Massive hemothorax in a pregnant patient with neurofibromatosis type 1

Abstract Background Reports of spontaneous hemothorax in patients with neurofibromatosis type 1 are scarce despite the severe complication. We herein present the first case of hemothorax in a neurofibromatosis type 1 patient during pregnancy and discuss the difficulty associated with its diagnosis and treatment. Case presentation A 39-year-old female at 34 weeks gestation presented with sudden left back pain and dyspnea. Chest radiography revealed massive left pleural effusion. Computed tomography showed bleeding from the intercostal artery. Although the patient appeared hemodynamically stable, the fetus was in a critical condition. Emergency caesarean section was performed within 1 hour. Subsequently, we performed endovascular coil embolization of the intercostal artery. While this intensive treatment saved the patient, her fetus could not be rescued. Conclusions Patients with neurofibromatosis type 1 may develop massive hemothorax without gross lesions. In late pregnancy, sufficient infusion and quick hemostasis are essential and can be lifesaving.

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Massive Hemothorax in A Pregnant Patient With Neurofibromatosis Type 1

10.21203/rs.3.rs-75605/v1 ◽

2020 ◽

Author(s):

Kumiko Hashimoto ◽

Yuji Nomata ◽

Takayuki Fukui ◽

Akira Takada ◽

Kunio Narita

Keyword(s):

Neurofibromatosis Type 1 ◽

Pregnant Patient ◽

Neurofibromatosis Type ◽

Emergency Cesarean ◽

First Case ◽

Left Pleural Effusion ◽

Pregnancy And Postpartum ◽

Massive Hemothorax ◽

Endovascular Coil Embolization

Abstract Background: Spontaneous hemothorax in a patient with neurofibromatosis type 1 is a less common though lethal complication, and there is no report which occurred in the pregnant period. We here present the first case of hemothorax in a neurofibromatosis type 1 patient during pregnancy and discus on the difficulty in diagnosis and treatment choice.Case Presentation: A 39-year-old female at 34 weeks gestation presented with sudden left back pain and dyspnea. Chest radiography revealed massive left pleural effusion. Although the patient was hemodynamically stable, the fetus was in a critical condition. Emergency cesarean section and endovascular coil embolization of the aneurysm of the intercostal artery were performed successfully. The patient’s condition improved immediately, and she was discharged 1 month later. Conclusions: A neurofibromatosis type 1 patients have the potential to develop massive hemothorax without gross lesions, especially late in pregnancy and postpartum.

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Neurofibromatosis type 1 with subarachnoid haemorrhage from the left vertebral arteriovenous fistula: case presentation and literature review

BMJ Case Reports ◽

10.1136/bcr-2020-239880 ◽

2021 ◽

Vol 14 (2) ◽

pp. e239880

Author(s):

Toshinori Nishizawa ◽

Takahiro Tsuchiya ◽

Yoshihiro Terasawa ◽

Yasuhiro Osugi

Keyword(s):

Neck Pain ◽

Subarachnoid Haemorrhage ◽

Arteriovenous Fistula ◽

Neurofibromatosis Type 1 ◽

Epidural Haematoma ◽

Neurofibromatosis Type ◽

Sudden Onset ◽

Vertebral Arteriovenous Fistula ◽

First Case

We present the case of a 47-year-old woman with neurofibromatosis type 1 (NF1) with subarachnoid haemorrhage (SAH) from the left vertebral arteriovenous fistula, along with a review of previous cases. Our patient had a family history of NF1 and presented to the emergency department with a sudden-onset severe headache and neck pain. CT scan showed SAH. CT angiography revealed a left vertebral arteriovenous fistula and an epidural haematoma. She underwent direct surgery and was discharged without neurologic deficits. To our knowledge, this is the first case of SAH caused by perimedullary drainage of a vertebral arteriovenous fistula associated with NF1. In a literature search, we identified 40 cases of vertebral arteriovenous fistula associated with NF1. The majority of vertebral arteriovenous fistulas occurred on the left side and in women. Patients with vertebral arteriovenous fistula typically experience neck pain, radiculopathy, radiculomyelopathy and bruits.

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Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis Type 1 and middle cerebral artery aneurysm treated with endovascular coil embolization

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.7.peds11151 ◽

2011 ◽

Vol 8 (4) ◽

pp. 346-352 ◽

Cited By ~ 11

Author(s):

Michael J. Ellis ◽

Samuel Cheshier ◽

Sunjay Sharma ◽

Derek Armstrong ◽

Cynthia Hawkins ◽

...

