Випадок остеоїд-остеоми у хворої на синдром Елерса-Данлоса

Abstract

У випадку, що описаний у статті було діагностовано остеоїд-остеому на фоні Елерса-Данлоса та численної супутньої патології. Синдром Елерса-Данлоса представляє собою гетерогенну групу спадкових захворювань сполучної тканини, загальними клінічними ознаками яких є гіпермобільність суглобів, підвищена розтяжність щкіри і крихкість тканин. Остеоїд-остеома доброякісна пухлина кісткової тканини, основна локалізація якої - діафіз довгих кісток. Виходячи з того, що вищезазначена патологія є доволі рідкою, вивчення випадку комбінації цих двох захворювань може бути не тільки цікавим, але й корисним для лікарів різних спеціальностей.

Ehlers-Danlos syndrome (EDS) represents a heterogenic group of hereditary diseases of the connective tissue with such common clinical features as hypermobility of joints, excessive extensibility of skin and fragility of tissues. Osteoid osteoma is a benign tumour of the bone tissue, with its main localization in the diaphysis of the long bones, or sometimes in the pelvic bones. In the case, described in the article, osteoid osteoma was diagnosed in the setting of Ehlers-Danlos syndrome and multiple concomitant pathologies. Goal of research. Analyze literature data and introduce doctors to the acquired own experience as to the clinical course and the morphological diagnostics of osteoid osteoma in a patient with EDS. Materials and methods of research. Literature data, materials of the case history, clinical examination of the patient, macro- and microscopic investigation of the removed tumour tissue (optical microscopy of histologic specimen, stained with hematoxylin and eosin) were used. Research results. A 17 year old female patient with Ehlers-Danlos syndrome complained of pains in the area of the pelvis joint for two years. The pains aggravated to the uttermost at night time. She was operated as an osteoid osteoma was suspected in the upper third of her left thigh bone. The macroscopically removed tumour represented a small size lump, with an irregular shape, of a dirty-red colour. On section the tumour tissue had a lobulated structure. On microscopic examination of the presented samples, the diagnosis of “osteoid osteoma” was confirmed. A great number of osteoid chordae and trabecules, interweaving with each other and surrounded by aggregations of osteogenic tissue with disintegration features were found. The fibrous stroma had a great number of blood vessels. In the postoperative period, the patient complained of marked pains in the area of the postoperative wound. She received symptomatic, antibacterial and vascular therapy. In the setting of the conducted therapy, a moderate lowering of the pain syndrome was achieved. At the attempt of erection of the patient, aggravation of the pain syndrome and orthostatic lowering of the arterial pressure were noticed. Sutures were removed on the 14th postoperative day. The patient, being in satisfactory condition, was discharged from the hospital to follow outpatient therapy. Conclusion. On the basis, that the pathology mentioned above is rather rare, studying a case of combination of these two diseases can be not only interesting, but also useful for doctors of different specialties. The special feature, which can be noted, is a rather long recovery period after the surgical intervention.

В описанном в статье случае была диагностирована остеоид-остеома на фоне синдрома Элерса-Данлоса и многочисленной сопутствующей патологии. Синдром Элерса-Данлоса представляет собой гетерогенную группу наследственных заболеваний соединительной ткани, общими клиническими признаками которых являются гипермобильность суставов, повышенная растяжимость кожи и хрупкость тканей. Остеоид-остеома - доброкачественная опухоль костной ткани, основная локализация которой - диафиз длинных костей. Исходя из того, что вышеупомянутая патология является достаточно редкой, изучение случае комбинации этих двух заболеваний может быть не только интересным, но и полезным для врачей различных специальностей.