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Total Knee Arthroplasty in a Patient With Neurofibromatosis 1.

ABSTRACT: Neurofibromatosis 1 (NF1) is a rare genetic syndrome that leads to the development of neurofibromas and increases the risk of malignancy, including malignant peripheral nerve sheath tumors. Patients with NF1 often have other orthopaedic manifestations, including short stature, osteopenia, and dysplasia. A 47-year-old patient with a history of NF1 and multiple neurofibromas of the right lower extremity presented with a severe valgus deformity, instability, and osteoarthritis of the right knee that was debilitating to daily life. Over time, the patient lost proprioception and potentially some sensation to the right knee with neurofibroma formation, leading to the development of Charcot arthropathy of the right knee with secondary osteoarthritis. The preoperative workup consisted of a magnetic resonance imaging of the knee to confirm no malignancy was present and templating to ensure the standard implant size was amenable for the patient. A primary total knee arthroplasty was performed with a cemented-stemmed hinged knee implant. At 6 months post-surgery, the patient had a dramatic improvement in her pain and quality of life.

SUBMITTER: Hodo T

PROVIDER: S-EPMC11342787 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature

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Total Knee Arthroplasty in a Patient With Neurofibromatosis 1.

Hodo Thomas T Sherman William W Sanchez Santiago S Anudu Edmund E Sanchez Fernando F

Arthroplasty today 20240803

Neurofibromatosis 1 (NF1) is a rare genetic syndrome that leads to the development of neurofibromas and increases the risk of malignancy, including malignant peripheral nerve sheath tumors. Patients with NF1 often have other orthopaedic manifestations, including short stature, osteopenia, and dysplasia. A 47-year-old patient with a history of NF1 and multiple neurofibromas of the right lower extremity presented with a severe valgus deformity, instability, and osteoarthritis of the right knee tha ...[more]

PMID: 39185400

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Project description:BackgroundThere is an existing perception that obesity has a negative impact on complications following total knee arthroplasty (TKA). However, data on the impact of obesity levels on patient-reported outcomes (PROMs) is sparse. We investigated the association between different obesity classes with PROMs among patients who underwent TKA.MethodsWe performed retrospective secondary analyses on data extracted from the total joint replacement data repository (Alberta, Canada) managed by the Alberta Bone and Joint Health Institute (ABJHI). Patients had WOMAC and EQ5D scores measured at baseline in addition to 3 and/or 12 months following TKA. Patients were stratified according to the World Health Organization (WHO) classification, into five body mass index (BMI) groups of normal, overweight, BMI class I, BMI class II, and BMI class III. The association between BMI and mean changes in WOMAC subscales (pain, function, and stiffness) and EQ-5D-5L index over the time intervals of baseline to 3 months and 3 to 12 months following TKA was assessed. Linear mixed-effects models were used, and the models were adjusted for age, sex, length of surgery, comorbidities, year of surgery, and geographical zone where the surgery was performed.ResultsMean age was 65.5 years (SD = 8.7). Postoperatively, there was a significant improvement (p < 0.001) in WOMAC subscales of patient-reported pain, function, and stiffness, as well as EQ-5D-5L regardless of BMI group. Although, patients in BMI class II and class III reported significantly improved pain 3 months after TKA compared to those with normal BMI, all BMI groups attained similar level of pain reduction at 12 months after TKA. The greatest improvement in all WOMAC subscales, as well as EQ5D index, occurred between baseline and 3 months (adjusted p < 0.0001).ConclusionThe findings indicate that patients reported improved pain, function, and stiffness across all BMI groups following TKA. Patients with BMI classified as obese reported similar benefits to those with BMI classified as normal weight. These results may help health care providers to discuss expectations regarding the TKA recovery in terms of pain, function, and quality of life improvements with their TKA candidates.

Dataset Information

Total Knee Arthroplasty in a Patient With Neurofibromatosis 1.

Publications

Total Knee Arthroplasty in a Patient With Neurofibromatosis 1.

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