Keyword(s):

Peripheral Nerve ◽

Middle Cerebral Artery ◽

Cerebral Artery ◽

Neurofibromatosis Type 1 ◽

Coil Embolization ◽

Neurofibromatosis Type ◽

Nerve Sheath Tumor ◽

Nerve Sheath ◽

Endovascular Coil Embolization

Among the neoplastic conditions that affect patients with neurofibromatosis Type 1 (NF1) are malignant peripheral nerve sheath tumors (MPNSTs), which typically arise from peripheral nerves of the limbs, trunk, and lumbar and brachial plexuses. Ionizing radiation is an established risk factor for MPNST development, especially in susceptible patients such as those with NF1. Patients with NF1 are also at risk for intracranial aneurysms, which are increasingly being successfully managed with endovascular therapies. The authors describe the case of a 9-year-old, previously healthy girl who presented in extremis with a right frontal intracerebral hemorrhage resulting from a ruptured right middle cerebral artery (MCA) trifurcation aneurysm. Following urgent decompressive craniectomy, the patient underwent endovascular coil embolization of the MCA aneurysm without complication. Given her mother's history of NF1, the child underwent genetic testing, which disclosed signs positive for NF1. The patient recovered well, but follow-up MR imaging and MR angiography performed at 14 months demonstrated a large frontotemporal mass encasing the right MCA trifurcation. The patient underwent frontotemporal craniotomy and subtotal resection of the mass, which was histologically found to be an intracranial MPNST. The patient received chemotherapy and focal radiation therapy and remains alive at 6 months postresection. To the authors' knowledge, this represents the only known case of intracranial neoplasm arising in the region of an intracranial aneurysm repaired by endovascular coil embolization. While patients with NF1 represent a population with genetic susceptibility to radiation-induced tumors, the pathogenesis of intracerebral MPNSTs remains poorly understood.

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A case of neurofibromatosis type 1 showing massive hemothorax due to intercostal aneurysm rupture

The Journal of the Japanese Association for Chest Surgery ◽

10.2995/jacsurg.33.736 ◽

2019 ◽

Vol 33 (7) ◽

pp. 736-743

Author(s):

Nobutaka Kawamoto ◽

Takashi Anayama ◽

Marino Yamamoto ◽

Hironobu Okada ◽

Ryouhei Miyazaki ◽

...

Keyword(s):

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Aneurysm Rupture ◽

Massive Hemothorax

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Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type 1 with Successful Transarterial Embolization

Korean Journal of Radiology ◽

10.3348/kjr.2013.14.1.86 ◽

2013 ◽

Vol 14 (1) ◽

pp. 86 ◽

Cited By ~ 13

Author(s):

Keerati Hongsakul ◽

Sorracha Rookkapan ◽

Pramot Tanutit ◽

Songklod Pakdeejit ◽

Apiradee Songjamrat ◽

...

Keyword(s):

Neurofibromatosis Type 1 ◽

Transarterial Embolization ◽

Neurofibromatosis Type ◽

Massive Hemothorax

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Brain metastasis of Wilms tumor with diffuse anaplasia and complex cytogenetic phenotype in a child with neurofibromatosis Type 1

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.7.peds1119 ◽

2011 ◽

Vol 8 (4) ◽

pp. 353-356

Author(s):

Marianna Shvartsbeyn ◽

Luigi Bassani ◽

Irina Mikolaenko ◽

Jeffrey H. Wisoff

Keyword(s):

Brain Metastasis ◽

Neurofibromatosis Type 1 ◽

Wilms Tumor ◽

Metastatic Tumor ◽

Neurofibromatosis Type ◽

Radiological Features ◽

First Case ◽

History Of ◽

The Brain

The authors report the first case of a Wilms tumor (WT) with diffuse anaplasia metastatic to the brain in a 13-year-old girl with a history of neurofibromatosis Type 1. At presentation, the metastatic tumor had radiological features that suggested a meningioma. Histologically it was characterized by striking anaplasia and features similar to the patient's previously resected WT with diffuse anaplasia.

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Severe Hypercholesterolemia: A Unique Presentation of Non-Hodgkin’s Lymphoma in a Patient with Neurofibromatosis Type 1

Case Reports in Gastrointestinal Medicine ◽

10.1155/2014/579352 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Kenechukwu Chudy-Onwugaje ◽

Nnaemeka Anyadike ◽

Yuriy Tsirlin ◽

Ira Mayer ◽

Rabin Rahmani

Keyword(s):

Obstructive Jaundice ◽

Neurofibromatosis Type 1 ◽

Hodgkin’S Lymphoma ◽

Neurofibromatosis Type ◽

Hodgkin's Lymphoma ◽

Initial Manifestation ◽

Non Hodgkin’S Lymphoma ◽

First Case ◽

Non Hodgkin's Lymphoma

We report a case of non-Hodgkin’s lymphoma (NHL) with an unusual initial manifestation as severe hypercholesterolemia and obstructive jaundice in a patient with neurofibromatosis type 1 (NF 1). NHL should be considered in the evaluation of obstructive jaundice alone or in combination with severe hypercholesterolemia. Relief of biliary obstruction led to the resolution of hypercholesterolemia in our 59-year-old male patient, followed by doxorubicin-based chemotherapy for the underlying lymphoma. NF 1 is a genetic condition that results from a defect in a tumor-suppressor gene and it is likely that this led to the development of NHL in our patient. It is important that clinicians are familiar with the gastrointestinal manifestations of NF 1, especially its association with intra-abdominal malignancies, when treating patients with a personal or family history. To the best of our knowledge, this is the first case of NHL presenting initially as severe hypercholesterolemia and it is also one of the few instances where NHL has been reported in association with NF 1.

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