scholarly journals Improved cephalic index following early cranial vault remodeling in patients with isolated nonsyndromic sagittal synostosis

2021 ◽  
Vol 50 (4) ◽  
pp. E7
Author(s):  
Arvid Frostell ◽  
Maryam Haghighi ◽  
Jiri Bartek ◽  
Ulrika Sandvik ◽  
Bengt Gustavsson ◽  
...  

OBJECTIVE Isolated nonsyndromic sagittal synostosis (SS) is the most common form of craniosynostosis in children, accounting for approximately 60% of all craniosynostoses. The typical cranial measurement used to define and follow SS is the cephalic index (CI). Several surgical techniques have been suggested, but agreement on type and timing of surgery is lacking. This study aimed to evaluate the authors’ institutional experience of surgically treating SS using a modified subtotal cranial vault remodeling technique in a population-based cohort. Special attention was directed toward the effect of patient age at time of surgery on long-term CI outcome. METHODS A retrospective analysis was conducted on all patients with isolated nonsyndromic SS who were surgically treated from 2003 to 2011. Data from electronic medical records were gathered. Eighty-two patients with SS were identified, 77 fulfilled inclusion criteria, and 72 had sufficient follow-up data and were included. CI during follow-up after surgery was investigated with ANOVA and a linear mixed model. RESULTS In total, 72 patients were analyzed, consisting of 16 females (22%) and 56 males (78%). The mean ± SD age at surgery was 4.1 ± 3.1 months. Blood transfusions were received by 81% of patients (26% intraoperatively, 64% postoperatively, 9% both). The mean ± SD time in the pediatric ICU was 1.1 ± 0.25 days, and the mean ± SD total hospital length of stay was 4.6 ± 2.0 days. No patient required reoperation. The mean ± SD CI increased from 69 ± 3 to 87 ± 5 for patients who underwent surgery before 45 days of age. Surgery resulted in a larger increase in CI for patients who underwent surgery at a younger age compared with older patients (p < 0.05, Tukey’s HSD test). In the comparison of patients who underwent surgery before 45 days of age with patients who underwent surgery at 45–90, 90–180, and more than 180 days of age, the linear mixed model estimated a long-term loss of CI of 3.0, 5.5, and 7.4 points, respectively. CONCLUSIONS The modified subtotal cranial vault remodeling technique used in this study significantly improved CI in patients with SS. The best results were achieved when surgery was performed early in life.

2019 ◽  
Vol 24 (2) ◽  
pp. 200-208
Author(s):  
Ravindra Arya ◽  
Francesco T. Mangano ◽  
Paul S. Horn ◽  
Sabrina K. Kaul ◽  
Serena K. Kaul ◽  
...  

OBJECTIVEThere is emerging data that adults with temporal lobe epilepsy (TLE) without a discrete lesion on brain MRI have surgical outcomes comparable to those with hippocampal sclerosis (HS). However, pediatric TLE is different from its adult counterpart. In this study, the authors investigated if the presence of a potentially epileptogenic lesion on presurgical brain MRI influences the long-term seizure outcomes after pediatric temporal lobectomy.METHODSChildren who underwent temporal lobectomy between 2007 and 2015 and had at least 1 year of seizure outcomes data were identified. These were classified into lesional and MRI-negative groups based on whether an epilepsy-protocol brain MRI showed a lesion sufficiently specific to guide surgical decisions. These patients were also categorized into pure TLE and temporal plus epilepsies based on the neurophysiological localization of the seizure-onset zone. Seizure outcomes at each follow-up visit were incorporated into a repeated-measures generalized linear mixed model (GLMM) with MRI status as a grouping variable. Clinical variables were incorporated into GLMM as covariates.RESULTSOne hundred nine patients (44 females) were included, aged 5 to 21 years, and were classified as lesional (73%), MRI negative (27%), pure TLE (56%), and temporal plus (44%). After a mean follow-up of 3.2 years (range 1.2–8.8 years), 66% of the patients were seizure free for ≥ 1 year at last follow-up. GLMM analysis revealed that lesional patients were more likely to be seizure free over the long term compared to MRI-negative patients for the overall cohort (OR 2.58, p < 0.0001) and for temporal plus epilepsies (OR 1.85, p = 0.0052). The effect of MRI lesion was not significant for pure TLE (OR 2.64, p = 0.0635). Concordance of ictal electroencephalography (OR 3.46, p < 0.0001), magnetoencephalography (OR 4.26, p < 0.0001), and later age of seizure onset (OR 1.05, p = 0.0091) were associated with a higher likelihood of seizure freedom. The most common histological findings included cortical dysplasia types 1B and 2A, HS (40% with dual pathology), and tuberous sclerosis.CONCLUSIONSA lesion on presurgical brain MRI is an important determinant of long-term seizure freedom after pediatric temporal lobectomy. Pediatric TLE is heterogeneous regarding etiologies and organization of seizure-onset zones with many patients qualifying for temporal plus nosology. The presence of an MRI lesion determined seizure outcomes in patients with temporal plus epilepsies. However, pure TLE had comparable surgical seizure outcomes for lesional and MRI-negative groups.


