Sudden Postpartum Death: An Unexpected Complication of Neurofibromatosis
RD Boyum, JN Elliot, MJ Sexton. Naval Hospital Pensacola, Pensacola, FL; Naval Health Clinic Cherry Point, Cherry Point, NC
Background: Neurofibromatosis type 1 (NF1) is associated with vascular abnormalities including stenosis, aneurysms, and vessel wall dysplasia, sometimes resulting in vessel rupture. NF1 has also been associated with spontaneous hemothorax and sudden death, and spontaneous hemothorax has been reported in pregnant patients with NF1. Furthermore, neurofibromas in patients with NF1 have been noted to increase in size during pregnancy. We present a case of NF1 resulting in maternal mortality secondary to mediastinal vessel rupture and subsequent massive hemothorax in the early post-partum period.
Design: Autopsy was performed with internal gross and microscopic examination of the chest.
Results: A 26-year-old with a known history NF1 delivered a healthy male infant following an uneventful pregnancy and labor course remarkable only for mild persistent elevation of the systolic blood pressure. At 27 hours postpartum, the patient complained of left sided pleuritic chest pain, exhibited signs of shock, and died despite extensive resuscitative efforts. Autopsy revealed left hemothorax, causing marked compression of the left lung. The middle and posterior mediastinum contained hemorrhage, and a ruptured arterial aneurysm was located in the mediastinum. The wall of the affected artery was focally necrotic. Adjacent to and completely surrounding the ruptured artery and nearby vessels and nerves was a diffuse neurofibroma (vascular neurofibromatosis) as confirmed by immunohistochemical studies. Granulation tissue was noted at the site of the rupture.
Conclusions: The patient in this report died from a sudden massive hemothorax and associated repiratory compromise and hemorrhagic shock. The cause of the hemmorhage was rupture of an arterial aneurysm in the mediastinum. The aneurysm apparently formed due to ischemic necrosis of the arterial wall, secondary to encasement of the artery by a diffuse neurofibroma. Granulation tissue at the site of rupture indicates that the initial hemorrhage occured between 1 and 7 days prior to death, and the patient's rapid demise occured after the initial, relatively slow, mediastinal hemorrhage extended into the pleural cavity and rapidly accelerated.
Wednesday, March 11, 2009 1:00 PM
Poster Session VI # 14, Wednesday Afternoon