Spontaneous evolution of a massive hematoma caused by type 1 neurofibromatosis: Case report
ConclusionSurgery in NF1 vascular involvement should be considered on a case by case basis especially when the risk of iatrogenesis is high. Surgeons need to be aware of hemorrhagic risks, which could occur because of vessel friability.
ConclusionThe differential diagnosis of neurofibromatosis should be advanced in cases of spontaneous bleeding. In patients diagnosed with neurofibromatosis, the risk of spontaneous bleeding due to the possibility of aneurysmal formation should be considered.
In this report, the authors review the mechanisms, subtypes, and approaches to treatment of DIC with a focus on the bleeding subtype. If this subtype is refractory to blood product administration (> 24 hours), recombinant factor VIIa is a safe and effective option. A wound VAC can be safely utilized with exposed dura if deemed necessary by the surgeon; however, the volume and characteristics of the output should be closely monitored. The use of unconventional surgical solutions may provide options to mitigate the morbidity associated with refractory DIC in spine surgery. PMID: 31604327 [PubMed - as supplied by publisher]
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...
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...
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...
Publication date: Available online 5 April 2019Source: Cirugía Española (English Edition)Author(s): Noa de la Fuente, Manuel Rodríguez Blanco, Gemma Cerdán, Vicenç Artigas
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.
, Artigas V PMID: 30293759 [PubMed - as supplied by publisher]
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.
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]