A rare case of intervertebral disc calcification combined with ossification of the posterior longitudinal ligament in a child: a case report and literature review.

BACKGROUND: Intervertebral disc calcification (IDC) combined with calcification in children has been sporadically reported, while ossification of the posterior longitudinal ligament (OPLL) in the cervical spine in pediatric patients is exceedingly rare. The aim of this study is to investigate the potential prognosis and outcomes associated with this condition. CASE PRESENTATION: We present an unusual case involving a 10-year-old Chinese child diagnosed with calcified cervical disc herniation and ossification of the posterior longitudinal ligament. Conservative treatment measures were implemented, and at the 1-month and 6-month follow-up, the patient's pain exhibited significant improvement. Subsequent cervical MRI and CT scans revealed the complete disappearance of OPLL and substantial absorption of the calcified disc. During the three-month follow-up, CT demonstrated slight residual disc calcification, however, the patient remained asymptomatic with no discernible limitation in cervical motion. CONCLUSIONS: We conducted a comprehensive review of several cases presenting with the same diagnosis. It is noteworthy that IDC combined with OPLL in children constitutes a rare clinical entity. Despite imaging indications of potential spinal canal occupation, the majority of such cases demonstrate complete absorption following conservative treatment, with OPLL exhibiting a faster absorption rate than calcified discs.

Orthostatic hypotension as an unusual presentation of spinal calcium pyrophosphate deposition disease: case report and review of literature.

Calcium pyrophosphate crystal deposition disease (CPPD), also known as pseudogout, with spinal involvement, is associated with clinical manifestations of acute nerve compression or chronic spinal stenosis. Precipitation of crystals of calcium pyrophosphate dihydrate in connective tissues can lead to acute inflammatory arthritis, degenerative chronic arthropathies, and radiographic evidence of cartilage calcification. We present a case of an 87-year-old woman, with unstudied chronic polyarthralgia and symptomatic orthostatic hypotension. It were documented acute calcium pyrophosphate deposition wrist arthritis, and cervical CT and MRI was suggestive of spinal involvement of CPPD. Workup excluded other causes of OH. Surgical approach could be indicated to minimize the symptoms, but it was contra-indicated due to the patient's performance status, so histological diagnosis was not possible. Muscle atrophy played an important part in the rapid progression of this insidious chronic disease. Conservative and symptomatic treatment achieve scarce short-term clinical improvement. Spinal involvement of CPPD was thought to be rare but recent studies show a higher prevalence than expected. We call for attention to the extent of structural changes that may occur when not early diagnosed nor treated. High clinical suspicion is required and this is, to our knowledge, the first report of orthostatic hypotension as a presentation of CPPD.

Lateral meniscus with tears or with histologic calcification does not increase the risk of lateral osteoarthritis after medial unicompartmental arthroplasty.

PURPOSE: Meniscal tears or histological meniscal calcifications (in the absence of radiological chondrocalcinosis) are frequent in osteoarthritis. Whether lateral meniscal lesions influence clinical outcomes after medial unicompartmental knee arthroplasty (UKA) is unknown. METHODS: We analyzed 130 patients (130 knees) with medial unicompartmental knee arthroplasties between 2005 and 2015. These 130 knees had full articular cartilage thickness in the lateral compartment and no radiological chondrocalcinosis on preoperative radiographs. The lateral meniscus was analyzed with preoperative MRI and a biopsy of the anterior horn at the time of surgery. Synovial fluid was collected and analyzed for calcium pyrophosphate dihydrate crystal deposition (CPPD crystals). Lateral meniscal tears were untreated when detected on MRI or during surgery, with the hypothesis that these tears on the opposite compartment would remain asymptomatic in medial UKA. At average 10-year follow-up, patients were evaluated with clinical and radiographic outcome, with a focus on the risk of joint space narrowing of the lateral femorotibial compartment. RESULTS: CPPD crystals were present in the synovial fluid of 70 knees. Lateral meniscal tears were seen on MRI in 34 (49%) normal meniscuses of the 60 knees without CPPD crystals and in six other knees without histological meniscal calcification despite CPPD crystals. Histological calcification was present on 61 lateral meniscuses with 53 meniscal tears. The results showed no significant differences in the clinical outcomes between knees with lateral meniscal tears or lateral meniscal histological chondrocalcinosis or both lesions and those without these conditions. Additionally, radiographic progression of osteoarthritis in the opposite femorotibial compartment of the knee was not more frequent in patients with these meniscal issues. The ten year cumulative survival rates, measured by the need for total knee arthroplasty, were 91% for knees without meniscal lesions and 92% for knees with these lesions. CONCLUSION: On this basis, treatment of meniscal tears of the lateral compartment and routine aspiration of the knee to assess for birefringent crystals in the planning of medial UKA do not appear necessary.

