Efficacy and Surgical Complications in the Treatment of Scoliotic Patients with Ehlers-Danlos Syndrome: A Literature Review Efficacy and Surgical Complications in with A

Objective: To compile the current knowledge of the surgical approach performed in the treatment of scoliosis in Ehlers-Danlos patients. Summary of Background Data: Ehlers-Danlos syndrome (EDS) has a low incidence in the population and is often associated with the development of scoliosis during the growth. Few articles are reported in the literature describing the effectiveness and the risks associated with the surgical treatment of scoliosis in EDS patients. Such approach has been shown to increase life expectancy but is largely controversial because of the high rate of complications and morbidity. Due to the lack of knowledge about this disease, appropriate recommendations are needed to propose an efficient approach for this kind of surgery. Methods: A literature search was conducted using database. The Medical Subject Headings keywords used in this research were “Ehlers-Danlos syndrome” associated with a combination of the following terms “spine”, “scoliosis” or “ktphoscoliosis”, “spinal fusion”, ‘spinal surgery”, “surgery”. Articles reporting the surgical management of scoliosis in EDS patients and the associated were selected and analyzed individually. Results: On 10 articles reviewed, 9 were mentioning surgical complications. These articles were about 41 patients. Orthopedic treatment has failed, with a rapid degradation of scoliosis in puberty in all cases. A stable arthrodesis has been achieved in 35 of 41 patients, which represents a rate of surgical success of 90%. Vascular lesions occurred in 13 of 41 patients (33%). Paraplegia occurred in 5 patients, which represents 12%. There has been high rate of blood loss in every surgery performed, with an average of 3101 mL. Conclusion: Surgery of scoliosis in EDS patients gives encouraged results, with a good correction of deformity and a stable fusion. However, it presents a high risk of neurological and vascular complications. Some precautions are mandatory for the surgery. the Treatment of Scoliotic Patients Ehlers-Danlos Syndrome: Literature

the 1660s, other cases of patients with skin hyperextensibility and abnormal flexibility were presented byJob Janszoon van Meek'ren to the Leyde Academy. Before and during the late nineteenth century, many cases of patients with such cutaneous and musculoskeletal features were observed and considered as performers around the world [1]. In the 20 th century, 2 physicians, Danish dermatologist Edward Lauritz Ehlers, and French dermatologist Henri-Alexandre Danlos, were the first to officially describe and publish a review of patients presenting skin hyperextensibility, joints laxity and skin fragility. As an evidence, the syndrome was rapidly named Ehlers-Danlos Syndrome by the scientific society. Many groups of patients were then observed and categorized depending on the severity of their joints mobility, skin laxity and musculoskeletal conditions. Classification of EDS syndrome was proposed in the 1960s with nine subcategories. Since 1997 the new nosology of Villefranche distinguishes 6 types of EDS presented in (Table 1) [2]. Table 1: Classification of EDS according to the nosology of Villefranche (Beighton et al., 1998).

Results
According to our research criteria only 10 articles published within the last 20 years reporting the surgical management of spinal deformities in EDS patients were selected. Most of these publications refer to complications associated with the surgical treatment of kyphoscoliosis in EDS patients (n = 9) and report 41 cases of operated patients. The mean age of the global population was 13 years old with respectively 13 males and 28 females ( Table   2). Ehlers-Danlos syndrome has a low prevalence (from 1 to 9 cases per 100 000 newborns) making it a rare disease and limits the number of reported cases. Therefore few articles have been written about spinal arthrodesis in scoliotic EDS patients . In

Efficacy of Surgical Treatment
In this literature review, most of the patients had an orthopedic treatment during childhood, at the time when they started walking.

Neurological Complications
Literature suggests that EDS patients have a higher risk of neurological complications. Given the few cases reported, the rate of such complications is difficult to evaluate even if it seems to be higher than 0,7% of the iatrogenic paralysis in classic scoliosis surgery in the global population. Paraplegia occurred in 5 patients, which represents 12% of the population (Table 3). Vogel et al.
suggests that type IV EDS patients have a higher risk to develop several neurological complications due to the laxity of bones and muscles, which allows a higher correction of deformation, more than the vascular and neurologic tissue can support [6]. It is also supposed that EDS patients have a higher risk of scoliosis angulation than the other scoliotic patients, which induces a higher risk of neurological complications.

Choice of Surgical Approach and Technique
In non-EDS patients, lumbar anterior approach has a low risk of vascular complications. But due to vessels fragility, EDS patients are often exposed to vascular injuries. Yang et al.  Table 4: Bloss loss associated with surgical treatment of scoliotic EDS patients.

Discussion
Cases

Conclusion
Spinal surgery for scoliotic EDS patients demonstrated good clinical results, with a good correction of deformity and a stable fusion. This is supposed to be a best way to extant the quality of life of EDS patients. However, this kind of treatment presents a high risk of neurological and vascular injuries. Some precautions are mandatory before this surgery.