BACKGROUND: Free fat graft has been used for the treatment of congenital hand differences. However, there have been a few reports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differences was investigated. METHODS: Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactyly were treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen bones with longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis and free fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly release with free fat graft was performed five times on four hands. RESULTS: In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvement of the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. The mean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomy group and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvement in one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four for severely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in one case. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separated bones. CONCLUSIONS: Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinal epiphyseal bracket and Madelung deformity. Free fat graft is also useful to prevent reunion of the bones after separation of osseous syndcatyly, if the grafted fat is securely filled into the space between the separated bones.
Adipose Tissue/*transplantation ; Adolescent ; Child ; Child, Preschool ; Female ; Fingers/*abnormalities/surgery ; Foot Deformities, Congenital/surgery ; Hallux Varus/surgery ; Hand Deformities, Congenital/*surgery ; Humans ; Male ; Osteotomy ; Statistics, Nonparametric ; Syndactyly/*surgery ; Treatment Outcome

BACKGROUND: Free fat graft has been used for the treatment of congenital hand differences. However, there have been a few reports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differences was investigated. METHODS: Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactyly were treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen bones with longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis and free fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly release with free fat graft was performed five times on four hands. RESULTS: In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvement of the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. The mean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomy group and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvement in one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four for severely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in one case. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separated bones. CONCLUSIONS: Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinal epiphyseal bracket and Madelung deformity. Free fat graft is also useful to prevent reunion of the bones after separation of osseous syndcatyly, if the grafted fat is securely filled into the space between the separated bones.
Adipose Tissue/*transplantation ; Adolescent ; Child ; Child, Preschool ; Female ; Fingers/*abnormalities/surgery ; Foot Deformities, Congenital/surgery ; Hallux Varus/surgery ; Hand Deformities, Congenital/*surgery ; Humans ; Male ; Osteotomy ; Statistics, Nonparametric ; Syndactyly/*surgery ; Treatment Outcome

Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.
Forearm/abnormalities/*surgery ; Hand Deformities, Congenital/*surgery ; Humans ; Joints/*transplantation ; Metatarsophalangeal Joint/surgery ; Radius/abnormalities/*surgery

Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.
Forearm/abnormalities/*surgery ; Hand Deformities, Congenital/*surgery ; Humans ; Joints/*transplantation ; Metatarsophalangeal Joint/surgery ; Radius/abnormalities/*surgery

Pollicization substitutes a functioning finger for a deficient thumb. The most indication is thumb hypoplasia with absence or instability of the carpometacarpal joint. However, there are additional causes that may negate thumb function, such as trauma, macrodactyly, multi-fingered hand, and a mirror hand. The technique of pollicization represents a consolidation of contributions from surgeons over the last 100 years. A meticulous stepwise approach from incision to closure is necessary to optimize outcome. Following pollicization, cortical plasticity and motor relearning play a pivotal role in function following pollicization with connections and adjacent sprouting from nearby cortical and/or subcortical territories. Occupational therapy is necessary to encourage large object acquisition followed by smaller objects and ultimately fine pinch. Pollicization is more reliable in patients with isolated thumb hypoplasia and a mobile index finger with robust extrinsic and intrinsic muscle-tendon units compared to and patients with radial forearm deficiencies and diminished index mobility.
Fingers/abnormalities/*surgery ; Hand Deformities, Congenital/rehabilitation/*surgery ; Humans ; Orthopedic Procedures/*methods/rehabilitation ; Reconstructive Surgical Procedures/*methods/rehabilitation ; Thumb/abnormalities/*surgery

Pollicization substitutes a functioning finger for a deficient thumb. The most indication is thumb hypoplasia with absence or instability of the carpometacarpal joint. However, there are additional causes that may negate thumb function, such as trauma, macrodactyly, multi-fingered hand, and a mirror hand. The technique of pollicization represents a consolidation of contributions from surgeons over the last 100 years. A meticulous stepwise approach from incision to closure is necessary to optimize outcome. Following pollicization, cortical plasticity and motor relearning play a pivotal role in function following pollicization with connections and adjacent sprouting from nearby cortical and/or subcortical territories. Occupational therapy is necessary to encourage large object acquisition followed by smaller objects and ultimately fine pinch. Pollicization is more reliable in patients with isolated thumb hypoplasia and a mobile index finger with robust extrinsic and intrinsic muscle-tendon units compared to and patients with radial forearm deficiencies and diminished index mobility.
Fingers/abnormalities/*surgery ; Hand Deformities, Congenital/rehabilitation/*surgery ; Humans ; Orthopedic Procedures/*methods/rehabilitation ; Reconstructive Surgical Procedures/*methods/rehabilitation ; Thumb/abnormalities/*surgery

Within the Oberg, Manske, Tonkin (OMT) classification, thumb duplications are a failure of formation and/or differentiation affecting the radial-ulnar axis of the hand plate. The Wassel description of seven types of thumb duplication provides a good structure from which an approach to management is based. The aim of surgical reconstruction is to obtain a stable, mobile thumb of adequate size and appropriate shape. The most common form of reconstruction is removal of the lesser digit and reconstruction of the dominant digit. Surgical techniques address the problems of deviation, instability and lack of size. The disadvantages of the Bilhaut-Cloquet procedure, these being joint stiffness and a nail ridge, may be lesser concerns when reconstruction of one digit will not create a satisfactory thumb of adequate mobility, stability, alignment and size. Complicated problems of triphalangism, triplication, ulnar dimelia and the rare circumstance in which neither of the duplicated thumbs may be adequately reconstructed present specific challenges which demand alternative techniques.
Hand Deformities, Congenital/classification/*surgery ; Humans ; Orthopedic Procedures/*methods ; Patient Care Planning ; *Reconstructive Surgical Procedures ; Thumb/*abnormalities/*surgery

Within the Oberg, Manske, Tonkin (OMT) classification, thumb duplications are a failure of formation and/or differentiation affecting the radial-ulnar axis of the hand plate. The Wassel description of seven types of thumb duplication provides a good structure from which an approach to management is based. The aim of surgical reconstruction is to obtain a stable, mobile thumb of adequate size and appropriate shape. The most common form of reconstruction is removal of the lesser digit and reconstruction of the dominant digit. Surgical techniques address the problems of deviation, instability and lack of size. The disadvantages of the Bilhaut-Cloquet procedure, these being joint stiffness and a nail ridge, may be lesser concerns when reconstruction of one digit will not create a satisfactory thumb of adequate mobility, stability, alignment and size. Complicated problems of triphalangism, triplication, ulnar dimelia and the rare circumstance in which neither of the duplicated thumbs may be adequately reconstructed present specific challenges which demand alternative techniques.
Hand Deformities, Congenital/classification/*surgery ; Humans ; Orthopedic Procedures/*methods ; Patient Care Planning ; *Reconstructive Surgical Procedures ; Thumb/*abnormalities/*surgery

For hand surgeons, the treatment of children with congenital differences of the upper extremity is challenging because of the diverse spectrum of conditions encountered, but the task is also rewarding because it provides surgeons with the opportunity to impact a child's growth and development. An ideal classification of congenital differences of the upper extremity would reflect the full spectrum of morphologic abnormalities and encompass etiology, a guide to treatment, and provide prognoses. In this report, I review current classification systems and discuss their contradictions and limitations. In addition, I present a modified classification system and provide treatment principles. As our understanding of the etiology of congenital differences of the upper extremity increases and as experience of treating difficult cases accumulates, even an ideal classification system and optimal treatment strategies will undoubtedly continue to evolve.
Hand Deformities, Congenital/*classification/pathology/*surgery ; Humans

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult