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  1. Wang CW, Tassaneeyakul W, Chen CB, Chen WT, Teng YC, Huang CY, et al.
    J Allergy Clin Immunol, 2021 04;147(4):1402-1412.
    PMID: 32791162 DOI: 10.1016/j.jaci.2020.08.003
    BACKGROUND: Co-trimoxazole, a sulfonamide antibiotic, is used to treat a variety of infections worldwide, and it remains a common first-line medicine for prophylaxis against Pneumocystis jiroveci pneumonia. However, it can cause severe cutaneous adverse reaction (SCAR), including Stevens-Johnson syndrome, toxic epidermal necrolysis, and drug reaction with eosinophilia and systemic symptoms. The pathomechanism of co-trimoxazole-induced SCAR remains unclear.

    OBJECTIVE: We aimed to investigate the genetic predisposition of co-trimoxazole-induced SCAR.

    METHODS: We conducted a multicountry case-control association study that included 151 patients with of co-trimoxazole-induced SCAR and 4631 population controls from Taiwan, Thailand, and Malaysia, as well as 138 tolerant controls from Taiwan. Whole-genome sequencing was performed for the patients and population controls from Taiwan; it further validated the results from Thailand and Malaysia.

    RESULTS: The whole-genome sequencing study (43 case patients vs 507 controls) discovered that the single-nucleotide polymorphism rs41554616, which is located between the HLA-B and MICA loci, had the strongest association with co-trimoxazole-induced SCAR (P = 8.2 × 10-9; odds ratio [OR] = 7.7). There were weak associations of variants in co-trimoxazole-related metabolizing enzymes (CYP2D6, GSTP1, GCLC, N-acetyltransferase [NAT2], and CYP2C8). A replication study using HLA genotyping revealed that HLA-B∗13:01 was strongly associated with co-trimoxazole-induced SCAR (the combined sample comprised 91 case patients vs 2545 controls [P = 7.2 × 10-21; OR = 8.7]). A strong HLA association was also observed in the case patients from Thailand (P = 3.2 × 10-5; OR = 3.6) and Malaysia (P = .002; OR = 12.8), respectively. A meta-analysis and phenotype stratification study further indicated a strong association between HLA-B∗13:01 and co-trimoxazole-induced drug reaction with eosinophilia and systemic symptoms (P = 4.2 × 10-23; OR = 40.1).

    CONCLUSION: This study identified HLA-B∗13:01 as an important genetic factor associated with co-trimoxazole-induced SCAR in Asians.

  2. Chiu CK, Wang WJ, Lee YJ, Chung WH, Chan CYW, Kwan MK
    Spine J, 2023 Nov;23(11):1700-1708.
    PMID: 37453514 DOI: 10.1016/j.spinee.2023.07.006
    BACKGROUND CONTEXT: Patients with adolescent idiopathic scoliosis (AIS) have higher prevalence of abnormal or dysplastic pedicles.

    PURPOSE: To investigate the prevalence and distribution of narrow dysplastic and fully corticalized pedicles in Asian AIS patients with major main thoracic curves.

    DESIGN: Retrospective study.

    PATIENT SAMPLE: A total of 6,494 pedicles in 191 patients were measured and evaluated.

    OUTCOME MEASURES: The primary outcomes measures were the pedicle width measurements (total transverse pedicle width, transverse cancellous width, total transverse cortical width) and classification of pedicles. Demographic data (age, gender, height, weight, body mass index), proximal thoracic Cobb angle, main thoracic Cobb angle and lumbar Cobb angle were also obtained.

    METHODS: AIS patients with major (largest Cobb angle) main thoracic curves and had computed tomography (CT) scans prior to corrective spine surgery were reviewed. The pedicles were classified as Grade A: cancellous channel >4 mm; Grade B: cancellous channel 2 to 4 mm; Grade C: cancellous channel <2 mm or corticalized pedicle >4 mm; Grade D: corticalized pedicle ≤4 mm. Grades B, C, and D were dysplastic pedicles while grades C and D were narrow dysplastic pedicles.

    RESULTS: The prevalence of dysplastic pedicles (grades B, C, and D) was 61.7%. There were 22.6% narrow dysplastic pedicles (grades C and D) and 4.1% fully corticalized pedicles (grade D). In the thoracolumbar region, there was a sharp transition from larger and less dysplastic pedicles at T11 and T12 to narrower and more dysplastic pedicles at L1 and L2 (narrow dysplastic pedicles at T11: 3.1%, T12: 3.1%, L1: 39.8% and L2: 23.6%). Higher prevalences of narrow dysplastic pedicles were located at right T3 to T5 (71.2%-83.7%) and left T7-T9 (51.3%-61.2%). Higher prevalences of fully corticalized pedicles were located at right T3 to T5 (20.9%-34.0%) and left T7 to T8 (11.0%-12.0%). These were the concave pedicles of proximal thoracic and main thoracic curves, respectively.

