Guillain-Barré syndrome associated with resistant cytomegalovirus infection after face transplantation.

A 39-year-old male, who received a facial allograft (cytomegalovirus [CMV] donor-seropositive, recipient-seronegative), developed multidrug-resistant CMV infection despite valganciclovir prophylaxis (900 mg/day) 6 months post transplantation. Lower extremity weakness with upper and lower extremity paresthesias developed progressively 11 months post transplantation, coinciding with immune control of CMV. An axonal form of Guillain-Barré syndrome was diagnosed, based on electrophysiological evidence of a generalized, non-length-dependent, sensorimotor axonal polyneuropathy. Treatment with intravenous immunoglobulin led to complete recovery without recurrence after 6 months.

Transformation of Face Transplants: Volumetric and Morphologic Graft Changes Resemble Aging After Facial Allotransplantation.

Facial allotransplantation restores normal anatomy to severely disfigured faces. Although >30 such operations performed worldwide have yielded promising short-term results, data on long-term outcomes remain scarce. Three full-face transplant recipients were followed for 40 months. Severe changes in volume and composition of the facial allografts were noted. Data from computed tomography performed 6, 18 and 36 months after transplantation were processed to separate allograft from recipient tissues and further into bone, fat and nonfat soft tissues. Skin and muscle biopsies underwent diagnostic evaluation. All three facial allografts sustained significant volume loss (mean 19.55%) between 6 and 36 months after transplant. Bone and nonfat soft tissue volumes decreased significantly over time (17.22% between months 6 and 18 and 25.56% between months 6 and 36, respectively), whereas fat did not. Histological evaluations showed atrophy of muscle fibers. Volumetric and morphometric changes in facial allografts have not been reported previously. The transformation of facial allografts in this study resembled aging through volume loss but differed substantially from regular aging. These findings have implications for risk-benefit assessment, donor selection and measures counteracting muscle and bone atrophy. Superior long-term outcomes of facial allotransplantation will be crucial to advance toward future clinical routine.

Initial experience of dual maintenance immunosuppression with steroid withdrawal in vascular composite tissue allotransplantation.

Current immunosuppression in VCA is largely based on the experience in solid organ transplantation. It remains unclear if steroids can be reduced safely in VCA recipients. We report on five VCA recipients who were weaned off maintenance steroids after a median of 2 months (mean: 4.8 months, range 2-12 months). Patients were kept subsequently on a low dose, dual maintenance consisting of tacrolimus and mycophenolate mofetil/mycophenloic acid with a mean follow-up of 43.6 months (median = 40 months, range 34-64 months). Early and late acute rejections responded well to temporarily augmented maintenance, topical immunosuppression, and/or steroid bolus treatment. One late steroid-resistant acute rejection required treatment with thymoglobulin. All patients have been gradually weaned off steroids subsequent to the treatment of acute rejections. Low levels of tacrolimus (<5 ng/mL) appeared as a risk for acute rejections. Although further experience and a cautious approach are warranted, dual-steroid free maintenance immunosuppression appears feasible in a series of five VCA recipients.

Functional outcomes of face transplantation.

In this study we provide a compilation of functional impairments before and improvements after face transplantation (FT) of five FT recipients of our institution and all FTs reported in current literature. Functional outcome included the ability to smell, breath, eat, speak, grimace and facial sensation. Before FT, all our patients revealed compromised ability to breath, eat, speak, grimace and experience facial sensation. The ability to smell was compromised in two of our five patients. Two patients were dependent on tracheostomy and one on gastrostomy tubes. After FT, all abilities were significantly improved and all patients were independent from artificial air airways and feeding tubes. Including data given in current literature about the other 24 FT recipients in the world, the abilities to smell, eat and feel were enhanced in 100% of cases, while the abilities of breathing, speaking and facial expressions were ameliorated in 93%, 71% and 76% of cases, respectively. All patients that required gastrostomy and 91% of patients depending on tracheostomy were decannulated after FT. Unfortunately, outcomes remain unreported in all other cases and therefore we are unable to comment on improvements.

[Progress in face transplantation]. / Fortschritte in der Gesichtstransplantation.

Vascularised composite allotransplantation (VCA) is utilised for restoration of complex defects. In this context, restoration describes the replacement of destroyed tissue by identical anatomic structures. Up to date, over 150 VCAs including 31 face transplantations have been performed worldwide. Face transplantation is a life giving, rather than life saving procedure that is intended to significantly improve the patient's quality of life. Safe revascularisation as well as aesthetic and functional reintegration are the ultimate goals of face transplantation. The necessary lifelong immunosuppression with potentially life-threatening side effects imposes the need for a very strict risk-benefit ratio assessment and currently limits the indications of face transplantation. Different transplant centres use different protocols for induction and maintenance immunosuppression. Skin is the most immunogenic part of the vascularised composite allograft and has been the focus of intensive research efforts in order to replicate the success of immunosuppressive regimens for solid organ transplantation. Organ preservation during transfer from donor to recipient is another important field of research within VCA. The general public's originally rejecting attitude towards non-lifesaving VCA procedures has changed towards a general acceptance following the publication of promising results after the first cases of face transplantation. Further improvements of surgical techniques and immunosuppressive strategies will be important to drive these young and exciting procedures forward in the future.

[Recent update on tumour biology and treatment of Merkel Cell carcinoma]. / Aktuelles Update zur Tumorbiologie und Behandlung des Merkelzell-Karzinoms.

Merkel cell carcinoma (MCC) is a carcinoma arising from Merkel cells located in the basal layer of the epidermis. The skin of the head and neck is a common site for MCC, occurring generally in fair-skinned elderly patients. MCC is considered as the most lethal skin cancer. Radical surgical excision with pathological verification of complete removal of the tumour is the recommended treatment. Early MCC can be cured by surgery with or without postoperative radiation therapy, whereas advanced MCC is currently considered to be incurable. In the year 2008, a new polyoma virus was found in the tumour genome of the MCC tumours. MCPyV (Merkel cell polyoma virus) appears to be the first example of a human oncogenic polyoma virus. Specific mutations in the viral genome and its clonal integration to the tumour genome are strong evidence against MCPyV as being a passenger virus that secondarily infects MCC tumours. The purpose of this review article is to shed light on this rare skin cancer and introduce the latest advances in research on MCC.