Spontaneous hemothorax associated with neurofibromatosis type I: A review of the literature

Pulivarthi, Swaroopa; Simmons, Byron; Shearen, John; Gurram, Murali Krishna

doi:10.1055/s-0039-1700327

Cited by 6 publications

(6 citation statements)

References 6 publications

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“…[6,7] In the literature, all published cases of spontaneous massive hemothorax secondary to a neurofibroma have been shown to be related to von Recklinghausen's disease. [1] In this context, this is the first case of spontaneous massive hemothorax secondary to a neurofibroma in non-von Recklinghausen's disease.…”

Section: Discussionmentioning

confidence: 88%

“…Spontaneous massive hemothorax secondary to a neurofibroma is a rare and often lethal complication. [1] Spontaneous massive hemothorax usually occurs secondary to pulmonary infarction, arteriovenous fistula, ruptured aneurysm, and tumors. Numerous benign and malignant tumors have been blamed for the development of spontaneous massive hemothorax including angiosarcoma, chondrosarcoma, f ibrosarcoma, peripheral neuroectodermal tumor, teratoma, and neural tumors.…”

Section: Discussionmentioning

confidence: 99%

“…A concomitant mediastinal neoplasm related to spontaneous hemothorax is a rare finding. [1] Herein, we report a case of spontaneous hemothorax secondary to a neurofibroma of the mediastinum in non-von Recklinghausen's disease.…”

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confidence: 96%

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Spontaneous massive hemothorax related to a neurofibroma: A case report

Charfi¹

2019

Cardiovasc Surg Int

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A 33-year-old woman was admitted due to sudden-onset dyspnea and right-sided chest pain. Imaging studies revealed a right-sided hemothorax and an hyperdense mass of the posterior mediastinum. A monobloc and complete tumor resection was done by thoracotomy. The diagnosis of a neurofibroma was confirmed by the pathological exam. A careful examination of the patient did not suggest any signs of von Recklinghausen's disease. To the best of our knowledge, this is the first case of spontaneous massive hemothorax secondary to a neurofibroma in non-von Recklinghausen's disease.

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Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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Spontaneous massive hemothorax related to a neurofibroma: A case report

Charfi¹

2019

Cardiovasc Surg Int

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“…Interestingly, the surgical management described in all these cases occurred during the sub‐acute rather than the emergency period, where preoperative magnetic resonance imaging was organized prior to theatre. In some cases, the patient required thoracocentesis as a temporizing measure prior to definitive management 2–4 …”

Section: Figurementioning

confidence: 99%

Salvage thoracotomy for atraumatic tension haemothorax in a patient with neurofibromatosis type 1: an Australian experience

Bassin

2020

ANZ Journal of Surgery

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Salvage thoracotomy for atraumatic tension haemothorax in a patient with neurofibromatosis type 1: an Australian experience A 49-year-old male presented to the emergency department on Christmas night with severe chest pain and dyspnoea on a background of atraumatic back pain for the past day. His medical history included neurofibromatosis type 1 (NF1) and scoliosis. A computed tomography aortogram demonstrated a large left-sided tension haemothorax with suspected contrast extravasation adjacent to the scoliosis concavity at T7 (Fig. 1).The patient became haemodynamically unstable necessitating emergency intubation. A 36-Fr drain was concurrently inserted with the immediate drainage of 3 L of frank blood. Massive transfusion protocol was activated and the patient was taken to the operating theatre.An extended left posterolateral thoracotomy was made with the release of frank blood under tension. The area of bleeding was found in the left paraspinal T7 area adjacent to a presumed 3 × 3 cm neurofibroma that was friable and highly vascularized; this was controlled with packing and pressure with an improvement in haemodynamics. Upon intraoperative discussion with a spinal surgeon and interventional radiologist, the decision was made to place drains and proceed to angioembolization. However, increasing haemodynamic instability with active bleeding in the drains necessitated an immediate re-look thoracotomy that demonstrated renewed bleeding, particularly from a large transected intercostal artery branch adjacent to the lesion. Haemostasis was achieved with a combination of 3-0 prolene sutures and clips and the pleural cavity re-packed; the patient was then transferred to the intensive care unit in a stable condition with a total blood loss of 6 L. Given the prolonged period of resuscitation and operating time, the decision was made to proceed to angioembolization only if there was evidence of renewed bleeding.Upon return to theatre the following day, the raw surface was reinforced with topical sealants and the thoracotomy closed thereover. The patient had an uneventful recovery and was discharged 9 days post-admission without any neurological complication. He was well upon his 30-day follow-up.NF1, also known as Von Recklinghausen's disease, is the most common type of neurofibromatosis. It is an autosomal dominant disorder that is characterized by the development of multiple neurofibromas of the peripheral nerves, which subsequently have effects on the vasculature via proliferation, invasion or compression, and pigmentary abnormalities such as café-au-lait macules. 1 Spontaneous haemothorax is a rare but potentially fatal complication of NF1. Degbelo et al. conducted a literature review that has described 17 cases to date. The mean age is 44.13 years and is 2.2 times more common in women, with acute chest pain and dyspnoea as the most frequent presenting symptoms. The source of bleeding is often difficult to locate, requiring imaging with magnetic resonance imaging or computed tomography aortogram. 1 Three management o...

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“…In my opinion, the cause of bleeding in this case was possibly due to a ruptured neurofibroma or a direct invasion of the artery at the apical lung, evidenced by a sentinel clot adjacent to the tumor. [7] Treatment of spontaneous massive hemothorax in NF1 depends on the etiology of the bleeding. Endovascular treatment with embolization is one choice in the case of an arterial rupture related Website: www.ruralneuropractice.com to NF1.…”

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confidence: 99%

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Hongsakul

2014

Journal of Neurosciences in Rural Practice

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Spontaneous hemothorax associated with neurofibromatosis type I: A review of the literature

Cited by 6 publications

References 6 publications

Spontaneous massive hemothorax related to a neurofibroma: A case report

Spontaneous massive hemothorax related to a neurofibroma: A case report

Salvage thoracotomy for atraumatic tension haemothorax in a patient with neurofibromatosis type 1: an Australian experience

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