    CONCLUSION: There were 95.9% pedicles with cancellous channels (grades A, B, and C) can allow pedicle screw fixation and only 4.1% fully corticalized pedicles (grade D) that require an alternative method of fixation. For grade C pedicles (18.5%), pedicle screws can still be attempted with caution. Precautions should also be observed at the L1 and L2 levels as there was a transition to narrower pedicles.

  3. Chiu CK, Chin TF, Chung WH, Chan CYW, Kwan MK
    Spine (Phila Pa 1976), 2024 Jan 01;49(1):64-70.
    PMID: 37146062 DOI: 10.1097/BRS.0000000000004711
    STUDY DESIGN: Retrospective Study.

    OBJECTIVE: This study aims to investigate variation in the number of thoracic and lumbar vertebrae, the prevalence of lumbosacral transitional vertebra (LSTV) and the prevalence of cervical ribs among surgical patients with adolescent idiopathic scoliosis (AIS).

    SUMMARY OF BACKGROUND DATA: Due to variation in the number of thoracic or lumbar vertebrae, inaccurate identification of vertebral levels has been found to be a contributory factor to wrong-level surgery.

    METHODS: This was a retrospective study on AIS patients who underwent posterior spinal fusion. Demographic and anthropometric data (age, gender, height, weight, and body mass index), radiographic data (Lenke curve type, pre-operative Cobb angle, vertebra numbering of cervical, thoracic, and lumbar spine, presence of LSTV based on the Castellvi classification and the presence of cervical ribs) and clinical data were collected. Data were analysed and reported with mean and standard deviation for quantitative parameters and number and percentage for qualitative parameters. Multinomial logistic regression analyses were performed to identify factors associated with the outcomes of interest.

    RESULTS: A total of 998 patients met inclusion criteria, of which 135 (13.5%) were males and 863 (86.5%) were females. The vertebrae number varied between 23 to 25 total vertebrae with 24 vertebrae considered as the typical number of vertebrae. The prevalence of atypical number of vertebrae (23 or 25) was 9.8% (98 patients). We found a total of 7 different variations in number of cervical, thoracic, and lumbar vertebrae (7C11T5L, 7C12T4L, 7C11T6L, 7C12T5L, 7C13T4L, 7C12T6L, and 7C13T5L) with 7C12T5L considered as the typical vertebrae variation. The total prevalence of patients with atypical vertebrae variation was 15.5% (155 patients). Cervical ribs were found in 2 (0.2%) patients while LSTV were found in 250 (25.1%) of patients. The odds of 13 thoracic vertebrae were higher in males (OR 5.17; 95% CI: 1.25, 21.39) and the odds of 6 lumbar vertebrae were higher in LSTV (OR 3.93; 95% CI: 2.58, 6.00).

    CONCLUSION: In this series, we identified a total of 7 different variations in the number of cervical, thoracic, and lumbar vertebrae. The total prevalence of patients with atypical vertebrae variation was 15.5%. LSTV was found in 25.1% of the cohort. It is important to ascertain atypical vertebrae variations rather than the absolute number of vertebrae because variants such as 7C11T6L and 7C13T4L may still have typical numbers of vertebrae in total. However, due to the differences in the number of morphologically thoracic and lumbar vetrebrae, there may still be a risk of inaccurate identification.

  4. Chiu CK, Wang WJ, Lee YJ, Chung WH, Chan CYW, Kwan MK
    Spine J, 2024 Feb 24.
    PMID: 38408520 DOI: 10.1016/j.spinee.2024.02.014
    BACKGROUND CONTEXT: The widths of medial and lateral pedicle walls in the normal spine of middle-aged and elderly adults had been investigated and these studies found that medial pedicle wall was thicker than lateral pedicle wall. However, none had evaluated the widths of medial and lateral pedicle walls on adolescent or young adult scoliotic spines.

    PURPOSE: This study aims to identify the distribution and variation of medial and lateral pedicle wall widths throughout the different vertebral levels of the scoliotic spine and its differences according to age, gender, body mass index (BMI), maturity, curve types and curve severity in adolescent idiopathic scoliotic (AIS) patients with major thoracic curves.

    STUDY DESIGN: Retrospective Study PATIENT SAMPLE: A total of 6230 pedicles (right: 3064, left: 3166) from 191 patients were included in this study with 264 (right: 183, left: 81) fully corticalized pedicles excluded from analysis.

    OUTCOME MEASURES: Demographic data were age, gender, height, weight, BMI, Risser grade, Lenke curve types and Cobb angles. Main outcome measures were medial and lateral pedicle wall widths. Associations between pedicle wall widths and demographic data were calculated.

    METHODS: This was a sub-analytical retrospective study done on the same patient population as the previously published study on pedicle grading. The data was obtained from the main computed tomography (CT) scan pedicle study dataset. Medial and lateral pedicle wall widths were measured in the axial slices of CT scans from T1 to L5 vertebrae.

