scholarly journals Neurofibromatosis Type 1 (von Recklinghausen's disease) in Pregnancy: cesarean delivery outcomes – case report

2014 ◽  
Vol 01 (02) ◽  
pp. 62-66
Author(s):  
Brindusa Lungu ◽  
Silvia Baican ◽  
Zenovica Badarau ◽  
Zinica Lungu
2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Monia Attia ◽  
Houda Gharsalli ◽  
Haifa Rmili ◽  
Meriem Affes ◽  
Soumaya Ben Saad ◽  
...  

Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen’s disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. It is generally a benign disease, but has the potential for rare and fatal complications, such as spontaneous hemothorax. 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. Electrocoagulation and clot removal were performed.  Evolution was favorable for both patients. Spontaneous hemothorax is a rare and potentially life threatening NF1’s complication. A tumor like neurofibroma or more rarely vascular involvement of large or small caliber arteries may be at hemothorax’s origin.


1970 ◽  
Vol 1 (1) ◽  
Author(s):  
Siti Handayani ◽  
Kristaninta Bangun ◽  
Fernita Leo Soetjipto Soepodo

Neurofibromatosis type 1 is a rare disease which can manifest itself by the development of plexiform neurofibromatosis, with craniofacial deformities. In this paper, we propose special consideration in excision nasolabial fold mass to help lifting procedures in neurofibromatosis patients. We are reporting two case, both presented with von Recklinghausen’s disease, a 21 years old male patient with neurofibromatosis in his right hemifacial for which he had been operated on five times previously at other center, and a 24 years old male with neurofibromatosis in his left hemifacial. After several stages of reconstruction which started with nasolabial fold mass excision, the result was satisfactory, by using the multiple stage repair, it was done until the end result is similar to anatomical form. The mass debulking procedures for these patients, which started with Nasolabial Fold mass excisison are continued with durable lifting procedures. This method gives results a satisfactory lifting procedure.


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