The Journal of laryngology and otology
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Review Case Reports
Laryngeal manifestations of pachyonychia congenita: a clinical case and discussion on management for the otolaryngologist.
Pachyonychia congenita is a rare keratinising disorder, which typically presents during the first three years of life and usually affects the nails and palmoplantar surfaces. It can involve the larynx and potentially result in life-threatening airway obstruction. ⋯ Patients with pachyonychia congenita and laryngeal involvement can have a varied presentation, ranging from hoarseness to acute airway obstruction. Management can be a challenge, requiring early evaluation, regular surveillance and aggressive treatment. This paper reports our experience in managing and treating the laryngeal complications of a child with pachyonychia congenita.
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Practice Guideline
Investigation and management of the unknown primary with metastatic neck disease: United Kingdom National Multidisciplinary Guidelines.
This is the official guideline endorsed by the specialty associations involved in the care of head and neck cancer patients in the UK. It discusses the evidence base pertaining to the management of metastatic neck disease in the setting of an unknown primary and provides recommendations on the work up and management for this group of patients receiving cancer care. Recommendations • All patients presenting with confirmed cervical lymph node metastatic squamous cell carcinoma and no apparent primary site should undergo: ○ Positron emission tomography-computed tomography whole-body scan. (R) ○ Panendoscopy and directed biopsies. (R) ○ Bilateral tonsillectomy. (R) • Tongue base mucosectomy can be offered if facilities and expertise exists. (G) • Concomitant chemotherapy with radiation should be considered in patients with an unknown primary. (R) • Concomitant chemotherapy with radiation should be offered to suitable patients in the post-operative setting, where indicated. (R) • Neo-adjuvant chemotherapy can be used in gross 'unresectable' disease. (R) • Patients should be followed up at least two months in the first two years and three to six months in the subsequent years. (G) • Patients should be followed up to a minimum of five years with a prolonged follow up for selected patients. (G) • Positron emission tomography-computed tomography scan at three to four months after treatment is a useful follow-up strategy for patients treated by chemoradiation therapy. (R).
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Practice Guideline
Aetiology and risk factors for head and neck cancer: United Kingdom National Multidisciplinary Guidelines.
This is the official guideline endorsed by the specialty associations involved in the care of head and neck cancer patients in the UK. It discusses the aetiology and risk factors for head and neck cancer and the recommended interventions appropriate for each risk factor. Recommendations • Recent evidence synthesis from National Institute for Health and Care Excellence suggests that the following brief interventions for smoking cessation work should be used: ○ Ask smokers how interested they are in quitting (R) ○ If they want to stop, refer them to an intensive support service such as National Health Service Stop Smoking Services (R) ○ If they are unwilling or unable to accept a referral, offer a stop smoking aid, e.g. pharmacotherapy. (R) • Brief interventions are effective for hazardous and harmful drinking. (R) • Specialist interventions are effective in people with alcohol dependence. (R) • Most people with alcohol dependence can undergo medically assisted withdrawal safely at home, after risk assessment. (R) • Management of leukoplakia is not informed by high-level evidence but consensus supports targeted use of biopsy and histopathological assessment. (R) • The management of biopsy proven dysplastic lesions favours: ○ advice to reduce known environmental carcinogens such as tobacco and alcohol (R) ○ surgical excision when the size of the lesions and the patient's function allows (R) ○ long-term surveillance. (R) • Fanconi anaemia patients should: ○ be followed up in a multidisciplinary specialist Fanconi anaemia clinic (G) ○ have quarterly screening for head and neck squamous cell carcinoma and an aggressive biopsy policy (G) ○ receive prophylactic vaccination against high risk human papilloma virus (G) ○ receive treatment for head and neck squamous cell carcinoma with surgery alone where possible. (G).