Supplemental Digital Content is available in the text.Summary:Early-onset carpal tunnel syndrome (CTS) is a well-known manifestation of mucopolysaccharidoses (MPS) due to excessive deposition of glycosaminoglycans in soft tissues. Standard treatment has been carpal tunnel release surgery, with the conventional technique of dividing the transverse carpal ligament. With advancement of treatments for MPS, these patients now have a longer life expectancy and are presenting with recurrent CTS. Management of recurrent CTS in these patients is not well studied. Here, we report 2 cases of recurrent CTS in MPS patients after a carpal tunnel release operation. We describe the findings on repeat operations and propose a unique technique for treating CTS in MPS patients to minimize recurrence during the initial CTS surgery. Our method involves resection of a portion of the transverse carpal ligament and use of a hypothenar fat pad flap over the median nerve.

BACKGROUND: Neurofibromatosis type 1 is a hereditary disease that can lead to pseudoarthrosis of various long bones. Rarely, pseudoarthrosis affects the forearm, and to the best of our knowledge, this is the first reported case of pseudarthrosis of the distal radius treated by this unique method. METHODS: We present a case of a 7-year-old male who underwent multiple operations to treat pseudoarthrosis of the distal radius. RESULTS: After failed conservative and operative fixation augmented with autologous iliac crest bone grafting, the patient underwent a successful double-barrel vascularized free fibula graft while preserving the physis of the distal radius. CONCLUSION: Double-barrel vascularized free fibula bone graft can be successfully used as the definitive treatment in refractory cases of distal forearm pseudarthrosis and we believe that our technique can be applied to all cases of pseudarthrosis in other patients with a similar presentation and lesion location.

Background

Neurofibromatosis type 1 is a hereditary disease that can lead to pseudoarthrosis of various long bones. Rarely, pseudoarthrosis affects the forearm, and to the best of our knowledge, this is the first reported case of pseudarthrosis of the distal radius treated by this unique method.

Methods

We present a case of a 7-year-old male who underwent multiple operations to treat pseudoarthrosis of the distal radius.

Results

After failed conservative and operative fixation augmented with autologous iliac crest bone grafting, the patient underwent a successful double-barrel vascularized free fibula graft while preserving the physis of the distal radius.

Conclusion

Double-barrel vascularized free fibula bone graft can be successfully used as the definitive treatment in refractory cases of distal forearm pseudarthrosis and we believe that our technique can be applied to all cases of pseudarthrosis in other patients with a similar presentation and lesion location.