Extratemporal facial nerve branching patterns: systematic review of 1497 cases.

OBJECTIVE: The aim of this systematic review was to analyse the complex anatomy of the extratemporal portion of the facial nerve with an accurate description of the branching patterns based on the Davis classification. METHOD: Medline, ScienceDirect and the Cochrane Library databases as well as other sources were searched by two independent reviewers. RESULTS: Analysis of 21 studies with a total of 1497 cases showed that type III is the most common branching pattern accounting for 26.8 per cent of cases. The type I pattern, previously considered as the normal anatomy in most textbooks, was the fourth most common branching pattern at 16.3 per cent. The majority of specimens (96.4 per cent) were found to have a bifurcated main trunk, and only 3.2 per cent were found with a trifurcated main trunk. CONCLUSION: Surgeons should be aware of anatomical variations in the course of the facial nerve. An early identification of the branching pattern during surgery reduces the risk for iatrogenic facial nerve injury.

Twenty-year experience with salvage total laryngectomy: lessons learned.

OBJECTIVE: The purpose of this study was to evaluate the outcome of salvage total laryngectomy and identify areas for further improvement. METHOD: A retrospective analysis of all patients who underwent salvage total laryngectomy between January 1999 and December 2018 was performed. RESULTS: Thirty-one patients were identified. The most common primary tumour site was the glottis (83.8 per cent). Early stage (T1-T2) disease was identified in 83.9 per cent of cases. Overall survival at 2 and 5 years post-salvage total laryngectomy was 71 per cent and 45 per cent, respectively. Disease-free survival at 2 and 5 years post-salvage total laryngectomy was 65 per cent and 42 per cent, respectively. The rate of post-salvage total laryngectomy pharyngocutaneous fistula was 29 per cent. CONCLUSION: More than half of patients will not survive beyond five years after salvage total laryngectomy. Regional recurrence was the most common form of failure and death. From this study, elective lateral and central neck dissection is advocated in patients with early laryngeal cancer who present with an advanced recurrence.

A rare case of jugular bulb diverticulum presenting as Meniere's disease, treated with embolization.

BACKGROUND:   Jugular bulb diverticulum constitutes a rare clinical entity that may present with variable symptoms. When obstructing the endolymphatic duct, it may cause sensorineural hearing loss and vertigo, mimicking Meniere's disease. Diagnosis is based on computed tomography and magnetic resonance imaging scans, with magnetic resonance and digital subtraction angiography being more specific and detailed diagnostic methods. Treatment options include surgical decompression, endovascular embolization, or stenting. Description of the case: We report a jugular bulb diverticulum case manifesting as Meniere's disease and treated with endovascular embolization. The procedure was uneventful, and the patient's vertigo gradually disappeared over two months. No medical treatment was required for the next 2.5 years as the patient remained asymptomatic with no more vertigo attacks. CONCLUSION: Endovascular embolization can be the method of choice in selected cases of jugular bulb diverticulum. HIPPOKRATIA 2019, 23(4): 172-174.

Prognostic utility of angiogenesis and hypoxia effectors in patients with operable squamous cell cancer of the larynx.

Angiogenesis is active in localised laryngeal squamous cell carcinoma. We assessed relative messenger RNA (mRNA) and immunohistochemical (IHC) expression of Vascular Endothelial Growth Factors (VEGF) A, B, C, their receptors VEGFR1, 2, 3, Neuropilins 1, 2 (NRP1, 2) and Hypoxia-Inducible Factor 1A (HIF1A) in paraffin-embedded localised laryngeal carcinomas. In 289 patients with T3-4 (77.8%), node-negative (84.1%) tumours of the larynx, high VEGFA and VEGFR1 mRNA correlated with advanced T stage, while low VEGFB and VEGFC mRNA with alcohol abuse and supraglottic primary, respectively (p<0.05). Age <55 was associated with high IHC expression of VEGFA, C and poor tumour differentiation with high IHC VEGFA. At a median follow-up of 74.5months, patients with VEGFR1-high tumours had significantly poorer disease-free survival (Hazard Ratio [HR] 1.93, p=0.008) and shorter overall survival (OS, HR 1.71, p=0.041). An association with dismal OS was seen for high VEGFR3 tumoural mRNA expression (HR 1.76, p=0.02). IHC expression of VEGF family proteins in the tumour was not prognostic and had poor concordance with mRNA expression (kappa<0.1, p=NS). In multivariate analysis, node-positive status, non-supraglottic localization, high VEGFR1 mRNA and high IHC VEGFA expression were significantly associated with relapse, while node-positive status, high VEGFR1 and VEGFC mRNA expression in the tumour with risk of death. In laryngeal cancer, upregulated mRNA expression of VEGFR1 and VEGFC is associated with poor patient outcome.

