Weiner Dennis S, Jonah David, Kopits Steven
Department of Pediatric Orthopaedic Surgery, Children's Hospital Medical Center of Akron, Akron, OH, USA.
J Pediatr Orthop. 2008 Jan-Feb;28(1):60-7. doi: 10.1097/BPO.0b013e31815ff2d9.
Surgical correction of the foot and ankle in patients with diastrophic dysplasia is extremely difficult secondary to the markedly distorted pathoanatomy. Several authors have described superficially some of the clinical and radiographic findings typical of the foot and ankle with diastrophic dysplasia; however, no description of the specific osseous deformities has been described in the literature. The purpose of this article was to provide such a model, detailing the nature of deformity of each of the bones and joints in the foot and ankle and their relationship to each other from a pathoanatomical standpoint.
A 3-dimensional sculpted model and detailed drawings were developed based on radiographs, computed tomography reconstructions, and direct observation both in and out of surgery. Fifty-three patients representing 106 feet formed the basis of this analysis (age, 3 days to 32 years). An additional 12 feet of the senior author's cases provided further confirmation of these deformities.
Superficially, the apparent deformity most closely resembles a Z-type foot or serpentine foot. The overall deformity bears no true resemblance to the idiopathic clubfoot. Typically, the hindfoot is in severe equinus with the subtalar joint being deformed into valgus and moving more posteriorly. In contrast to the idiopathic clubfoot, the navicular was markedly angulated laterally on the talus. The medial cuneiform was deviated medially on the navicular articulation. The forefoot was foreshortened and in marked varus positioning with varus of the entire metatarsals. The second to fifth metatarsals bend and curve near their bases as they tilt toward the first metatarsal.
The disturbed pathoanatomy of the diastrophic dysplasia foot and ankle reflects the difficulties in achieving any substantial surgical correction without customization. Surgical management of the foot and ankle in diastrophic dysplasia must be individualized and based on a clear understanding of the unique segmental malalignment of the foot and ankle.
由于病理解剖结构严重扭曲，矫正脊柱发育不良患者的足踝畸形极为困难。几位作者曾简要描述过脊柱发育不良患者足踝的一些典型临床和影像学表现；然而，文献中尚未对具体的骨性畸形进行描述。本文旨在提供这样一个模型，从病理解剖学角度详细阐述足踝部各骨骼和关节的畸形性质及其相互关系。
基于X线片、计算机断层扫描重建图像以及手术内外的直接观察，制作了一个三维雕刻模型和详细的图纸。对53例患者（共106只足）进行分析（年龄3天至32岁）。资深作者的另外12例病例的足部进一步证实了这些畸形。
从表面看，明显的畸形最类似于Z型足或蛇形足。整体畸形与特发性马蹄内翻足并无真正相似之处。通常，后足处于严重马蹄足畸形，距下关节外翻并向后移位。与特发性马蹄内翻足不同，舟骨在距骨上明显向外侧成角。内侧楔骨在与舟骨的关节处向内侧偏移。前足缩短，整个跖骨呈明显内翻位。第二至第五跖骨在靠近基部处弯曲并向第一跖骨倾斜时呈弧形。
脊柱发育不良患者足踝部紊乱的病理解剖结构反映了在未进行定制的情况下实现任何实质性手术矫正的困难。脊柱发育不良患者足踝部的手术治疗必须个体化，并基于对足踝部独特节段性排列不齐的清晰理解。