    RESULTS: A total of 6230 pedicles (right: 3064, left: 3166) from 191 patients were included in this study with 264 (right: 183, left: 81) fully corticalized pedicles excluded from analysis. Right-sided medial pedicle wall widths were narrower from T4-T10 (0.75 ± 0.23 mm) compared to T1-T3 (0.89 ± 0.28 mm) and T11-L5 (0.92 ± 0.30 mm). Left-sided medial pedicle wall widths were narrower from T4 to T7 (0.76 ± 0.24 mm) compared to T1-T3 (0.88 ± 0.26 mm) and T8-L5 (0.90 ± 0.27 mm). Medial cortical wall widths were significantly thicker compared to lateral cortical wall widths for all vertebras from T1 to L5 (right medial 0.85 ± 0.28 mm vs lateral 0.64 ± 0.26 mm (p<0.001), left medial 0.86 ± 0.26 mm vs lateral 0.64 ± 0.26 mm (p<0.001)). The left medial pedicle wall widths were marginally significantly (p<0.001) thicker than right side (right medial 0.85 ± 0.28 mm vs left 0.86 ± 0.26 mm). Then main notable significant differences were located at the periapical region of the thoracic curve between T7 to T10 with left concave medial pedicle width being thicker than right convex medial pedicle width. The thinnest medial pedicle walls were located at right concave T7 (0.73 ± 0.24 mm) and T8 (0.73 ± 0.23 mm). We generally found no significant associations between the medial and lateral pedicle wall widths with age, gender, BMI, Risser grade, Cobb angle and curve types.

    CONCLUSION: Knowledge on the widths of medial and lateral pedicle walls, their distribution and differences in a scoliotic spine is important for pedicle screw fixation especially during pedicle probing to find the pedicle channel. The medial pedicle wall widths were significantly thicker than lateral pedicle wall widths in AIS patients with major thoracic curves. The right concave periapical region had the thinnest medial pedicle walls.

  5. Wang YH, Chen CB, Tassaneeyakul W, Saito Y, Aihara M, Choon SE, et al.
    Clin. Pharmacol. Ther., 2019 01;105(1):112-120.
    PMID: 29569740 DOI: 10.1002/cpt.1071
    Specific ethnic genetic backgrounds are associated with the risk of Stevens-Johnson syndrome / toxic epidermal necrolysis (SJS/TEN) especially in Asians. However, there have been no large cohort, multiple-country epidemiological studies of medication risk related to SJS/TEN in Asian populations. Thus, we analyzed the registration databases from multiple Asian countries who were treated during 1998-2017. A total 1,028 SJS/TEN cases were identified with the algorithm of drug causality for epidermal necrolysis. Furthermore, those medications labeled by the US Food and Drug Administration (FDA) as carrying a risk of SJS/TEN were also compared with the common causes of SJS/TEN in Asian countries. Oxcarbazepine, sulfasalazine, COX-II inhibitors, and strontium ranelate were identified as new potential causes. In addition to sulfa drugs and beta-lactam antibiotics, quinolones were also a common cause. Only one acetaminophen-induced SJS was identified, while several medications (e.g., oseltamivir, terbinafine, isotretinoin, and sorafenib) labeled as carrying a risk of SJS/TEN by the FDA were not found to have caused any of the cases in the Asian countries investigated in this study.
  6. Chen WT, Wang CW, Lu CW, Chen CB, Lee HE, Hung SI, et al.
    J Invest Dermatol, 2018 07;138(7):1546-1554.
    PMID: 29458119 DOI: 10.1016/j.jid.2018.02.004
    Dapsone-induced hypersensitivity reactions may cause severe cutaneous adverse reactions, such as drug reaction with eosinophilia and systemic symptoms (DRESS). It has been reported that HLA-B*13:01 is strongly associated with dapsone-induced hypersensitivity reactions among leprosy patients. However, the phenotype specificity and detailed immune mechanism of HLA-B*13:01 remain unclear. We investigated the genetic predisposition, HLA-B*13:01 function, and cytotoxic T cells involved in the pathogenesis of dapsone-induced severe cutaneous adverse reactions. We enrolled patients from Taiwan and Malaysia with DRESS and maculopapular eruption with chronic inflammatory dermatoses. Our results showed that the HLA-B*13:01 allele was present in 85.7% (6/7) of patients with dapsone DRESS (odds ratio = 49.64, 95% confidence interval = 5.89-418.13; corrected P = 2.92 × 10-4) but in only 10.8% (73/677) of general population control individuals in Taiwan. The level of granulysin, the severe cutaneous adverse reaction-specific cytotoxic protein released from cytotoxic T cells, was increased in both the plasma of DRESS patients (36.14 ± 9.02 ng/ml, P < 0.05) and in vitro lymphocyte activation test (71.4%, 5/7 patients) compared with healthy control individuals. Furthermore, dapsone-specific cytotoxic T cells were significantly activated when co-cultured with HLA-B*13:01-expressing antigen presenting cells in the presence of dapsone (3.9-fold increase, compared with cells with no HLA-B*13:01 expression; P < 0.01). This study indicates that HLA-B*13:01 is strongly associated with dapsone DRESS and describes a functional role for the HLA-restricted immune mechanism induced by dapsone.
  7. Chung WH, Anuar AA, Lee KJ, Hasan MS, Chiu CK, Chan CYW, et al.
    J Orthop Surg (Hong Kong), 2020 9 11;28(3):2309499020945014.
    PMID: 32909907 DOI: 10.1177/2309499020945014
    Superior mesenteric artery (SMA) syndrome is a rare but serious complication following scoliosis surgery. Early diagnosis and management are key factors for successful conservative treatment to avoid the need for emergency laparotomies which causes higher morbidity or even mortality. We report two adolescent idiopathic scoliosis patients with Cobb angle of 49° and 132°, respectively, and low body mass index who presented with SMA syndrome following posterior spinal fusion from T2 to L3 and were treated successfully with conservative management. Abdominal radiographs showed distended gastric shadow. Computed tomography angiography of the abdomen showed decreased aortomesenteric angle and SMA-aorta distance. Both patients were treated successfully with conservative treatment which included three principles: gastric decompression with nasogastric tube, correction of electrolytes imbalance, and nutritional support with low volume, high calorie nutritional supplement. Both patients were started with small but frequent meals. Surgeries were not required in both cases. Early diagnosis and management are the key factors to successful treatment in SMA syndrome. Patients with SMA can be treated successfully with conservative treatment comprising of nasogastric decompression, electrolyte correction, and nutritional support with small but frequent meals.
  8. Chung WH, Chiu CK, Wei Chan CY, Kwan MK
    Acta Orthop Traumatol Turc, 2020 Sep;54(5):561-564.
    PMID: 33155569 DOI: 10.5152/j.aott.2020.19144
    Growth hormone secreting pituitary tumor or gigantism has not been previously reported to be associated with rapid progression of scoliosis in the literature. However, there are some reports indicating scoliosis can be worsened by growth hormone therapy in children and adolescents. A 19-year-old boy was referred to our institution for the treatment of a right thoracolumbar scoliosis. The Cobb angle had worsened from 29° to 83° over two years' duration. He attained puberty at the age of 13. He had a previous history of slipped upper femoral epiphysis (SUFE), which was operated in 2015, with no clinical features of gigantism. Preoperative assessment was performed. He was diagnosed with growth hormone secreting pituitary macroadenoma by magnetic resonance imaging with a high serum level of insulin-like growth factor-I (IGF-I). Computed tomography (CT) of the pancreas showed a pancreatic endocrine tumor. The patient was later diagnosed with multiple endocrine neoplasia type 1 (MEN 1). He underwent endoscopic endonasal excision of the pituitary mass and distal pancreatectomy. This case indicates that growth hormone secreting pituitary macroadenoma could result in rapid progression of scoliosis.
  9. Chan CYW, Ch'ng PY, Lee SY, Chung WH, Chiu CK, Kwan MK
    Global Spine J, 2023 Mar;13(2):443-450.
    PMID: 33691529 DOI: 10.1177/2192568221998642
    STUDY DESIGN: Retrospective.