2021 ◽  
Vol 147 (3) ◽  
pp. 661-671
Author(s):  
Michael Alperovich ◽  
Christopher M. Runyan ◽  
Kyle S. Gabrick ◽  
Robin T. Wu ◽  
Chelsea Morgan ◽  
...  

Author(s):  
Seok Jong Chung ◽  
Yun Joong Kim ◽  
Han Soo Yoo ◽  
Jin Ho Jung ◽  
KyoungWon Baik ◽  
...  

Abstract Background To investigate the relationship between temporalis muscle thickness (TMT) at baseline as a surrogate marker for sarcopenia and long-term motor outcomes in patients with Parkinson’s disease (PD). Methods We enrolled 249 patients with drug-naïve early-stage PD (119 males and 130 females, follow-up &gt; 3 years). Baseline TMT of each patient was measured on the axial plane of T1-weighted images. The association between baseline TMT and long-term motor outcomes in PD was assessed using Cox regression models for levodopa-induced dyskinesia (LID), wearing-off, and freezing of gait (FOG) and a linear mixed model for the longitudinal increases in levodopa-equivalent dose (LED) per body weight over time. Statistical analyses were performed separately for sex if an interaction effect between TMT and sex was assumed. Results TMT differed substantially between the sexes, and male PD patients had higher TMT (6.69 ± 1.39 mm) than female PD patients (5.64 ± 1.34 mm, p &lt; 0.001). Cox regression models demonstrated that baseline TMT was not associated with the risk of developing LID, wearing-off, or FOG during the follow-up period. The linear mixed model was applied separately for sex and demonstrated that higher TMT at baseline was associated with slower increases in LED per body weight in male PD patients, but not in female PD patients. Conclusions This study demonstrated that baseline TMT could be an indicator of the longitudinal requirement for dopaminergic medications in male patients with PD, suggesting that sarcopenia may have a detrimental effect on disease progression in PD in a sex-specific manner.


Author(s):  
Marloes A. H. M. Michels ◽  
Kioa L. Wijnsma ◽  
Roel A. J. Kurvers ◽  
Dineke Westra ◽  
Michiel F. Schreuder ◽  
...  

Abstract Background C3 glomerulopathy (C3G) is a rare kidney disorder characterized by predominant glomerular depositions of complement C3. C3G can be subdivided into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). This study describes the long-term follow-up with extensive complement analysis of 29 Dutch children with C3G. Methods Twenty-nine C3G patients (19 DDD, 10 C3GN) diagnosed between 1992 and 2014 were included. Clinical and laboratory findings were collected at presentation and during follow-up. Specialized assays were used to detect rare variants in complement genes and measure complement-directed autoantibodies and biomarkers in blood. Results DDD patients presented with lower estimated glomerular filtration rate (eGFR). C3 nephritic factors (C3NeFs) were detected in 20 patients and remained detectable over time despite immunosuppressive treatment. At presentation, low serum C3 levels were detected in 84% of all patients. During follow-up, in about 50% of patients, all of them C3NeF-positive, C3 levels remained low. Linear mixed model analysis showed that C3GN patients had higher soluble C5b-9 (sC5b-9) and lower properdin levels compared to DDD patients. With a median follow-up of 52 months, an overall benign outcome was observed with only six patients with eGFR below 90 ml/min/1.73 m2 at last follow-up. Conclusions We extensively described clinical and laboratory findings including complement features of an exclusively pediatric C3G cohort. Outcome was relatively benign, persistent low C3 correlated with C3NeF presence, and C3GN was associated with higher sC5b-9 and lower properdin levels. Prospective studies are needed to further elucidate the pathogenic mechanisms underlying C3G and guide personalized medicine with complement therapeutics. Graphical abstract


2020 ◽  
Vol 26 (2) ◽  
pp. 113-121
Author(s):  
Ema Zubovic ◽  
Gary B. Skolnick ◽  
Sybill D. Naidoo ◽  
Mark Bellanger ◽  
Matthew D. Smyth ◽  
...  

