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Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis)

Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome

Hashem, Ahmed M., MD*†; Djohan, Risal, MD*; Bernard, Steven, MD*; Hendrickson, Mark, MD*; Schwarz, Graham, MD*; Gharb, Bahar B., MD, PhD*; Rampazzo, Antonio, MD, PhD*; Hoffman, Gary S., MD, MS, MACR; Doumit, Gaby, MD, MSc*; Bergfeld, Wilma, MD§; Zins, James E., MD*; Siemionow, Maria, MD, PhD, DSc; Papay, Francis, MD*; Gastman, Brian, MD*

doi: 10.1097/SAP.0000000000001735
Transplantation Surgery and Research
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Background We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. This account (1) highlights the technical details of face transplantation for this unique indication, (2) reports the 3-year posttransplant outcome, and (3) describes relevant immunological aspects.

Methods A Le Fort III near-total face and near-total scalp transplant was performed after extensive trauma and subsequent bone and soft tissue infection in a patient with GPA. Incisions were planned along facial aesthetic subunits. The vascular pedicle comprised the facial and superficial temporal arteries bilaterally. The functioning left eye was preserved and fitted into the donor tissues.

Results The procedure took 21 hours, and transfusion was limited to 4 units of packed red cells. Early medical and surgical complications were successfully treated. At 3 years, acceptable aesthetic outcome was achieved with adequate color match and scalp hair growth. The patient has recovered light touch, temperature, and 2-point discrimination and has evidence of symmetric cheek elevation albeit with limited eyelid and frontalis function. GPA relapse did not occur. Four acute rejections were fully reversed.

Conclusions This case represents a new underlying disease (trauma + GPA) leading to face transplantation and a unique clinical scenario where allografting was indicated for potentially life-threatening and sight-preserving reasons and not for mere functional and aesthetic concerns. Despite complexity, 3-year clinical outcome is encouraging, and the patient is no longer at risk for dural exposure, meningitis, and related morbidity.

From the *Department of Plastic Surgery, Cleveland Clinic, Cleveland, OH;

Division of Plastic Surgery, Cairo University, Cairo, Egypt;

Departments of Rheumatology, and

§Dermatology, Cleveland Clinic, Cleveland, OH; and

Department of Orthopedics, University of Illinois, Chicago, IL.

Received April 29, 2018, and accepted for publication, after revision October 5, 2018.

Conflicts of interest and sources of funding: This work was supported by the Armed Forces Institute of Regenerative Medicine, Department of Defense, under award no. W81XWH-08-2-0034. The US Army Medical Research Acquisition Activity, Fort Detrick, MD, is the awarding and administering acquisition office. Opinions, interpretations, conclusions, and recommendations are those of the authors and are not necessarily endorsed by the Department of Defense. The authors declare no conflict of interest.

Reprints: Brian Gastman, MD, Department of Plastic Surgery—Head and Neck Surgery, Cleveland Clinic, Mail Code A-60, 9500 Euclid Ave, Cleveland, OH 44195. E-mail: gastmab@ccf.org.

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