Acute Gastrointestinal Bleeding, Multiple GIST and Intestinal Ganglioneuromatosis in a Patient With Neurofibromatosis

Publication date: Available online 5 April 2019Source: Cirugía Española (English Edition)Author(s): Noa de la Fuente, Manuel Rodríguez Blanco, Gemma Cerdán, Vicenç Artigas
Source: Cirugia Espanola - Category: Surgery Source Type: research

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We reported two cases of patients with a history of von Recklinghausen's disease. Both of them suffered sudden chest pain. Chest-X ray revealed a hemi-thoracic opacity. Enhanced chest computed tomography showed massive hemothorax, but no evidence of tumors or an obvious bleeding point in the thorax. After we had ensured a stable hemodynamic condition, we performed video-assisted thoracic surgery to remove the hematoma. No evidence of bleeding was noticed in the first patient whereas an active bleeding was observed in the second patient. We could not determine which vessel was responsible of the hemorrhage. Electrocoagulati...
Source: Monaldi Archives for Chest Disease - Category: Respiratory Medicine Tags: Monaldi Arch Chest Dis Source Type: research
This study aimed to report a case of gastrointestinal stromal tumor in the jejunal region in a patient with type 1 neurofibromatosis, followed-up for two years, who underwent laparoscopic segmental enterectomy and diagnosis determined by histopathology and immunohistochemistry. The diagnosis of small intestine gastrointestinal stromal tumor is challenging because of its low incidence, nonspecific symptoms, relative inaccessibility of the small intestine to conventional endoscopic examination, broad spectrum of radiological appearances, and the fact that the nature of the mass is difficult to determine with imaging examinat...
Source: Journal of Coloproctology - Category: Gastroenterology Source Type: research
Gingival enlargement, although frequently encountered in clinical settings, is rarely associated with systemic diseases or syndromes. Among the diverse pathological conditions of neurofibromatosis type 1 (NF-1), minor manifestations in the orofacial region are occasionally overlooked. Herein, the authors present an unusual case of gingival neurofibroma in a patient with NF-1 associated with characteristic osseous defects in the alveolus in the long-term course of 17 years from the first examination. A 5-year-old boy with NF-1 was referred for the evaluation of gingival enlargement in the posterior left maxilla. An incisal...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Brief Clinical Studies Source Type: research
ConclusionAlthough TAE with gelatine particles and balloon occlusion were reliable haemorrhage control methods in debulking surgery for lower limb DPN, optimal haemorrhage control technique, compatible with haemostasis and wound healing, was desired.
Source: International Journal of Surgery Case Reports - Category: Surgery Source Type: research
, Artigas V PMID: 30293759 [PubMed - as supplied by publisher]
Source: Cirugia eEspanola - Category: Surgery Authors: Tags: Cir Esp Source Type: research
We report a fatal case of massive hemothorax due to a spontaneous rupture of the left pulmonary artery branch micro-aneurysm in a NF 1 patient. Indeed, spontaneous rupture of these pathologic vessels is very rare in clinical practice and the literature, but, for its potentially life-threatening complications, there is the need for it to be taken into account in differential diagnosis. The origin of bleeding was first confirmed by computed tomography angiography (CTA). The patient ’s condition worsened suddenly leading to pulmonary hemorrhage and death. A clinical autopsy was required to assess the definitive cause of death.
Source: Forensic Science, Medicine, and Pathology - Category: Forensic Medicine Source Type: research
CONCLUSIONS Intracerebral OPG hemorrhages have a fatal outcome in 20% of cases. Age, hormonal status, neurofibromatosis involvement, and histopathological diagnosis have been suggested as risk factors for hemorrhage, but this cannot be reliably established from the present series. The goals of surgery should be patient survival and prevention of further neurological and ophthalmological deterioration. PMID: 29424646 [PubMed - as supplied by publisher]
Source: Journal of Neurosurgery - Category: Neurosurgery Authors: Tags: J Neurosurg Source Type: research
There have been anecdotal reports of vasculopathy associated with Neurofibromatosis Type 2 (NF2). Given the increasing use of bevacizumab, a vascular endothelial growth factor inhibitor which results in an increased risk of bleeding, it is important to ascertain if there is a predisposition to vascular abnormalities in NF2. In our unit NF2 patients undergo annual MRI brain and internal auditory meatus imaging. We noted incidental intracranial aneurysms in some patients and sought to determine the prevalence of intracranial aneurysms in our cohort of NF2 patients. We conducted a retrospective audit of the MRI images of 104 ...
Source: American Journal of Medical Genetics Part A - Category: Genetics & Stem Cells Authors: Tags: ORIGINAL ARTICLE Source Type: research
We report the case of a 28-year-old male patient presented with a large back-pack shaped plexiform neurofibroma. The tumor was successfully excised using the pineapple technique, with minimal intraoperative blood loss. To our opinion, this is the first reported case of excision of a plexiform neurofibroma combined with multi-layered hemostatic suture technique. This novel application proves the efficacy and the potential of the technique, extending its feasibility to the excision of any type of vascular tumor or extensive cancerous masses.Level of Evidence: Level V, therapeutic study
Source: European Journal of Plastic Surgery - Category: Cosmetic Surgery Source Type: research
Abstract: Type-1 neurofibromatosis, a common autosomal dominant disease, is also known as von Recklinghausen disease. Surgical procedures to treat this condition are challenging because of the brittleness of the surrounding blood vessels and soft tissues that bring the risk of causing fatal bleeding. With improvements in neurovascular embolization procedures, some literatures have been published about the application of preoperative embolization for neurofibromatosis. This case report describes a 60-year-old female with Type-1 neurofibromatosis, who presented giant facial neurofibromas with intratumoral hemorrhage on both ...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Clinical Studies Source Type: research
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