OBJECTIVECombined metopic-sagittal craniosynostosis is traditionally treated with open cranial vault remodeling and fronto-orbital advancement, sometimes in multiple operations. Endoscopic treatment of this multisuture synostosis presents a complex challenge for the surgeon and orthotist.METHODSThe authors retrospectively analyzed the preoperative and 1-year postoperative CT scans of 3 patients with combined metopic-sagittal synostosis, all of whom were treated with simultaneous endoscope-assisted craniectomy of the metopic and sagittal sutures followed by helmet therapy. Established anthropometric measurements were applied to assess pre- and postoperative morphology, including cranial index and interfrontal divergence angle (IFDA). Patients’ measurements were compared to those obtained in 18 normal controls.RESULTSTwo boys and one girl underwent endoscope-assisted craniectomy at a mean age of 81 days. The mean preoperative cranial index was 0.70 (vs control mean of 0.82, p = 0.009), corrected postoperatively to a mean of 0.82 (vs control mean of 0.80, p = 0.606). The mean preoperative IFDA was 110.4° (vs control mean of 152.6°, p = 0.017), corrected postoperatively to a mean of 139.1° (vs control mean of 140.3°, p = 0.348). The mean blood loss was 100 mL and the mean length of stay was 1.7 days. No patient underwent reoperation. The mean clinical follow-up was 3.4 years.CONCLUSIONSEndoscope-assisted craniectomy with helmet therapy is a viable single-stage treatment option for combined metopic-sagittal synostosis, providing correction of the stigmata of trigonoscaphocephaly, with normalization of the cranial index and IFDA.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H Rai ◽  
F Alfonso ◽  
M Maeng ◽  
C Bradaric ◽  
J Wiebe ◽  
...  

Abstract Background Bioresorbable scaffolds (BRS) are novel devices designed to overcome the long-term limitations of permanent metallic stent implantation. Optical coherence tomography (OCT) surveillance can provide important insights on the process of vessel wall healing at follow-up. Purpose We sought to compare OCT-assessed healing at 6 months after implantation of everolimus-eluting BRS and everolimus-eluting metallic stents (EES) in patients treated for acute myocardial infarction (AMI). Methods ISAR-Absorb MI is a multicentre, 2:1 randomized trial comparing outcomes of patients with AMI stented with BRS or conventional EES. Angiographic surveillance was planned for all patients at 6–8 months follow-up. Patients who had OCT surveillance at follow-up were included for the present analysis. Analysis of contiguous OCT cross-sections- 1 mm apart was performed at a centralized core laboratory. Tissue characterisation using a 256-level grey-scale signal intensity (GSI) analysis was also performed for all neointimal regions of interest (ROI) with thickness of 100 to 400 μm. ROI's were classified as mature using a standard cut-off GSI score of 109.7. Generalised linear mixed model (GLMM) was used as appropriate. Statistical analysis was performed using R software. Data is presented as numbers, percentages or median (Interquartile range, IQR). Results Median follow-up interval was 216 days. 70 patients in the BRS arm and 33 patients in the EES arm were available for analysis. Stented length was 19.8 mm (13.6, 24.5) and 22.3 mm (16.7, 26.4) in BRS and EES arms respectively (p=0.73). Minimum lumen area [5.13 (3.95, 6.71) vs. 4.83 (3.63, 6.92) mm2] and minimum stent area [5.78 (4.88, 7.34) vs. 6.36 (4.70, 7.45) mm2] were comparable between BRS and EES. 2,262 frames (1,529 in BRS, 733 in EES) with 20,033 struts (12,704 in BRS, 7,329 in EES) were assessed. Overall strut coverage was better with BRS compared to EES (97.5% vs. 91.1% respectively, p<0.001). Malapposed (1.1% vs. 0.5%, p=0.54) and uncovered struts (7.3% vs. 1.3%, p<0.001) were more common with EES. Neointimal coverage was comparable amongst both stent groups [85.5 (61.9, 124.1) vs. 71.5 (33.4, 133.0) μm in BRS and EES groups respectively, p=0.50]. GSI analysis in 95 cases (65 cases, 2,233 ROIs in BRS; 30 cases, 1,210 ROIs in EES) showed that immature ROIs were numerically more common in the EES group as compared to the BRS group (75.4 vs. 57.0% respectively; p=0.35). Two-year clinical follow-up and analysis of correlation of clinical outcomes with OCT findings will also be available for presentation at ESC Congress 2019. Conclusions In selected patients undergoing OCT imaging at 6–8 months after implantation of BRS and conventional EES for AMI, we observed generally favourable healing characteristics with high rates of strut coverage, low rates of strut malapposition and fewer areas of immature neointimal areas with BRS in comparison to EES. Acknowledgement/Funding The study was predominantly funded by Deutsches Herzzentrum München and in part by a grant from Abbott Vascular


2021 ◽  
Vol 4 (2) ◽  
pp. V14
Author(s):  
Catherine Y. Wang ◽  
Alisha R. Bonaroti ◽  
Brandon A. Miller ◽  
James Liau

Sagittal craniosynostosis, the most common form of craniosynostosis, affects 1 per 1000 live births. The main surgical treatments include endoscopic suturectomy and open cranial vault remodeling. This video describes an open reconstruction method, including strip resection of the sagittal suture, biparietal craniotomies with spiral cut cranioplasty, and barrel staves of the posterior occiput. Ideally used between 4 and 15 months of age, this approach takes advantage of the flexibility of the cranial bones to expand, allowing for immediate and long-term increases of the parietal width and correction of cosmetic deformity, without necessitating the use of cranial molding devices postoperatively. The video can be found here: https://vimeo.com/516699203


2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.


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