Juvenile hyaline fibromatosis is a rare autosomal recessive disease characterized by large cutaneous nodules, especially around the head and neck and often involving the lips. The effects become increasingly severe with age and also include joint contractures, gingival hypertrophy and osteolytic lesions. We describe the anaesthetic management of two sisters with this disease. Safe maintenance of a patent airway is the principal anaesthetic challenge.

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We applaud the critically important and excellent editorial by Pandit et al. on the recognition of oesophageal intubation [1]. However, as Dominic Bell pointed out in his 2003 editorial [2], oesophageal intubation is only one cause of difficulty with ventilation, which should be defined as any situation in which high perceived airway pressure and difficulty with ventilation is associated with reducing arterial oxygen saturation.This generic situation must be solved in a logical and systematic way. It is paramount to first exclude oesophageal displacement of the tracheal tube, followed by obstruction in any part of the breathing system, including the tracheal tube.