Considered rare, the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a distinct clinical entity, associating skin manifestations and osteoarticular symptoms. Anterior chest wall pain centered at sternoclavicular and sternocostal joints is an important and characteristic clinical finding that can lead to its diagnosis. Radiologists and clinicians must be aware of synovitis-acne-pustulosis-hyperostosis-osteitis syndrome as it can mimic some of the more common disease entities such as Paget\'s disease. We report the case of a 63-year-old male patient, with no significant medical history, who presented to the dermatology department, with severe palmar and plantar pustulosis associated with polyarthralgia. Computerized tomography scan showed sternoclavicular hyperostosis, in favor of SAPHO syndrome, with regression of clinical symptoms after non-steroidal anti-inflammatory drug treatment.

A series of 12 contiguous caudal vertebrae of an ichthyodectiform fish from the Smoky Hill Chalk Member of the Niobrara Formation is described. The vertebral centra exhibit extensive overgrowth of pathological bone and there is additional pathological bone within the centra and intervertebral spaces, which together resulted in the coossification of most centra. The extent of the pathology is greatest on preural vertebrae 1-3 and decreases anteriorly, which suggests that the pathology began posteriorly and progressed anteriorly. In addition to the pathological overgrowth on bones, the specimen preserves features interpreted as calcified and/or ossified soft tissues associated with the neural and haemal canals. The pathologies are unlike previously described examples of bony pathologies in fish, and it is suggested that they resulted from combined bacterial and fungal infections. As the pathologies developed, they would have adversely impacted the fish\'s swimming and feeding abilities, and presumably eventually led to the fish\'s death.

En plaque meningioma is a rare type of meningioma characterized by an infiltrative nature, sheet-like growth, and at times invading the bone. We report here a case of en plaque meningioma. The patient was a 66-year-old woman presenting with headache and painful bilateral proptosis. Cerebral magnetic resonance imaging revealed a bilateral en plaque meningioma showed as a bilateral hyperostotic of greater wing sphenoid bone associated with bilateral thickening and enhancement of the dura in the anterior temporal area and the retroclival region invading bilaterally the cavernous sinus, the prechiasmatic portion of the optic nerve, and the lateral rectus muscle of the orbit through the superior orbital fissure causing bilateral exophthalmia. Due to invasion of the cavernous sinus and the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal followed by adjuvant radiotherapy frequently produces good functional and cosmetic results, and over a 3-year period, follow-up magnetic resonance imaging scans showed no obvious signs of recurrence.

Teaching point: Benign hyperostosis of the rib is a benign entity consisting of a stress phenomenon that should not be confused with Paget, fibrous dysplasia, or osteoblastic metastasis.

BACKGROUND: Osteopetrosis is a rare hereditary disease that can be transmitted in an autosomal recessive or autosomal dominant.
METHODS: Here, we report a case of trochanteric fracture in an 18-year-old boy with an anatomical plate. At the last follow-up, 24 months after surgery, the fracture had healed well, and the patient was not restricted in his activities.
CONCLUSIONS: Osteopetrosis is a rare bone disease that is mainly caused by osteoclast dysfunction. It results from a remodelling defect that leads to hypermineralization of the skeleton, resulting in bone fragility. Both surgical and nonsurgical management have advantages and disadvantages. Thus, open reduction and anatomic plate fixation remain effective management modalities for trochanteric fractures in osteopetrosis patients.
CONCLUSIONS: For our patient and as described in the literature, the complication rate decreases as some principles are respected with better consolidation of the osteoporotic fracture.

UNASSIGNED: To report our experience with patient specific implants for one-step orbit reconstruction following hyperostotic SWM removal and to describe the evolution of the technique through three surgical cases. Methods: Three cases of one-step SWM removal and orbit reconstruction are described. All cases are given consecutively to describe the evolution of the technique. Hyperostotic bone resection was facilitated by electromagnetic navigation and cutting guides (templates). Based on a 3D model, silicone molds were made using CAD/CAM. Then PMMA implant was fabricated from these molds. The implant was adjusted and fixed to the cranium with titanium screws after tumor removal. Results: Following steps of the procedure changed over these series: hyperostotic bone resection, implant thickness control, implant overlay features, anatomic adjustments, implant fixation. The proptosis resolved in all cases. In one patient the progressive visual acuity deterioration was recognized during the follow-up. No oculomotor disturbances and no tumor regrowth were found at the follow-up.
UNASSIGNED: CAD/CAM technologies enable creation of implants of any size and configuration, and thereby, to increase the extent of bony resection and lower the risk of tumor progression. The procedure is performed in one step which decreases the risk of postoperative morbidity.

BACKGROUND: The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare condition. Its treatment remains a challenge for clinicians, and often yields mixed results.
METHODS: We report the case of a 51-year-old Caucasian woman who presented with SAPHO syndrome with mainly axial involvement. She had been treated with sulfasalazine and anti-inflammatory drugs for many years without any success. A few weeks after starting treatment with tofacitinib, both clinical and biological parameters dramatically improved. Imaging also showed considerable regression of the vertebral and pelvic lesions. However, tofacitinib had to be discontinued due to the occurrence of pulmonary embolism. Consequently, recurrence of bone pain and biologic inflammation was rapidly observed.
CONCLUSIONS: Anti-JAKs are an interesting treatment option in the management of SAPHO syndrome that need further clinical trials and assessment for validating response.

暂无摘要。

OBJECTIVE: Synovitis acne pustulosis hyperostosis osteitis (SAPHO) is a rare heterogeneous disease of unknown aetiopathology. Externally validated and internationally agreed diagnostic criteria or outcomes and, as a result, prospective randomised controlled trials in SAPHO are absent. Consequently, there is no agreed treatment standard. This study aimed to systematically collate and discuss treatment options in SAPHO.
METHODS: Following \'Preferred Reporting Items for Systematic Reviews and Meta-Analyses\' guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded.
RESULTS: A total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement.
CONCLUSIONS: No single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.

Melorheostosis is a rare benign bone pathology involving bone dysplasia and hyperostosis. The disease can be recognized with a characteristic radiographic feature of radiopaque lesions dripping along a long bone\'s diaphysis. The aberrant bone formation and development manifests mainly as pain, edema, and paresthesia of the affected limb. Severe cases may report limb deformity as well as limited range of motion. Until now, there have been approximately 300 cases reported about melorheostosis worldwide and its diverse clinical picture and age distribution. In Vietnam, there is only one known case of melorheostosis discovered incidentally via radiography. The scarcity of cases presents a challenge within the medical community in recognizing and diagnosing the condition, and a delayed diagnosis can lead to severe contracture and compromised limb motility. In this article, we reported an 82-year-old case of polyostotic melorheostosis with late onset and predominant edema, affecting the sternum, the ribs, and multiple bones of the right extremities and presented our clinical approach for a geriatric patient with chronic limb edema. Our case is distinctive in terms of anatomical location as well as the predominant 20-year non-pitting edema. A prompt diagnosis was made upon the classic dripping candle wax radiographic features emphasizing the role of plain X-ray in establishing the diagnosis without extraneous utilization of other modalities and invasive procedures. Exclusion of other causes of chronic edema such as lymphadenopathy, malignancy as well as parasitic infection is of clinical importance.