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Facial plexiform neurofibromatosis: A surgical challenge
Bharat B Dogra1, Karamvir Singh Rana
1Department of Surgery, Padmashri Dr. D Y Patil Medical College Hospital and Research Centre, Pimpri, Pune, India.
Indian Dermatology Online Journal
|August 29, 2013
Summary
Plexiform neurofibromatosis in the face causes significant disfigurement. Surgical removal offers temporary improvement, but regrowth is common, highlighting challenges in managing this neurofibromatosis type 1 manifestation.
Area of Science:
- Plastic Surgery
- Oncology
- Genetics
Background:
- Plexiform neurofibromatosis is a common manifestation of neurofibromatosis type 1 (NF1).
- Facial involvement can lead to severe disfigurement and functional impairment.
- Surgical management is challenging due to tumor infiltration and high recurrence rates.
Observation:
- Two cases of facial plexiform neurofibromatosis are presented.
- Patients exhibited significant facial disfigurement, including visual obstruction and ptosis.
- Associated NF1 stigmata like café au lait macules and neurofibromas were noted.
Findings:
- Subtotal tumor excision and facial skin redraping were performed.
- Both patients experienced tumor regrowth within six months post-surgery.
- This indicates the limitations of current surgical approaches for facial plexiform neurofibromas.
Implications:
- Effective long-term management strategies for facial plexiform neurofibromatosis are needed.
- Further research into adjuvant therapies or novel surgical techniques is warranted.
- Improved outcomes require addressing the infiltrative nature and recurrence potential of these tumors.