Case report: crowned dens syndrome in a patient with seronegative rheumatoid arthritis.

Chondrocalcinosis (CC) is the one of the most common crystal pyrophosphate disease associated arthritis in the elderly. It has been shown to coexist with seronegative and seropositive rheumatoid arthritis (RA), yet mostly with seronegative RA. Among the localisation of CC, the deposition in the ligaments surrounding the odontoid process may remain asymptomatic for years or may lead to and acute severe symptomatology, which may mimic several clinical illnesses among which meningitis (fever, severe pain, acute phase reactants). This is called the 'crowned dens syndrome (CDS)', which has been reported to represent an important percentage of acute neck pain needing hospital admission in neurosurgery units. In this case, the rapid demonstration of 'crowned dens' through CT scan may allow to avoid lumbar puncture and cerebrospinal fluid examination. The coexistence of RA and CDS is very rare, and rarely reported in the literature, yet it may represent a clinical challenge. We describe here one case that while on therapy with methotrexate (MTX) and naproxen (NPX) had an acute neck pain, and peripheral arthritis flare, that responded well to colchicine given along with MTX and NPX.

C9 immunostaining as a tissue biomarker for periprosthetic joint infection diagnosis.

Background: Culture-negative periprosthetic joint infections (PJI) are often false diagnosed as aseptic implant failure leading to unnecessary revision surgeries due to repeated infections. A marker to increase the security of e PJI diagnosis is therefore of great importance. The aim of this study was to test C9 immunostaining of periprosthetic tissue as a novel tissue-biomarker for a more reliable identification of PJI, as well as potential cross-reactivity. Method: We included 98 patients in this study undergoing septic or aseptic revision surgeries. Standard microbiological diagnosis was performed in all cases for classification of patients. Serum parameters including C-reactive protein (CRP) serum levels and white blood cell (WBC) count were included, and the periprosthetic tissue was immunostained for C9 presence. The amount of C9 tissue staining was evaluated in septic versus aseptic tissue and the amount of C9 staining was correlated with the different pathogens causing the infection. To exclude cross-reactions between C9 immunostaining and other inflammatory joint conditions, we included tissue samples of a separate cohort with rheumatoid arthritis, wear particles and chondrocalcinosis. Results: The microbiological diagnosis detected PJI in 58 patients; the remaining 40 patients were classified as aseptic. Serum CRP values were significantly increased in the PJI cohort. Serum WBC was not different between septic and aseptic cases. We found a significant increase in C9 immunostaining in the PJI periprosthetic tissue. To test the predictive value of C9 as biomarker for PJI we performed a ROC analyses. According to the Youden's criteria C9 is a very good biomarker for PJI detection with a sensitivity of 89% and a specificity of 75% and an AUC of 0.84. We did not observe a correlation of C9 staining with the pathogen causing the PJI. However, we observed a cross reactivity with the inflammatory joint disease like rheumatoid arthritis and different metal wear types. In addition, we did not observe a cross reactivity with chondrocalcinosis. Conclusion: Our study identifies C9 as a potential tissue-biomarker for the identification of PJI using immunohistological staining of tissue biopsies. The use of C9 staining could help to reduce the number of false negative diagnoses of PJI.

