Baek Goo Hyun, Kim Jae Kwang, Chung Moon Sang, Lee Sang Ki
Department of Orthopedic Surgery, College of Medicine, Seoul National University, Seoul, Korea.
Int Orthop. 2008 Apr;32(2):263-7. doi: 10.1007/s00264-006-0293-6. Epub 2007 Feb 15.
Congenital lateral ray deficiency is considered to be a manifestation of fibular hemimelia. However, we have noted patients with absent lateral ray but stable knee and ankle joints, and named this condition terminal hemimelia of the lower extremity. This study was undertaken to further define this group of patients and to compare these patients with fibular hemimelia patients. Four boys and one girl of mean age six years two months were in the terminal hemimelic group and four boys and three girls of mean age eight years seven months in the fibular hemimelic group at the final evaluation. Clinical features commonly observed in the fibular hemimelia such as knee valgus, knee instability, tibial bowing, ball and socket ankle, ankle instability, tarsal coalition, leg length inequality were compared between both groups. Terminal hemimelia of the lower extremity was the same as fibular hemimelia in clinical features below the ankle joint. However, terminal hemimela was found to be milder than fibular hemimelia in terms of limb shortening. The clinical features above the ankle joint were different between both groups. Knees and ankles were stable, and gait disturbance were rarely noticed in patients with terminal hemimelia of the lower extremity.
先天性外侧射线缺如被认为是腓骨半肢畸形的一种表现。然而,我们注意到一些患者虽无外侧射线,但膝关节和踝关节稳定,我们将这种情况命名为下肢末端半肢畸形。本研究旨在进一步明确这组患者,并将这些患者与腓骨半肢畸形患者进行比较。最终评估时,下肢末端半肢畸形组有4名男孩和1名女孩,平均年龄为6岁2个月;腓骨半肢畸形组有4名男孩和3名女孩,平均年龄为8岁7个月。比较了两组中常见于腓骨半肢畸形的临床特征,如膝外翻、膝关节不稳定、胫骨弓形、球窝踝关节、踝关节不稳定、跗骨联合、下肢长度不等。下肢末端半肢畸形在踝关节以下的临床特征与腓骨半肢畸形相同。然而,在肢体缩短方面,末端半肢畸形比腓骨半肢畸形轻。两组踝关节以上的临床特征不同。下肢末端半肢畸形患者的膝关节和踝关节稳定,很少注意到步态障碍。