Multistage surgical treatment of early-onset scoliosis in patients with Ehlers-Danlos syndrome: A series of observations

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BACKGROUND: Ehlers–Danlos syndrome (EDS) is a group of hereditary pathological conditions caused by various disorders of collagen biosynthesis. The study analyzed the results of multistage surgical treatment of early scoliosis in patients with severe spinal deformities due to EDS. No similar observations have been found in the literature.

CLINICAL CASES: Four patients with a verified diagnosis of EDS and progressive spinal deformities were subjected to multistage surgical treatment using the VEPTRII instrumentation, which included periodic distractions and “final” spinal fusion with segmental instrumentation. Stage-by-stage surgical treatment was initiated from the age of 3 to 6 years. In 3 of 4 cases, the kyphotic component prevailed over the scoliotic one (86°–140° vs. 21°–110°). The number of staged distractions ranged from 6 to 10. The age of the final stage (correction and dorsal fusion) was 9–14 years (surgery was performed in three of four cases). The primary correction was 30°–56°, the loss of correction before the final stage was 14°–35°, and the correction during the final stage was 22°–40°. A significant correction of the frontal and sagittal imbalances of the spine was noted. Blood loss during the “final” fusion was 540–750 mL, and the operation time was 310–350 min. Ten complications occurred, of which 9 were associated with implants and disappeared during staged distractions. No neurological and vascular complications occurred.

DISCUSSION: Scoliosis occurring in the first decade of life in patients with EDS is characterized by early-onset, rapid progression, and tendency to form a significant kyphotic component of spinal deformity.

CONCLUSIONS: Multistage treatment of early scoliosis in patients with EDS using VEPTRII tools allows for obtaining quite satisfactory results and has not severe complications. The “final” fusion gives a significant corrective effect; however, new research and accumulation of data are needed to optimize the treatment process.

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作者简介

Mikhail Mikhaylovskiy

Novosibirsk Research Institute of Traumatology & Orthopedics

编辑信件的主要联系方式.
Email: MMihailovsky@niito.ru
ORCID iD: 0000-0002-4847-100X
SPIN 代码: 5828-8306
Scopus 作者 ID: 57028305800
Researcher ID: C-5483-2017

MD, PhD, Dr. Sci. (Med.), Professor

俄罗斯联邦, Novosibirsk

Vasiliy Suzdalov

Novosibirsk Research Institute of Traumatology & Orthopedics

Email: vsuzdalov@mail.ru
ORCID iD: 0000-0003-2581-1638
SPIN 代码: 5287-6560
Scopus 作者 ID: 57203745429
Researcher ID: AAP-2266-2020

MD, PhD, Cand. Sci. (Med.)

俄罗斯联邦, Novosibirsk

参考

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2. Fig. 1. Spondylograms of patient P.: a, b — before surgical treatment (scoliosis — 71°, countercurvature — 53°, kyphosis — 86°, lumbar lordosis — smoothed, frontal imbalance to the right — 41 mm, sagittal imbalance — +6 mm); c, d — after primary correction (scoliosis — 44°, countercurvature — 41°, kyphosis — 56°, lumbar lordosis — 45°, frontal imbalance to the right — 4 mm, sagittal imbalance — +20 mm); e, f — before the “final” fusion (scoliosis — 72°, countercurvature — 69°, kyphosis — 76°, lumbar lordosis — 67°, frontal imbalance to the right — 24 mm, sagittal imbalance — +5 mm); g, h — after “final” fusion (scoliosis — 65°, countercurvature — 55°; kyphosis — 54°, lumbar lordosis — 36°, frontal imbalance to the right — 15 mm, sagittal imbalance — –12 mm)

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3. Fig. 2. Spondylograms of patient T.: a, b — before surgical treatment (scoliosis — 110°, kyphosis — 142°, lumbar lordosis — 71°, frontal imbalance to the left — 7 mm, sagittal imbalance — +5 mm); c, d — after primary correction (scoliosis — 75°, kyphosis — 104°, lumbar lordosis — 47°, frontal imbalance to the left — 34 mm, sagittal imbalance — +17 mm); e, f — before the “final” fusion (scoliosis — 96°, kyphosis — 139°, lumbar lordosis — 81°, frontal imbalance to the left — 29 mm, sagittal imbalance — +40 mm); g, h — after “final” fusion (scoliosis — 82°, kyphosis — 99°, lumbar lordosis — 83°, frontal imbalance to the left — 25 mm, sagittal imbalance — –12 mm)

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4. Fig. 3. Spondylograms of patient S.: a, b — before surgical treatment (scoliosis — 41°, countercurvature — 21°, kyphosis — 103°, lumbar lordosis — 66°, frontal imbalance to the right — 18 mm, sagittal imbalance — +10 mm) ; c, d — after primary correction (scoliosis — 33°, countercurvature — 16°, kyphosis — 47°, lumbar lordosis — 50°, frontal imbalance to the right — 14 mm, sagittal imbalance — –15 mm); e, f — before the “final” fusion (scoliosis — 56°, countercurvature — 49°, kyphosis — 77°, lumbar lordosis — 36°, frontal imbalance to the left — 5 mm, sagittal imbalance — +5 mm); g, h — after “final” fusion (scoliosis — 33°, countercurvature — 38°, kyphosis — 39°, lumbar lordosis — 48°, frontal imbalance to the left — 5 mm, sagittal imbalance — –5 mm)

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