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      Complications of Posterior Fusion for Atlantoaxial Instability in Children With Down Syndrome

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          Abstract

          Objective

          To clarify the complications of posterior fusion for atlantoaxial instability (AAI) in children with Down syndrome and to discuss the significance of surgical intervention.

          Methods

          Twenty pediatric patients with Down syndrome underwent posterior fusion for AAI between February 2000 and September 2018 (age, 6.1±1.9 years). C1–2 or C1–3 fusion and occipitocervical fusion were performed in 14 and 6 patients, respectively. The past medical history, operation time, estimated blood loss (EBL), duration of Halo vest immobilization, postoperative follow-up period, and intra- and perioperative complications were examined.

          Results

          The operation time was 257.9±55.6 minutes, and the EBL was 101.6±77.9 mL. Complications related to the operation occurred in 6 patients (30.0%). They included 1 major complication (5.0%): hydrocephalus at 3 months postoperatively, possibly related to an intraoperative dural tear. Other surgery-related complications included 3 cases of superficial infections, 1 case of bone graft donor site deep infection, 1 case of C2 pedicle fracture, 1 case of Halo ring dislocation, 1 case of pseudoarthrosis that required revision surgery, and 1 case of temporary neurological deficit after Halo removal at 2 months postoperatively. Complications unrelated to the operation included 2 cases of respiratory infections and 1 case of implant loosening due to a fall at 9 months postoperatively.

          Conclusion

          The complication rate of upper cervical fusion in patients with Down syndrome remained high; however, major complications decreased substantially. Improved intra- and perioperative management facilitates successful surgical intervention for upper cervical instability in pediatric patients with Down syndrome.

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          Most cited references35

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          Posterior C1-C2 fusion with polyaxial screw and rod fixation.

          A novel technique of atlantoaxial stabilization using individual fixation of the C1 lateral mass and the C2 pedicle with minipolyaxial screws and rods is described. In addition, the initial results of this technique on 37 patients are described. To describe the technique and the initial clinical and radiographic results for posterior C1-C2 fixation with a new implant system. Stabilization of the atlantoaxial complex is a challenging procedure because of the unique anatomy of this region. Fixation by transarticular screws combined with posterior wiring and structural bone grafting leads to excellent fusion rates. The technique is technically demanding and has a potential risk of injury to the vertebral artery. In addition, this procedure cannot be used in the presence of fixed subluxation of C1 on C2 and in the case of an aberrant path of the vertebral artery. To address these limitations, a new technique of C1-C2 fixation has been developed: bilateral insertion of polyaxial-head screws in the lateral mass of C1 and through the pars interarticularis into the pedicle of C2, followed by a fluoroscopically controlled reduction maneuver and rod fixation. After posterior exposure of the C1-C2 complex, the 3.5-mm polyaxial screws are inserted in the lateral masses of C1. Two polyaxial screws are then inserted into the pars interarticularis of C2. Drilling is guided by anatomic landmarks and fluoroscopy. If necessary, reduction of C1 onto C2 can be accomplished by manipulation of the implants, followed by fixation to the 3-mm rod. For definitive fusion, cancellous bone can be added. No structural bone graft or wiring is required. In selected cases, e.g., C1-C2 subluxation or fractures in young patients in whom only temporary fixation is necessary, the instrumentation can be removed after an appropriate time. Because the joint surfaces stay intact, the patient can regain motion in the C1-C2 joints. Thirty-seven patients underwent this procedure. No neural or vascular damage related to this technique has been observed. The early clinical and radiologic follow-up data indicate solid fusion in all patients. Fixation of the atlantoaxial complex using polyaxial-head screws and rods seems to be a reliable technique and should be considered an efficient alternative to the previously reported techniques.
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            Intrinsic abnormalities of lymphocyte counts in children with down syndrome.

