Spontaneous haemothorax associated with von Recklinghausen's disease: review of occurrence in Japan

Abstract

was no history of trauma. A chest radiograph showed a left pleural effusion (fig 1). A plain computed tomographic (CT) scan showed a left pleural effusion with high density areas Abstract suspicious of blood. There was no evidence The case history is presented of a 61 year of an aneurysm. Numerous subcutaneous old man with von Recklinghausen's disease tumours were seen on the face, trunk, and who developed a spontaneous haemo-limbs. There were no abnormal neurological thorax. In spite of being asymptomatic findings although there was tachycardia for five days after drainage, he died as a (170/min) and the blood pressure was 120/ result of fatal sudden re-bleeding. The post 80 mm Hg. Atrial fibrillation was observed mortem examination showed dissection on the ECG and the haemoglobin level was and rupture of the left subclavian artery. 11.2 g/dl. Microscopically, disarrangement of An intercostal drainage tube was inserted and smooth muscle and decrease of elastic approximately 1000 ml of blood was removed. fibre was observed in the ruptured artery. After drainage the serous bloody discharge deHaemothorax in patients with von Reck-creased to below 100 ml per day. Radiologically linghausen's disease may require thora-there was no evidence of an increase in the cotomy, even if the condition of the patient pleural effusion. appears to be stable.Five days after admission he suddenly col- (Thorax 1997;52:577-578) lapsed and his blood pressure was unrecordable. Although cardiopulmonary resuscitation Keywords: haemothorax, von Recklinghausen's disease, was immediately performed, he died. Blood neurofibromatosis.did not flow out from the chest drainage tube. The chest radiograph taken two hours before cardiac arrest also showed no increase in the Von Recklinghausen's disease is transmitted by pleural effusion. an autosomal dominant gene and is charThe post mortem examination revealed acterised by multiple skin tumours and ab-about 4000 ml of blood in the thoracic cavity. normal cutaneous pigmentation. Benign The tumorous lesion with haematoma, about Department of schwannomas, osseous system involvement, 5 cm in diameter, in the apex was thought to five days after drainage but who died due to served at the ruptured portion. Neurofibromas sudden re-bleeding. The cause of arterial rup-were not seen at the ruptured portion. Hachioji Medicalture was different from those previously re-

NF1 Regulates a Ras-Dependent Vascular Smooth Muscle Proliferative Injury Response

“…4 Increasing evidence indicates that NF1 vasculopathy can be extremely serious, even causing sudden death. [5][6][7] Analysis of US death certificates showed that vascular disease was a cause of death Ͼ7 times more often than expected among NF1 patients Ͻ30 years of age. 8 Despite this, many individuals with NF1-related vascular disease are not brought to clinical attention, so its frequency probably is underestimated.…”

Ruptured Aneurysm of the Subclavian Artery in a Patient With von Recklinghausen's Disease

“…5 There have been 11 cases of von Recklinghausen's disease, including the present case, that presented with ruptured aneurysm or spontaneous rupture of the subclavian artery (Table 1). 6,7,[9][10][11][12][13][14] Symptoms related to compression occurred in 4 patients, hemothorax developed in 4 patients, and 2 of them died from uncontrollable bleeding from the artery. Nine patients underwent surgery, including emergency surgery in 7 patients.…”

Spontaneous Hemothorax Associated with von Recklinghausen’s Disease: Report of a Case

“…1,4) In the thoracic cavity, ruptures of intercostal, subclavian, and internal mammary artery aneurysms 5) have been reported in patients with VRD. In the present case, definitive pathological and radiological findings could not be obtained, and the etiology of the spontaneous hemothorax remains unclear.…”

“…However, even if the condition of patient appears stable, we now consider that earlier timing of operation might be favorable in order to avoid the risk of re-bleeding as fatal sudden re-bleeding has been reported 5 days after chest tube drainage. 1) In addition, retained hemothorax can lead to infection, chronic fibrothorax, lung entrapment, and impaired pulmonary function. Therefore, we recommend earlier indication of exploratory VATS procedure or endovascular procedure for spontaneous hemothorax associated with VRD.…”

“…Different associated complications have been described such as musculoskeletal anomalies and neoplasms (schwannoma, meningioma, etc.). 1) Vascular lesions associated with VRD are rare, but can be fatal. Such lesions are characterized by stenosis, occlusion, aneurysm, pseudoaneurysm, and rupture or fistula formation in small, medium, and large arteries.…”