    PURPOSE: To evaluate the relationship between shoulder/ neck imbalance with distal adding-on phenomenon and to identify other risk factors in Lenke 1 and 2 (non-AR curves) adolescent idiopathic scoliosis (AIS) patients.

    METHODS: 100 Lenke 1 and 2 AIS patients with lowest instrumented vertebra (LIV) cephalad to or at L1 were recruited. Medial shoulder/ neck balance was represented by T1-tilt and cervical axis (CA). Lateral shoulder balance was represented by clavicle angle (Cla-A) and radiographic shoulder height (RSH). Distal adding-on phenomenon was diagnosed when there was disc wedging below LIV of >5o at final follow-up. Predictive factors and odds ratio were derived using univariate and multivariate logistic regression analysis.

    RESULTS: Mean age of this cohort was 15.9 ± 4.4 years. Mean follow-up duration was 30.9 ± 9.6 months. Distal adding-on phenomenon occurred in 19 patients (19.0%). Only Risser grade, preoperative CA and final follow-up lumbar Cobb angle were the independent factors. A positive preoperative CA deviation increased the odds of distal adding-on by 5.4 times (95% CI 1.34-21.51, P = 0.018). The mean immediate postoperative T1-tilt, CA, RSH and Cla-A were comparable between the group with distal adding-on and the group without.

    CONCLUSION: Distal adding-on phenomenon occurred in 19.0% of patients. Preoperative "Cervical Axis" was an important factor and it increased the risk of distal adding-on by 5.4 times. Other significant predictive factors were Risser grade and lumbar Cobb angle at final follow-up. Immediate postoperative shoulder or neck imbalance was not a significant factor for postoperative distal adding-on phenomenon.

