Author(s)
Sarek Alexander Shen BS
Aria Jafari MD
Bharat A. Panuganti MD
David M. Chan MD
Bruce H. Campbell MD
Susan R. Cordes MD
Affiliation(s)
University of California San Diego
Abstract:
Educational Objective: At the conclusion of this presentation, the participants should be able to describe clinical presentation and management of a patient with complex facial deformity resulting from giant hemifacial plexiform neurofibroma in a resource limited setting. Objectives: Plexiform neurofibromas (PN) are rare, painless, benign neoplasms resulting from abnormal growth of peripheral nerve sheaths and surrounding connective tissue. The condition is pathognomonic for neurofibromatosis type 1 and lesions are most commonly found in the head and neck. Given this location, PNs can result in functional impairment and social stigma. Intervention is typically surgical, with the goal of improving quality of life through enhanced cosmesis and restoration of function. We present a case of giant hemifacial plexiform neurofibroma and discuss the sequential management in a resource limited setting. Study Design: Case report. Methods: A 32 year old female with progressive right sided facial deformity since childhood presented to a public hospital in Eldoret, Kenya. The patient described social isolation and impaired vision from right eye due to ptosis. Biopsy of the tumor revealed PN. She underwent subcutaneous debulking of the right malar and upper lip regions, restoration of nasolabial fold, and resection of excess skin. Two years later, she underwent upper and lower lid blepharoplasty, lateral canthal fixation, fascia lata sling, neck lift and alar base reduction. Results: The patient reported significant functional and cosmetic improvement and enhanced quality of life after sequential, staged reconstructive surgeries. Conclusions: PNs are rare, benign tumors with potentially significant functional and cosmetic consequences. Surgical intervention is the mainstay of treatment, with emphasis on improved cosmesis and restoration of function. Sequential, staged reconstructive procedures with close followup may be successful in the treatment of this condition. Further research is necessary to establish consistent surgical approaches, indications for treatment, and timing of intervention, particularly in a resource limited setting.