Andrea Gallo

CO2 laser cordectomy for early-stage Glottic carcinoma: A long-term follow-up of 156 cases

Objectives To define when laser resection of early‐stage glottic carcinoma is indicated and to compare the results obtained by laser surgery with other therapeutic options.

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.

Cogan's syndrome: An autoimmune inner ear disease

OBJECTIVES The objective of our study was to review our current knowledge of the aetiopathogenesis of Cogans syndrome, including viral infection and autoimmunity, and to discuss disease pathogenesis with relevance to pharmacotherapy. SYSTEMATIC REVIEW METHODOLOGY Relevant publications on the aetiopathogenesis and pharmacotherapy of Cogans syndrome from 1945 to 2012 were analysed. RESULTS AND CONCLUSIONS Cogans syndrome is a rare autoimmune vasculitis, and its pathogenesis is unknown. Infection, but primarily autoimmunity, may play contributing roles in the pathogenesis of this disease. It is characterised by ocular and audiovestibular symptoms similar to those of Menieres syndrome. Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism. The immunologic theory is based on the release of auto-antibodies against corneal, inner ear and endothelial antigens, and of anti-nuclear cytoplasmic auto-antibodies (ANCA). Corticosteroids are the first line of treatment, and multiple immunosuppressive drugs have been tried with varying degrees of success. Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogans syndrome.

Vogt–Koyanagi–Harada syndrome

OBJECTIVES The objectives of this study are to review our current knowledge of the aetiopathogenesis of Vogt-Koyanagi-Harada syndrome, including viral infection, genetic factors and immunomediated mechanisms, and to discuss pathogenesis and its relevance to pharmacotherapy. SYSTEMATIC REVIEW METHODOLOGY Relevant publications from 1965 to 2012 on the aetiopathogenesis and pharmacotherapy of VKHS were analysed. RESULTS AND CONCLUSION Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The disease occurs more frequently among people with darker skin pigmentation. Asians, Native Americans, and Hispanics are most frequently affected. It predominates in patients aged between 20 and 50years, and females are affected more frequently, with a female:male ratio of 2:1. The classic clinical course is characterised by bilateral panuveitis, hypoacusis, and meningitis, in addition to cutaneous involvement with poliosis, vitiligo, and alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes. VKHS classically begins with vague systemic symptoms suggestive of a viral infection, although a clear association between a specific viral agent and the disease has not been established. Genetic factors may play an important role in the loss of self-tolerance in VKHS. The HLA-DRB1*0405 allele is the main susceptibility allele for VKHS. Early and aggressive systemic corticosteroids are still the primary initial therapy for VKHS. Ocular complications may require an intravitreous injection of corticosteroids. Despite proper treatment with steroids, a number of patients experience recurrent attacks or steroid-associated complications. Thus, non steroid immunomodulatory therapy (IMT) has become necessary for the treatment of VKHS.

Endoscopic treatment of esthesioneuroblastoma: A minimally invasive approach combined with radiation therapy

OBJECTIVE: In a retrospective analysis we discuss our experience in the treatment of olfactory neuroblastoma (ON), a rare tumor of the sinonasal tract. STUDY DESIGN AND SETTING: Nine patients with olfactory neuroblastoma were treated at the Bolognini Hospital of Seriate and at the Department of Otolaryngology of “La Sapienza” University of Rome combining endoscopic surgery and postoperative radiation therapy. All patients were staged according to Kadish criteria. No chemotherapy was administered in any cases. Local radiation therapy was started postoperatively in all the cases. RESULTS: All patients, over an average follow-up which ranged from 26 through 60 months, are actually alive with non evidence of disease (NED). CONCLUSIONS: Despite the short follow-up in this small series of patients, we suggest endoscopic surgery combined with postoperative radiation therapy as an alternative to the conventional modalities of treatment for esthesioneuroblastoma in selected cases.