  10. Mihara Y, Chung WH, Mohamad SM, Chiu CK, Chan CYW, Kwan MK
    Eur Spine J, 2021 03;30(3):653-660.
    PMID: 33486626 DOI: 10.1007/s00586-020-06701-3
    PURPOSE: Knowledge on the factors affecting the correction rate (CR) aids in the surgical planning among severe idiopathic scoliosis (IS) patients. This study aimed to investigate the independent factors affecting CR among patients with severe IS (Cobb angle ≥ 90°) who underwent single-staged posterior spinal fusion (PSF).

    METHODS: We retrospectively reviewed 128 severe IS patients who underwent single-staged PSF. Factors including age, height, weight, body mass index, Risser sign, Lenke subtypes, preoperative major Cobb angle, side bending major Cobb angle, side bending flexibility (SBF), motion segments of the major curve, AR curve, number of levels fused, screw density, operative time and postoperative major Cobb angle were analysed using linear regression analysis.

    RESULTS: The mean age was 15.5 ± 4.5 years with mean Risser sign of 3.1 ± 1.6. The mean preoperative Cobb, SBF, postoperative Cobb and CR were 102.8 ± 12.3°, 37.5 ± 13.7%, 44.4 ± 13.5° and 57.2 ± 10.8%, respectively. From stepwise multiple linear regression analysis, SBF, Risser sign and AR curve were the independent predictive factors for CR, with R2 value of 0.345 (p 

  11. Chan CYW, Tan YWE, Chandren JR, Chandirasegaran S, Chung WH, Chiu CK, et al.
    Spine J, 2024 Mar 16.
    PMID: 38499066 DOI: 10.1016/j.spinee.2024.03.012
    BACKGROUND CONTEXT: Preoperative supine radiographs are mandatory in the new adult idiopathic scoliosis (AdIS) classification. Supine radiographs are easily reproducible and highly predictive of side bending radiographs. However, few studies evaluated the use of supine radiographs in predicting postoperative curve correction after posterior spinal fusion (PSF) in AdIS.

    PURPOSE: To investigate the use of supine and side bending (SB) radiographs in predicting postoperative curve correction in AdIS patients who underwent PSF.

    STUDY DESIGN: Retrospective study.

    PATIENT SAMPLE: 93 AdIS patients who underwent PSF between 2022 and 2023 were included.

    OUTCOME MEASURES: Demographic data were age, gender, height, weight, body mass index (BMI), Risser grade, Lenke curve types and Cobb angles. Main outcome measures were preoperative and immediate postoperative Cobb angle (proximal thoracic [PT], main thoracic [MT] and thoracolumbar/lumbar [TL/L] curves), Supine Cobb angle and Flexibility rate (PT, MT and TL/L), and Correction rate (PT, MT and TL/L).

    METHODS: Correlation study was performed between Supine Cobb angle vs. postoperative Cobb angle for PT, MT and TL/L curves. A predictive formula was derived from the correlation plots.

    RESULTS: A total of 93 subjects were included in our study with a median age of 24.7 years and comprised of 80 females (86.0%). Preoperative Supine Cobb angle (r=0.835, r=0.881, r=0.767, p

  12. Chung WH, Mihara Y, Toyat SS, Chiu CK, Hasan MS, Saw A, et al.
    Malays Orthop J, 2021 Nov;15(3):99-107.
    PMID: 34966502 DOI: 10.5704/MOJ.2111.015
    Introduction: To report the indications and early treatment outcomes of pre-operative halo-pelvic traction in patients with neurofibromatosis associated with severe proximal thoracic (PT) spinal deformity.

    Materials and methods: We reviewed four patients with neurofibromatosis with severe PT spinal deformity. Case 1, a 16-year-old male presented with severe PT kyphoscoliosis (scoliosis: 89°, kyphosis: 124°) and thoracic myelopathy. Case 2 was a 14-year-old, skeletally immature male who presented with a PT lordoscoliosis (scoliosis: 85°). Case 3, a 13-year-old male, presented with severe PT kyphoscoliosis (scoliosis: 100°, kyphosis: 95°). Case 4, a 35-year-old gentleman, presented with severe PT kyphoscoliosis (scoliosis: 113°, kyphosis: 103°) and thoracic myelopathy. All patients underwent pre-operative halo-pelvic traction. After a period of traction, all patients underwent posterior spinal fusion (PSF) with autologous bone grafts (local and fibula bone grafts) and recombinant human bone morphogenetic protein-2 (rhBMP-2).

    Results: Both patients with thoracic myelopathy regained near normal neurological status after halo-pelvic traction. Following traction, the scoliosis correction rate (CR) ranged from 18.0% to 38.9%, while the kyphosis CR ranged from 14.6% to 37.1%. Following PSF, the scoliosis CR ranged from 24.0% to 58.8%, while the kyphosis CR ranged from 29.1% to 47.4%. The total distraction ranged from 50-70mm. Duration of distraction ranged from 26-95 days. The most common complication encountered during halo-pelvic traction was pin-related e.g. pin tract infection, pin loosening and migration, osteomyelitis, and halo-pelvic strut breakage. No patients had cranial nerve palsies or neurological worsening.

