Elsevier

The Lancet

Volume 372, Issue 9639, 23–29 August 2008, Pages 639-645
The Lancet

Articles
Repair of the lower and middle parts of the face by composite tissue allotransplantation in a patient with massive plexiform neurofibroma: a 1-year follow-up study

https://doi.org/10.1016/S0140-6736(08)61277-5Get rights and content

Summary

Background

The risk to benefit ratio of face transplantation with a composite tissue allograft remains debatable, although this procedure is technically feasible. We report here a 1-year follow-up of a patient who underwent face transplantation with a composite tissue allograft.

Methods

On Jan 21, 2007, a 29-year-old man with neurofibromatosis type 1 underwent resection of a massive plexiform neurofibroma diffusely infiltrating the middle and lower part of his face. The main goal was to restore both the cutaneous appearance and function of the face, including, in particular, control of orbicularis oculi and oris muscle contraction. The issues of immunosuppressive therapy, psychological outcome, and social reintergration were addressed, together with the monitoring of graft rejection by biopsies of the skin and mucosa.

Findings

The initial postoperative course was uncomplicated. Two episodes of clinical rejection occurred on days 28 and 64. The second episode was associated with cytomegalovirus infection. Both episodes resolved favourably, with no further clinical signs of rejection, making the reduction of immunosuppressive treatment possible. A year after surgery, the functional outcome was very good, with successful sensory and motor reinnervation in the transplanted territory. Psychological recovery was excellent, with complete social reintegration.

Interpretation

This case demonstrates the feasibility of surgically removing a large part of the face and replacing it with a composite tissue allograft. This facial repair procedure, which seems to have a satisfactory risk to benefit ratio, could be offered in rare and selected cases.

Funding

Programme Hospitalier de Recherche Clinique.

Introduction

The risk to benefit ratio of face transplantation using a composite tissue allograft (CTA) remains debatable,1 although the procedure seems to be technically feasible. However, reports of cases that have assessed the risk to benefit ratio of the procedure are rare.2 The need for large doses of immunosuppressive drugs to ensure CTA survival and the need for intense psychological support to prevent distress has raised ethical concerns, preventing many teams from attempting this type of transplant.

Most facial defects can be reconstructed using autologous tissues and standard plastic surgery techniques. However, in some cases, conventional plastic and reconstructive surgery cannot provide satisfactory results to severely disfigured patients. The patients concerned have generally been disfigured by burns, ballistic trauma, tumours, or congenital deformities.3 Congenital deformities can have a genetic origin, as in the case of neurofibromatosis type 1 (NF1), which is an autosomal dominant genetic disease with an incidence of 1 in 2500–3300.4 Plexiform neurofibromas associated with NF1 occur in about 10% of cases, can be disfiguring, and have a major effect on quality of life.5 Conventional surgery often has little to offer patients with these conditions, and face transplantation has been identified as a possible alternative treatment. We report here a 1-year follow-up of a patient with deforming plexiform neurofibroma associated with NF1 who underwent face transplantation with a CTA. We focus on the management of post-transplantation immunosuppression and on functional and psychosocial outcomes.

Section snippets

Methods

In 2002, we asked the French National Ethics Advisory Committee for Life Sciences and Health to give advice about facial repair using a CTA. The committee's response was delivered in 2004: this kind of transplant could be proposed for severely disfigured patients—cases of total destruction of the mouth and the nose for example—and should be presented to the patient as highly experimental. Preclinical anatomical studies had already been done to optimise the surgical technique.6, 7 A clinical

Results

The only postoperative complication seen was transient steroid-induced confusion, which was treated with 25–50 mg of chlorpromazine for 5 days. The swelling resolved within 1 week. The tracheotomy cannula was removed on day 8 and the patient was able to speak and eat by day 10.

The follow-up immunosuppressive regimen is described in figure 3. Early biopsies of skin and mucosa (days 2, 7, 14, and 21) showed no lymphocyte infiltration, corresponding to grade 0 on the acute rejection scale. Mild

Discussion

Our case confirms that face transplantation is surgically feasible and effective for the correction of specific disfigurement, due in this case to a genetic disorder. The procedure involved three technical challenges: the removal of a disfiguring tumour, plexiform neurofibroma, which is difficult to debulk due to its vascularity;10 the need to procure the face of a donor with a beating heart; and the transplantation itself.

Blood loss is a known, life-threatening complication of surgery for

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