Posterior thoracolumbar fusion in a patient with Kleefstra Syndrome related scoliosis: The first case reported.

Arthrodesis Kleefstra syndrome Scoliosis Spine deformities Spine surgery Surgical correction

Journal

Journal of clinical orthopaedics and trauma
ISSN: 0976-5662
Titre abrégé: J Clin Orthop Trauma
Pays: India
ID NLM: 101559469

Informations de publication

Date de publication:
Historique:
received: 06 06 2020
revised: 13 07 2020
accepted: 20 07 2020
entrez: 4 9 2020
pubmed: 4 9 2020
medline: 4 9 2020
Statut: ppublish

Résumé

Spine deformities could be considered a possible manifestation of the childhood hypotonia, typical feature of Kleefstra Syndrome (KS). There is a paucity of literature describing posterior spinal fusion in the Kleefstra syndrome patient. For patients who develop severe scoliotic curve, bracing is often ineffective and surgery is recommended. We report the first corrective surgery for scoliosis in one patient with KS. We describe a case of 13-year-old female with severe developmental scoliosis in KS. Preoperative examination showed a thoracolumbar scoliosis with left convex thoracic curve (T3-T9, 97°) and right convex thoracolumbar curve (T9-L3, 88°). Posterior correction, pedicle screw fixation and bone graft fusion T3-L5 was performed. Postoperatively, the thoracic curve was corrected to 33° while the thoracolumbar one to 26° and better standing posture was obtained. Six month follow-up images showed no loosening of the hardware. The patient is still in our follow-up program. Scoliosis seems to be a rare evenience of the severe hypotonia of patients with KS. We report the first case of scoliosis in KS treated successfully with surgery. Corrective surgery for spinal deformity, such as scoliosis, could help in posture and improve the quality of life especially in complicated patients such as syndromic ones.

Identifiants

pubmed: 32879587
doi: 10.1016/j.jcot.2020.07.017
pii: S0976-5662(20)30341-6
pmc: PMC7452239
doi:

Types de publication

Case Reports

Langues

eng

Pagination

956-960

Informations de copyright

© 2020 Delhi Orthopedic Association. All rights reserved.

Déclaration de conflit d'intérêts

The Authors declare that there is no conflict of interests.

Références

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Auteurs

Andrea Perna (A)

Istituto di Clinica Ortopedica, Fondazione Policlinico Universitario A. Gemelli, Catholic University, Rome, Italy.
Istituto di Clinica Ortopedica, Università Cattolica del Sacro Cuore, Rome, Italy.

Maria Beatrice Bocchi (MB)

Istituto di Clinica Ortopedica, Fondazione Policlinico Universitario A. Gemelli, Catholic University, Rome, Italy.
Istituto di Clinica Ortopedica, Università Cattolica del Sacro Cuore, Rome, Italy.

Luca Proietti (L)

Istituto di Clinica Ortopedica, Fondazione Policlinico Universitario A. Gemelli, Catholic University, Rome, Italy.
Istituto di Clinica Ortopedica, Università Cattolica del Sacro Cuore, Rome, Italy.

Classifications MeSH