Spinal Fusion in Patients With Classic Amyoplasia and General Arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita is a group of conditions characterized by joint contractures affecting 2 or more joints. This study describes results of spinal fusion in patients with classic amyoplasia and general arthrogryposis. METHODS: IRB approved retrospective review of patients with a diagnosis of classic amyoplasia and general arthrogryposis who had a primary definitive posterior spinal fusion between 1990 and 2017 at a single pediatric institution. Patients with distal and syndromic arthrogryposis were excluded as well as patients treated with growth-sparing spinal instrumentation. The Modified Clavien-Dindo-sink (MCDS) classification system was used to describe postoperative complications. RESULTS: Over the 28-year period, 342 patients were diagnosed with amyoplasia and general arthrogryposis. Among the 342 patients, 60 (18%) had scoliosis, and 22 (6% of the cohort and 37% of those with scoliosis) were treated surgically. Six patients had growth-sparing techniques, initial fusion elsewhere, or ˂1 year of follow-up, leaving 15 patients. Of the 15, 9 (60%) had a posterior spinal fusion (PSF) and 6 (40%) had a combined anterior spinal fusion (ASF)/PSF. The ASF/PSF group was significantly younger at surgery, had a greater American Society of Anesthesiologists status, longer surgery duration, and lower implant density. The average preoperative major coronal deformity in ASF/PSF patients (108 degrees) was greater than patients treated with PSF alone (88 deg). There were 11 complications in 7 patients, with the most common being deep infection requiring reoperation (5/11, 45%). There was 1 instance (1/11, 9%) of each: prolonged intensive care unit admission (>72 h), superficial wound dehiscence, symptomatic implants requiring removal/revision, coronal plane progression requiring extension of fusion, recurrent pneumothorax requiring return to OR, and pseudoarthrosis leading to implant failure (without revision). Complications occurred in 1/9 (11%) PSF-only patients and 6/6 (100%) ASF/PSF patients with all 6 ASF/PSF patients requiring at least 1 reoperation. The average coronal correction was 48% in the PSF-only group and 28% in the ASF/PSF group. CONCLUSION: Complication rates after spinal fusion for scoliosis in arthrogryposis multiplex congenita patients are high, especially in patients undergoing ASF/PSF, deep infection is common, and major coronal plane curve correction is modest. LEVEL OF EVIDENCE: II Retrospective Study.

Comparison of treatment outcomes between growth-sparing instrumentation and definitive one-stage fusion for EOS patients ages 6-10 years.

PURPOSE: To compare objective outcomes for EOS patients age 6-10 years treated by growth-sparing (GS) surgery or definitive one-stage correction and fusion (DF). METHODS: We reviewed surgical, radiographic, PFT's, and EOSQ-24 outcomes for EOS patients > age 6 at index surgery treated at a single institution, minimum 2-year follow-up. Neuromuscular diagnoses were excluded. RESULTS: 47 patients underwent index surgery between age 6 and 10.9 years. Twenty-one had DF, 26 had GS surgery (13 MCGR, 13 TGR). Diagnoses included 15 congenital, 15 idiopathic, 17 syndromic. Age at index was 9.1 years DF, 7.8 GS (p < .001). Follow-up was 63-78 months. 18/26 GS cases converted to DF, 13 due to complications, which occurred in 8/21 DF cases vs 19/26 GS (p = .016). DF patients had fewer post-index surgeries (0.6 vs 3.7, p < .001). At follow-up there were no differences in curve magnitudes, %correction, T1-12/T1-S1 segment lengths, EOSQ-24 scores or PFTs. 18 patients converting to DF after initial GS had equal outcomes as DF initially. 31 patients > age 8 at index ("tweeners") were studied separately. 13 had GS surgery (7 MCGR), 18 had DF. At > 60 months follow-up, curve magnitudes, spine lengths, PFT's, or EOSQ scores were equivalent. DF patients had fewer procedures and complications. CONCLUSION: For patients age 6-10.9 years, outcomes were no different at > 5 year follow-up between DF and GS groups. DF patients had fewer total surgeries and complications. Equal outcomes also occurred for tweeners. As a result, GS treatment does not appear to benefit patients > age 8.

A thoracoscopic anterior approach to the spine for adolescent idiopathic scoliosis does not have a detrimental effect on pulmonary function at 2 years compared to posterior-only surgery.

