Spontaneous apical lung herniation presenting as a neck lump in a patient with Ehlers-Danlos syndrome.

Ehlers-Danlos syndrome (EDS) is a heritable group of disorders of connective tissue characterised by skin hyperlaxity, joint hypermobility and tissue fragility. We report on a 49-year-old man with EDS who presented with spontaneous swelling at the root of the right neck during coughing. The size and site of the lesion led to diagnostic uncertainty. Subsequent computerised tomography (CT) scanning during a Valsalva manoeuvre demonstrated a large apical lung hernia arising through the costo-vertebral fascia into the root of the neck. No previous reports have documented spontaneous apical lung herniation in patients with EDS. This case serves to remind all practitioners dealing with the head and neck to remember the apical lung as a potential source of pathology in the neck.