PDF(Pubmed)
Abstract：
UNASSIGNED: Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term \"terminal hemimelia\" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia.
UNASSIGNED: In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared.
UNASSIGNED: Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years.
UNASSIGNED: Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening.
UNASSIGNED: level IV.
UNASSIGNED: In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared.
UNASSIGNED: Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years.
UNASSIGNED: Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening.
UNASSIGNED: level IV.
摘要：
■腓骨半位炎表示腓骨发育不全/发育不全的后轴纵向缺陷；术语“末端半位炎”保留给腓骨正常的后轴纵向缺陷患者。我们旨在描述末端半乳球菌的特征。
■总共，回顾了30例腓骨正常或发育不良并在1992年至2022年间访问我们机构的后轴纵向缺陷患者。将患者分为终末期半球症和经典腓骨半球症组，并比较了他们的人口统计学特征以及临床和影像学检查结果。
■股骨缩短，膝盖外翻,和胫骨脊柱发育不全在末端半乳畸形（n=13）中的发生率低于经典腓骨半乳畸形（n=17）（分别为p=0.03，p<0.001和p=0.003）。终末期半球组患者均未出现膝关节不稳,而12%的经典腓骨半乳症患者有。在两组中通常观察到球窝踝关节和无侧线。然而,在终末半球症中观察到的tar骨联盟频率较低（p=0.004）。所有终末期半球症患者均表现出无痛的脚，没有踝关节不稳定。尽管在成熟时的肢体长度差异平均为40.4mm的末端半乳和67.0mm的经典腓骨半乳（p<0.001），终末期半乳病患者，除了一个,表现出>20毫米的肢体长度差异。然而,46%的患者接受了肢体长度均衡手术，主要是单级胫骨延长术,平均年龄为11.2岁。
■末端半球症可能表现出比经典腓骨半球症更温和的表型。它主要与踝关节以下腓骨半角的症状重叠，并表现为肢体长度差异。然而,相当数量的终末期半乳症患者需要肢体长度均衡程序，例如单级胫骨延长术。
■四级。
■总共，回顾了30例腓骨正常或发育不良并在1992年至2022年间访问我们机构的后轴纵向缺陷患者。将患者分为终末期半球症和经典腓骨半球症组，并比较了他们的人口统计学特征以及临床和影像学检查结果。
■股骨缩短，膝盖外翻,和胫骨脊柱发育不全在末端半乳畸形（n=13）中的发生率低于经典腓骨半乳畸形（n=17）（分别为p=0.03，p<0.001和p=0.003）。终末期半球组患者均未出现膝关节不稳,而12%的经典腓骨半乳症患者有。在两组中通常观察到球窝踝关节和无侧线。然而,在终末半球症中观察到的tar骨联盟频率较低（p=0.004）。所有终末期半球症患者均表现出无痛的脚，没有踝关节不稳定。尽管在成熟时的肢体长度差异平均为40.4mm的末端半乳和67.0mm的经典腓骨半乳（p<0.001），终末期半乳病患者，除了一个,表现出>20毫米的肢体长度差异。然而,46%的患者接受了肢体长度均衡手术，主要是单级胫骨延长术,平均年龄为11.2岁。
■末端半球症可能表现出比经典腓骨半球症更温和的表型。它主要与踝关节以下腓骨半角的症状重叠，并表现为肢体长度差异。然而,相当数量的终末期半乳症患者需要肢体长度均衡程序，例如单级胫骨延长术。
■四级。