PURPOSE: This study aims to examine pulmonary function outcomes in patients with adolescent idiopathic scoliosis (AIS) undergoing posterior spinal fusion (PSF) with an anterior thoracoscopic release compared to those undergoing PSF alone. METHODS: A retrospective review of patients with AIS over a 9-year period at a single institution compared 2 groups: PSF with video-assisted thoracoscopic surgery (PSF/VATS) and patients undergoing a posterior spinal fusion (PSF) alone. Standard radiographs and Forced Expiratory Volume (FEV1) and Forced Vital Capacity (FVC) were obtained preoperatively and at regular follow up periods up to 2-year post-operatively. Within group and between-group comparisons were performed. RESULTS: There were 110 patients in the study: 12 in the PSF/VATS cohort and 98 in the PSF only cohort. The PSF/VATS group were younger (12.6 vs. 14.6, p = 0.003) and had larger coronal curves (80.8° vs. 60.7°, p = 0.001), and worse preoperative FVC (64.7% vs. 79.6%, p = 0.018) and FEV1 (62.3% vs. 77.6%, p = 0.003). At 2 years, the percent coronal Cobb correction was greater in the PSF/VATS group (67.9% vs. 48.4%, p < 0.001) with greater improvement in thoracic height (32.8 mm vs. 20.7 mm, p = 0.028). While the 2-year PFTs were the same for FEV1% (75.8% vs. 81.8%, p = 0.368) and FVC% (77.3% vs. 83.7%, p = 0.562), there was greater percent improvement over the 2 years in the PSF/VATS cohort: FEV1% (13.5% vs. 4.2%, p = 0.082) and FVC% (12.7% vs. 4.1%, p = 0.112). CONCLUSION: AIS patients who have a VATS approach in addition to PSF have greater coronal plane correction and improved pulmonary function compared to PSF alone despite more severe spinal deformity and worse baseline pulmonary function.

Angelman and Prader-Willi Syndromes: Sister Imprinting Disorders With High Complication Rates Following Spinal Deformity Surgery.

We sought to examine the modern surgical treatment of spinal deformity associated with sister imprinting disorders, Prader-Willi syndrome (PWS) and Angelman syndrome (AS), with emphasis on the specific complications encountered in these patient populations. Fifteen patients with PWS and 5 patients with AS who underwent surgical intervention for spinal deformity between 2000 and 2018 were identified. Postoperative complications were classified using the modified Clavien-Dindo-Sink (CDS) system and further categorized into specific subtypes including excessive drainage, dehiscence, implant failure, infection, and delayed wound healing. Perioperative and final follow-up radiographic data were analyzed. Mean age at surgery was 12.9 years (range, 4-21 years) with mean follow-up of 46.1 months (range, 1-145 months). There were postoperative complications in 17 patients (85%). Ten major complications (CDS ≥ 3) occurred in 9 patients (45%). These included 5 infections requiring reoperation, 1 seroma requiring drainage, 2 severe cervical-thoracic deformities requiring reoperation, 1 implant failure requiring reoperation, and 1 death secondary to fungal sepsis and thromboembolic disease. Eight additional patients (40%) had minor complications (CDS 1 or 2). Eight intraoperative complications occurred in 5 patients (25%), including loss of neuromonitoring signals and cerebrospinal fluid leaks. Surgical intervention for scoliosis in PWS and AS continues to have high complication rates secondary to medical and behavioral comorbidities found in these patient populations. The exact etiology of the high complication rates encountered cannot be definitively stated, but both syndromes frequently present with a number of unique features that may predispose patients to develop surgical complications. [Orthopedics. 2023;46(4):e223-e229.].

Incidence and significance of findings on spinal MRIs in a paediatric population with spinal column complaints.

PURPOSE: We sought to identify correlations between working diagnosis, surgeon indication for obtaining spinal MRI and positive MRI findings in paediatric patients presenting with spinal disorders or complaints. METHODS: Surgeons recorded their primary indication for ordering a spinal MRI in 385 consecutive patients. We compared radiologist-reported positive MRI findings with surgeon response, indication, working diagnosis and patient demographics. RESULTS: The most common surgeon-stated indications were pain (70) and coronal curve characteristics (63). Radiologists reported 137 (36%) normal and 248 (64%) abnormal MRIs. In total, 58% of abnormal reports (145) did not elicit a therapeutic or investigative response, which we characterized as 'clinically inconsequential'. In all, 42 of 268 (16%) presumed idiopathic scoliosis patients had intradural pathology noted on MRI.Younger age (10.3 years versus 12.0 years) was the only significant demographic difference between patients with or without intradural pathology. Surgeon indication 'curve magnitude at presentation' was associated with intradural abnormality identification. However, average Cobb angles between patients with or without an intradural abnormality was not significantly different (39° versus 37°, respectively). Back pain without neurological signs or symptoms was a negative predictor of intradural pathology. CONCLUSION: Radiologists reported a high frequency of abnormalities on MRI (64%), but 58% of those were deemed clinically inconsequential. Patients with MRI abnormalities were two years' younger than those with a normal or inconsequential MRI. 'Curve magnitude at presentation' in presumed idiopathic scoliosis patients was the only predictor of intrathecal pathology. 'Pain' was the only indication significantly associated with clinically inconsequential findings on MRI. LEVEL OF EVIDENCE: III.