    Conclusion: Pre-operative correction of severe PT spinal deformities could be performed safely and effectively with the halo-pelvic device prior to definitive surgery.

  13. Wong TS, Abu Bakar J, Chee KH, Hasan MS, Chung WH, Chiu CK, et al.
    Spine (Phila Pa 1976), 2019 02 15;44(4):E252-E257.
    PMID: 30086081 DOI: 10.1097/BRS.0000000000002828
    STUDY DESIGN: Case report.

    OBJECTIVE: To describe the technical difficulties on performing posterior spinal fusion (PSF) on a pacemaker-dependent patient with complete congenital heart block and right thoracic scoliosis.

    SUMMARY OF BACKGROUND DATA: Congenital complete heart block requires pacemaker implantation at birth through thoracotomy, which can result in scoliosis. Corrective surgery in this patient was challenging. Height gain after corrective surgery may potentially cause lead dislodgement. The usage of monopolar electrocautery may interfere with the function of the implanted cardiac device.

    METHODS: A 17-year-old boy was referred to our institution for the treatment of right thoracic scoliosis of 70°. He had underlying complete congenital heart block secondary to maternal systemic lupus erythematosus. Pacemaker was implanted through thoracotomy since birth and later changed for four times. PSF was performed by two attending surgeons with a temporary pacing inserted before the surgery. The monopolar electrocautery device was used throughout the surgery.

    RESULTS: The PSF was successfully performed without any technical issues and complications. Postoperatively, his permanent pacemaker was functioning normally. Three days later, he was recovering well and was discharged home from hospital.

    CONCLUSION: This case indicates that PSF can be performed successfully with thoughtful anticipation of technical difficulties on a pacemaker-dependent patient with underlying congenital heart block.

    LEVEL OF EVIDENCE: 5.

  14. Chan CYW, Chung WH, Mihara Y, Lee SY, Ch'ng PY, Hasan MS, et al.
    J Orthop Surg (Hong Kong), 2020 8 9;28(3):2309499020936005.
    PMID: 32762498 DOI: 10.1177/2309499020936005
    PURPOSE: Various surgical strategies including combined approach and spinal osteotomies in severe rigid scoliosis had been reported with significant perioperative complication rates. The use of single-staged posterior spinal fusion (PSF) utilizing a dual attending surgeon strategy for severe rigid scoliosis has not been widely reported.

    METHODS: This was a retrospective study aimed to evaluate the perioperative outcome of single-staged PSF in severe rigid idiopathic scoliosis patients (Cobb angle ≥90° and ≤30% flexibility). Forty-one patients with severe rigid idiopathic scoliosis who underwent single-staged PSF were included. The perioperative outcome parameters were operation duration, intraoperative blood loss, intraoperative hemodynamic parameters, preoperative and postoperative hemoglobin, transfusion rate, patient-controlled anesthesia morphine usage, length of postoperative hospital stay, and perioperative complications. Radiological parameters included preoperative and postoperative Cobb angle, correction rate, side-bending flexibility, and side-bending correction index.

    RESULTS: The mean age was 16.9 ± 5.6 years. The mean preoperative Cobb angle was 110.8 ± 12.1° with mean flexibility of 23.1 ± 6.3%. The mean operation duration was 215.5 ± 45.2 min with mean blood loss of 1752.6 ± 830.5 mL. The allogeneic blood transfusion rate was 24.4%. The mean postoperative hospital stay was 76.9 ± 26.7 h. The mean postoperative Cobb angle and correction rate were 54.4 ± 12.8° and 50.9 ± 10.1%, respectively. The readmission rate in this cohort was 2.4%. Four perioperative complications were documented (9.8%), one somatosensory evoke potential signal loss, one superficial infection, one lung collapse, and one superior mesenteric artery syndrome.

    CONCLUSIONS: Severe rigid idiopathic scoliosis treated with single-staged PSF utilizing a dual attending surgeon strategy demonstrated an average correction rate of 50.9%, operation duration of 215.5 min, and postoperative hospital stay of 76.9 h with a 9.8% perioperative complication rate.

  15. Kwan MK, Loh KW, Chung WH, Chiu CK, Hasan MS, Chan CYW
    BMC Musculoskelet Disord, 2021 May 04;22(1):413.
    PMID: 33947368 DOI: 10.1186/s12891-021-04225-5
    BACKGROUND: There has been a growing interest in using all pedicle screw construct in posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) surgery in recent years. However, studies focusing on perioperative outcome and complications utilizing only pedicle screw system in AIS population are lacking. This study aims to evaluate perioperative outcomes and to determine the prevalence of major and minor complications following single-staged PSF for AIS.

    METHODS: In this retrospective study of prospectively collected data, 1057 AIS patients operated between 2012 and 2019 were included. Main outcome measures were operative time, intraoperative blood loss, allogeneic blood transfusion rate, length of hospital stay after surgery, complication rate, and mean drop of haemoglobin (Hb) level. We documented the number of fusion levels, screw density, and postoperative radiographic parameters.

