Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage

Rumbak, Dania M.; Mowrey, Wenzhu; Schwartz, Sandra Kasper; Sarwahi, Vishal; Djukic, Aleksandra; Killinger, James S.; Katyal, Chhavi

doi:10.1177/0883073815585352

Cited by 13 publications

(17 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In fact, a study comparing patients with RTT and cerebral palsy showed that the incidence of respiratory failure and prolonged use of positive pressure ventilation were significantly more common in RTT girls after posterior spinal fusion [96]. Similar results were obtained in a previous report investigating the same parameters following surgical treatment for scoliosis in RTT girls and patients affected by adolescent idiopathic scoliosis and neurologic scoliosis [97]. RTT patients undergoing spine surgery were also evaluated in another study aimed at identifying the perioperative adverse events associated with the surgical procedure [98].…”

Section: Interactions Of Bone With Other Organ Systems In Rttsupporting

confidence: 80%

Altered Bone Status in Rett Syndrome

Pecorelli

Cordone

Schiavone

et al. 2021

Life

View full text Add to dashboard Cite

Rett syndrome (RTT) is a monogenic neurodevelopmental disorder primarily caused by mutations in X-linked MECP2 gene, encoding for methyl-CpG binding protein 2 (MeCP2), a multifaceted modulator of gene expression and chromatin organization. Based on the type of mutation, RTT patients exhibit a broad spectrum of clinical phenotypes with various degrees of severity. In addition, as a complex multisystem disease, RTT shows several clinical manifestations ranging from neurological to non-neurological symptoms. The most common non-neurological comorbidities include, among others, orthopedic complications, mainly scoliosis but also early osteopenia/osteoporosis and a high frequency of fractures. A characteristic low bone mineral density dependent on a slow rate of bone formation due to dysfunctional osteoblast activity rather than an increase in bone resorption is at the root of these complications. Evidence from human and animal studies supports the idea that MECP2 mutation could be associated with altered epigenetic regulation of bone-related factors and signaling pathways, including SFRP4/WNT/β-catenin axis and RANKL/RANK/OPG system. More research is needed to better understand the role of MeCP2 in bone homeostasis. Indeed, uncovering the molecular mechanisms underlying RTT bone problems could reveal new potential pharmacological targets for the treatment of these complications that adversely affect the quality of life of RTT patients for whom the only therapeutic approaches currently available include bisphosphonates, dietary supplements, and physical activity.

show abstract

Section: Interactions Of Bone With Other Organ Systems In Rttsupporting

confidence: 80%

Altered Bone Status in Rett Syndrome

Pecorelli

Cordone

Schiavone

et al. 2021

Life

View full text Add to dashboard Cite

show abstract

“…The literature does not contain any studies which evaluate the corrections possible in Rett syndrome. This may be because of its rarity, or the technical challenges which have historically been present in managing these patients, the perioperative morbidity, and difficulty in monitoring their neurological status during the procedures [21,22].…”

Section: Discussionmentioning

confidence: 99%

Correcting Scoliosis in Rett Syndrome

Rocos¹,

Zeller²

2021

Cureus

View full text Add to dashboard Cite

Rett syndrome is a rare disorder characterised by severe scoliosis in 80% of cases. In this retrospective case series, we analysed the radiographic, clinical, and functional outcomes of consecutive patients treated for scoliosis associated with Rett syndrome. We sought to understand the results of the treatment of scoliosis in Rett syndrome and evaluate the need to fuse to the pelvis. MethodsA retrospective case series was used to analyse the radiographic, clinical, and functional outcomes of consecutive patients treated for Rett syndrome scoliosis between the ages of 10 and 8 years in a single tertiary paediatric spinal unit. Cases were identified through departmental and neurophysiological records, and patients were excluded if the diagnosis of Rett syndrome was not confirmed. ResultsSeven eligible cases were identified. At presentation, the mean coronal Cobb angle was 90.9°, mean sagittal Cobb 72.0°, and pelvic obliquity 24.5°. The mean post-operative improvement in coronal Cobb was 53.2° and pelvic obliquity reduced to 5.8°. These did not change during a mean follow up of 3.5 years. None showed any post-operative complications. Three patients with a mean 16.1° pelvic obliquity underwent a fusion to L5. The postoperative result in those cases remained stable at 3.5 years mean follow-up and full skeletal maturity. ConclusionOur data suggests that with modern technology, severe curves can be safely treated. Fusion to the pelvis is not necessary in patients with mild, flexible pelvic obliquity.

show abstract

“…There were 133 patients with adolescent idiopathic scoliosis, 48 patients with neurologic scoliosis, and 8 patients with Rett syndrome. They found that patients with Rett syndrome undergoing scoliosis surgery have higher rates of respiratory failure and longer ventilation times in the postoperative period when compared with both adolescent idiopathic scoliosis and neurologic scoliosis patients [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Minimally Invasive Scoliosis Surgery: A Novel Technique in Patients with Neuromuscular Scoliosis

Sarwahi

Amaral

Wendolowski

et al. 2015

BioMed Research International

Self Cite

View full text Add to dashboard Cite

Minimally invasive surgery (MIS) has been described in the treatment of adolescent idiopathic scoliosis (AIS) and adult scoliosis. The advantages of this approach include less blood loss, shorter hospital stay, earlier mobilization, less tissue disruption, and relatively less pain. However, despite these significant benefits, MIS approach has not been reported in neuromuscular scoliosis patients. This is possibly due to concerns with longer surgery time, which is further increased due to more levels fused and instrumented, challenges of pelvic fixation, size and number of incisions, and prolonged anesthesia. We modified the MIS approach utilized in our AIS patients to be implemented in our neuromuscular patients. Our technique allows easy passage of contoured rods, placement of pedicle screws without image guidance, partial/complete facet resection, and all standard reduction maneuvers. Operative time needed to complete this surgery is comparable to the standard procedure and the majority of our patients have been extubated at the end of procedure, spending 1 day in the PICU and 5-6 days in the hospital. We feel that MIS is not only a feasible but also a superior option in patients with neuromuscular scoliosis. Long-term results are unavailable; however, short-term results have shown multiple benefits of this approach and fewer limitations.

show abstract

Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage

Cited by 13 publications

References 22 publications

Altered Bone Status in Rett Syndrome

Altered Bone Status in Rett Syndrome

Correcting Scoliosis in Rett Syndrome

Minimally Invasive Scoliosis Surgery: A Novel Technique in Patients with Neuromuscular Scoliosis

Contact Info

Product

Resources

About