The resulting dopamine deficiency in the basal ganglia leads to a movement disorder that is characterized by classical parkinsonian motor symptoms. Parkinsons disease is recognized as the most common neurodegenerative disorder after Alzheimers disease. PD ethiopathogenesis remains to be elucidated and has been connected to genetic, environmental and immunologic conditions. The past decade has provided evidence for a significant role of the immune system in PD pathogenesis, either through inflammation or an autoimmune response. Several autoantibodies directed at antigens associated with PD pathogenesis have been identified in PD patients. This immune activation may be the cause of, rather than a response to, the observed neuronal loss. Parkinsonian motor symptoms include bradykinesia, muscular rigidity and resting tremor. The non-motor features include olfactory dysfunction, cognitive impairment, psychiatric symptoms and autonomic dysfunction. Microscopically, the specific degeneration of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies, which are brain deposits containing a substantial amount of α-synuclein, have been recognized. The progression of Parkinsons disease is characterized by a worsening of motor features; however, as the disease progresses, there is an emergence of complications related to long-term symptomatic treatment. The available therapies for Parkinsons disease only treat the symptoms of the disease. A major goal of Parkinsons disease research is the development of disease-modifying drugs that slow or stop the neurodegenerative process. Drugs that enhance the intracerebral dopamine concentrations or stimulate dopamine receptors remain the mainstay treatment for motor symptoms. Immunomodulatory therapeutic strategies aiming to attenuate PD neurodegeneration have become an attractive option and warrant further investigation.

Goodpasture's syndrome: A clinical update

Goodpastures syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest confined to specific target organs, such as the lung and kidney. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death.

Kawasaki disease: An evolving paradigm

Kawasaki disease (KD) is a self-limited childhood systemic vasculitis that exhibits a specific predilection for the coronary arteries. KD predominantly affects young children between the ages of 6months and 4years. Incidence rates in Asians are up to 20 times higher than Caucasians. The aetiology of KD is not known. One reasonable open hypothesis is that KD is caused by an infectious agent that produces an autoimmune disease only in genetically predisposed individuals. The typical presentation of KD is a young child who has exhibited a high swinging fever for five or more days that persists despite antibiotic and/or antipyretic treatment. The lips are dry and cracked. There is a characteristic strawberry tongue, and a diffuse erythema of oropharyngeal mucosal surfaces. Lymphadenopathy is usually unilateral and confined to the anterior cervical triangle. Coronary aneurysms generally appear during the convalescence phase (beginning during the second week). The absence of any laboratory tests for KD means that the diagnosis is made by the presence of a constellation of clinical features. The aim of echocardiography is to assess the presence of coronary artery dilatation or aneurysm formation. Effective therapies exist for most patients with acute KD, but the exact mechanisms of action are not clear. Treatment with aspirin and intravenous immunoglobulins (IVIG) are first-line therapies. However, options are plentiful for the children who fail this treatment, but these treatments are not as beneficial. Some centres attempt to salvage resistant patients using intravenous pulsed doses of methylprednisolone. Other centres use infliximab or combinations of these approaches.

Sudden sensorineural hearing loss: A vascular cause? Analysis of prothrombotic risk factors in head and neck

Abstract Objective: This aim of this study was to determine the prevalence of thrombophilic risk factors in sudden sensorineural hearing loss, central retinal vein occlusion, and stroke associated with small vessel disease, with the purpose of investigating and reinforcing the vascular hypothesis in the pathogenesis of sudden sensorineural hearing loss. Design: Case-control study. Genetic and acquired risk factors of these three groups were compared with healthy controls. Study sample: Forty-nine, 60, and 101 patients affected respectively by sudden sensorineural hearing loss, central retinal vein occlusion, or stroke associated with small vessel disease, enrolled during a three-year period were compared with 210 healthy controls. Results: The frequency of hyperhomocysteinemia (homocysteine ≥ 15 μmol/L) was higher in each disease group than in controls. A statically significant, albeit weak, correlation between the MTHFR C677T mutation and hyperhomocysteinemia was found in all three diseases. Conclusions: Hyperhomocysteinemia proved to be a risk factor for sudden sensorineural hearing loss. Based on these results, we propose to analyse homocysteine in sudden sensorineural hearing loss patients and, if its values are high, to evaluate the presence of MTHFR C677T mutation.

Role of Genetic and Acquired Prothrombotic Risk Factors in Genesis of Sudden Sensorineural Hearing Loss

The methylenetetrahydrofolate reductase C677T mutation, factor V G1691A (factor V Leiden) mutation, prothrombin G20210A mutation and 8 other laboratory values associated with increased thrombotic risk were analyzed in 40 patients with sudden sensorineural hearing loss (SSHL). The results were compared with those obtained from 120 controls not affected by SSHL. We found a statistically significant higher frequency of hyperhomocysteinemia in the SSHL group compared with controls, and that this was also associated with the presence of homozygosity for the MTHFR C677T mutation. The study results suggest that SSHL might be caused, among other factors, by a combination of these 2 variables. We suggest that this analysis of the MTHFR C677T mutation should be further investigated to establish the etiology of SSHL, and that the same analysis should be taken into account in those patients with high levels of homocysteine.

Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The disease predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). Microscopic polyangiitis was considered to be a disease entity by Savage et al. in 1985. Microscopic polyangiitis has a reported low incidence and a slight male predominance. The aetiology of MPA remains unknown. There is, however, increased evidence that MPA is an autoimmune disease in which ANCAs, particularly those reacting with MPO, are pathogenic. MPA belongs to the systemic vasculitides, indicating that multiple organs can be affected. The major organs involved in MPA are the kidneys and the lungs. As expected for an illness that affects multiple organ systems, patients with MPA can present with a myriad of different symptoms. Ear, nose and throat (ENT) manifestations are not considered to be clinical symptoms of MPA, but in the majority of populations described, ENT involvement was found in surprisingly high percentages. MPA is part of the ANCA-associated vasculitides, which are characterized by necrotizing vasculitis of small vessels. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies.

How to optimize laryngeal and hypopharyngeal exposure in transoral robotic surgery

OBJECTIVE The aim of this study was to present the various strategies adopted in our center to improve and overcome problems with exposure of the operative field in 48 patients who underwent TORS for the treatment of laryngeal and hypopharyngeal cancer. METHODS We present our operative and preoperative treatment protocols for patients undergoing TORS for laryngeal and hypopharyngeal cancer. In particular, we emphasize the role of preoperative exposure assessment and the usefulness of simple measures to overcome problems with exposure of the operative field. RESULTS In 12 patients (25%), we experienced difficult laryngeal-hypopharyngeal exposure. However the correct positioning of the robotic arms, the proper use of the laryngeal and tongue blade and some simple maneuvers, such as the anterior traction of the tongue and the partial epiglottectomy, ensured the feasibility of TORS with negative margins in all patients. CONCLUSION In TORS, the exposure of larynx and hypopharynx can be difficult, but the adoption of certain methods may make it possible in most patients. An accurate preoperative evaluation under general anesthesia is the primary strategy for reducing the number of cases terminated intraoperatively. Currently, TORS is not feasible in all patients, but in our opinion, reductions in the size of robotic equipment and development of new devices will extend the application of TORS to a larger number of patients.