Madelung-Launois-Bensaude lipomatosis is a rare disease characterized by an enormous accumulation of fat, mainly in the region of the neck. A case is presented to illustrate the course of the disease and its clinical features. In particular, the necessity for a bilateral conservative neck dissection is emphasized to avoid early recurrence.
Recurrent respiratory papillomatosis is a human papillomavirus-mediated condition characterised by the development of benign squamous papillomata of the respiratory tract. Malignant transformation of recurrent respiratory papillomatosis, while rare, carries a poor prognosis and there are limited data surrounding treatment options, particularly in inoperable disease. We present the case of a 64-year-old male who developed malignant airway obstruction secondary to primary tracheal squamous cell carcinoma in the setting of a 5-year history of recurrent laryngotracheal papillomatosis, requiring placement of tracheostomy while on veno-venous extracorporeal membranous oxygenation. He was managed with cisplatin-based definitive chemoradiotherapy and had a complete metabolic response on post-treatment positron emission tomography/computed tomography, and remains free of recurrent squamous cell carcinoma at 16 months following treatment. This case supports the use of combined chemoradiotherapy as a potential therapeutic option for patients with primary tracheal squamous cell carcinoma, and emphasises the challenges associated with the long-term management of recurrent respiratory papillomatosis.
Chronic non-granulomatous supraglottitis (CNGS) is a rare disorder of the supraglottic larynx, characterised by chronic supraglottic inflammation in the absence of granulomata, vasculitis, neoplasia, autoimmune disease or infective changes on histology. We present the case of a male adolescentwho attended with progressively worsening exertional dyspnoea, stridor and symptoms of obstructive sleep apnoea. Flexible nasendoscopy revealed marked supraglottic subepithelial thickening sparing the glottis and subglottis, confirmed on microlaryngoscopy. MRI of the head and neck demonstrated diffuse, homogenous supraglottic oedema. At the peak of his symptomology, the patient was admitted for further investigations and intravenous steroid therapy, and switched to prolonged oral steroids on discharge. Tracheostomy was avoided. After 3 months, he was successfully weaned from steroids to azathioprine with gradual symptomatic improvement. This case represents the first successful use of a steroid-sparing agent in the management of CNGS.
Application of load-bearing osteosynthesis plates is the current gold-standard management for complex mandibular fractures. Traditionally, this has required a transcutaneous submandibular approach, carrying with it the risk of damage to the facial nerve and obvious extraoral scarring. The existing literature describes the use of computer-assisted design and manufacturing technology through external vendors to aid transoral mandibular reconstruction. However, the reliance on third-party manufacturers comes with significant drawbacks, notably increased financial costs and manufacturing delays. We describe our experience in using point-of-care three-dimensional-printed surgical models to aid with the application of mandibular reconstruction plates. Utilising a virtual three-dimensional reconstruction of the patient’s preoperative computed tomography facial bones, we fabricate a custom model of the patient’s mandible with the department’s in-house three-dimensional printer. Stock plates are subsequently pre-bent and adapted to the three-dimensional model, with plate and screw position marked and screw lengths measured with callipers. By using a custom three-dimensional-printed surgical model to pre-contour the plates, we are able to position stock reconstruction plates via a transoral approach. Moreover, our unit’s utilisation of in-house computer-assisted design and manufacturing software and hardware allows us deliver a same-day turnaround for both surgical planning and performing the operation. Patient-specific surgical planning guides can facilitate the safe and efficient transoral application of mandibular reconstruction plates. Moreover, the use of point-of-care computer-assisted design and manufacturing technology ensures timely and cost-effective manufacturing of the necessary biomodel.
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