20 Years of Radiographic Imaging: Crystalline Deposits Causing Severe Arthropathy and Erosions.

Urate crystal gout arthritis and calcium pyrophosphate deposition disease (CPPD) are crystalline arthropathies seen in middle age to elderly patients, but are also seen in the active duty military population. Flares of either can be identified by acute joint pain, associated swelling, tenderness, and warmth. Definitive diagnosis involves synovial analysis from arthrocentesis. Gout and CPPD are common inflammatory joint diseases. Both arthropathies presenting themselves in the same joint are rather rare. An elderly female with a history of gout presented to the hospital with severe hip pain. She was on urate-lowering therapy at the time, and uric acid levels on admission were not significantly elevated. Radiographic imaging of her hip demonstrated periarticular cartilage calcifications. A review of radiographic imaging over the last 20 years found significant erosive arthropathy in multiple joints and radiographic evidence of chondrocalcinosis, suggesting CPPD. Synovial analysis was not obtained during this admission as the patient declined procedures due to her elderly age. Her condition improved with oral steroids. Few literatures have demonstrated that gout and CPPD are common crystal arthropathies that can occur concomitantly in the same joint. A 20-year review of imaging in an elderly female with known gout arthropathy found that she had radiographic evidence of concomitant CPPD-associated damage to many of her joints. Clinicians should be aware of the different erosive arthropathies, their corresponding imaging findings, evaluation for underlying metabolic disorders if appropriate, and the possibility that they may occur in the same joint. Early prevention can reduce joint destruction later in life.

Acute Calcium Pyrophosphate Crystal Arthritis of the Wrist Elicited by Anti-COVID-19 Vaccination After Carpal Tunnel Release.

BACKGROUND Calcium pyrophosphate dihydrate deposition disease includes a variety of clinical syndromes, including acute calcium pyrophosphate (CPP) crystal arthritis. Most patients with CPP crystal arthritis have a primary/idiopathic form presenting with severe pain, swelling, and stiffness. COVID-19 infection, which originated in China in December 2019, required extraordinary efforts to develop and test new vaccines to halt the pandemic. The Vaxzervria vaccine has shown excellent safety and efficacy in phase 3 trials with a mechanism based on the expression of the SARS-CoV-2 spike protein gene coding for the S-antigen, which stimulates the immune response. CASE REPORT We describe an acute event of crystal arthritis after a carpal tunnel syndrome release followed by administration of the second dose of anti-COVID-19 Vaccine Oxford-AstraZeneca (ChAdOx1 nCoV-19). Medical treatment resulted in full resolution of the symptoms in 2 weeks. CONCLUSIONS Although most episodes of acute arthritis happen spontaneously, certain factors may trigger the acute CPP crystal arthritis such as intercurrent illnesses or surgeries. Although the association between carpal tunnel syndrome and CPP arthritis has been known for over 40 years, surgical release of the carpal ligament has always been associated with full resolution of symptoms. This is the first case report describing an exacerbation after carpal canal release, concomitant with the administration of the vaccine. According to our opinion, the vaccination associated with a prior surgery in the same anatomical site could have synergically triggered the arthritis flare-up, in a predisposed patient, with a mechanism still unknown.

Outcomes and resource utilization in calcium pyrophosphate deposition disease patients who underwent total knee arthroplasty: a cross-sectional analysis.

This cross-sectional study aims to evaluate the predictors, outcomes, and resource utilization of total knee arthroplasty (TKA) in calcium pyrophosphate deposition disease (CPPD) patients. We used the US National Inpatient Sample database to identify CPPD and non-CPPD who underwent TKA from 2006 to 2014. Data collection included patient demographics and comorbidities. Outcomes following TKA included in-hospital mortality, complications, length of hospitalization, hospital charges, and disposition. Among the 5,564,005 patients who have undergone TKA, 11,529 (0.20%) had CPPD, with a median age of 72 years, and 53.7% were females. Compared with non-CPPD, patients with CPPD were more likely to be older (mean 72 vs 66 years; p < 0.001), male, white, and have Medicare insurance. CPPD patients were more likely to have ≥ 2 comorbidities calculated by the Charlson Comorbidity Index and discharge to an inpatient/rehabilitation facility. Regarding inpatient complications, myocardial infarction and knee reoperation were significantly more common in CPPD patients. TKA in CPPD patients was associated with significantly higher odds of increased length of stay (> 3 days) than those without CPPD (OR 1.43, 95% CI 1.37-1.49). There was no significant difference in the in-hospital mortality even after adjusting for possible confounders. CPPD patients who underwent TKA were more likely to have a longer hospital stay and discharge to a non-home setting than non-CPPD. Also, CPPD patients had a higher comorbidity burden and risk for myocardial infarction and reoperation.Key Points• This is the largest study to analyze data of CPPD patients who underwent TKA and compare them with non-CPPD patients, using a large nationwide database.• Compared to non-CPPD patients, TKA in CPPD patients is associated with a greater length of stay and disposition to a nursing/rehabilitation facility.• In-hospital complications such as myocardial infarction and reoperation were more frequently observed in CPPD patients than non-CPPD.• The results of this study should alert healthcare providers to develop strategies in order to improve outcomes of CPPD patients undergoing TKA.

