CLINICAL VARIANT OF OSSIFYING MYOSITIS IN PEDIATRIC PRACTICE

H. Senatorova, T. Frolova, A. Senatorova, O. Kikhtenko, N. Osman
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Abstract

Introduction. Ossifying myositis is a pathological process in muscles characterized by the formation of ossification in soft tissues. At present, the etiological factors of the disease remain not fully elucidated. The triggering factors of the disease are considered to be traumatic injuries, invasive medical manipulations against the background of genetic predisposition.Aim. Invite attention of general practitioners and pediatricians to a rare disease, namely progressive ossifying fibrodysplasia in children and the peculiarities of its diagnosis.Results. The article presents a clinical case of progressive ossifying fibrodysplasia (Munchmeyer's disease) in a 4-year-old girl. At birth, the child was diagnosed with a foot deformity characteristic of this pathology (shortening of the first metatarsal finger, flexion-rotation contracture of both feet). The clinic of the disease manifested itself at the age of 3 years, when, after falling on the back, a dense formation was noticed in the area of the left shoulder blade. Half a year after the fall, swelling and pain appeared in the sacro-coccygeal region of the spine. The girl was consulted by an orthopedist, dermatologist, and oncologist. During the examination of the child, characteristic clinical features of progressive ossifying fibrodysplasia were revealed, namely, deformation and fixed position of the chest, tense neck muscles, sharp limitation of movements in all parts of the spine, limitation of bending in the left elbow joint, clinodactyly, valgus deformity of the big toes. During the ultrasound examination, the following changes were diagnosed: swelling of muscle tissue in the neck area, subscapular area on the left and sacrococcygeal joint; multiple hypoechoic formations of irregular shape, heterogeneous echo structure with hyperechoic inclusions with an acoustic shadow; a focal change in the muscle structure in the form of a loss of the characteristic pinnate structure of the perimysium. The diagnosis was confirmed histologically. No characteristic changes were found in clinical and biochemical studies. The girl is under supervision. No worsening of the child's condition has been recorded over the past four years.
小儿骨化性肌炎的临床表现
介绍。骨化性肌炎是一种以软组织骨化形成为特征的肌肉病理过程。目前,该病的病因尚未完全阐明。该疾病的触发因素被认为是外伤性损伤,在遗传易感性背景下的侵入性医疗操作。引起全科医生和儿科医生对一种罕见疾病的关注,即儿童进行性骨化性纤维发育不良及其诊断特点。本文报告一个4岁女童进行性骨化性纤维发育不良(Munchmeyer病)的临床病例。出生时,患儿被诊断为该病理特征的足部畸形(第一跖指缩短,双脚屈曲-旋转挛缩)。该疾病的临床表现在3岁时,当仰卧后,在左肩胛骨区域发现致密的形成。坠落半年后,骶尾骨区出现肿胀和疼痛。这个女孩接受了骨科医生、皮肤科医生和肿瘤科医生的咨询。检查时发现患儿进行性骨化性纤维发育不良的临床特征为胸部变形固定,颈部肌肉紧张,脊柱各部位活动受限,左肘关节屈曲受限,拇趾斜指,大脚趾外翻畸形。超声检查时诊断如下变化:颈部、左侧肩胛下、骶尾关节肌肉组织肿胀;不规则形状、非均匀回声结构的多个低回声地层,伴有声影的高回声内含物;肌萎缩肌肉结构的局灶性变化,表现为肌围膜特有的羽状结构的丧失经组织学检查确诊。临床及生化检查未见特征性变化。这个女孩在监督之下。在过去四年中,没有记录显示这名儿童的病情恶化。
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