    RESULTS: There were 917 females and 140 males. Majority were Lenke 1 curve type (46.9%). Mean age was 15.6 ± 3.7 years, with mean BMI of 18.6 ± 3.2 kg/m2. Mean operative time was 146.8 ± 49.4 min. Average intraoperative blood loss was 952.9 ± 530.4 ml with allogeneic blood transfusion rate of 5%. Mean screw density was 1.27 ± 0.21 screws per fusion level. Average hospital stay after surgery was 3.5 ± 0.9 days. Twenty-four complications were documented: twelve superficial infections (1.14%), five transient neurological deficits (0.47%), two deep infections (0.19%), two superior mesenteric artery syndrome, and one case each (0.09%) for massive intraoperative blood loss, intraoperative seizure, and lung atelectasis.

    CONCLUSION: AIS patients treated with single-staged PSF using pedicle screw construct had a 0.95% rate of major complications and 1.32% rate of minor complications. Rate of neurologic complication was 0.47% while non-neurologic postoperative complications was 1.80% with infection being the leading complication at 1.32%.

  16. Mihara Y, Chung WH, Chiu CK, Hasan MS, Lee SY, Ch'ng PY, et al.
    Spine (Phila Pa 1976), 2020 Mar 15;45(6):381-389.
    PMID: 31574058 DOI: 10.1097/BRS.0000000000003274
    STUDY DESIGN: Retrospective study from a prospectively collected database.

    OBJECTIVE: To compare the perioperative outcome between after-hours and daytime surgery carried out by a dedicated spinal deformity team for severe Idiopathic Scoliosis (IS) patients with Cobb angle ≥ 90°.

    SUMMARY OF BACKGROUND DATA: There were concerns that after-hours corrective surgeries in severe IS have higher morbidity compared to daytime surgeries.

    METHODS: Seventy-one severe IS patients who underwent single-staged Posterior Spinal Fusion (PSF) were included. Surgeries performed between 08:00H and 16:59H were classified as "daytime" group and surgeries performed between 17:00H and 06:00H were classified as "after-hours" group. Perioperative outcome parameters were average operation start time and end time, operation duration, intraoperative blood loss, intraoperative hemodynamic parameters, preoperative and postoperative hemoglobin, blood transfusion rate, total patient-controlled anesthesia (PCA) morphine usage, length of postoperative hospitalization, and complications. Radiological variables assessed were preoperative and postoperative Cobb angle, side bending flexibility, number of fusion levels, number of screws used, Correction Rate, and Side Bending Correction Index.

    RESULTS: Thirty patients were operated during daytime and 41 patients were operated after-hours. The mean age was 16.1 ± 5.8 years old. The mean operation start time for daytime group was 11:31 ± 2:45H versus 19:10 ± 1:24H for after-hours group. There were no significant differences between both groups in the operation duration, intraoperative blood loss, intraoperative hemodynamic parameters, postoperative hemoglobin, hemoglobin drift, transfusion rate, length of postoperative hospitalization, postoperative Cobb angle, Correction Rate, and Side Bending Correction Index. There were four complications (1 SSEP loss, 1 massive blood loss, and 2 superficial wound infections) with no difference between daytime and after-hours group.

    CONCLUSION: After-hours elective spine deformity corrective surgeries in healthy ambulatory patients with severe IS performed by a dedicated spinal deformity team using dual attending surgeon strategy were as safe as those performed during daytime.

    LEVEL OF EVIDENCE: 4.

  17. Lee SY, Ch'ng PY, Wong TS, Ling XW, Chung WH, Chiu CK, et al.
    Global Spine J, 2023 Apr;13(3):752-763.
    PMID: 33823628 DOI: 10.1177/21925682211007795
    STUDY DESIGN: Cross-sectional.

    OBJECTIVES: To evaluate the perception and satisfaction among adolescent idiopathic scoliosis (AIS) patients on Neck Tilt with Medial Shoulder Imbalance and Lateral Shoulder Imbalance.

    METHODS: Lenke 1 and 2 AIS patients were stratified into 6 groups of preoperative/ postoperative Balanced (B), Lateral Shoulder Imbalance (LSI) (>2 cm), and Neck Tilt with Medial Shoulder Imbalance (NT) (Grade 3). Patients were interviewed using Modified Neck and Shoulder Appearance and SRS-22r questionnaires. T1 tilt, Cervical Axis (CA), Clavicle Angle (Cla-A) and Radiographic Shoulder Height (RSH) were measured.