Supracricoid Laryngectomy With Cricohyoidopexy (CHP) in the Treatment of Laryngeal Cancer: A Functional and Oncologic Experience

Subtotal laryngectomy with cricohyoidopexy (CHP) results in a satisfactory cure rate in selected T1b, T2, and T3 supraglottic and glottic cancers. The clinical, oncologic, and functional results of this type of surgery have led to an ever‐increasing number of patients undergoing this procedure. This study involved 70 patients operated on from 1984 to 1993 using this surgical procedure. This report also discusses the clinical (cTNM) and pathologic (pTNM) classifications of CHP cases. Fifty‐two of these patients were evaluated after a follow‐up of more than 3 years. Lymph node treatment protocol comprised 51 functional ipsilateral neck dissections (FNDs), 9 bilateral FNDs, 1 radical ipsilateral dissection, and 1 radical ipsilateral and contralateral FND. There were 6 recurrences (5 local, 1 neck); after CHP physiologic deglutition was recovered in most of the patients (95.5%), and all but 6 (92%) were decannulated. Results were excellent after phoniatric rehabilitation. This surgical procedure may be indicated for selected laryngeal cancers and allows for the preservation of laryngeal functions even when the neoplastic lesions are advanced. However, care must be taken when evaluating the infiltration of the paraglottic space, as that infiltration represents the main cause of failure in this type of surgery.

Evolution of precancerous laryngeal lesions: A clinicopathologic study with long-term follow-up on 259 patients

A wide spectrum of lesions ranging from dysplasia to in situ carcinoma have to be considered when dealing with laryngeal precancerous conditions. Recently the concept of laryngeal intraepithelial neoplasia (LIN) was introduced.

Sudden sensorineural hearing loss: An autoimmune disease?

OBJECTIVES To review our current knowledge of the pathogenesis of sudden sensorineural hearing loss, including viral infection, vascular occlusion and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy. SYSTEMATIC REVIEW METHODOLOGY Relevant publications on the pathogenesis of sudden sensorineural hearing loss from 1944 to 2010 were analysed. RESULTS AND CONCLUSIONS Sudden sensorineural hearing loss is defined as hearing loss of 30 dB in three sequential frequencies over 3 days or less. It can be an isolated symptom or the presenting symptom of a systemic disease. The aetiology and pathogenesis remain unknown. Detailed investigation typically reveals a specific cause in about 10% of patients. Proposed theories of causation include viral infections, vascular occlusion and immune system-mediated mechanisms. A variety of therapies have been proposed based on the various proposed aetiologies.

Meniere's disease might be an autoimmune condition?

OBJECTIVES To review our current knowledge of the pathogenesis of Menieres disease, including viral infection and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy. SYSTEMATIC REVIEW METHODOLOGY Relevant publications on the aetiopathogenesis, molecular biology, genetics and histopathology of Menieres disease from 1861 to 2011 were analysed. RESULTS AND CONCLUSIONS Menieres disease is characterised by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure. The aetiology and pathogenesis remain unknown. Proposed theories of causation include viral infections and immune system-mediated mechanisms. The immune response in Menieres disease is focused on inner ear antigens. Approximately one-third of Menieres disease cases seem to be of an autoimmune origin although the immunological mechanisms involved are not clear. The diagnosis of autoimmune inner ear disease is based either on clinical criteria or on a positive response to steroids. The antiviral approach has virtually eliminated the use of various surgical methods used in the past. Steroid responsiveness is high, and with prompt treatment, inner ear damage may be reversible. The administration of etanercept improves or stabilises symptoms in treated patients. Treatment of antiphospholipid syndrome can be directed toward preventing thromboembolic events by using antithrombotic medications. Only warfarin has been shown to be effective. Gene therapy can be used to transfer genetic material into inner ear cells using viral vectors and to protect, rescue, and even regenerate hair cells of the inner ear.

Ectopic Lingual Thyroid as Unusual Cause of Severe Dysphagia

Ectopic lingual thyroid is an uncommon embryological aberration characterized by the presence of thyroid tissue located in a site different from the pretracheal region as in the normal. Lingual thyroid is the most frequent ectopic location of the thyroid gland, although its clinical incidence varies between 1:3000 and 1:10,000. We present the case of a 26-year-old woman who presented severe dysphagia caused by a mass located on the base of the tongue in the midline. An endoscopic partial removal of the ectopic tissue allowed her to rapidly regain her swallowing capacity. The literature regarding the incidence and diagnosis of lingual thyroid is reviewed and the possibilities of treatment discussed.