Correction of Congenital Kyphosis

Enguer Beraldo Garcia^1,2,3,5*, Liliane Faria Garcia^2,3, Enguer Beraldo Garcia Jr³, Marcos Felipe Camarinha^3,4, Roberto Garcia Gonçalves^2,5, Juliana Garcia Camarinha³, Eduardo Beraldo Garcia^2,5, Saulo Terror Giesbrecht^2,5 & Ramires Fahfe Sampaio Santos

¹Graduate in Master's and Doctorate, Federal University MG, Brazil
²Spine Group of the Santa Casa, Belo Horizonte, MG, Brazil
³Institute of the Vertebral Spine of Belo Horizonte, MG, Brazil
⁴Member of the Brazilian Society of Clinical Neurophysiology, Brazil
⁵Member of the Brazilian Society of the Spine, Brazil

*Correspondence to: Dr. Enguer Beraldo Garcia, Institute of the Vertebral Spine, Av. Francisco Sales, 427, Floresta, CEP: 30150220, Minas Gerais, Brazil.

Copyright © 2018 Dr. Enguer Beraldo Garcia, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Introduction
Bingold et al., in 1953, defined congenital kyphosis as a deformity consequent to the presence of one or more anomalous vertebrae, with an inclination of the vertebral spine anteriorly, determining an angular deformity with posterior convexity [1].

Lonstein, in 1999, defined congenital deformities of the spine as being an abnormal development of vertebrates present at birth. Thus, affected children tend to have a curvature observed much earlier than do patients with idiopathic scoliosis [2].

Lomberd et al., in 1938, described the first case of paraplegia resulting from spinal cord compression by congenital kyphosis, demonstrated by myelography [3].

Winter et al., in 1973, presented a radiological classification: Type I - partial or total absence of one or more vertebral bodies; Type II - lack of segmentation between vertebral bodies; Type III - the association of Type I and Type II defects [4].

Williams et al., in 2014, in association with fourteen experienced surgeons, performed a system of consensus classification for scoliosis and early onset. It is used to identify three main variables (greater scoliosis angle, etiology, kyphosis and an annual progression variable). In the end, the classification consisted of: congenital, neuromuscular, syndromic and idiopathic, (C, M, S, I), greater angle of scoliosis (1, 2, 3, or 4), kyphosis (-, N, or +, and annual progression modifier (P0, P1, P2). This classification system would serve as a basis for guiding ongoing research efforts and standardizing communication in the clinical setting [5].

Winter et al., in 1985, presented a study of ninety-four patients submitted to surgical treatment of congenital kyphosis, which were reviewed. Those authors state that surgical intervention is best performed early to minimize the number of spinal levels requiring treatment. Early surgery includes excision of the hemivertebra, in which the abnormal vertebra is removed, allowing the spine to become almost completely stretched, and the adjacent portions of the spine to grow normally [6].

Feng et al., In 2016, point out that the main treatment for congenital scoliosis is the resection of the posterior hemivertebra with bilateral transpedicular fixation [7].

The Cobb technique measures the angle of the spine curvatures in the sagittal and coronal planes. The calculation of the angle between the lines, respectively traced, that touch the upper terminal plate of the cranial vertebra and the lower end plate of the caudal vertebra. The two lines perpendicular to these will meet to form the angle to be measured [8].

Objective
The objective of the present study is to evaluate the outcome of the surgical treatment of a patient with congenital kyphosis.

Materials and Methods
The specialists of the Vertebral Spine Institute and the Santa Casa Vertebral Spine Group, both in Belo Horizonte, Brazil, performed a case report, a 12-year-old female patient with severe congenital kyphosis, in a retrospective study. The case is part of a series of 29 patient operated in the Service with 3^rd generation instruments.

A radiographic study was carried out by means of panoramic views of the vertebral spine in orthostatic position in the posterior-anterior, profile incidences. The images were measured by the Cobb method in the pre and postoperative periods.

Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) were performed before and after surgery.