Identification and validation of gene expression models that predict clinical outcome in patients with early-stage laryngeal cancer.

BACKGROUND: Despite improvement in therapeutic techniques, patients with early-stage laryngeal cancer still recur after treatment. Gene expression prognostic models could suggest which of these patients would be more appropriate for testing adjuvant strategies. MATERIALS AND METHODS: Expression profiling using whole-genome DASL arrays was carried out on 56 formalin-fixed paraffin-embedded tumor samples of patients with early-stage laryngeal cancer. We split the samples into a training and a validation set. Using the supervised principal components survival analysis in the first cohort, we identified gene expression profiles that predict the risk of recurrence. These profiles were then validated in an independent cohort. RESULTS: Gene models comprising different number of genes identified a subgroup of patients who were at high risk of recurrence. Of these, the best prognostic model distinguished between a high- and a low-risk group (log-rank P<0.005). The prognostic value of this model was reproduced in the validation cohort (median disease-free survival: 38 versus 161 months, log-rank P=0.018), hazard ratio=5.19 (95% confidence interval 1.14-23.57, P<0.05). CONCLUSIONS: We have identified gene expression prognostic models that can refine the estimation of a patient's risk of recurrence. These findings, if further validated, should aid in patient stratification for testing adjuvant treatment strategies.

Laryngeal tuberculosis presenting as supraglottic carcinoma: case report and literature review.

Laryngeal tuberculosis is a rare extra-pulmonary manifestation of tuberculosis, and frequently presents with tumour-like symptoms and clinical findings. Uncommon clinical features are to be expected by the ENT specialist, who should be aware of these recently changing presentations. Despite the dramatic reduction of the incidence of the disease during the last few decades, a perceptible increase in case reports has lately been noted and may prove significant. Treatment remains conservative and long-term follow-up is suggested. A relevant case of laryngeal tuberculosis presenting as a supraglottic carcinoma is presented, its diagnosis and management are detailed, and suggestions are offered based on a literature review.

Classic (Mediterranean) Kaposi's sarcoma of the true vocal cord: a case report and review of the literature.

Kaposi's sarcoma (KS) is a rare subcutaneous lesion linked mainly with patients suffering from acquired immunodeficiency syndrome. The aim of the present study is to present the first documented case of classic Kaposi's sarcoma (CKS) located in the right true vocal cord. A 62 year old male presented with cough and hoarseness for 2 months. Clinical examination revealed a nodule on the right vocal cord. The patient underwent surgery and the lesion was removed and biopsied. The histopathology report showed that the lesion was KS but with no complete removal of the lesion, since the surgical margins of the nodule were not healthy. The patient, although fully informed, refused any further treatment. Further laboratory tests were performed, revealing an HIV-negative immunodeficiency profile. Although (Mediterranean) CKS is not an aggressive malignancy, surgery with complete removal of the affected area is indicated when it is applicable. Moreover, conservative treatment and follow up of the patient is essential in order to prevent relapse or other primary lesions.

Factors influencing tumour relapse after total laryngectomy.

