Effectiveness of the Chiari Health Index for Pediatrics instrument in measuring postoperative health-related quality of life in pediatric patients with Chiari malformation type I.

OBJECTIVE: The authors' previously published work validated the Chiari Health Index for Pediatrics (CHIP), a new instrument for measuring health-related quality of life (HRQOL) for pediatric Chiari malformation type I (CM-I) patients. In this study, the authors further evaluated the CHIP to assess HRQOL changes over time and correlate changes in HRQOL to changes in symptomatology and radiological factors in CM-I patients who undergo surgical intervention. Strong HRQOL evaluation instruments are currently lacking for pediatric CM-I patients, creating the need for a standardized HRQOL instrument for this patient population. This study serves as the first analysis of the CHIP instrument's effectiveness in measuring short-term HRQOL changes in pediatric CM-I patients and can be a useful tool in future CM-I HRQOL studies. METHODS: The authors evaluated prospectively collected CHIP scores and clinical factors of surgical intervention in patients younger than 18 years. To be included, patients completed a baseline CHIP captured during the preoperative visit, and at least 1 follow-up CHIP administered postoperatively. CHIP has 2 domains (physical and psychosocial) comprising 4 components, the 3 physical components of pain frequency, pain severity, and nonpain symptoms, and a single psychosocial component. Each CHIP category is scored on a scale, with 0 indicating absent and 1 indicating present, with higher scores indicating better HRQOL. Wilcoxon paired tests, Spearman correlations, and linear regression models were used to evaluate and correlate HRQOL, symptomatology, and radiographic factors. RESULTS: Sixty-three patients made up the analysis cohort (92% Caucasian, 52% female, mean age 11.8 years, average follow-up time 15.4 months). Dural augmentation was performed in 92% of patients. Of the 63 patients, 48 reported preoperative symptoms and 42 had a preoperative syrinx. From baseline, overall CHIP scores significantly improved over time (from 0.71 to 0.78, p < 0.001). Significant improvement in CHIP scores was seen in patients presenting at baseline with neck/back pain (p = 0.015) and headaches (p < 0.001) and in patients with extremity numbness trending at p = 0.064. Patients with syringomyelia were found to have improvement in CHIP scores over time (0.75 to 0.82, p < 0.001), as well as significant improvement in all 4 components. Additionally, improved CHIP scores were found to be significantly associated with age in patients with cervical (p = 0.009) or thoracic (p = 0.011) syrinxes. CONCLUSIONS: The study data show that the CHIP is an effective instrument for measuring HRQOL over time. Additionally, the CHIP was found to be significantly correlated to changes in symptomatology, a finding indicating that this instrument is a clinically valuable tool for the management of CM-I.

Deformational brachycephaly: the clinical utility of the cranial index.

OBJECTIVE: The incidence of deformational brachycephaly has risen since the "Back to Sleep" movement in 1992 by the American Academy of Pediatrics. Brachycephaly prevalence and understanding the dynamic nature of the pediatric skull have not been explored in relation to the cranial index (CI). The objective of the study was to determine the prevalence of brachycephaly, via the CI, with respect to time. METHODS: The authors conducted a retrospective review of 1499 patients ≤ 19 years of age who presented for trauma evaluation with a negative CT scan for trauma (absence of bleed) in 2018. The CI was calculated using CT at the lateral-most point of the parietal bone (cephalic width), and the distance from the glabella to the opisthocranion (cephalic length). Brachycephaly was defined as a CI ≥ 90%. RESULTS: The mean CI was 82.6, with an average patient age of 6.8 years. The prevalence of deformational brachycephaly steadily decreased from 27% to 4% from birth to > 2 years of life. The mean CI was statistically different between ages < 12 months, 12-24 months, and > 24 months (F[2,1496] = 124.058, p < 0.0005). A simple linear regression was calculated to predict the CI based on age; the CI was found to decrease by 0.038 each month. A significant regression equation was found (F[1,1497] = 296.846, p < 0.0005), with an R2 of 0.140. CONCLUSIONS: The incidence of deformational brachycephaly is common in infants but decreases as the child progresses through early childhood. Clinicians can expect a significant decrease in mean CI at 12 and 24 months. Additionally, these regression models show that clinicians can expect continued improvement throughout childhood.

Management of Suprasellar Arachnoid Cysts in Children: A Systematic Literature Review Highlighting Modern Endoscopic Approaches.

