scholarly journals Massive spontaneous hemothorax, giant intrathoracic meningocele, and kyphoscoliosis in neurofibromatosis type 1

2014 ◽  
Vol 6 (1) ◽  
pp. 33 ◽  
Author(s):  
AyodejiSalman Yusuf ◽  
Ashok Pillai ◽  
SajeshK Menon ◽  
Dilip Panikar
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Spontaneous Hemothorax ◽  
Intrathoracic Meningocele

Large intrathoracic meningocele in a patient with neurofibromatosis type 1

2013 ◽  
Vol 22 (2) ◽  
pp. 219-221 ◽  
Author(s):  
Tao Zuo ◽  
Zhengyi Ni ◽  
Mi Zhou ◽  
Chaolin Huang ◽  
Zhongwen Tang ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Intrathoracic Meningocele

Massive Spontaneous Hemothorax in Neurofibromatosis Type 1

CHEST Journal ◽  
2016 ◽  
Vol 150 (4) ◽  
pp. 63A ◽  
Author(s):  
Tousif Kabir ◽  
Atasha Asmat ◽  
Dokev Basheer Ahmed Aneez
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Spontaneous Hemothorax

A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele

2003 ◽  
Vol 10 (2) ◽  
pp. 268-272 ◽  
Author(s):  
S Ebara ◽  
Y Yuzawa ◽  
T Kinoshita ◽  
J Takahashi ◽  
I Nakamura ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Intrathoracic Meningocele

scholarly journals Spontaneous hemothorax: A rare lethal pulmonary complication of neurofibromatosis type 1

2021 ◽  
pp. 433-435
Author(s):  
Aditya Nath Shukla ◽  
Ashok Kumar Singh ◽  
Saket Nigam
Keyword(s):  
Hemorrhagic Shock ◽  
Neurofibromatosis Type 1 ◽  
Autosomal Dominant ◽  
Pulmonary Complication ◽  
Neurofibromatosis Type ◽  
Autosomal Dominant Disease ◽  
Spontaneous Hemothorax ◽  
Dominant Disease ◽  
Abnormal Pigmentation

Neurofibromatosis 1 (NF1), also called von Recklinghausen’s disease, is an autosomal dominant disease characterized by multiple non-cancerous tumors of nerves and skin, and areas of abnormal pigmentation. Vasculopathy and spontaneous hemothorax are rare complications, but potentially lethal, which necessitates quick and decisive intervention to save the life of the patient. Here, we present a case of spontaneous massive haemothorax leading to hemorrhagic shock in a 66-year-old woman with neurofibromatosis type-1. She was investigated and managed successfully.

scholarly journals Surgical repair of a large intrathoracic meningocele associated with neurofibromatosis type 1 after failed cystoperitoneal shunts: illustrative case

2021 ◽  
Vol 2 (10) ◽  
Author(s):  
Christopher H. F. Sum ◽  
Lai-Fung Li ◽  
Benedict B. T. Taw ◽  
Wai-Man Lui ◽  
Ko-Yung Sit ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Muscle Flap ◽  
Neurofibromatosis Type ◽  
Definitive Treatment ◽  
Illustrative Case ◽  
Thoracoscopic Approach ◽  
Regional Flap ◽  
Definitive Repair ◽  
Intrathoracic Meningocele

BACKGROUND Surgical treatment of intrathoracic meningoceles, commonly associated with neurofibromatosis type 1 (NF1), aims to reduce sac size for symptomatic relief. The procedures can be divided into cerebrospinal fluid diversion and definitive repair. The authors describe the management of an intrathoracic meningocele in a 56-year-old female with preexisting NF1. OBSERVATIONS The patient presented with progressive dyspnea. Magnetic resonance imaging revealed a left hemithoracic meningocele arising from the thecal sac at C7–T2. Two attempts at diversion by cystoperitoneal shunts resulted in recurrence. For definitive repair, T2–3 costotransversectomy was performed, and intradural closure of the meningocele opening was performed utilizing spinal dura and autologous fascia lata graft. Trapezius muscle regional flap was turned for reinforcement. Persistent leak warranted reoperation 7 days later. A transthoracic approach was undertaken using video-assisted thoracoscopic resection of the sac at aortic arch level, with reinforcement by latissimus dorsi flap and synthetic materials. Mechanical pleurodesis was performed. Intradural repair of the meningocele opening was revised. LESSONS Inherent dural abnormality makes repair difficult for meningoceles associated with NF1. A combined intradural and thoracoscopic approach with regional muscle flap and synthetic material reinforcement is a unique method for definitive treatment. Some essential points of perioperative management are highlighted.

scholarly journals MON-322 Intrathoracic Meningocele Associated with Neurofibromatosis Type 1: A Case Report

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Pedro Franco ◽  
Delane Wajman ◽  
Julia Couto ◽  
Manuela Rocha-Braz ◽  
Cristina Bueno ◽  
...  
Keyword(s):  
Case Report ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Intrathoracic Meningocele

scholarly journals Hemostasis Surgery of Spontaneous Hemothorax Complicating Neurofibromatosis Type 1

2021 ◽  
pp. 1-4
Author(s):  
Abdennadher Mahdi ◽  
Abdennadher Mahdi ◽  
Ben Saad Soumaya ◽  
Zribi Hazem ◽  
Zairi Sarra ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Hemodynamic Instability ◽  
Endovascular Embolization ◽  
Vascular Lesions ◽  
Spontaneous Hemothorax ◽  
Life Threatening ◽  
Continuous Bleeding

Vascular lesions in Von Recklinghausen’s disease also known as Neurofibromatosis type 1 (NF1), are rare but have a fatal and potentially life threatening complications such as spontaneous hemothorax. An emergent thoracotomy is indicated when there is an active bleeding associated unstable hemodynamic status. Despites surgery is laborious and unpredictable but it have a merit to stop hemorrhage. A conservative management with endovascular embolization or non-operative approach have also been reported in case of hemodynamic stability. We report two case report of spontaneous hemothorax in patient with Recklinghausen disease. A chest tube was immediately inserted for two patients. Due to continuous bleeding and hemodynamic instability (Patient 1), and the increase of pleural effusion volume (Patients 1 & 2), emergent surgery of thorax was done with favourable post-operative follow up.

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