Laryngeal granular cell tumour: a very rare diagnosis for a child presenting with hoarse voice in the UK.

We present a rare case of a paediatric laryngeal granular cell tumour (GCT) of the vocal cord. GCTs are rare clinical entities, thought to arise from Schwann cells. There are only a handful of paediatric laryngeal GCTs in the literature, and therefore, little is known regarding their natural history or preferred management strategies. In this case report, we discuss the incidence, aetiopathogenesis, presentation and management of a laryngeal GCT in an 11-year-old girl, who presented with dysphonia. We believe that this case will aid otolaryngologists in the management of paediatric patients with the common dysphonia.

Rare and unusual case of familial focal dermal hypoplasia (Goltz syndrome) presenting to otolaryngology in the UK.

Goltz syndrome or focal dermal hypoplasia (FDH), is an X-linked dominant condition which predominantly involves the skin, limbs and eyes. In otolaryngology, FDH has been poorly described, but can result in increased symptoms of obstructive sleep apnoea requiring surgery. There have also been documented cases of mixed severe hearing loss secondary to congenital ossicular anomalies. More frequently, patients present to the ear-nose-throat clinic with symptoms of dysphagia, secondary to papillomatosis. A 36-year-old woman presented with pain, irritation and dysphagia with a known diagnosis of FDH. She was subsequently investigated with an oesophago-gastro-duodenoscopy, Barium Swallow and an MRI neck scan with contrast. Lymphoid hyperplasia was found on investigation and the patient underwent a panendoscopy with CO2 laser to the lesion with good clinical outcome. This case report highlights the need for multidisciplinary team involvement to ensure full consideration of management options.

An extremely rare and atypical paediatric presentation of a maxillary sinus haemangioma in the UK.

We present a rare and unusual case of a 16-year-old girl, with no significant medical history, presenting with right nasal obstruction and suspected sinusitis with occasional epistaxis and haemoptysis. On examination, she had a mass lesion in the right nasal cavity, with no evidence of other pathology on assessment of the ears, nose, throat or head and neck. A CT scan revealed an opacified right maxillary sinus with polypoidal mucosa, extending and passing through the accessory ostium into the right nasal cavity. Examination under anaesthesia with functional endoscopic sinus surgery and excision of the lesion was subsequently undertaken. Histological analysis confirmed the mass lesion as a haemangioma. This case report is the first to present a maxillary haemangioma presenting as nasal obstruction with intermittent sinusitis symptoms in a child. The authors discuss the incidence, presentation and management of maxillary haemangiomas in the paediatric population.

Acute presentation of an intraoral dermoid cyst causing airway compromise in a young child.

A 4-month-old boy presented with a cystic swelling at the floor of the mouth causing acute airway compromise. The only previous history of note, was a tongue tie release at 3 days old. CT scan suggested a dermoid cyst with extensive floor of mouth abscess. He had an excision of the cyst and drainage of the superimposed abscess and made a good recovery. The histology report revealed a dermoid cyst which is a rare diagnosis in a child, particularly within the oral cavity. Early treatment is required to remove these lesions especially when they cause airway compromise or swallowing difficulties. This is the first case to our knowledge which suggests tongue tie release procedures causes a predisposition to the development of dermoid cysts in the oral cavity.

Hoarse voice: a rare presentation of tuberculosis lymphadenitis.

A man aged 37 years from Indian descent presented to an inner city London hospital with hoarseness of voice, fatigue, night sweats and a 2 kg weight loss. Laryngoscopy demonstrated a left vocal fold palsy. Extensive investigations were undertaken with disseminated malignancy being primarily investigated. CT/MRI scanning showed non-specific necrotic mediastinal lymph nodes. Tuberculin skin testing, cytology and PCR were eventually undergone. Tests yielded results consistent with tuberculosis (TB) infection and the patient was started on standard TB treatment protocol, with excellent results. TB infection can result in multiple atypical presentations of disease, including hoarseness of voice secondary to vocal cord paralysis. This is a rare but important presentation for community clinician's to consider, especially in populations where TB is epidemiologically thought to be prevalent.

Silastic injection for vocal fold medialization resulting in a false-positive finding on F18 FDG-PET/CT.

A false-positive uptake of F18-fluorodeoxyglucose (FDG) on positron-emission tomography/computed tomography (PET/CT) can result in confusion and misinterpretation of scans. Such uptakes have been previously described after injection of polytetrafluoroethylene (Teflon) into the vocal folds. Similarly, vocal fold injection of silicone elastomer (Silastic) can result not only in a false-positive FDG uptake on PET/CT, but also in chronic inflammation. We report a case of increased FDG uptake in a vocal fold after Silastic injection that was misinterpreted as a malignancy in a 70-year-old woman who had metastatic carcinoma of the stomach.