OBJECTIVE: Suprasellar arachnoid cysts are a rare but important pediatric neurosurgical pathology with unknown ideal management. They have been previously managed with techniques including open craniotomy with microsurgical fenestration, cystoperitoneal shunting, endoscopic ventriculocystostomy, and endoscopic ventriculocystocisternostomy (VCC), without a consistent consensus on the best surgical approach. We present an overview of the literature on surgical management of suprasellar arachnoid cysts. METHODS: A literature search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted for all articles evaluating treatment modalities for suprasellar arachnoid cysts, using PubMed, OVID, and Web of Science. RESULTS: Twenty-five articles on management of suprasellar arachnoid cysts in children were identified. Few published studies exist that examine different types of surgical management across a single institution. The majority of studies reported best clinical outcomes in patients treated with endoscopic approaches when compared with microsurgical fenestration or cystoperitoneal shunting, reporting lower rates of infection, shunt dependence, and need for revision in addition to better resolution of clinical symptoms. Furthermore, most studies argue that VCC is superior to ventriculocystostomy, offering better long-term improvement of clinical symptoms and lower rates of failure. CONCLUSIONS: This study examines the current literature on suprasellar arachnoid cyst surgical management to conclude that an endoscopic approach in comparison with other approaches has the best outcomes. Of the endoscopic options available, VCC provides patients with the best long-term resolution of symptoms and the lowest need for revision. These findings should be further investigated with larger multicenter studies to further compare different surgical techniques and outcomes.

Pediatric cranial deformations: demographic associations.

OBJECTIVE: No study has established a relationship between cranial deformations and demographic factors. While the connection between the Back to Sleep campaign and cranial deformation has been outlined, considerations toward cultural or anthropological differences should also be investigated. METHODS: The authors conducted a retrospective review of 1499 patients (age range 2 months to less than 19 years) who presented for possible trauma in 2018 and had a negative CT scan. The cranial vault asymmetry index (CVAI) and cranial index (CI) were used to evaluate potential cranial deformations. The cohort was evaluated for differences between sex, race, and ethnicity among 1) all patients and 2) patients within the clinical treatment window (2-24 months of age). Patients categorized as "other" and those for whom data were missing were excluded from analysis. RESULTS: In the CVAI cohort with available data (n = 1499, although data were missing for each variable), 800 (56.7%) of 1411 patients were male, 1024 (79%) of 1304 patients were Caucasian, 253 (19.4%) of 1304 patients were African American, and 127 (10.3%) of 1236 patients were of Hispanic/Latin American descent. The mean CVAI values were significantly different between sex (p < 0.001) and race (p < 0.001). However, only race was associated with differences in positional posterior plagiocephaly (PPP) diagnosis (p < 0.001). There was no significant difference in CVAI measurements for ethnicity (p = 0.968). Of the 520 patients in the treatment window cohort, 307 (59%) were male. Of the 421 patients with data for race, 334 were Caucasian and 80 were African American; 47 of the 483 patients with ethnicity data were of Hispanic/Latin American descent. There were no differences between mean CVAI values for sex (p = 0.404) or ethnicity (p = 0.600). There were significant differences between the mean CVAI values for Caucasian and African American patients (p < 0.001) and rate of PPP diagnosis (p = 0.02). In the CI cohort with available data (n = 1429, although data were missing for each variable), 849 (56.8%) of 1494 patients were male, 1007 (67.4%) of 1283 were Caucasian, 248 (16.6%) of 1283 were African American, and 138 patients with ethnicity data (n = 1320) of Hispanic/Latin American descent. Within the clinical treatment window cohort with available data, 373 (59.2%) of 630 patients were male, 403 were Caucasian (81.9%), 84 were African American (17.1%), and 55 (10.5%) of 528 patients were of Hispanic/Latin American descent. The mean CI values were not significantly different between sexes (p = 0.450) in either cohort. However, there were significant differences between CI measurements for Caucasian and African American patients (p < 0.001) as well as patients of Hispanic/Latin American descent (p < 0.001) in both cohorts. CONCLUSIONS: The authors found no significant associations between cranial deformations and sex. However, significant differences exist between Caucasian and African American patients as well as patients with Hispanic/Latin American heritage. These findings suggest cultural or anthropological influences on defining skull deformations. Further investigation into the factors contributing to these differences should be undertaken.