Pratap Rishabh, Raj Gaurav
Department of Radiodiagnosis, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh 226010, India.
Radiol Case Rep. 2023 Feb 15;18(4):1613-1616. doi: 10.1016/j.radcr.2023.01.083. eCollection 2023 Apr.
Macrodystrophia lipomatosa is a rare form of nonhereditary congenital localized gigantism involving upper and lower limbs and is characterized by overgrowth of all the mesenchymal elements predominantly fibro-adipose component, in the distribution of a particular nerve, usually median nerve. It usually presents with progressive painless overgrowth of the involved limb, toe, or digit and is associated with macrodactyly. It might cause limitation of the movement of the involved part. Imaging has an important role in diagnosing this condition and differentiating it from malignant mimics. Imaging findings include hypertrophy of the mesenchymal elements of the involved digits and/or limbs predominantly fibro adipose component with associated overgrowth of the phalanges. In this case report, we present a case of unilateral involvement of index finger and thumb with associated macrodactyly.
巨指（趾）脂肪营养不良是一种罕见的非遗传性先天性局限性巨大症，累及上肢和下肢，其特征是所有间充质成分过度生长，主要是纤维脂肪成分，分布于特定神经，通常是正中神经。它通常表现为受累肢体、脚趾或手指进行性无痛性过度生长，并伴有巨指（趾）。它可能导致受累部位活动受限。影像学在诊断这种疾病并将其与恶性模仿疾病相鉴别方面具有重要作用。影像学表现包括受累手指和/或肢体的间充质成分肥大，主要是纤维脂肪成分，并伴有指骨过度生长。在本病例报告中，我们展示了一例示指和拇指单侧受累并伴有巨指（趾）的病例。
