Radiographs and MRI of the Cervical Spine in Juvenile Idiopathic Arthritis: A Cross-Sectional Retrospective Study

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease among children. In some patients, cervical spine arthritis remains a serious and chronic manifestation of JIA. The aim of this study was to assess the frequency of cervical spine lesions on radiographs and MRI in JIA patients with clinical signs of cervical spine involvement and to verify if with the addition of MRI, the use of radiographs could be abandoned. Methods: This retrospective study evaluated consecutive 34 children (25 girls; aged 6–18 years, median 15.5 years) with JIA and with clinical involvement of cervical spine. In each patient, both radiographs and MRI of the cervical spine were performed. Imaging findings were correlated with clinical and laboratory data. Results: The cervical spine was affected in 35% of patients. The most frequent lesions were subaxial subluxations (SAS; 24%), apophyseal joint ankylosis (9%), and C1/C2 joint lesions (9%). Anterior atlanto-axial subluxation (AAS) was diagnosed only by radiography, and most of the SAS were seen on radiography, whereas only a few on MRI. Reversely, C1/C2 soft tissue involvement were seen on MRI only. Cervical spine involvement was associated with raised ESR (p = 0.012) and CRP (p = 0.014). Conclusions: The cervical spine lesions are still frequent complication of JIA affecting up to 35% of JIA patients. Most of them develop serious complications, such as AAS and ankylosis. Despite advantages of MRI in terms of the imaging of the atlanto-axial region radiography shows superiority in diagnosis of AAS and SAS.

Chronic osteomyelitis of the hand by Mycobacterium kansasii in an immunocompromised patient

Atypical mycobacteria are pathogens that uncommonly infect the hand. These organisms are capable of causing extensive bone damage in the hand. Mycobacterium kansasii is a slow growing non-tuberculous Mycobacterium. It is the second most common non-tuberculous Mycobacterium that mainly affects the hand and joints. Immunosuppressed patients are more likely to develop infection. Because extrapulmonary involvement of M. kansasii is rare, skin and soft tissue infections are infrequent; osteomyelitis is even rarer. Immunosuppressed patients are more likely to develop infection. A history of trauma is frequent. There is a delay in diagnosis antibiotics have been given with no response. Imaging studies are recommended in the diagnostic approach, with magnetic resonance imaging being the best option to show bone and soft tissue involvement. Infected tissue culture has greater sensitivity for diagnosis. Treatment for musculoskeletal involvement consists of multiple susceptibility-based antibiotics and antiretroviral therapy in HIV coinfection, combined with surgical management with incision and drainage.

Phase 1a/b Study of Monoclonal Antibody CAEL-101 (11-1F4) in Patients with AL Amyloidosis

Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by pathologic deposition of immunoglobulin light chains as amyloid fibrils in vital organs, leading to organ impairment and eventual death. That the process is reversible was evidenced in an in vivo experimental model in which fibril-reactive chimeric monoclonal antibody (mAb) 11-1F4 directly targeted human light-chain amyloid deposits and effected their removal via a phagocyte-mediated response. To determine tolerability and potential amyloidolytic effect of this agent (now designated mAb CAEL-101), we conducted a phase 1a/b study involving 27 patients, most of whom had manifestations of organ involvement. This was an open label study in which phase 1a patients received mAb CAEL-101 as a single intravenous infusion, with escalating dose levels from 0.5 mg/m2 to 500 mg/m2 to establish the maximum tolerated dose (MTD). In phase 1b, the antibody was administered as a graded series of four weekly infusions. For both phases, there were no drug-related serious adverse events or dose-limiting toxicities among recipients and the MTD was not reached. Majority of patients had deep hematologic responses but persistent organ disease prior to treatment. Fifteen of 24 patients (63%) who manifested cardiac, renal, hepatic, gastrointestinal, or soft tissue involvement had a therapeutic response to mAb CAEL-101 as evidenced by serum biomarkers or objective imaging modalities with median time to response of 3 weeks. Infusions of mAb CAEL-101 were well-tolerated and, for the majority, resulted in improved organ function, notably for those with cardiac impairment. This trial was registered at www.clinicaltrials.gov as NCT02245867.

SAPHO Syndrome Mimicking Infectious Spondylodiscitis and Bone Metastasis

The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) includes diseases with similar osteoarticular manifestations and skin conditions. Making this diagnosis is not always obvious, especially when the clinical presentation does not fit the typical pattern of the disease or it occurs in a particular field. We described three cases where the diagnosis was difficult. A 46 year-old woman presented with cervical pain. The cervical X-ray showed the aspect of an ivory C5 vertebra. The patient had, however, preserved general condition, no signs of underlying neoplasia, nor other joint complaints. Blood analysis was normal. Tomography did not find any suspect lesion but showed sclerosis and hyperostosis of the manubrium. Scintigraphy showed the characteristic “bullhead” appearance. A 61-year-old woman had thoracic and lumbar pain. MRI showed spondylodiscitis in D3-D4, D4-D5, D5-D6, D6-D7, and L1-L2 with paraspinal soft tissue involvement, simulating infectious spondylodiscitis. Infectious investigations and discovertebral biopsy performed twice were negative. SAPHO syndrome was then suspected. Bone scintigraphy showed uptake in the chondrosternal articulations and D4 to D7 vertebrae. The diagnosis of SAPHO was established. The third case was a 46-year-old man with a lung adenocarcinoma. Staging for metastatic disease, a TAP tomography was performed and showed osteosclerosis of D8 to D12 and intra-articular bridges in the sacroiliac joints. MRI and scintigraphy eliminated malignancy and confirmed the diagnosis of SAPHO. In our cases, imaging findings could facilitate differentiating SAPHO syndrome from other diseases.