    RESULTS: A total of 120 Lenke 1 and 2 AIS patients were recruited. NT patients were aware and unhappy with their abnormal neck with medial shoulder appearances (P < .001). Similarly, LSI patients were aware and unhappy with their abnormal lateral shoulder appearances (P < .001). NT group had larger preoperative/ postoperative T1 tilt (9.2 ± 5.0°; 9.5 ± 5.3°) and CA (4.6 ± 3.1°; 7.0 ± 2.0°) (P < .01). LSI group had larger preoperative/ postoperative Cla-A (4.8 ± 2.8°; 4.0 ± 1.3°) and RSH (20.1 ± 9.4 mm; 17.0 ± 6.6 mm) (P < .001). Postoperative B group scored higher in overall SRS-22r scores, self-image and satisfaction domains (4.2 ± 0.3; 4.0 ± 0.5; 4.4 ± 0.5) while preoperative LSI scored the lowest comparatively (3.5 ± 0.4; 2.4 ± 0.5; 3.3 ± 0.5) (P < .001).

    CONCLUSION: NT and LSI were major concerns among AIS patients. Both NT and LSI groups were unhappy with their appearances. NT group had larger T1 tilt/ CA whereas LSI group had larger Cla-A/ RSH. Postoperative B group scored higher in overall SRS-22r scores, self-image and satisfaction domains.

  18. Tsai MH, Muir AM, Wang WJ, Kang YN, Yang KC, Chao NH, et al.
    Neuron, 2020 Apr 22;106(2):237-245.e8.
    PMID: 32097630 DOI: 10.1016/j.neuron.2020.01.027
    Lissencephaly (LIS), denoting a "smooth brain," is characterized by the absence of normal cerebral convolutions with abnormalities of cortical thickness. Pathogenic variants in over 20 genes are associated with LIS. The majority of posterior predominant LIS is caused by pathogenic variants in LIS1 (also known as PAFAH1B1), although a significant fraction remains without a known genetic etiology. We now implicate CEP85L as an important cause of posterior predominant LIS, identifying 13 individuals with rare, heterozygous CEP85L variants, including 2 families with autosomal dominant inheritance. We show that CEP85L is a centrosome protein localizing to the pericentriolar material, and knockdown of Cep85l causes a neuronal migration defect in mice. LIS1 also localizes to the centrosome, suggesting that this organelle is key to the mechanism of posterior predominant LIS.
  19. Chan CYW, Chong JSL, Lee SY, Ch'ng PY, Chung WH, Chiu CK, et al.
    Spine (Phila Pa 1976), 2020 Dec 01;45(23):1661-1667.
    PMID: 32756286 DOI: 10.1097/BRS.0000000000003641
    STUDY DESIGN: Prospective study.

    OBJECTIVE: To determine the parents'/patients' perception on the informed consent process prior to posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) patients.

    SUMMARY OF BACKGROUND DATA: Understanding parents/patients perspective on the process is important in order to achieve the goal of consent and prevent medico-legal implications.

    METHODS: Fifty AIS patients operated between August 2019 and November 2019 were prospectively recruited. Parents'/patients' perceptions on three sections were evaluated: the process of the informed consent, specific operative risk which they were most concerned with and the accountability of surgeons for the surgical risks. These data were ranked and scored using a 5-point Likert Scale. Preferences were reported in mean and standard deviation. Differences in terms of preferences were studied using One-way analysis of variance (ANOVA) analysis and deemed significant when P 

  20. Chung WH, Eu WC, Chiu CK, Chan CYW, Kwan MK
    J Orthop Surg (Hong Kong), 2019 12 27;28(1):2309499019888977.
    PMID: 31876259 DOI: 10.1177/2309499019888977
    PURPOSE: To describe the reduction technique of thoracolumbar burst fracture using percutaneous monoaxial screws and its radiological outcomes compared to polyaxial screws.

    METHODS: All surgeries were performed by minimally invasive technique with either percutaneous monoaxial or percutaneous polyaxial screws inserted at adjacent fracture levels perpendicular to both superior end plates. Fracture reduction is achieved with adequate rod contouring and distraction maneuver. Radiological parameters were measured during preoperation, postoperation, and follow-up.

    RESULTS: A total of 21 patients were included. Eleven patients were performed with monoaxial pedicle screws and 10 patients performed with polyaxial pedicle screws. Based on AO thoracolumbar classification system, 10 patients in the monoaxial group had A3 fracture type and 1 had A4. In the polyaxial group, six patients had A3 and four patients had A4. Total correction of anterior vertebral height (AVH) ratio was 0.30 ± 0.10 and 0.08 ± 0.07 in monoaxial and polyaxial groups, respectively (p < 0.001). Total correction of posterior vertebral height (PVH) ratio was 0.11 ± 0.05 and 0.02 ± 0.02 in monoaxial and polyaxial groups, respectively (p < 0.001). Monoaxial group achieved more correction of 13° (62.6%) in local kyphotic angle compared to 8.2° (48.0%) in polyaxial group. Similarly, in regional kyphotic angle, 16.5° (103.1%) in the monoaxial group and 8.1° (76.4%) in the polyaxial group were achieved.

    CONCLUSIONS: Monoaxial percutaneous pedicle screws inserted at adjacent fracture levels provided significantly better fracture reduction compared to polyaxial screws in thoracolumbar fractures.

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