Spontaneous haemothorax in a patient with diffuse cafe-au-lait macules

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease associated with multiorgan involvement. Arterial involvement in this disease has been reported to be only 3.6%. There is limited data available on spontaneous haemothorax associated with NF1, with only 38 cases found in the literature. Treatment is either conservative or by surgical thoracotomy, depending on the haemodynamic stability of the patient. A 46-year-old man with diffuse cafe-au-lait macules presented with hypotension, dyspnoea, cough and chest pain. He was found to have a left haemothorax on radiological imaging and underwent a thoracostomy that drained over a litre of bloody fluid. He required two emergency thoracotomies to control the bleeding, during which, a bleeding soft tissue lesion was seen in the patient's thoracic cavity. The patient was eventually discharged uneventfully. We present the first reported case of spontaneous haemothorax in a suspected NF1 patient in Singapore who underwent surgical management with a favourable outcome. We hope to highlight the importance of early recognition of this life-threatening condition, as patient deterioration can be rapid and difficult to control.