Endoscopic resection of a mucocele of the crista galli.

Mucoceles expand by the process of bone resorption and new bone formation, which leads to local destruction and displacement of adjacent structures. We present the case of a 47-year-old woman who presented with recurrent headaches, nausea, and vomiting, all of which were believed to have occurred secondary to a mucocele of the crista galli. To the best of our knowledge, only 1 similar case has been previously reported in the world literature; in that case, the crista galli was drained externally. In our patient, resolution of symptoms was achieved by endoscopic dacryocystorhinostomy, endoscopic frontal sinusotomy, and drainage of the mucocele under image guidance. We recommend that an endoscopic surgical approach be considered for the management of mucoceles of the crista galli.

A rare case of bilateral nasolabial cysts.

Nasolabial cysts are rare non-odontogenic cystic lesions representing around 0.7% of all maxillofacial cysts. They usually present as unilateral painless swellings, sometimes with epiphora and dacryocystitis as well as pain in cases of rapid growth or infection. We have reviewed the literature and present an extremely rare case of bilateral nasolabial cysts in a young Afro-Caribbean man presenting with chronic nasal blockage, epiphora and rhinorrhoea. We describe our successful surgical management using a sublabial approach for complete excision, leading to a disease-free outcome at 6 months follow-up. Other modalities have been described, from endoscopic marsupialisation to simple aspiration. However, with the exception of complete surgical excision, all other surgical techniques are associated with a high recurrence rate. We therefore advocate complete surgical excision as described below for optimal results.

Construct validity of the ovine model in endoscopic sinus surgery training.

OBJECTIVES/HYPOTHESIS: To demonstrate construct validity of the ovine model as a tool for training in endoscopic sinus surgery (ESS). STUDY DESIGN: Prospective, cross-sectional evaluation study. METHODS: Over 18 consecutive months, trainees and experts were evaluated in their ability to perform a range of tasks (based on previous face validation and descriptive studies conducted by the same group) relating to ESS on the sheep-head model. Anonymized randomized video recordings of the above were assessed by two independent and blinded assessors. A validated assessment tool utilizing a five-point Likert scale was employed. Construct validity was calculated by comparing scores across training levels and experts using mean and interquartile range of global and task-specific scores. Subgroup analysis of the intermediate group ascertained previous experience. Nonparametric descriptive statistics were used, and analysis was carried out using SPSS version 21 (IBM, Armonk, NY). RESULTS: Reliability of the assessment tool was confirmed. The model discriminated well between different levels of expertise in global and task-specific scores. A positive correlation was noted between year in training and both global and task-specific scores (P < .001). Experience of the intermediate group was variable, and the number of ESS procedures performed under supervision had the highest impact on performance. CONCLUSIONS: This study describes an alternative model for ESS training and assessment. It is also the first to demonstrate construct validity of the sheep-head model for ESS training.

Feasibility of a synthetic temporal bone for training in mastoidectomy: face, content, and concurrent validity.

OBJECTIVE: To investigate the face, content, and concurrent validity of the synthetic Pettigrew temporal bone (PTB) for mastoidectomy training as compared with cadaveric temporal bone (CTB). STUDY DESIGN: A prospective evaluation study. METHODS: Participants were invited to perform a step-by-step modified radical mastoidectomy using both bones and complete a 22-item, 5-point Likert scale questionnaire. The questionnaire is divided into 4 domains: face validity (FV), global content (GC), task-specific content (TSC), and curriculum recommendation (CR). RESULTS: Thirty-six experts and 89 trainees completed all tasks, 63 using CTB and 62 using PTB. The PTB median FV was 4 (IQR: 4-5), GC of 4 (IQR: 4-5), TSC of 4 (IQR: 3-4), and CR of 4 (IQR: 4-5). The CTB was rated significantly higher than PTB by both groups in all domains; CTB FV: 5 (IQR: 4-5), GC: 5 (IQR: 4-5), TSC: 5 (IQR: 4-5), and CR: 5 (IQR: 5-5), p < 0.001 for each. Trainees rated PTB significantly higher than experts in all domains. There was no statistically significant difference between experts and trainees in rating the CTB in all domains. PTB gives similar haptic feedback to CTB, allows the use of suction and irrigation, has the important landmarks painted for identification, and contains articulating ossicles. The facial nerve anatomy was found to be inaccurate around the region of the second genu. CONCLUSION: Participants found PTB to be valid for teaching some, yet not all, aspects of mastoid surgery, and experts agreed that it could improve global transferrable otologic skills. It is essential that the facial nerve anatomy is addressed before recommending this model.