OBJECTIVES: To determine the prognostic factors predictive of tumour recurrence after surgical treatment for laryngeal carcinoma with total laryngectomy. STUDY DESIGN/METHODS: Retrospective review of 308 patients with laryngeal carcinoma who underwent total laryngectomy in the ENT Department of AHEPA University Hospital between 01/01/1992 and 31/12/1999. In 238 patients, total laryngectomy was performed as primary treatment of laryngeal carcinoma, and in 70 others as treatment of tumour recurrence following radiotherapy or partial surgery. Follow-up was standardized, following a strict protocol, the mean follow-up time was 68 months. RESULTS: During post-operative follow-up, recurrences were observed in 96 of 308 patients (31%). The relapse rates were 27% (65 of 238) for patients treated with primary total laryngectomy, and 44% (31 of 70) for those treated for recurrence following previous treatment. The difference in relapse rates was statistically significant. In 39 of 238 (16%) cases treated with primary total laryngectomy cervical lymph node infiltration was present at diagnosis and radical or modified neck dissection was performed. The tumour recurrence rate in this group was 46% (18 of 39), while in metastatic node-free patients the relapse rate was 24% (47 of 199) [p < 0.05]. Primary laryngectomy was effective in 82% of glottic, 70% of supraglottic, and only 59% of transglottic carcinoma. Concerning primary tumour extension at the time of surgery, total laryngectomy proved effective in 85% of T2 tumours, 81% of T3, and only 55% for T4. The higher recurrence rates for supraglottic and transglottic tumours seem related mainly to the higher rates of cervical lymph node metastasis at diagnosis. The majority of tumour recurrences were observed during the first two years of post-operative follow-up. Thus, 76% of the 308 patients remained disease-free after the first year of post-operative follow-up, 68% after the second year, and 67% after the fifth follow-up year. Of the 96 recurrences documented until now, 91 were loco-regional (19 at the tracheostomy), and only 5 involved distant metastases. Sixteen of the 308 patients (5%) subjected to total laryngectomy have since developed second primary neoplasms, most often involving the lungs (10 patients). CONCLUSIONS: Prognostic factors for recurrence following total laryngectomy include: performance of total laryngectomy as salvage surgery, degree of tumour extension, infiltration of cervical lymph nodes at the time of initial diagnosis, poor initial tumour differentiation and trans-glottic/sub-glottic tumour localization.

Wegener's granulomatosis presenting with bilateral facial nerve palsy.

Primary manifestation of Wegener's granulomatosis in the mucosa of the middle ear is rather rare, and has been reported as presenting with serous otitis media, chronic otitis media, sensorineural hearing loss, and, in rare instances, unilateral facial palsy. Bilateral facial palsy has never been reported. This last fact constitutes the interest in our report of a 23-year-old female patient who presented with symptoms of recurrent bilateral otitis media, eventually developing sensorineural hearing loss and bilateral facial palsy. Soon thereafter neurological symptoms appeared and lung extension was noted. Histological examination of repeated biopsies taken from the nasal and middle ear mucosa was not conclusive for the suspected disease, and c-ANCA titers were also initially repeatedly negative. Eventually, positive lung biopsy and elevated c-ANCA titers when the patient had developed pulmonary granulomas confirmed the diagnosis of Wegener's granulomatosis. Mastoid surgery with facial nerve decompression of the most severely afflicted side did not result in the recovery of facial nerve function. Medical therapy with corticosteroids and cyclophosphamide improved the clinical picture but were ineffective in improving the bilateral sensorineural hearing loss and the facial paralysis on the operated side. We would contribute to the literature a unique case of bilateral facial nerve palsy due to Wegener's granulomatosis.

Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea.

Intrasphenoidal encephalocele is a rare clinical entity. In the international literature only 16 cases have been reported up today, with female predominance. Clinically they manifest at middle and advanced ages (40-67 years), when spontaneous CSF rhinorrhoea or recurrent meningitis occurs. We present our case, a 46 years old female, who had CSF rhinorrhoea from the right vestibule for 10 months. The diagnosis was based on the history and the high-resolution brain and skull base CT-scanning in conjunction with opaque fluid injection in the subarachnoidal space through a lumbar puncture. She was successfully treated with an operation, through an endonasal trans-ethmoid microendoscopic approach, using the Draf and Stammberger technique. We discuss the pathogenesis of the intrasphenoidal encephalocele, the existence of small occult defects in the skull base, which cause, at the middle and advanced ages, CSF fistula with spontaneous CSF rhinorrhoea and/or recurrent meningitis. Finally we emphasize the advantages of the endonasal surgical approach for the treatment of this condition.