Posterior spinal fusion in patients with Ehlers–Danlos syndrome: A report of six cases

Rabenhorst, Brien M; Garg, Sumeet; Herring, John A.

doi:10.1007/s11832-012-0393-3

Cited by 21 publications

(15 citation statements)

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“…All 3 articles emphasized blood loss measurements, which averaged 1272 mL intraoperatively. One reported death postoperatively from vascular compromise was discussed as well [ 24 , 26 , 27 ].…”

Section: Resultsmentioning

confidence: 99%

“…Jasiewicz et al [ 26 ] found that 4 of 11 patients treated for their scoliosis required reoperation secondary to imbalance and instrumentation failure. Rabenhorst et al [ 27 ], looked at 6 patients with Ehlers-Danlos, reported that one patient died following PSF secondary to intra-abdominal bleeding. The authors also noted the need for removal of hardware over one year postoperatively secondary to infection in two patients [ 27 ].…”

Section: Resultsmentioning

confidence: 99%

“…Rabenhorst et al [ 27 ], looked at 6 patients with Ehlers-Danlos, reported that one patient died following PSF secondary to intra-abdominal bleeding. The authors also noted the need for removal of hardware over one year postoperatively secondary to infection in two patients [ 27 ].…”

Section: Resultsmentioning

confidence: 99%

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Complications associated with surgical repair of syndromic scoliosis

et al. 2015

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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.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Complications associated with surgical repair of syndromic scoliosis

et al. 2015

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“…Furthermore, meticulous hemostasis is needed in these patients [1]. Characteristically, posterior spinal fusion for scoliosis has been associated with excessive bleeding [14,17], leading to death in a patient with postoperative intraabdominal bleeding [17]. Although surgical closure might be complicated by tearing of the skin edges by the sutures, careful multilayered closure with nylon sutures and wound closure strips instead of staples can prevent possible future wound hematoma, wound dehiscence, and resulting infections [21].…”

Section: Discussionmentioning

confidence: 99%

Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations

Grigoriou

Boris

Dormans

2015

Clinical Orthopaedics &Amp; Related Research

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Background Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware. Case Description We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin. Literature Review POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation. Clinical Relevance These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

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“…Rabenhorst et al [12] сообщили о шести оперированных (средний возраст 14 лет), причем во всех случа-ях диагноз подтвержден результатами генетической экспертизы. Применяли различные варианты дорсального вер-тебрального инструментария, интрао-перационный мониторинг спинного мозга проводили в большинстве слу-чаев.…”

Section: рисunclassified

Surgical Correction of Spinal Deformities in Patients With Ehlers-Danlos Syndrome

Михайловский¹,

Новиков²,

Васюра³

et al. 2018

Hir. pozvonoč.

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Objective. To analyze surgical treatment results in patients with severe progressive spinal deformities owing to EhlersDanlos syndrome. Material and Methods. A total of nine patients with spinal deformities owing to Ehlers-Danlos syndrome were operated on during the period of 2003-2013. Mean age of patients was 12.5 years. One patient undergoes multistage treatment with VEPTR instrumentation, and eight patients underwent corrective posterior surgery using third-generation segmental instrumentation. Posterior correction was preceded by anterior stage including discectomy and interbody fusion with bone autograft in six cases. Results. The mean primary curve was 95.2° before surgery, 50.9° immediately after surgery, and 59.1° at final followup. The kyphotic component was 93.7°, 53.9°, and 59.6°, respectively. The coronal trunk imbalance was 28.3 mm before surgery, 39.4 mm immediately after surgery, and 22.0 mm at final follow-up. Complications were observed in three patients: right-sided pneumothorax in one case, and instability of the cranial anchor of instrumentation system, which required reoperation in two cases. There were neither pyogenic or neurologic complications, nor massive bleeding. Conclusion. Spinal deformities caused by Ehlers-Danlos syndrome are rare, though rapidly progressive disorders, which attain significant magnitude. Timely surgical treatment allows achieving satisfactory correction of both scoliosis and kyphosis, though can cause vascular and neuriological complications, especially with the anterior approach.

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Posterior spinal fusion in patients with Ehlers–Danlos syndrome: A report of six cases

Abstract: The fragility of the EDS patient population cannot be overlooked. Despite a conservative surgical approach, half of our patients experienced complications. The surgeon choosing to operate on EDS patients must do so with extreme caution.

Cited by 21 publications

References 7 publications

Complications associated with surgical repair of syndromic scoliosis

Complications associated with surgical repair of syndromic scoliosis

Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations

Surgical Correction of Spinal Deformities in Patients With Ehlers-Danlos Syndrome

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