Plexiform Neurofibromatosis: Our Experience at Rural Setup with Review of Literature

Authors

  • Keerthi N. Department of General Surgery, Sri Devarajurs Medical College, Tamaka, Kolar
  • Sreeramulu P.N. Department of General Surgery, Sri Devarajurs Medical College, Tamaka, Kolar
  • Rakesh N. Department of General Surgery, Sri Devarajurs Medical College, Tamaka, Kolar
  • Harsha R. Department of General Surgery, Sri Devarajurs Medical College, Tamaka, Kolar

DOI:

https://doi.org/10.37591/rrjos.v5i2.1447

Abstract

 

Neurofibromatosis 1 (NF1), also known as von Recklinghausen disease, is an autosomal-dominant condition caused by mutations of the NF1 gene, which is located at chromosome 17. The average life expectancy of patients with NF1 is probably reduced by 10–15 years, and malignancy is the most common cause of death [1]. We present a case of 28 year old female with NF-1 who presented to our OPD with a huge painless swelling over the lower back of four years duration. Patient later noticed multiple swellings over the abdomen and the right arm, which increased gradually in size. Histopathological diagnosis of plexiform neurofibromatosis was made and confirmed. Surgical excision with reconstruction was done.


Keywords: Neurofibromatosis, NF-1 gene, plexiform, swelling, Recklinghausen disease

Cite this Article
Keerthi N, Sreeramulu PN, Rakesh N, et
al. Plexiform Neurofibromatosis: Our
Experience at Rural Setup with Review
of Literature. Research and Reviews:
Journals of Surgery. 2016; 5(2): 21–23p.

Published

2019-04-17

Issue

Section

Case Study