Ann Thorac Surg 2006;82:1500-1501
© 2006 The Society of Thoracic Surgeons
Case Reports
Massive Spontaneous Hemothorax Associated With Von Recklinghausen's Disease
Mohammad Vaziri, MDa,*,
Meetra Mehrazma, MDb
a Department of Thoracic Surgery, Hazrat Rasool Hospital, Iran University of Medical Sciences, Tehran, Iran
b Department of Pathology, Hazrat Rasool Hospital, Iran University of Medical Sciences, Tehran, Iran
Accepted for publication February 2, 2006.
* Address correspondence to Dr Vaziri, Thoracic Surgery Ward-Hazrat Rasool Hospital-Niayesh Ave-Shahrara, Tehran, 1445713131 Iran (Email: dr_m_vaziri{at}yahoo.com).
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Abstract
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A 30-year-old woman with Von Recklinghausen's disease was admitted to our hospital because of sudden onset of dyspnea and syncope. A chest roentgenogram showed a massive right pleural effusion and insertion of an intercostal tube drained 1,600 cc of blood. A computed tomographic chest scan with contrast revealed a hyperdense mass in the right paravertebral area. At thoracotomy, retained clotted hemothorax and continued bleeding from tumor vessels was noted. The apex of the right hemithorax and the tumor location was packed. The pathologic diagnosis was ganglioneuroma and follow-up of the patient for 2 years after re-thoracotomy and removal of the packs revealed no complication and morbidity. We report this case to emphasize the importance of early recognition and prompt surgical intervention in spontaneous hemothorax associated with Von Recklinghausen's disease.
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Introduction
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Spontaneous hemothorax is a rare and life threatening complication of neurofibromatosis. It is usually due to vasculopathy presenting as aneurysmal alterations in large vessels such as the aorta or dysplastic features in smaller vessels [1]. Presence of a concomitant mediastinal tumor causing spontaneous hemothorax is a rare finding in this disease, and we report a 30-year-old woman with this unusual complication to emphasize the consideration of such diagnosis and prompt thoracotomy even if the patient remains stable.
A 30-year-old woman with Von Recklinghausen's disease was admitted to our hospital due to sudden onset of dyspnea and syncope. Physical examination revealed an agitated patient with tachycardia (pulse rate = 130/min), tachypnea (respiratory rate = 30/min), blood pressure of 80/50 mm Hg, slight fullness in the right supraclavicular fossa, and decreased breath sounds in the right lung field. No other abnormality was noted. The patient's weight was 55 kg; she was not married, and her mother and grandfather were also affected by neurofibromatosis with no morbidity.
Chest roentgenogram revealed a large amount of right-sided pleural effusion (Fig 1) and 1,600 cc blood was drained by inserting a chest tube. A hyperdense mass and residual hemothorax was noted in the computed tomographic chest scan (Fig 2). Laboratory tests including coagulation studies were normal except for a low hemoglobin level of 7 g/dL. Due to the presence of a massive hemothorax (> 20 cc/kg), residual clotted hemothorax and the finding of a hypervascular mediastinal tumor, prompt thoracotomy was performed. Continued bleeding from tumor vessels (probably due to spontaneous rupture) was noted, and the inability to achieve a stable hemodynamic status despite the transfusion of 8 units of blood prompted us to pack the area with three large laparotomy pads (Fig 3). The patient's condition was too unsatisfactory to allow us to resect the lesion and perhaps to lose time. The first priority was to save the patient's life, and the safest and simplest way at that time was by "packing." Three weeks later the patient tolerated an uneventful re-thoracotomy and removal of the packs with no morbidity; the patient has been followed for 2 years with no complication. Waiting such a long time before removal of the packs was partly due to the reluctance of the patient to undergo a reoperation. The packs were removed without difficulty and there was no re-bleeding. More importantly, there was no obvious lesion to resect, and the disappearance of the tumor (perhaps due to the rupture and losing its vascular supply-necrosis, or degeneration) was thoroughly confirmed later by postoperative computed tomographic chest scans. The final pathologic diagnosis of the tumor, which has been confirmed by some other unrelated pathologists, was ganglioneuroma (Fig 4).
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Comment
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Massive spontaneous hemothorax is a very uncommon and life-threatening manifestation of a presenting disease. It is a known complication of Von Recklinghausen's disease mainly due to vascular involvement. Dramatic presentations such as sudden death [2], dissection and rupture of the subclavian artery leading to fatal re-bleeding [3], and spontaneous rupture of an intercostal artery or an intercostal vein [4] have been reported in this regard. Another important and rare reported source of spontaneous hemothorax is benign and malignant thoracic tumors including angiosarcoma, chondroblastoma [5], pleural fibrosarcoma, peripheral neuroectodermal tumor [6], and teratoma of the mediastinum [7]. Simultaneous occurrence of neurofibromatosis and an uncommon mediastinal tumor, such as ganglioneuroma leading to spontaneous hemothorax, is quite rare. The expected mediastinal tumor in Von Recklinghausen's disease is a tumor of nerve sheath origin namely neurofibroma, and 4% of patients with this disease develop malignant schwannoma, which has been reported to cause massive hemothorax [4]. We emphasize prompt thoracotomy in any patient with Von Recklinghausen's disease who presents with sudden onset of pleural effusion and consideration of a mediastinal tumor in the differential diagnosis.
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References
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Abstract
Introduction
