Cervicofacial necrotising fasciitis (CNF) is a potentially fatal infection which can occasionally present in the head and neck. An early diagnosis and aggressive treatment is imperative for minimising the associated mortality and morbidity. The early clinical features are usually non-specific which makes it difficult to differentiate it from other less serious infections. Necrosis of the skin is a late feature. Although it is more common in the immunocompromised, it can also affect normal individuals. We discuss our experience of five patients with CNF, review of literature and algorithm for early diagnosis of CNF. With experience, we were able to diagnose the subsequent cases early and minimise the mortality and morbidity. In conclusion, the incidence of CNF has increased in the last decade partly due to an increased clinical awareness. Early intervention is essential to minimise the mortality and morbidity. It should be managed by a team of at least otolaryngologists, intensivist, microbiologist and plastic surgeons; cardiothoracic surgeons may be required. Treatment involves early aggressive surgical debridement/fasciotomy, intravenous antibiotics and general metabolic support in the intensive care unit.
The additional measures involved in pharyngo-laryngectomy do not confer any functional disadvantage, compared with total laryngectomy, but only if the procedure is non-circumferential. Functional results of circumferential pharyngo-laryngectomy are worse than those of both non-circumferential pharyngo-laryngectomy and total laryngectomy. If oncologically possible and safe, it is better to keep a pharyngo-laryngectomy non-circumferential.
(1) Background: Mucopolysaccharidoses (MPS) are a heterogeneous group of lysosomal storage disorders caused by the absence of enzymes required for degradation of glycosaminoglycans (GAGs). GAGs deposition in tissues leads to progressive airway narrowing and/or tortuosity. Increased longevity of patients has posed newer problems, especially the airway. This study aims to characterise various airway abnormalities in adult MPS from a regional centre and proposes a method to quantify the severity of the airway disease. (2) Methods: Retrospective analysis by case notes review, clinical examination, endoscopy, cross-sectional imaging, 3-dimensional reconstruction, and physiological investigations were used to assess the airway abnormalities. Quantitative assessment of the airway severity was performed a validated questionnaire of 15 parameters to derive Salford Mucopolysaccharidosis Airway Score (SMAS). (3) Results: Thirty-one adult MPS patients (21M/ 9F; median 26.7 years; range 19–42 years) were reviewed. There were 9 MPS I, 12 MPS II, 2 MPS III, 5 MPS IV, 2 MPS VI, and 1 MPS VII. Airway abnormalities in each MPS type are described. Patients scoring more than 35 on SMAS had some form of airway intervention. The area under curve of 0.9 was noted at a score of 25, so SMAS more than 25 may predict a difficult airway and potential to have complications. Pearson’s correlation between SMAS and height, weight, BMI were poor (p < 0.05). (4) Conclusions: Airway abnormalities in adult MPS are varied and complex. Assessment of the airway should be holistic and include multiple parameters. An objective multidimensional score such as SMAS may help to predict and manage difficult airways warranting further investigation and validation.
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