下肢畸形伴血小板减少-桡骨缺失综合征的两期治疗。

IF 1.1 4区 医学 Q3 ORTHOPEDICS Acta orthopaedica et traumatologica turcica Pub Date : 2022-11-01 DOI:10.5152/j.aott.2022.21397
Mehmet Akdemir, Çağdaş Biçen, Mustafa Özkan
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引用次数: 0

摘要

目的:本研究的目的是评估两阶段治疗无桡骨血小板减少症患者的上、下肢畸形的效果。方法:4例患者(女3例,男1例),平均年龄1.8岁(范围1 ~ 4岁)。这些患者的随访时间平均为5.5年。4例患者均有双侧桡骨纵向缺损,仅有2例患者有双侧固定膝挛缩。实施两期手术。治疗桡骨纵向缺损的手术包括在第一阶段用Ilizarov支架撑开,然后在第二阶段进行集中。首先使用Ilizarov支架治疗膝关节挛缩,然后在第二次手术中进行腘绳肌腱转移。术前和术后随访时,通过测量手-前臂角度、手-前臂位置和尺弓来评估桡骨纵向缺损。通过术前和术后随访测量角度来评估膝关节挛缩。结果:患者术前评估、术后早期、末次随访时手前臂角均值分别为82.60、5、80、11.10 (P < 0.001)。手-前臂位置值分别为-14.25 mm、+11 mm和+7.1 mm (P < 0.001)。尺弓值分别为7.3°、4.5°和2.9°(P < 0.001)。复发的桡骨纵向缺陷畸形需要手术发生1例。在其他3例患者中,一些畸形复发,但不需要手术干预。此外,1例膝关节屈曲挛缩患者挛缩复发,不需要手术干预。患者下肢和上肢均无循环障碍或皮肤坏死。结论:两期治疗是治疗下肢和上肢畸形伴无桡骨血小板减少综合征的可靠方法。然而,复发仍然是一个主要问题。证据等级:IV级,治疗性研究。
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Two-stage treatment of extremity deformities associated with thrombocytopenia-absent radius syndrome.

Objective: The aim of this study was to evaluate the results of 2-stage treatment of upper and lower extremity deformities in patients with thrombocytopenia absent radius syndrome.

Methods: Four patients (3 female, 1 male) with a mean age of 1.8 years (range 1-4) were included in the study. The patients were followed up for an average of 5.5 years. All 4 patients had bilateral radial longitudinal deficiency, whereas only 2 patients had bilateral fixed knee contractures. A 2-stage surgical procedure was implemented. The surgical procedure performed for radial longitudinal deficiency consisted of distraction with an Ilizarov frame in the first stage, followed by centralization performed in the second stage. Knee contractures were first treated using an Ilizarov frame, followed by a hamstring tendon transfer in the second procedure. Radiological evaluation of the radial longitudinal deficiency was done by measuring hand-forearm angle, hand-forearm position, and ulnar bowing preoperatively and at postoperative follow-ups. Knee contracture was evaluated by measuring the angle preoperatively and at postoperative follow-ups.

Results: The mean hand-forearm angle values of patients at preoperative assessment, early postoperative period, and at the last follow-ups were 82.60, 5,80, and 11.10, respectively (P < .001). The hand-forearm position values were -14.25 mm, +11, and +7.1 mm, respectively (P < .001). The ulnar bowing values were 7.3°, 4.5°, and 2.9°, respectively (P < .001). Recurrence of the radial longitudinal deficiency deformity requiring surgery occurred in 1 patient. In the other 3 patients, some deformity recurred but did not require surgical intervention. In addition, 1 patient with knee flexion contracture had a recurrence of the contracture that did not require surgical intervention. There was no circulatory disorder or skin necrosis in the lower or upper extremities of the patients.

Conclusion: This study has shown us that two-stage treatment is a reliable method for lower and upper extremity deformities accompanying thrombocytopenia absent radius syndrome. However, recurrence is still a major problem.

Level of evidence: Level IV, Therapeutic Study.

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来源期刊
CiteScore
2.00
自引率
0.00%
发文量
66
审稿时长
>12 weeks
期刊介绍: Acta Orthopaedica et Traumatologica Turcica (AOTT) is an international, scientific, open access periodical published in accordance with independent, unbiased, and double-blinded peer-review principles. The journal is the official publication of the Turkish Association of Orthopaedics and Traumatology, and Turkish Society of Orthopaedics and Traumatology. It is published bimonthly in January, March, May, July, September, and November. The publication language of the journal is English. The aim of the journal is to publish original studies of the highest scientific and clinical value in orthopedics, traumatology, and related disciplines. The scope of the journal includes but not limited to diagnostic, treatment, and prevention methods related to orthopedics and traumatology. Acta Orthopaedica et Traumatologica Turcica publishes clinical and basic research articles, case reports, personal clinical and technical notes, systematic reviews and meta-analyses and letters to the Editor. Proceedings of scientific meetings are also considered for publication. The target audience of the journal includes healthcare professionals, physicians, and researchers who are interested or working in orthopedics and traumatology field, and related disciplines.
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