Cardiothoracic Surgery: Student/Resident Case Report Poster - Cardiac and Thoracic Surgery |

Massive Spontaneous Hemothorax in Neurofibromatosis Type 1 FREE TO VIEW

Tousif Kabir, MBBS; Atasha Asmat, MMed; Dokev Basheer Ahmed Aneez, MBBS
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Tan Tock Seng Hospital, Singapore, Singapore

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):63A. doi:10.1016/j.chest.2016.08.070
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SESSION TITLE: Student/Resident Case Report Poster - Cardiac and Thoracic Surgery

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterised by multiple café au lait macules, benign neurofibromas, skeletal abnormalities, and learning disabilities [1]. Massive spontaneous hemothorax is a rare, potentially fatal complication of this disease.

CASE PRESENTATION: We present two cases of massive spontaneous hemothorax associated with NF-1. The first patient was a 42-year-old lady with known NF-1 who presented with a 2 day history of chest pain. Computed tomography (CT) scan of the thorax demonstrated a large left pleural effusion with mediastinal shift. Chest tube insertion drained fresh blood. An emergent posterolateral thoracotomy was performed, and a tumour was noted overlying the first rib. This was successfully resected. Histopathological diagnosis confirmed a benign neurofibroma. She made an uneventful recovery and was discharged home well. The second patient was a 46 year old man with no known medical history who presented with chest pain and hypotension. Clinical examination revealed multiple café au lait macules on his trunk. A CT thorax performed as diagnostic workup for suspected sepsis revealed a massive left pleural effusion. A chest tube was placed which similarly drained fresh blood. On emergent thoracotomy, an actively bleeding ruptured and fragile blood vessel was seen near the 2nd rib. This was ligated with pledgetted sutures, but the patient had recurrent bleeding two hours later requiring a re-look thoracotomy. He subsequently made an uneventful recovery and was discharged well.

DISCUSSION: There have been previous reports of massive spontaneous hemothorax in NF-1. While rare, this complication is associated with a high mortality rate of 28%. Females are affected more often (63%) and there is a left-sided predominance (61%). The source of bleeding is usually the intercostal arteries (30%) and subclavian arteries (26%) which can be invaded by a neurofibroma or become aneurysmal. In 4 cases, the source of bleed could not be identified definitively. Therapeutic approaches include open surgery or interventional radiological procedures. Procedures performed at thoracotomy include suture ligation, resection of the neurofibroma or packing. Some haemodynamically stable patients undergo endovascular intervention with coil embolisation or stenting.

CONCLUSIONS: Spontaneous hemothorax is an unusual complication in NF-1 patients. One must have a high index of suspicion for this and once diagnosed, therapeutic measures must be instituted rapidly to avoid potentially catastrophic consequences. Stable patients may be considered for endovascular intervention, but in the event of deterioration they should be referred emergently for surgery.

Reference #1: National Institutes of Health consensus development conference: neurofibromatoses. Arch Neurol 1988;45:575-8.

Reference #2: Brasfield RD, Das Gupta TK (1972) Von Recklinghausen’s disease: a clinicopathological study. Ann Surg 175:86-104

DISCLOSURE: The following authors have nothing to disclose: Tousif Kabir, Atasha Asmat, Dokev Basheer Ahmed Aneez

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