Chondrocalcinosis does not affect functional outcome and prosthesis survival in patients after total or unicompartmental knee arthroplasty: a systematic review.

PURPOSE: There are contentious data about the role calcium pyrophosphate (CPP) crystals and chondrocalcinosis (CC) play in the progression of osteoarthritis (OA), as well as in the outcomes after knee arthroplasty. Hence, the purpose of this systematic review was to analyse the clinical and functional outcome, progression of OA and prosthesis survivorship after unicompartmental knee arthroplasty (UKA) and total knee arthroplasty (TKA) in patients with CC compared to patients without CC. METHODS: A systematic review of the literature in PubMed, Medline, Embase and Web of Science was performed using the "Preferred Reporting Items for Systematic Reviews and Meta-Analysis" (PRISMA) guidelines. Articles which reported the outcome and survival rates of prosthesis after TKA or UKA in patients with CC were included. RESULTS: A total of 3718 patient knees were included in eight selected publications, with a median sample sizes of 234 knees (range 78-1000) and 954 knees (range 408-1500) for publications including UKA and TKA, respectively. At time of surgery, the mean age was 69 years and the prevalence for CC ranged from 12.6 to 36%. Chondrocalcinosis did not significantly influence the functional and clinical outcome, the implant survival as well as the radiologic progression of OA disease after UKA and TKA. CONCLUSION: The presence of CPP crystals in tissue samples, synovial fluid or evidence of calcifications on preoperative radiographs did not significantly influence the postoperative functional and activity scores. It also had no significant influence on prosthesis survival rate, whether it was a UKA or a TKA. This study shows that the impact of a subclinical form of chondrocalcinosis may not be of clinical relevance in the context of arthroplasty. LEVEL OF EVIDENCE: IV.

Polymyalgia Rheumatica in a Patient with Pseudogout and Dementia.

BACKGROUND The differential diagnosis of generalized pain includes reactivity associated with bacterial and viral infections, autoimmune rheumatic disease, and orthopedic diseases. Obtaining a detailed medical history and establishing an accurate diagnosis are difficult in elderly patients with dementia. In addition, the differential diagnosis between polymyalgia rheumatica and pseudogout is often difficult. Thus, in our work, we examined the importance of interviewing the family of an elderly patient with dementia. CASE REPORT We report the case of an 88-year-old woman with dementia and a history of recurrent pseudogout who presented with a 12-day history of fever and generalized pain. Physical examination findings revealed warmth and swelling in the shoulder joints and right knee. Blood tests indicated increased inflammatory marker levels. The primary working impression was oligo-articular pseudogout. Based on family interview, the patient was seen to manifest atypical symptoms, including movement difficulty. Joint ultrasound findings showed inflammation of the left long head of the biceps attachment. Further, right knee arthrocentesis detected no calcium pyrophosphate crystals. After obtaining a detailed medical history from the patient's family and conducting other diagnostic tests, the patient was finally diagnosed with polymyalgia rheumatica, rather than oligo-articular pseudogout, with rapid improvement after undergoing low-dose prednisolone treatment. CONCLUSIONS Family interviews can be helpful for obtaining correct diagnosis in elderly patients with dementia.

