18-month outcomes of heterologous bilateral hand transplantation in a child: a case report.

BACKGROUND: Although heterologous vascular composite allotransplantation has become a burgeoning treatment option for adult amputees, there have been no successful cases previously reported in children. Here, we describe the surgical, immunological, and neurorehabilitation details with functional outcomes 18 months after heterologous bilateral hand and forearm transplantation in an 8-year-old child with quadrimembral amputations and a previous kidney transplant. METHODS: 2 years of extensive preparation by medical and surgical teams preceded the hand-forearm transplantation of this child. The initial immunosuppressive protocol included thymoglobulin, tacrolimus, prednisone, and mycophenolate mofetil. In July, 2015, our vascularised composite allotransplantation team did the first bilateral hand and forearm transplantation in a child, an 8-year-old boy with previous living-related kidney transplantation. The surgery included four teams working simultaneously on the donor and recipient limbs, aided by customised cutting guides that aimed to reduce ischaemia time. Following an extended length of time in hospital, skin biopsies and close monitoring of renal function and drug concentrations occurred weekly for the first 3 months and were slowly tapered to monthly, and then quarterly. Skin biopsies were also done when tissue rejection was suspected. Paediatric-specific rehabilitation techniques were applied to promote patient engagement during rehabilitation. Progress was assessed by monthly sensory and motor function tests during routine clinic visits and with serial functional brain imaging studies, including structural brain MRI, magnetoencephalography and transcranial magnetic stimulation. FINDINGS: The surgery lasted 10 h and 40 min. Vascular revision of the ulnar artery was required a few hours postoperatively. There were no further immediate postsurgical complications. Rejection episodes occurred throughout the first year but were reversed. An increase in serum creatinine led to the addition of sirolimus at 3 months after transplantation with concomitant reduction in tacrolimus targets. Sensibility to light touch was present by 6 months after transplantation. Intrinsic hand muscle innervation was present by 7-10 months after transplantation. At 18 months, the child had exceeded his previous adapted abilities. As of 18 months after transplantation surgery he is able to write and feed, toilet, and dress himself more independently and efficiently than he could do before transplantation. He remains on four immunosuppressive medications and functional neuroimaging studies have shown motor and somatosensory cortical reorganisation. INTERPRETATION: Hand transplantation in a child can be surgically, medically, and functionally successful under carefully considered circumstances. Long-term data on the functional trajectory, neurological recovery, psychological sequelae, and the potential late effect of immunosuppression are still needed to support broader implementation of paediatric vascular composite allotransplantation. FUNDING: The Children's Hospital of Philadelphia.

Limb deficiency and prosthetic management. 1. Decision making in prosthetic prescription and management.

UNLABELLED: This self-directed learning module highlights decision making in prosthetic management in pediatric and adult patients. This chapter reviews classification of congenital limb deficiency and management of congenital pediatric upper-extremity amputees. Differences in management of amputees of various ages from infancy to old age is discussed. Case examples are used to formulate prosthetic prescriptions for transradial limb deficiency as well as knee disarticulation and transtibial level amputations. Common prosthetic gait deviations and anticipated functional levels are evaluated. OVERALL ARTICLE OBJECTIVE: To discuss prosthetic prescription and management in congenital limb deficiency and acquired amputations for patients of various ages.

Limb deficiency and prosthetic management. 2. Aging with limb loss.

UNLABELLED: This self-directed learning module highlights the issues faced by people aging with limb loss. It is part of the study guide on limb deficiency and vascular rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on the impact that limb loss has on health and physical function throughout the life span. Case examples are used to illustrate what effect limb loss in childhood or young adulthood has on the incidence and management of new impairments or disease processes commonly associated with aging. OVERALL ARTICLE OBJECTIVE: To discuss the impact of early-life limb loss on the incidence and management of physiologic and functional changes associated with aging.

Limb deficiency and prosthetic management. 3. Complex limb deficiency.

UNLABELLED: This self-directed learning module highlights rehabilitation and prosthetic issues associated with complex limb deficiencies. It is part of the chapter on acquired limb deficiencies in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article discusses rehabilitation and prosthetic management of patients with amputations for complex limb deficiencies secondary to trauma. Mechanisms of injury, prosthetic issues, prosthetic components, and potential problems in prosthetic fitting will be discussed. OVERALL ARTICLE OBJECTIVE: To evaluate common problems associated with complex limb deficiency.

Advances in amputee care.

UNLABELLED: This self-directed learning module highlights the recent innovations in amputee care. It is part of the study guide on limb deficiency in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This focused review article describes the key elements of a successful comprehensive amputee program, the basic surgical considerations for upper- and lower-extremity amputation, and some of the more recent advances in prosthetic components. Further, an update is given on issues such as hand transplantation and the integration of robotics and artificial muscles for people with limb loss. OVERALL ARTICLE OBJECTIVE: (a) To discuss current issues and advances in the care of patients with amputation, (b) to describe the key elements in designing a comprehensive amputee care program, and (c) to discuss surgical considerations of limb preservation and amputation levels.

