To compare the key functional results (regarding swallowing and voice rehabilitation) in patients treated by pharyngo-laryngectomy with flap reconstruction, versus standard, wide-field, total laryngectomy. We studied 97 patients who had undergone total laryngectomy and pharyngo-laryngectomy with flap reconstruction. The main outcome measures were swallowing (i.e. solid food, soft diet, fluid or enteral feeding) and fluent voice development. There were 79 men and 18 women, with follow up of one to 19 years. Voice (p = 0.037) and swallowing (p = 0.041) results were significantly worse after circumferential pharyngo-laryngectomy than after non-circumferential pharyngo-laryngectomy. There was no significant difference in voice (p = 0.23) or swallowing (p = 0.655) results, comparing total laryngectomy and non-circumferential pharyngo-laryngectomy. The presence of a post-operative fistula significantly influenced voice (p = 0.001) and swallowing (p = 0.009) outcomes. 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.

Mucopolysaccharidoses (MPS) are a heterogeneous group of disorders that results in the absence or deficiency of lysosomal enzymes, leading to an inappropriate storage of glycosaminoglycans (GAGs) in various tissues of the body such as bones, cartilage, heart valves, arteries, upper airways, cornea, teeth, liver and nervous system. Clinical manifestations can become progressively exacerbated with age and affect their quality of life. Developments in advanced supportive treatment options such as enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT) may have improved patients' life span. Adult MPS patients require specialist clinical surveillance long-term. In many cases, in addition to the MPS-related health problems, they may develop age-related complications. Considering the complexity of their clinical manifestations and lack of guidelines on the management of adult MPS disorders, multispecialty and multidisciplinary teams' care is essential to diagnose and treat health problems that are likely to be encountered. This review presents non-cardiac clinical manifestations, their pathophysiology, management and long-term outcomes in adult MPS patients.