التفاصيل البيبلوغرافية
| العنوان: |
Neurofibroma: Case Series with Clinical Features and Recommendations. |
| المؤلفون: |
Khatri N; Dr. Ruth K. M. Pfau Civil Hospital Karachi, Dow University of Health Sciences, Karachi, Pakistan., Raza ML; Al Fatima Hospital, Gulshan e Iqbal, Karachi, Pakistan; Department of Health Management, IoBM, Karachi, Pakistan., Aijaz A; Dr. Ruth K. M. Pfau Civil Hospital Karachi, Dow University of Health Sciences, Karachi, Pakistan., Ramesh R; Dr. Ruth K. M. Pfau Civil Hospital Karachi, Dow University of Health Sciences, Karachi, Pakistan., Gianchand N; Dr. Ruth K. M. Pfau Civil Hospital Karachi, Dow University of Health Sciences, Karachi, Pakistan., Khan FAA; Dr. Ruth K. M. Pfau Civil Hospital Karachi, Dow University of Health Sciences, Karachi, Pakistan. |
| المصدر: |
Acta neurologica Taiwanica [Acta Neurol Taiwan] 2024 Sep 30; Vol. 33(3), pp. 112-121. |
| نوع المنشور: |
Journal Article |
| اللغة: |
English |
| بيانات الدورية: |
Publisher: Color Design Country of Publication: China (Republic : 1949- ) NLM ID: 9815355 Publication Model: Print Cited Medium: Print ISSN: 1028-768X (Print) Linking ISSN: 1028768X NLM ISO Abbreviation: Acta Neurol Taiwan Subsets: MEDLINE |
| أسماء مطبوعة: |
Original Publication: Taipei : Color Design, [1996- |
| مواضيع طبية MeSH: |
Neurofibromatosis 1*/complications , Neurofibromatosis 1*/diagnosis , Neurofibromatosis 1*/pathology , Neurofibroma, Plexiform*/complications , Neurofibromatoses*/complications , Neurofibroma*/diagnosis , Neurofibroma*/complications , Neurofibroma*/pathology , Hamartoma*/complications , Eye Neoplasms*/complications, United States ; Humans ; Cafe-au-Lait Spots/complications ; Cafe-au-Lait Spots/diagnosis ; Cafe-au-Lait Spots/pathology |
| مستخلص: |
Neurofibroma is an autosomal benign disorder. It can be localized, diffuse or invasive like plexiform neurofibroma that involves the nerves, muscle, tissues, skeleton. It represents itself as a destructive variant of neurofibroma, mostly present as orbital or periorbital neurofibroma or may be associated with autosomal dominant disease. Clinical diagnosis of neurofibromatosis (NF) according to National Institutes of Health (NIH) criteria should have more than two of the seven features including lisch nodules, cafe'- au-lait spots, plexiform neurofibroma, optic glioma, freckling, first degree relative with NF or dysplasia of cortical bones. However, proper early diagnosis is still crucial due to its various presentation such as cheek mass, painless swelling on skin, chalazion, intratracheal tumor, genital swelling or ptosis. It is reported that neurofibroma often represents as ocular or facial swelling. Here we are presenting features of neurofibroma of eight cases of patients from Civil Hospital, Karachi. These cases had main complain of overhanging skin mass mainly on orbital or periorbital region that damage the area and with poor daily activities. Multiple nodules on face and body along with them Cafe'-au-lait spots and lisch nodules were main signs. While, other signs i.e. ptosis, pterygium, telecanthus and muddy discoloration of conjunctiva need further evaluation for correlation with neurofibromatosis. Debulking surgery was planned for most of the cases but the huge disfigurement caused by overhanging skin mass and nodules made it a challenge for plastic surgeons to provide good outcomes with minimum damage. Keywords: neurofibroma; lisch nodules; ptosis; Cafe'-au-lait spot; periorbital; overhanging skin. |
| تواريخ الأحداث: |
Date Created: 20231116 Date Completed: 20240214 Latest Revision: 20240214 |
| رمز التحديث: |
20240214 |
| PMID: |
37968860 |
| قاعدة البيانات: |
MEDLINE |
