Outcomes of limb reconstruction in treating achterman type I fibular hemimelia based on Ilizarov technique

doi:10.3877/cma.j.issn.2096-0263.2023.02.001

Abstract

Abstract:

Objective

To explore the clinical outcomes of combination of Ilizarov technique and composite surgeries for treatment of congenital fibula hemimelia, and to summarize the key points of limb reconstruction management for such deformity.

Method

Twenty patients with congenital fibula hemimelia were treated surgically and retrospectively studied in Department of Orthopedics, Shanghai Jiao Tong University affiliated Sixth People's Hospital since 2014. Patients included 12 males and 8 females, with an age of (17.5 ± 7.0) y (4 to 26 y). According to Achterman classification, there were 14 cases of type IA and 6 cases of type IB. Single Ilizarov technique or combined intramedullary assistance was applied for limb length deficiency; For genu valgus or valgus ankle deformities, close-wedge osteotomy and internal fixation were applied for adult patients, and hemiepiphysiodesis for pediatric patients; Contracted iliotibial band, fibular tendons and Achilles' tendon were released and elongated; Overlapping toes were corrected by tendon transfer.

Results

Twenty patients were followed up for (36.1±8.7) months. The limb was lengthened for (7.2±1.0) cm, with average EFI (15.9±6.3) d/cm and average HI (21.7±2.8) d/cm. All obvious limb length discrepancy, genu valgus [GVA: (14.9±3.4)° and (2.1±1.7)° preoperatively and at last follow-up, t=13.510, P<0.05], tibial angulation [TBA: (3.3±3.3)° and (0.8±0.7)° preoperatively and at last follow-up, t=3.490, P<0.05] and ankle and foot deformities [VAA: (9.2±3.4)° and (1.2±1.3)° preoperatively and at last follow-up, t=9.785, P<0.05] were corrected at the final follow-up. Clinical outcomes indicated significantly improved limb function compared with preoperative grading. [LLRS AIM: (10.1±1.2) points and (2.0±1.4) points preoperatively and at last follow-up, t=21.140, P<0.05].

Conclusion

Congeinital fibular hemimelia is a rare and complicated syndrome with co-exist limb length deficiency, abnormal alignment of lower-limb and foot and ankle deformities. Combination of Ilizarov technique and composite surgeries can bring satisfying outcomes for treatment of congenital fibula hemimelia and improve the quality of life of the patients.

Key words: Fibular hemimelia, Ilizarov technique, Composite surgery, Limb lengthening, Osteotomic orthopedics

Rui Zhang, Shenghe Liu, Hongjiang Ruan, Yifan Yu, Jia Xu, Qinglin Kang. Outcomes of limb reconstruction in treating achterman type I fibular hemimelia based on Ilizarov technique[J]. Chinese Journal of Geriatric Orthopaedics and Rehabilitation(Electronic Edition), 2023, 09(02): 65-72.

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References 35

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病例	性别	年龄	长度缺陷（cm）	膝外翻	踝外翻	跖列畸形
1	女	23	6	是	否	无
2	男	8	6	否	否	单跖列缺失
3	男	9	7	是	是	三跖列缺失，叠趾
4	男	4	3	是	是	无
5	男	11	6	是	是	无
6	女	21	7	是	否	无
7	男	23	6	是	是	双跖列缺失
8	女	23	7	是	是	单趾萎缩
9	男	19	6	是	是	双跖列缺失
10	女	23	5	是	是	双跖列缺失，叠趾
11	男	6	5	是	是	无
12	男	18	7	是	是	无
13	男	21	7	是	是	无
14	男	23	6	是	是	无
15	男	26	7	是	是	单跖列缺失，叠趾
16	女	17	6	是	是	双跖列缺失
17	女	9	5	是	是	三跖列缺失，叠趾
18	女	18	6	是	是	无
19	女	22	6	是	是	无
20	男	25	7	是	是	双跖列缺失
病例	Achterman分型	随访（mo）	延长部位	髓内结合	膝、踝外翻处理
1	IA	15	股骨	髓内钉	DFO
2	IA	22	胫骨	弹性钉	无
3	IB	38	胫骨	无	股骨内髁及内踝骨骺阻滞
4	IA	25	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
5	IA	46	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
6	IA	37	股骨	髓内钉	DFO
7	IA	28	胫骨	无	DFO、踝上截骨
8	IA	47	胫骨	无	DFO
9	IA	45	胫骨	髓内钉	DFO、踝上截骨
10	IB	36	胫骨	髓内钉	DFO、踝上截骨
11	IA	37	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
12	IA	44	股骨	无	DFO、踝上截骨
13	IA	35	股骨	无	DFO、踝上截骨
14	IA	40	胫骨	髓内钉	DFO、踝上截骨
15	IB	43	胫骨	髓内钉	DFO、踝上截骨
16	IB	40	胫骨	无	DFO、踝上截骨
17	IB	31	胫骨	无	股骨内髁及内踝骨骺阻滞
18	IA	28	股骨	髓内钉	DFO、踝上截骨
19	IA	43	股骨	髓内钉	DFO、踝上截骨
20	IB	42	胫骨	髓内钉	DFO、踝上截骨

病例	性别	年龄	长度缺陷（cm）	膝外翻	踝外翻	跖列畸形
1	女	23	6	是	否	无
2	男	8	6	否	否	单跖列缺失
3	男	9	7	是	是	三跖列缺失，叠趾
4	男	4	3	是	是	无
5	男	11	6	是	是	无
6	女	21	7	是	否	无
7	男	23	6	是	是	双跖列缺失
8	女	23	7	是	是	单趾萎缩
9	男	19	6	是	是	双跖列缺失
10	女	23	5	是	是	双跖列缺失，叠趾
11	男	6	5	是	是	无
12	男	18	7	是	是	无
13	男	21	7	是	是	无
14	男	23	6	是	是	无
15	男	26	7	是	是	单跖列缺失，叠趾
16	女	17	6	是	是	双跖列缺失
17	女	9	5	是	是	三跖列缺失，叠趾
18	女	18	6	是	是	无
19	女	22	6	是	是	无
20	男	25	7	是	是	双跖列缺失
病例	Achterman分型	随访（mo）	延长部位	髓内结合	膝、踝外翻处理
1	IA	15	股骨	髓内钉	DFO
2	IA	22	胫骨	弹性钉	无
3	IB	38	胫骨	无	股骨内髁及内踝骨骺阻滞
4	IA	25	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
5	IA	46	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
6	IA	37	股骨	髓内钉	DFO
7	IA	28	胫骨	无	DFO、踝上截骨
8	IA	47	胫骨	无	DFO
9	IA	45	胫骨	髓内钉	DFO、踝上截骨
10	IB	36	胫骨	髓内钉	DFO、踝上截骨
11	IA	37	股骨	弹性钉	股骨内髁及内踝骨骺阻滞
12	IA	44	股骨	无	DFO、踝上截骨
13	IA	35	股骨	无	DFO、踝上截骨
14	IA	40	胫骨	髓内钉	DFO、踝上截骨
15	IB	43	胫骨	髓内钉	DFO、踝上截骨
16	IB	40	胫骨	无	DFO、踝上截骨
17	IB	31	胫骨	无	股骨内髁及内踝骨骺阻滞
18	IA	28	股骨	髓内钉	DFO、踝上截骨
19	IA	43	股骨	髓内钉	DFO、踝上截骨
20	IB	42	胫骨	髓内钉	DFO、踝上截骨

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