Performed thoracofrenectomy was performed on the right, the osteotomy was performed on T11 / T12 and the anterior arthrosis area of the deformity. After three weeks the T11 / T12 level was submitted to osteotomy, T7 to L5 was fixed, the spinal cord was monitored, the deformity was reduced, and arthrodesis was performed only from T10 to L2, and lastly, the area was reviewed and levels were closed.

In the immediate postoperative period, cervico-thoracolumbar orthotic orthosis was indicated for four months.

After six months of performing the procedures, the terminal ends of the instrumentation were removed, with the fixation being only T10 to L2.

In the images below, the severity of the reported case deformity and the procedures that have been performed can be evaluated, see figures 1 to 7.

Discussion
The authors state that there is a tendency in young children with congenital deformities of the spine not to receive the optimal treatment. These curves should not be permitted to get worse. In many cases, early fusion is necessary to avoid severe worsening of the deformity [2,10].

These authors recommend the treatment of congenital kyphosis only with the use of the posterior pathway, performing osteotomies, neurological decompression, shortening of the vertebral canal, arthrodesis and transpedicular fixations [9-17].

Other authors defend the association of the posterior access approach for vertebral release, discectomy, osteotomies, vertebral mobilization, 360º arthrodesis, and lengthening of the vertebral canal at the site of the anterior osteotomy, without disregarding the possibility of anterior access [19,20,21].

Congenital Kyphosis is difficult to solve, and the procedures come with high risk of neurological deficits, each case with its own peculiarities.

In the presence of severe congenital kyphoscoliosis with formation and follow-up defects, anterior access is performed or not. The posterior access is performed, applying osteotomy, instrumentation, and monitoring the spinal cord judiciously. At this proper time to reduce, fortunately, the present authors have always had positive results. Considering joint correction, for kyphosis and scoliosis, using rigid instrumentation, this is considered the really challenging moment of correction.

In more severe, rigid, deforming cases, the previous anterior approach to the spine, for soft tissue release, osteotomy, neurological decompression and arthrodesis. Followed by the posterior pathway with osteotomies, transpedicular fixation, neurological decompression and arthrodesis. This association of procedures facilitates spinal stretching, avoids greater exposure and manipulation of neurological structures, makes possible the alignment of the spine, and can offer a quality result, which the present authors obtain frequently in the service.

After six months of performing the procedures in the patient reported, the terminal ends of the instrumentation was removed, fixation was only maintained in the arthrodesed area of T11 to L2. Thus, it was possible to preserve greater vertebral mobility.

In the case reported, the combination of anterior and posterior access was performed, showing excellent evolution.

The patient was reassessed after 15 months of the correction, she was asymptomatic, with preserved spinal function and excellent vertebral mobility.

Conclusion
The association of the two surgical approaches in this case was an excellent option.