Limb deficiency and prosthetic management. 4. Comorbidities associated with limb loss.

UNLABELLED: This self-directed learning module highlights common comorbidities found in people with amputations and their impact on functional outcome. It is part of the study guide on limb deficiency and vascular rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article focuses on prosthetic considerations, functional outcome, and potential complications for a woman with the comorbidities of stroke and diabetes who experiences a dysvascular amputation. Formulation of the differential diagnosis, management of limb pain, and evaluation of the potential psychosocial issues arising after amputation are also discussed. OVERALL ARTICLE OBJECTIVE: To analyze common comorbidities of people with amputations and to delineate their impact on functional outcome.

Stroke and neurodegenerative disorders. 1. Acute stroke evaluation, management, risks, prevention, and prognosis.

UNLABELLED: This self-directed learning module highlights recent developments in the acute care of stroke patients, prediction of outcome after stroke, evaluation of risk factors, secondary prevention of stroke, and the evaluation of the young adult with stroke. It is part of the study guide on stroke and neurodegenerative disorders in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article contains sections on the acute evaluation and management of the stroke patient, prediction of functional outcome after stroke, and secondary prevention of stroke. Special emphasis is given to the evaluation of the young adult with stroke. OVERALL ARTICLE OBJECTIVES: (a) To summarize the acute evaluation and management of stroke, particularly in the young stroke patient; and (b) to review the risk factors for stroke and secondary prevention measures.

Stroke and neurodegenerative disorders. 2. Stroke: comorbidities and complications.

UNLABELLED: This self-directed learning module highlights diagnosis and treatment of comorbidities and complications encountered by patients with stroke. It is part of the study guide on stroke and neurodegenerative disorders in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on cardiopulmonary complications and examines neurologic sequelae, risk factors for falls, and prevention strategies. It also discusses upper-limb pain, fatigue, and depression and highlights diagnosis and management of genitourinary complications. OVERALL ARTICLE OBJECTIVE: To summarize common comorbidities and complications encountered by patients after stroke.

Stroke and neurodegenerative disorders. 3. Stroke: rehabilitation management.

UNLABELLED: This self-directed learning module highlights common rehabilitation issues in stroke survivors. Topics include spasticity, constraint-induced movement therapy, partial body weight-supported treadmill training, virtual reality training, vestibular retraining, aphasia treatment, and cognitive retraining. It is part of the study chapter on stroke and neurodegenerative disorders in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. OVERALL ARTICLE OBJECTIVES: (a) To identify and review the treatment options for poststroke spasticity; (b) to review the use of body weight-supported treadmill training in stroke patients; (c) to describe virtual reality training as an adjunct in stroke rehabilitation; (d) to review vestibular rehabilitation; (e) to discuss advances in aphasia treatment; (f) to discuss cognitive retraining; and (g) to provide an update on treatment of neglect syndromes.

Stroke and neurodegenerative disorders. 4. Neurodegenerative disorders.

UNLABELLED: This self-directed learning module highlights diagnosis, treatment, and rehabilitation issues in patients with neurodegenerative disorders, including multiple sclerosis (MS), Parkinson's disease, and amyotrophic lateral sclerosis (ALS). It is part of the study guide on stroke and neurodegenerative disorders in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on the differential diagnosis, diagnostic evaluation, medical management, and rehabilitation issues in MS. Similarly, the differential diagnosis treatment and rehabilitation in Parkinson's disease is discussed. Electrodiagnosis, pharmacologic treatment, and rehabilitation options for ALS are also discussed. OVERALL ARTICLE OBJECTIVES: To review the differential diagnosis, evaluation, medical treatment, and rehabilitation management of patients with MS, Parkinson's disease, and ALS.

Rehabilitation of movement disorders.

UNLABELLED: This self-directed learning module highlights several movement disorders. These include dystonia, chorea, tremors, and myoclonus. A description of the clinical presentation and associated disease processes is presented. Although the discussion on treatment focuses on pharmacologic intervention, surgical options are presented when appropriate. Other movement disorders (ie, parkinsonism) are discussed elsewhere in the Study Guide. OVERALL ARTICLE OBJECTIVES: (a) To define the various symptoms and etiologies of dystonia; (b) to define chorea and its treatment; (c) to define tremors, including associated neurologic disorders, plus pharmacologic and potential surgical interventions; and (d) to describe the symptoms, classification, and treatment of primary and secondary myoclonus.

The rehabilitation of causalgia (complex regional pain syndrome-type II).

CRPS-type I or causalgia is a challenging pain syndrome and its pathogenesis remains controversial. Although its incidence is relatively low, the pain and suffering it causes can be severe and functionally debilitating. Early, accurate diagnosis permits initiation of appropriate therapeutic interventions and enhances the potential for successful treatment.