Enthesitis Related Arthritis: A Single Center Experience

Objective: Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis category, characterized by enthesitis, arthritis, and the risk of axial involvement. We aimed to summarize the demographics, clinical, and laboratory findings of enthesitis-related arthritis patients and to identify the distinguishing features of enthesitis-related arthritis patients with HLA B27 positive compared to the patients who were HLA B27 negative. Materials and Methods: This retrospective study included patients with Enthesitis-related arthritis who followed up between 2015 and 2018. Demographical, clinical, and laboratory data were retrospectively reviewed from the patient files and computerized medical charts. Results: A total of 72 patients diagnosed with enthesitis-related arthritis were included in the study. The male/female ratio was 2.1/1. Fifty-three (73%) of them presented with peripheral arthritis. The most commonly affected joint was knee (81.1%), followed by the ankle (43%), hips (32%), and wrist (5%). HLA B27 was positive in 36 (50%) patients. During follow-up, the number of patients who developed enthesitis-related arthritis -associated uveitis was 8 (11.1%). During follow-up, 56 patients with inflammatory back pain and/or sacroiliac tenderness underwent spinal MRI. Ten (17.8%) patients had only thoracal and/or lumbar involvement, 18 (32%) had only sacroiliitis, and 9 (16%) patients had both of them on spinal MRI. In comparison with HLA-B27-negative children, HLA-B27-positive patients were more likely to have enthesitis (16 (44.4%) vs 8 (22.2%), p=0.046), MRI proven sacroiliitis (19 (52.7%) vs 8 (22.2%), p=0.031), MRI proven spinal involvement (13 (36.1%) vs 6 (16.6%), p=0.031), and uveitis (8 (100%) vs 0(0%), p=0.014). During follow up, 65/72 (90.2 %) of them needed disease-modifying antirheumatic drugs (DMARD), and 51/72 (70.8%) needed anti-tumor necrosis factor-α (TNF-α) therapy. Conclusion: We found that patients who were HLA-B27- positive had significantly more enthesitis, MRI-proven sacroiliitis, MRI-proven spinal involvement, and acute anterior uveitis, in comparison to patients who were HLA B27 negative. It is crucial to carefully assess those patients with concern for enthesitis-related arthritis to determine the expected prognosis and make therapeutic decisions appropriately.

Contiguous Three-Level Vertebral Collapse in Thoracic Spine: A Novel Presentation of Chronic Recurrent Multifocal Osteomyelitis in 12 years old and Review of Literature

Introduction: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoimmune disorder of childhood and adolescence which often manifests as recurring episodes of inflammatory bone pains. Spinal involvement is rare; however, recent studies advocate full body magnetic resonance imaging in all suspected cases to pick up asymptomatic lesions early to prevent complications. Spinal involvement may manifest as fractures, scoliosis, or kyphotic deformity. Case Report: We present a case of a 12-year-old boy who had three-level involvement of thoracic spine, T6-T8, and was worked up and managed for pathological fracture of spine. He underwent biopsy for the same and was later diagnosed as CRMO. Here, we discuss the diagnostic challenges involved in CRMO, need for biopsy, and the management options available. Conclusions: Identifying CRMO is challenging and remains a diagnosis of exclusion. Nonsteroidal anti-inflammatory drugs often constitute the first line of treatment and other drugs such as bisphosphonates and biologics such as TNF-alpha antagonists are reserved for more severe cases. Although CRMO is considered a benign disease, recent data suggest up to 50% rate of residual impairments despite optimal management. Keywords: Chronic recurrent multifocal osteomyelitis, spine, contiguous, child.

Cryptococcal meningitis presenting as anterior spinal cord syndrome with accessory nerve palsy in immunocompetent patient: A case report

Background: Cryptococcus has a tropism for the nervous system with a higher prevalence of infection in immunosuppressed patients; it remains a major cause of human immunodeficiency virus (HIV)-related mortality worldwide. Neurological compromise caused by this microorganism mainly debuts as a meningeal syndrome, spinal involvement has been reported in literature, neuropathological assessments have found Cryptococci in spinal roots and meninges, with perineuritic adhesions probably explaining compromise lower cranial nerves and even spinal nerve roots. Case Description: 39-year-old male seronegative for HIV, with a surgical history of hydrocephalus treated with ventriculoperitoneal shut 1 year before, he presented with progressive weakness in the four extremities evolving to be disabling with bilateral accessory nerve palsy and loss of sensation below his neck. The MR imaging showed diffuse leptomeningeal thickening both supra and infratentorial and over the spinal canal up to C5 with a cystic formation shown in the craniocervical union causing compression of the medullary bulb. The patient underwent a medial suboccipital craniectomy with resection of the posterior arch of c1 for sampling and decompression, pathologically appears numerous spherical organisms that have a thick clear capsule and are surrounded by histiocytes forming a granuloma compatible with Cryptococcus. Postoperatively, the patient’s prior neurological deficits resolved. Conclusion: It is an infrequently suspected pathology in immunocompetent patients, usually requiring only antifungal treatment with adjustment of immunosuppressive or antiretroviral management. In special and rare situations like our case as presenting with lower cranial nerve and spinal involvement, surgical treatment is a priority for the resolution of the pathology and improves disabling neurological deficit.

Case Report: Kaposiform Hemangioendothelioma With Spinal Involvement

Introduction: Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor that mostly appears in infants and adolescents. KHE with spinal involvement is extremely rare. The aim of this study was to review the imaging features, clinical manifestations and treatment of KHE patients with spinal involvement.Materials and Methods: We reviewed patients with KHE who were admitted to Pediatric Surgery of West China Hospital of Sichuan University from April 2014 to August 2020, and the cases were evaluated.Results: Seven patients with spinal involvement were enrolled in the study, including four (57.1%) males and three (42.9%) females. The age at onset ranged from 1.0 day to 4.0 years, with an average of 1.6 years. Five (71.4%) had pain due to bone destruction, three patients (42.9%) had decreased range of motion (ROM), four (57.1%) patients had scoliosis, two (28.6%) patients developed claudication, and three patients (42.9%) presented with a soft tissue mass in the neck of the back. Five patients (71.4%) had the Kasabach-Merritt phenomenon (KMP), with a minimum platelet value of 8 × 109/L. All patients were treated with sirolimus, and showed regression of the lesion and/or normalization of the hematologic parameters.Conclusion: KHE with spinal involvement is difficult to diagnose due to its rarity and variable symptoms, which need to be recognized to start early treatment. The management of KHE with spinal involvement should be performed by a multidisciplinary team. Sirolimus can improve outcomes in patients with KHE with spinal involvement.