Bibliography

1. Bingold, A. C. (1953). Congenital kyphosis. J Bone Jt Surg., 35-B, 579-83.
2. Lonstein, J. E. (1999). Congenital spine deformities - Scoliosis, kyphosis, and lordoses. Orthopedic Clinics of North America, 30(3), 387-387.
3. Lomberd, P. & Le Genissel, M. (1938). Congenital Kyphoses. Rev Orthop., 25, 532-50.
4. Winter, R. B. & Wang, J. F. (1973). Congenital Kyphosis. lt's natural 2 history and treatment as observed in a study of 130 patients. J. Bone Jt Surg., 55(2), 223-56.
5. Williams, B. A., Matsumoto, H., McCalla, D. J., Akbarnia, B. A., Blakemore, L.C., Betz, R. R., et al. (2014). Development and Initial Validation of the Classification of Early-Onset Scoliosis (C-EOS). Journal of Bone and Joint Surgery - American Volume, 96(16), 1359-67.
6. Winter, R. B., Moe, J. H. & Lonstein, J. E. (1985). The surgical treatment of congenital kyphosis: A review of 94 patients age 5 years or older, with 2 years or more follow-up in 77 patients. Spine, 10(3), 224-231.
7. Feng, Y., Hai, Y., Zhao, S. & Zang, L. (2016). Hemivertebra resection with posterior unilateral intervertebral fusion and transpedicular fixation for congenital scoliosis: results with at least 3 years of follow-up. European Spine Journal, 25(10), 3274-3281.
8. Cobb, J. (1948). Outline for the study of scoliosis. Instr Course Lect., 5, 261.
9. Everitt, B. S. (1989). The analysis of contingency tables. London: Chapman and Hall.
10. Winter, R. B. & Moe, J. H. (1982). The results of spinal arthrodesis for congenital spine deformity in patients younger than 5 years old. J. Bone Jt Surg., 64(3), 419-32.
11. Feng, Y., Hai, Y., Zhao, S. & Zang, L. (2016). Hemivertebra resection with posterior unilateral intervertebral fusion and transpedicular fixation for congenital scoliosis: results with at least 3 years of follow-up. European Spine Journal, 25(10), 3274-3281.
12. Wang, S., Aikenmu, K., Zhang, J., Qiu, G., Guo, J., Zhang, Y. & Weng, X. (2017). The aim of this retrospective study is to evaluate the efficacy and safety of posterior-only vertebral column resection (PVCR) for the treatment of angular and isolated congenital kyphosis. European Spine Journal, 26(7), 1817 - 1825.
13. Chang, D. G., Yang, J. H., Lee, J. H., Kim, Y. H., Kim, J. H., Suh, S. W., Ha, K. Y. & Suk, S. I. (2016). Congenital Kyphoscoliosis in Monozygotic Twins: Ten-Year Follow-up Treated by Posterior Vertebral Column Resection (PVCR): A Case Report. Medicine, 95(17), 3499.
14. Spiro, A. S., Rupprecht, M., Stenger, P., Hoffman, M., Kunkel, P., Kolb, J. P., Rueger, J. M. & Stuecker, R. (2013). Surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach. Bone Joint J., 95-B(11), 1527-32.
15. Zeng, Y., Chen, Z., Qi, Q., Guo, Z., Li, W., Sun, C. & Liu, N. (2013). The posterior surgical correction of congenital kyphosis and kyphoscoliosis: 23 cases with minimum 2 years follow-up. European Spine Journal, 22(2), 372-8.
16. Atici, Y., Sökücü, S., Uzümcügil, O., Albayrak, A., Erdogan, S. & Kaygusuz, M. A. (2013). The results of closing wedge osteotomy with posterior instrumented fusion for the surgical treatment of congenital kyphosis. European Spine Journal, 22(6), 1368-74.
17. Bakaloudis, G., Lolli, F., Di Silvestre, M., Greggi, T., Astolfi, S., Martikos, K., Vommaro, F., Brodano, G. B., Cioni, A. & Giacomini, S. (2011). Thoracic pedicle subtraction osteotomy in the treatment of severe pediatric deformities. European Spine Journal, 20(Suppl. 1), 95-104.
18. Sugimoto, Y., Tanaka, M., Nakahara, R., Misawa, H., Kunisada, T. & Ozaki, T. (2012). Surgical Treatment for Congenital Kyphosis Correction Using Both Spinal Navigation and a 3-Dimensional Model. Acta Medica Okayama, 66(6), 499-502.
19. Akbar, M. & Wiedenhöfer, B. (2011). Correction of adolescent kyphosis: What is the state of the art? Orthopade., 40(8), 682-9.
20. Garrido, E., TomeBermejo, F., Tucker, S. K., Noordeen, H. N. N. & Morley, T. R. (2008). Short anterior instrumented fusion and posterior convex non-instrumented fusion of hemivertebra for congenital scoliosis in very young children. European Spine Journal, 17(11), 1507-1514.
21. Zhang, Z., Wang, H. & Liu, C. (2016). Compressive myelopathy in severe angular kyphosis: a series of ten patients. European Spine Journal, 25(6), 1897-1903.