Reconstruction of the mandible and maxilla

Mandibular and maxillary reconstruction is commonly required in clinical practice following major trauma or tumour resection. General principles of reconstruction should be followed: replace like with like and address functional and cosmetic deficiencies. Many reconstructive approaches are applicable to both mandibular and maxillary reconstruction and free vascularized tissue transfer is the accepted standard for both these anatomical sites with flaps such as the fibula or iliac crest. Advances in microsurgery allow high-quality, reproducible reconstructions to be performed by specialist teams. Reconstruction modality will be influenced by a range of factors such as defect site, size, and soft tissue involvement, as well as patient factors such as general health, dentition, age, and further planned treatment. Key factors to consider when reconstructing the midface and jaw include the airway, swallowing, speech, nutrition, and dental rehabilitation. These are also considered in this chapter. Computer modelling and surgical planning based on preoperative computed tomography scanning is now commonly used by many teams and this is also discussed.

OSTEOSÍNTESIS DE FRACTURA DE ESPINA TIBIAL ASISTIDA CON ARTROSCOPÍA Y TORNILLO DE COMPRESIÓN TIPO HERBERT, REPORTE DE UN CASO Y REVISIÓN DE LITERATURA.

Introduction Tibial spine fractures have a prevalence of 3 per 100,000 people annually. High-energy trauma is the leading cause, followed by low-energy trauma and 40% by multiple trauma. Imaging studies play a crucial role in establishing the diagnosis. It is important to understand that radiography alone does not allow a correct identification of the fracture, so it is necessary to complement it with a CT or MRI scan. The Meyers-McKeever classification divides fractures by their degree of displacement and comminution into 4 types and guides us in the therapeutic decision. The management of these fractures depends on the morphology, soft tissue involvement and the general condition of the patient. Surgical treatment is primarily considered for displaced fractures. Within this approach, the arthroscopy-assisted technique has reported excellent results. with a low complication rate, compared to open techniques, despite the few studies to define the standard Gold treatment. Case description A clinical case of a 32-year-old patient with a posterior tibial spine fracture is reported, who underwent surgery with arthroscopic-assisted osteosynthesis and a 4.5 x 4.0 Herbert-type compression screw with intraoperative arthroscopic images that demonstrated the restoration of joint congruence, without menisci or ligament injury, assessing intraoperative arches of motion from 0 to 90 degrees. In his mediate postsurgical has been started isometric physiotherapy with flexion and extension of the knee from 0 to 90 degrees plus strengthening of the iliac psoas and quadriceps and resume his activities in 2 months after his surgery. Conclusion At present, there is no consensus on the optimal surgical technique due to the lack of clinical trials. More studies of higher quality and sample size are necessary to establish the Gold Standard in the treatment of tibial spine fractures. However, we found that by using Herbert-type compression screws, timely compression of the fracture fragment is achieved in the anatomical reduction. An updated review of the subject and its therapeutic management is carried out.

Rare case of Mycobacterium marinum in a patient presenting with tenosynovitis

A 72-year-old man presented with tenosynovitis of the left hand’s extensor tendons that had been present for several months. He was initially treated with corticosteroids, first by local injection then systemically, but with no effect. When re-evaluated, the patient had developed a rash, and the symptoms had spread locally to surrounding structures. At this point, the patient added to the medical history that he had been stung by a sculpin a month before the debut of symptoms. Based on this, the patient’s involved area was biopsied, and subsequent microbiology findings proved consistent with Mycobacterium marinum infection. By the time of diagnosis, the patient had soft tissue involvement, arthritis and osteomyelitis with an overlying rash. This case emphasises the need for reassessment when treatment is not effective and for further investigations of the medical history to establish the correct diagnosis and treatment.

Atypical presentation of melorheostosis with soft tissues involvement: a case report

Background Melorheostosis is a skeletal disorder giving rise to a dripping wax appearance. The exact cause is still unclear, and the diagnosis is always challenging due to its wide differential diagnoses. Soft tissue involvement of melorheostosis has been reported in previous literatures but it is a rare phenomenon. Case presentation A 10-year-old child with melorheostosis presented with hematuria. Ultrasonography (USG) identified a cystic lesion in the right hemipelvis adjacent to the urinary bladder which was initially regarded as an ovarian cyst. Computed tomography (CT) confirmed the cystic lesion as a fusiform right internal iliac artery aneurysm, as well as multiple right retroperitoneal and right lower limb capillary hemangiomas with uterine involvement. Hence, the final diagnosis was atypical melorheostosis with vascular malformations. The patient was managed conservatively with Sirolimus therapy for the vascular anomalies. Conclusion Albeit the rare involvement of soft tissues, careful search for vascular malformation is recommended in melorheostosis.