            Down syndrome (DS) is associated with an increased frequency of infections, hematologic malignancies, and autoimmune diseases, suggesting that immunodeficiency is an integral part of DS that contributes significantly to the observed increased morbidity and mortality. We determined the absolute counts of the main lymphocyte populations in a large group of DS children to gain further insight into this immunodeficiency. In a large group of children with DS (n = 96), the absolute numbers of the main lymphocyte subpopulations were determined with 3-color immunophenotyping using the lysed whole-blood method. The results were compared with previously published data in healthy children without DS. In healthy children with DS, the primary expansion of T and B lymphocytes seen in healthy children without DS in the first years of life was severely abrogated. The T- lymphocyte subpopulation counts gradually reached more normal levels with time, whereas the B- lymphocyte population remained severely decreased, with 88% of values falling below the 10th percentile and 61% below the 5th percentile of normal. The diminished expansion of T and B lymphocytes strongly suggests that a disturbance in the adaptive immune system is intrinsically present in DS and is not a reflection of precocious aging. Thymic alterations have been described in DS that could explain the decreased numbers of T lymphocytes, but not the striking B lymphocytopenia, seen in these children.
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              Primary posterior fusion C1/2 in odontoid fractures: indications, technique, and results of transarticular screw fixation.

              Odontoid fractures, especially unstable type II fractures have a poor prognosis in respect to healing. Therefore, operative stabilization (posterior fusion C1/2 or anterior screw fixation) has been suggested for the treatment of unstable type II and for some unstable type III fractures. Compared to posterior fusion C1/2, anterior screw fixation has proven to be effective; it has the advantage of leaving the motion segment C1/2 intact, therefore preserving at least some C1/2 rotation. However, in some instances, this method of stabilization is not indicated. In these cases, posterior fusion C1/2 is the treatment of choice. Primary posterior fusion C1/2 is indicated in (a) odontoid fracture associated with comminution of one or both atlanto-axial joints; (b) fracture of the odontoid associated with an unstable Jefferson fracture; (c) unstable type III odontoid fracture, when immobilization in a halo jacket or plaster cast is not suitable, as in elderly people or polytraumatized patients; (d) atypical type II fractures (comminuted or with oblique fracture in the frontal plane); (e) irreducible fracture dislocation C1/2, e.g., several-weeks-old fracture; (f) unstable type II or shallow and unstable type III odontoid fracture, when marked thoracic kyphosis is associated with limited extension of the cervical spine; (g) unstable type II or shallow type III odontoid fracture in elderly people with degenerative narrow spinal canal; (h) pathologic fracture of the odontoid. In all these instances, posterior fusion C1/2 is the treatment of choice. We prefer the transarticular screw fixation technique. Compared to other posterior fusion techniques, it has the advantage of increased stability and allows effective stabilization of C1/2 in a reduced position as well as immediate ambulation with minimal head support. This technique can also be performed when the posterior arch of the atlas is fractured or absent. Our experience of 12 acute odontoid fractures, managed by this technique, is presented. At follow-up, all C1/2 fusions were united in reduced position.
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                Author and article information

                Journal
                Neurospine
                Neurospine
                NS
                Neurospine
                Korean Spinal Neurosurgery Society
                2586-6583
                2586-6591
                December 2021
                31 December 2021
                : 18
                : 4
                : 778-785
                Affiliations
                [1 ]Department of Orthopaedic Surgery, Kobe University Graduate School of Medicine, Kobe, Japan
                [2 ]Department of Orthopaedic Surgery, National Hospital Organization Kobe Medical Center, Kobe, Japan
                [3 ]Department of Orthopaedic Surgery, Kindai University Hospital, Osaka-Sayama, Japan
                [4 ]Department of Orthopaedic Surgery, Kobe Children’s Hospital, Kobe, Japan
                Author notes
                Corresponding Author Yoshiki Takeoka https://orcid.org/0000-0001-5360-5618 Department of Orthopaedic Surgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan Email: yoshiki_tkk@ 123456hotmail.com
                Author information
                http://orcid.org/0000-0001-5360-5618
                Article
                ns-2142720-360
                10.14245/ns.2142720.360
                8752718
                35000332
                57d45b45-5aa8-49ce-aa4a-228b5223d4b7
                Copyright © 2021 by the Korean Spinal Neurosurgery Society

                This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 23 July 2021
                : 8 September 2021
                : 20 October 2021
                Categories
                Original Article

                pediatric down syndrome,surgical complication,atlantoaxial instability,posterior fusion,atlantodental interval,cervical spine

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