Cervical myeloradiculopathy as an initial presentation of pseudogout.

Pseudogout, also known as calcium pyrophosphate deposition disease, is an inflammatory arthropathy that primarily occurs in the peripheral joints, such as the knee or elbow. Spinal pseudogout is uncommon, and neck pain is its most common clinical manifestation. However, cervical myeloradiculopathy as an initial presentation of pseudogout attack of the ligamentum flavum has rarely been reported in the literature. We report a case of a 65-year-old woman who presented with neck pain, bilateral finger numbness, and left-sided upper extremity weakness. Magnetic resonance images showed an epidural mass at the C4-5 level, compressing the spinal cord. Following laminectomy with removal of the calcified mass, the profound neurologic deficits gradually recovered. A pathological examination confirmed the diagnosis of cervical pseudogout.

Cervical myelopathy due to subaxial calcium pyrophosphate dihydrate (CPPD) deposition with simultaneous asymptomatic crowned dens syndrome: two case reports.

BACKGROUND: There are few reports of cervical myelopathy caused by an attack of subaxial calcium pyrophosphate dihydrate (CPPD) deposition. Moreover, there has been no report on cervical myelopathy by subaxial CPPD deposition with simultaneous asymptomatic crowned dens syndrome (CDS) at the same time. CASE PRESENTATION: The first case was a 68-year-old male complaining of cervical myelopathic symptoms. Plain radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) findings revealed spinal cord compression by calcified round lesions at C4 as well as a calcified lesion behind the dens. The second case was a 77-year-old female complaining of cervical myelopathic symptoms. Plain radiographs, CT and MRI findings revealed spinal cord compression by calcified round lesions at C3 and C4 as well as a calcified lesion behind the dens. In both cases, we believed that the calcified lesion behind the dens was an asymptomatic lesion. Therefore, the first patient received decompressive laminectomy of C3 and C4, removal of calcified round lesions, and posterior fixation from C3 to C5 due to associated kyphosis. The second patient underwent decompressive laminectomy of C3 and C4 and removal of calcified round lesions. Microscopic examination under polarized light showed dark blue calcifications with rhomboid crystals that were positively birefringent. The findings were consistent with those of CPPD. CONCLUSIONS: This is the first study to report cervical myelopathy caused by subaxial CPPD deposition with simultaneous asymptomatic CDS. Surgical removal of the subaxial CPPD deposition alone achieved a satisfactory surgical outcome without recurrence.

Whole exome sequencing of patients with diffuse idiopathic skeletal hyperostosis and calcium pyrophosphate crystal chondrocalcinosis.

OBJECTIVES: DISH/CC is a poorly understood phenotype characterised by peripheral and axial enthesopathic calcifications, frequently fulfilling the radiological criteria for Diffuse Idiopathic Skeletal Hyperostosis (DISH, MIM 106400), and in some cases associated with Calcium Pyrophosphate Dihydrate (CPPD) Chondrocalcinosis (CC). The concurrence of DISH and CC suggests a shared pathogenic mechanism. In order to identify genetic variants for susceptibility we performed whole exome sequencing in four patients showing this phenotype. MATERIALS AND METHODS: Exome data were filtered in order to find a variant or a group of variants that could be associated with the DISH/CC phenotype. Variants of interest were subsequently confirmed by Sanger sequencing. Selected variants were screened in a cohort of 65 DISH/CC patients vs 118 controls from Azores. The statistical analysis was performed using PLINK V1.07. RESULTS: We identified 21 genetic variants in 17 genes that were directly or indirectly related to mineralization, several are predicted to have a strong effect at a protein level. Phylogenetic analysis of altered amino acids indicates that these are either highly conserved in vertebrates or conserved in mammals. In case-control analyses, variant rs34473884 in PPP2R2D was significantly associated with the DISH/CC phenotype (p=0.028; OR=1.789, 95% CI= 1.060 - 3.021)). CONCLUSION: The results of the present and preceding studies with the DISH/CC families suggests that the phenotype has a polygenic basis. The PPP2R2D gene could be involved in this phenotype in an as yet unknown way.