Complications associated with surgical repair of syndromic scoliosis.

Benjamin J Levy,Eric D Fornari,Jacob F Schulz,Adam L Wollowick

doi:10.1186/s13013-015-0035-x

Benjamin J Levy, Eric D Fornari + Show 2 more

Open Access

https://doi.org/10.1186/s13013-015-0035-x

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Abstract

BackgroundThere are a number of syndromes that have historically been associated with scoliosis e.g.: Marfan, Down, and Neurofibromatosis. These syndromes have been grouped together as one etiology of scoliosis, known as syndromic scoliosis. While multiple studies indicate that these patients are at high risk for perioperative complications, there is a paucity of literature regarding the collective complication rates and surgical needs of this population.MethodsPubMed and Embase databases were searched for literature encompassing the surgical complications associated with the surgical management of patients undergoing correction of scoliosis in the syndromic scoliosis population. Following exclusion criteria, 24 articles were analyzed for data regarding these complications.ResultsThe collective complication rates and findings of these articles were categorized based on specific syndrome. The rates and types of complications for each syndrome and the special needs of patients with each syndrome are discussed. Several complication trends of note were observed, including but not limited to the universally nearly high rate of wound infections (>5% in each group), high rate of pulmonary complications in patients with Rett syndrome (29.2%), high rate (>10%) of dural tears in Marfan and Ehlers-Danlos syndrome patients, high rate (>20%) of implant failure in Down and Prader-Willi syndrome patients, and high rate (>25%) of pseudarthrosis in Down and Ehlers-Danlos patients.ConclusionsThough these syndromes have been classically grouped together under the umbrella term “syndromic,” there may be specific needs for patients with each of these ailments. Given the high rate of complications, further research is necessary to understand the unique needs for each of these patient groups in the preoperative, intraoperative, and postoperative settings.

Highlights

Scoliosis in the pediatric population is traditionally classified as idiopathic, congenital, neuromuscular, or syndromic in etiology
Syndromic scoliosis is generally recognized as scoliosis that is associated with a systemic disease
Diseases associated with scoliosis include, but are not limited to, Down syndrome, Marfan syndrome, neurofibromatosis, Rett syndrome, achondroplasia, EhlersDanlos syndrome, Prader-Willi syndrome, Friedrich’s ataxia, and Osteogenesis Imperfecta

Summary

Introduction

Scoliosis in the pediatric population is traditionally classified as idiopathic, congenital, neuromuscular, or syndromic in etiology. Diseases associated with scoliosis include, but are not limited to, Down syndrome, Marfan syndrome, neurofibromatosis, Rett syndrome, achondroplasia, EhlersDanlos syndrome, Prader-Willi syndrome, Friedrich’s ataxia, and Osteogenesis Imperfecta. In these conditions, scoliosis occurs at a rate significantly greater than that of the population at large. The surgical management of scoliosis is associated with significant complications including blood loss, infection, and neurologic compromise. It has previously been demonstrated that patients with non-idiopathic scoliosis, especially neuromuscular, have higher rates of infection, blood loss during surgery, and subsequent need for transfusion, than idiopathic scoliosis patients [1]. There are a number of syndromes that have historically been associated with scoliosis e.g.: Marfan, Down, and Neurofibromatosis. While multiple studies indicate that these patients are at high risk for perioperative complications, there is a paucity of literature regarding the collective complication rates and surgical needs of this population

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