Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome.

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.

Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I.

BACKGROUND: Granulomatosis with polyangiitis (Wegener granulomatosis) is a rare disease that commonly starts in the craniofacial region and can lead to considerable facial disfigurement. Granulomas and vasculitis, however, can involve many other tissues (especially pulmonary and renal). Dermatologic and subcutaneous components can lead to malignant pyoderma. METHODS: The authors describe a unique pathologic condition, where significant Le Fort type trauma was associated with subsequent development of granulomatosis with polyangiitis and malignant pyoderma. Successive operations to excise necrotic tissue and reconstruct the defects were followed by worsening inflammation and tissue erosions. Trauma and surgery in proximity to the eye and sinuses masked the initial clinical presentation and led to delay in diagnosis and disease progression. The resultant facial disfigurement and tissue loss were substantial. RESULTS: Despite multiple confounding factors, accurate diagnosis was eventually established. This was based on persistence of sinus inflammations in the absence of infective agents, proven sterility of lung lesions, and antineutrophil cytoplasmic antibody positivity with proteinase 3 specificity. Skin lesion biopsy specimens were identified as pyoderma gangrenosum and later as malignant pyoderma. Institution of immunosuppressive therapy allowed successful control of the disease and wound healing. The resulting craniofacial destruction, however, necessitated facial vascularized composite allotransplantation. CONCLUSION: Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.