Arthroplasty of a Charcot Knee in a Patient With Congenital Insensitivity to Pain

Abstract

Hereditary sensory and autonomic neuropathies (HSANs) include hereditary disorders that cause congenital insensitivity to pain. Moreover, patients diagnosed with such disorders are known to have genetic mutations that alter their deep pain sensation, making them more prone to developing bone and joint complications such as repetitive fractures, joint swelling, and Charcot arthropathy. Neuropathic arthropathy (Charcot joint) is a rare and relatively poorly understood condition; it is suggested to be caused by autonomic dysfunction and repetitive microtrauma and characterized by instability and joint destruction. Diagnosing the idiopathic Charcot joint is challenging and is considered to be a diagnosis of exclusion. In addition, there are limited cases of Charcot knees managed by arthroplasty. Patients with Charcot knees are commonly characterized by profound bone loss, diffuse synovitis, and instability in the knee joint. In this article, we report the case of a 13-year-old patient with known NTRK1 gene mutation who presented with recurrent knee joint swelling episodes and instability without pain. She was diagnosed with Charcot knee joint and underwent right hinged total knee replacement. At one-year follow-up, she continued to have good knee stability and an overall functional gait. Our findings suggest that managing Charcot knee joint with total knee replacement in patients with HSAN may show improvement in terms of stability, swelling, and overall gait.

Charcot neuroarthropathy due to Congenital Insensitivity to Pain With Anhidrosis: A case report and literature review

“…Four cases were treated surgically, two of which were treated by osteotomy and orthopedic approach 10,13 , one by lesion debridement 11 , and one by total knee arthroplasty. 12 In a short period of time, all of the above treatments showed limb function was signi cantly improved in all patients. However, at follow-up, the overall total postoperative complication rate requiring reoperation in these reported cases was 66.7% (Two cases were not reported with complications).…”

“…Although the surgical treatment of CN combined with CIPA remains controversial, several previous studies have reported on the e cacy of other surgical modalities in speci c cases. [10][11][12][13] As shown in Table 1, these reports describe various treatment procedures in patients with varying severity of CIPA with joint involvement in CN and differing outcomes. Four cases were treated surgically, two of which were treated by osteotomy and orthopedic approach 10,13 , one by lesion debridement 11 , and one by total knee arthroplasty.…”

Constrained Condylar Prostheses for the Treatment of Charcot Arthropathy: A Case Report and Literature Review

“…Rotating hinge prosthesis could provide better three‐dimensional freedom, and has been more frequently chosen for TKA to treat Charcot knee in the last 10 years (Figure 2 ). 20 , 21 Recently, more studies 5 , 6 , 9 , 10 , 22 , 23 , 24 , 25 , 26 , 27 , 28 reported the use of condylar constrained prostheses and rotating hinge prostheses with satisfied results for TKA in Charcot knee.…”

Bilateral Total Knee Arthroplasties in a Patient with Bilateral Below-Knee Amputations and Osseointegration Limb Replacements

“…We present an unusual case of a patient with congenital pain insensitivity. Congenital pain insensitivity is a group of hereditary sensory and autonomic neuropathies [18][19][20] known to result in multiple musculoskeletal complications including multiple fractures, joint subluxation, and Charcot arthropathy [21][22][23][24] . As a result of his pain insensitivity, he required bilateral below-knee amputations and subsequently underwent bilateral osseointegration limb reconstructions.…”