Living related hemi-face skin transplant using radial forearm free flap for a xeroderma pigmentosa patient: early outcome

Abstract

IntroductionXeroderma pigmentosa (XP) is a hereditary disease characterized by deficient repair of DNA damage that occurred on exposure of the skin to ultraviolet irradiation. The affected children have a propensity to develop multiple skin cancers mainly in the face and eventually die before the age of 20.HypothesisAllograft replacement of facial skin by a healthy skin from normal person might decrease the incidence of skin cancer development, the number of surgical procedures, and eventually might improve the survival of these miserable patients.MethodsAs Cadaveric organs are unavailable in our country. After approval from the ethical committee, confirmed agreement of the donor and the patient's guardian, a radial forearm free flap was transplanted from an ABO compatible mother to her 5 year old daughter with XP. The mother had an older daughter died from the same disease at the age of 14. The flap replaced skin of the hemi face that developed precancerous lesions. The girl was kept on adjusted doses of immunosuppressive drugs.ResultsThe flap survived, wounds healed uneventfully. The flap developed a reddish spot one and half month following transplant where baseline skin biopsy was taken. In the fifth months the girl presented with bad non salvageable rejection that ended up loosing the flap. On long term follow up, the girl started to develop skin lesion on the virgin half of the face. Our early cosmetic result replacing half of the facial skin was very promising. In addition the girl did not develop skin lesions in the operated site.ConclusionOur early cosmetic result was very promising. In addition to this, the girl did not develop skin lesions in the operated side of the face

Therapeutics of xeroderma pigmentosum: A PRISMA-compliant systematic review

“…However, there was consensus regarding skin-sparing resections and the use of skin grafts from donor areas that were not exposed to the sun and/or were poorly pigmented. 36,46,57,58 No study reported continuous, long-term follow-up to determine the incidence of recurrence or new lesions in the grafts or flaps used. This scientific scenario could be explained by the rarity of xeroderma pigmentosum, making it difficult to perform large randomized clinical trials and meta-analyses.…”

“…Twenty (80%) of the tumors were on the face, one was on the back and three on the forearms. All wounds were closed primarily or with split grafts Amin et al 46 Living related hemi-face skin transplant using radial forearm free flap for an XP patient: early outcome…”

Perioperative risk factors for vascularized composite allotransplantation: A systematic review and proposal of identity‐defining VCA

“…The already‐acknowledged disputable cases include the penis transplant, uterus transplant, tongue and cephalocervical flap transplant for cancer patients, and the comcomitant face and double hands transplant . In our opinion, the propriety of the thumb transplant, the palm transplant, and the facial flap transplant for xeroderma pigmentosa patient are also questionable . Clearly, the thumb and palm defects were not important enough to risk lifelong immunosuppression; and the xeroderma pigmentosa patient would die sooner with immunosuppression.…”

“…[41][42][43][44][45] In our opinion, the propriety of the thumb transplant, the palm transplant, and the facial flap transplant for xeroderma pigmentosa patient are also questionable. [46][47][48] Clearly, the thumb and palm defects were not important enough to risk lifelong immunosuppression; and the xeroderma pigmentosa patient would die sooner with immunosuppression.…”

Four-Quadrant Approach Squared: Ethical Analysis of Living Donation in Vascularized Composite Allotransplantation

“…Examples include abdominal wall tissue (for breast reconstruction), 9 omentum (for scalp repair), 10 uterus, 11 and fasciocutaneous radial forearm tissue (for hemifacial reconstruction). 12 Despite successful outcomes, these cases have raised legal and 1245e ethical concerns with respect to living donation in vascularized composite allotransplantation. Specifically, compared to solid organ transplantation, the benefits of live donor allotransplantation are less clear.…”