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Lipoid proteinosis is a rare autosomal recessive disorder caused by mutations in the extracellular matrix protein 1 gene. It is characterised by deposition of hyaline material in the skin and mucous membranes. This paper describes the management of two cases with laryngopharyngeal disease.
Two patients with a biopsy diagnosis of lipoid proteinosis were identified from the surgical pathology archive covering the period 2004–2016. Their notes were reviewed.
An adult male and an adult female were identified. Both had dysphonia and laryngopharyngeal lesions. The patients underwent interval laser microlaryngoscopy to debulk disease but minimise mucosal injury and scarring, using a ‘pepper pot’ technique. Both had adequate symptom control.
Lipoid proteinosis is a rare genetic condition, which typically presents in infancy with dysphonia and subsequent skin involvement. Two cases are presented to demonstrate that laryngotracheal symptoms can be controlled with interval laser debulking and the ‘pepper pot’ technique without causing stenosis.
Delays in head and neck cancer treatment lead to increased mortality, more extensive treatment and patient anxiety. We aim to treat all patients with cancer within 62 days of receipt of a referral. An analysis was conducted of those patients whose treatment had been delayed in order to identify factors associated with treatment delay.
In this retrospective case–control study, 50 patients whose treatment was delayed were identified and compared with 50 patients whose treatment was not delayed. Individual factors assessed included patient age, co-morbidity, tumour location and stage, the treatment agreed, the hospitals to which the patients were referred, and the clinicians they saw.
There was a significant association between referral to a non-head and neck cancer centre, or review by a non-head and neck multidisciplinary team member, and treatment delay.
In the context of centralisation of head and neck cancer services, it is important to consider delays that may be associated with a ‘hub and spoke’ model of service provision.