Ap. Griffiths et al., SPONTANEOUS HEMOTHORAX - A CAUSE OF SUDDEN-DEATH IN VON-RECKLINGHAUSENS-DISEASE, Postgraduate medical journal, 74(877), 1998, pp. 679-681
Vasculopathy is a relatively frequent but poorly recognised manifestat
ion of von Recklinghausen's neurofibromatosis. One of its more dramati
c presentations is as spontaneous haemothorax. Clinicians and patholog
ists should be aware of this syndrome as a cause of sudden death in pa
tients with neurofibromatosis.