Surgical management of bilateral parotid lipomatosis in a patient with HIV.

BACKGROUND: The long-term use of highly active antiretroviral therapy (HAART) in patients with human immunodeficiency virus (HIV) has led to sequelae including lipodystrophy syndrome (LDS). We present the first published case of surgical management of bilateral parotid lipomatosis in a patient with HIV on long-term HAART. METHODS: We undertook review of the case notes from the time of diagnosis with HIV and literature review of this topic. RESULTS: A 45-year-old man with HIV on HAART presented with a 4-year history of increasing bilateral facial swelling. He was asymptomatic apart from the stigmatizing cosmetic deformity. MRI revealed the parotid glands had been replaced by fat. He elected for surgery and parotid lipomatosis was diagnosed on histopathological examination. CONCLUSION: Lipohypertrophy in LDS is rare in the literature and this presentation of bilateral parotid lipomatosis secondary to HAART is only the third reported case, and the first to undergo surgical resection.

Craniofacial resection and its role in the management of sinonasal malignancies.

Sinonasal malignancy is rare, and its presentation is commonly late. There is a wide variety of pathologies with varying natural histories and survival rates. Anatomy of the skull base is extremely complex and tumors are closely related to orbits, frontal lobes and cavernous sinus. Anatomical detail and the late presentation render surgical management a challenging task. A thorough understanding of anatomy and pathology combined with modern neuroimaging and reliable reconstruction within a multidisciplinary team is imperative to carry out skull base surgery effectively. While endoscopic approaches are gaining credibility, clearly, it will be some time before meaningful comparisons with craniofacial resection can be made. Until then, craniofacial resection will remain the gold standard for managing the sinonasal malignancies of the anterior skull base, as it has proved to be safe and effective.

Rare sarcoma presented as sinusitis.

Myxofibrosarcoma (MFS) also known as myxoid variant of malignant fibrous histocytoma is one of the most common soft tissue sarcomas of the extremities in adult and elderly patients with rare occurrences in head and neck region. Low-grade MFS is unusual among low-grade sarcomas because it often recurs relentlessly and multiplies despite wide local resection with gross negative margins. We report a case of extreme rarity and a tumour of aggressive nature in the maxillary sinus, which presented with non-specific sinonasal symptoms and we present a review of the radiological and histopathological characteristics of this rare tumour and recent evidence of management.

An interesting collection of paraneoplastic syndromes in a patient with a malignant thymoma.

Paraneoplastic neurological syndromes are conditions that manifest as the remote effects of cancer. These are very rare, occurring in 1/10000 patients with a malignancy, and include Lambert-Eaton myasthenic syndrome, limbic encephalitis, subacute cerebellar ataxia, opsoclonus-myoclonus, Stiff-Person Syndrome, retinopathies, chronic gastrointestinal pseudo-obstruction and sensory neuropathy. This report describes a case of 41-year-old man who presented with elements of multiple paraneoplastic syndromes, including chronic gastrointestinal pseudo-obstruction, myasthenia gravis-Lambert-Eaton overlap syndrome and polymyositis, and who was subsequently found to have a malignant thymoma. There are only three reported cases in the literature describing cases of Lambert-Eaton myasthenic syndrome in association with a thymoma, and only one case of a myasthenia gravis-Lambert-Eaton overlap syndrome in a patient with thymoma. However, there are no documented cases in the literature of this constellation of syndromes in a patient with a malignant thymoma.

Balloon Sinuplasty: balloon-catheter dilation of paranasal sinus ostia for chronic rhinosinusitis.

More than 100 million patients worldwide suffer from chronic rhinosinusitis, and a considerable amount of money has been spent on research and treatments by healthcare providers. In the northern hemisphere, damp, temperate climates, along with higher concentrations of pollen, are associated with a higher prevalence of chronic rhinosinusitis. Owing to its persistent nature, the disease can become a significant cause of morbidity. If untreated, it can reduce quality of life and productivity. When medical treatment is not effective, surgery may offer an excellent outcome. Although functional endoscopic surgery has proven to be effective, many cases could be managed medically. Recent research has suggested that optimal medical treatment is as effective as surgery in patients with chronic rhinosinusitis at the end of 1 year. Balloon Sinuplasty (Acclarent, Inc., CA, USA) is a new technique in the management of sinusitis and is a hotly debated topic. It is a delicate, minimally invasive tool, and early research demonstrates promising outcomes in terms of safety and effectiveness. This novel technique has been approved by the US FDA. Recently, NICE raised no concerns regarding its safety and efficacy but will continue to review this procedure.