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.

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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.

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.

Two female (FL 1, FL 2) and one male (ML) 11-wk-old, intact, captive African lion cubs (Panthera leo leo) were presented with a history of mild vestibular signs. Initial serum vitamin A concentrations were low (140 nmol/L) for ML. Calvarial hyperostosis was confirmed using computed tomography (CT) of the head and cervical vertebrae in each cub. CT measurements were adapted in relation to the skull width. ML showed the most pronounced thickening of the tentorium cerebelli and occipital bone, represented by a tentorium cerebelli to skull width ratio (TCR) of 0.08 (FL 1: 0.06, FL 2: 0.05) and a basisphenoid to skull width ratio (BBR) of 0.07 (FL 1: 0.06, FL 2: 0.04). Magnetic resonance imaging (MRI) revealed cerebellar herniation and cervical intramedullary T2-weighted hyperintensity from C1, extending caudally for at least two cervical vertebrae in all cubs. Treatment was initiated with subcutaneous vitamin A supplementation and feeding of whole carcasses. Improvement in ataxia was noticed 3 wk later. Follow-up CT and MRI examinations were performed in ML after 3 and 8 mon. The affected bones appeared slightly less thickened and TCR and BBR had decreased to 0.05 after 3 mon. The cerebellum remained mildly herniated, accompanied by amelioration of cervical T2w hyperintensities. After 8 mon, evaluation and diagnostic imaging revealed further improvement regarding the neurologic status and measurements (TCR 0.05, BBR 0.04) despite persistence of a subtle cerebellar herniation. In conclusion, bone remodeling and improvement in clinical signs may be achievable in young lion cubs presented with calvarial hyperostosis and may be attributable to high-dose vitamin A supplementation.

Hidradenitis suppurativa (HS) is an autoinflammatory skin disorder of the terminal hair follicle, which can present in sporadic, familial, or syndromic form. A classification has been proposed for the latter, distinguishing cases associated with a known genetic condition, with follicular keratinization disorders or with autoinflammatory diseases. This review focuses on the clinical and genetic features of those entities (ie, pyoderma gangrenosum [PG], acne and HS; PG, acne, pyogenic arthritis and HS; psoriatic arthritis, PG, acne and HS; synovitis, acne, pustulosis, hyperostosis, osteitis; and so forth) for which the collective term HS-related autoinflammatory syndromes is proposed.

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.

BACKGROUND: Planar hyperostotic meningiomas account for 2-9% of intracranial meningiomas. They are characterized by planar node following the contours of the inner surface of the skull. Hyperostosis is present in most cases. Timely diagnosis of skull base tumors is usually simple due to early involvement of the cranial nerves. However, convexity meningiomas en plaque usually reach large dimensions that complicates surgery and radiotherapy.
OBJECTIVE: To analyze the current state of diagnosis, molecular biology and surgical treatment of hyperostotic meningiomas en plaque.
METHODS: A systematic review was performed in accordance with the PRISMA guidelines. Searching for literature data included the following keywords: «planar meningioma», «hyperostotic meningioma», «meningioma en plaque», «infiltrative meningioma». We reviewed the PubMed and Google Scholar databases until May 2023 and enrolled only full-text Russian-, English- or French-language reports.
CONCLUSIONS: Among primary 332 reports, 35 references met the inclusion criteria. We found less severity or absence of focal neurological symptoms, comparable incidence of intracranial hypertension and no histological differences between planar and nodular meningiomas. Analysis of molecular biological features of planar meningiomas, including cell cultures, is feasible. There is no consensus regarding surgical treatment and radiotherapy. Most publications are case reports.
CONCLUSIONS: The results of treatment of planar hyperostotic meningiomas, especially large and giant ones, are unsatisfactory. There is no a generally accepted algorithm for treating patients in the literature. This problem requires further research.
Плоскостные («en plaque») гиперостотические менингиомы составляют от 2 до 9% внутричерепных менингиом. Они характеризуются наличием плоскостного узла, повторяющего контуры внутренней поверхности черепа, и в большинстве случаев формируют гиперостоз. Если при локализации в области основания черепа возможна своевременная диагностика по причине раннего вовлечения черепных нервов, плоскостные менингиомы свода черепа на момент постановки диагноза могут достигать значительных размеров, что затрудняет как хирургическое, так и лучевое лечение.
UNASSIGNED: Анализ современного состояния проблемы диагностики, молекулярной биологии и хирургического лечения плоскостных гиперостотических менингиом свода черепа по данным литературы.
UNASSIGNED: Систематический обзор выполнен в соответствии с рекомендациями PRISMA. Проведен поиск по ключевым словам: «planar meningioma», «hyperostotic meningioma», «meningioma en plaque», «infiltrative meningioma» — в базах PubMed и Google Scholar на всю глубину по май 2023 г., в исследование включены только полнотекстовые публикации на русском, английском или французском языках.
UNASSIGNED: Из 332 выявленных ссылок критериям включения соответствовали 35 источников. Отмечаются меньшая выраженность или отсутствие очаговых неврологических симптомов, сопоставимая частота внутричерепной гипертензии и отсутствие гистологических различий при плоскостных по сравнению с узловыми менингиомами. Подчеркивается целесообразность изучения молекулярно-биологических особенностей плоскостных менингиом, в том числе в клеточных культурах. Нет консенсуса в отношении тактики хирургического и лучевого лечения. Большинство публикаций является описанием клинических случаев.
UNASSIGNED: Результаты лечения плоскостных гиперостотических менингиом свода черепа, особенно больших и гигантских, являются неудовлетворительными. Общепризнанный алгоритм лечения пациентов в литературе не представлен. Проблема требует дальнейших исследований.

Meningiomas are the most common feline primary brain tumours, and calvarial hyperostosis (CH) is frequently documented in association with this neoplastic entity. The clinical significance of and mechanisms driving the formation of CH in cats with meningiomas are poorly understood, although tumour invasion into the skull and tumour production of cytokines and enzymes have been implicated as causes of CH in humans. This retrospective study investigated relationships between signalment, MRI or CT imaging features, histopathologic tumour characteristics, alkaline phosphatase (ALP) isoenzyme concentrations, tumour expression of matrix metalloproteinases (MMP)-2, MMP-9, and interleukin-6 (IL-6), and progression free survival times (PFS) following surgical treatment in 27 cats with meningiomas with (n = 15) or without (n = 12) evidence of CH. No significant differences in breed, age, sex, body weight, tumour grade, tumour volume, peritumoral edema burden, ALP isoenzyme concentrations, tumour Ki-67 labelling indices or MMP-2 or MMP-9 expression and activity, or PFS were noted between cats with or without CH. There was a trend towards higher serum (p = .06) and intratumoral (p = .07) concentrations of IL-6 in cats with CH, but these comparisons were not statistically significant. Histologic evidence of tumour invasion into bone was observed in 5/12 (42%) with CH and in no (0/6) cats without CH, although this was not statistically significant (p = .07). Tumour invasion into bone and tumour production of IL-6 may contribute to the formation of meningioma associated CH in cats, although larger studies are required to further substantiate these findings and determine their clinical relevance.

暂无摘要。