Case-matched comparative analysis of spinal deformity correction in arthrogryposis multiplex congenita versus adolescent idiopathic scoliosis

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OBJECTIVE

As scoliosis in arthrogryposis multiplex congenita (AMC) is unusual and the number of cases reviewed in previous studies is also relatively small, no previous study exists that has directly compared the results of spinal deformity correction between AMC and adolescent idiopathic scoliosis (AIS) patients. The aim of this study was to compare the radiographic and clinical outcomes of surgical correction of spinal deformity associated with AMC versus AIS.

METHODS

Twenty-four adolescents with AMC were matched with 48 AIS patients in terms of Cobb angle of main curve, curve pattern, sex, age at surgery, Risser grade, and length of follow-up. Patients in both groups underwent posterior-only spinal correction and fusion procedures. The surgical outcomes and complications were analyzed and compared between the 2 groups.

RESULTS

In comparison to the AIS group, the AMC group had a significantly longer mean operation time (5.6 vs 4.4 hours, p = 0.002), more blood loss (1620 ± 250 ml vs 840 ± 260 ml, p < 0.001), and more fusion levels (14.1 ± 2.3 levels vs 12.4 ± 2.5 levels, p = 0.007) as well as a lower correction rate (44.3% ± 11.1% vs 70.8% ± 12.4%, p < 0.001) and a higher rate of loss of correction (5.0% ± 3.1% vs 2.1% ± 1.9%, p < 0.001). Nine patients in the AMC group had preoperative pelvic obliquity, which was corrected from a mean of 14.2° ± 8.4° to a mean of 4.3° ± 3.2° (p < 0.001) after the surgery. The thoracic lordosis and sagittal vertical axis were significantly improved in the AMC group. Notably, however, the AMC group was found to have higher rates of screw malpositioning (15.9% vs 9.5%, p = 0.002) and complications (8/24 [33.3%] vs 4/48 [8.3%], p = 0.016) as compared to the AIS group.

CONCLUSIONS

Correction of AMC-associated scoliosis tends to require a longer operating time and involve more fusion levels but results in less correction, more blood loss, and more complications, in comparison with AIS. In addition, more attention should be paid to pelvic obliquity and sagittal hyperlordosis in AMC patients.

ABBREVIATIONS AIS = adolescent idiopathic scoliosis; AMC = arthrogryposis multiplex congenita; C7PL = C7 plumb line; CSVL = center sacral vertical line; SRS = Scoliosis Research Society; SVA = sagittal vertical axis.

Article Information

Correspondence Xu Sun: Drum Tower Hospital, Nanjing University Medical School, Nanjing, China. drsunxu@163.com.

INCLUDE WHEN CITING Published online October 12, 2018; DOI: 10.3171/2018.7.PEDS18347.

L.X. and Z.C. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Representative case of AMC-associated scoliosis. This boy presented at the age of 14 years with AMC complicated with scoliosis. As shown in the photographs obtained before surgery (A), he had elbow and knee contractures. Preoperative anteroposterior and lateral radiographs (B) showed severe scoliosis in the coronal plane as well as a hyperlordotic lumbar spine in the sagittal plane. Ponte osteotomies were performed from T6 to T11 and fusion from T2 to L3 (C). Postoperatively, the coronal Cobb angle of the major curve decreased from 75° to 43°, resulting in significant improvement of his sagittal alignment. Follow-up radiographs obtained 2.5 years after surgery (D) showed that the sagittal alignment was well maintained, although some loss of scoliosis correction was noted in the coronal plane. Figure is available in color online only.

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    Representative case of AIS. This girl presented at the age of 13 years with AIS (preoperative radiographs, A). She was treated with posterior instrumentation and fusion from T4 to L2 (B). Comparison of preoperative and postoperative radiographs showed that the coronal Cobb angle of the major curve decreased from 77° to 22°. Follow-up radiographs obtained 2 years after surgery (C) showed good maintenance of both coronal and sagittal alignment.

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