John M. Hutson

Annals of surgery • December 18, 2024

Richard Wood, Shruthi Srinivas, Misel Trajanovska, Brennan Collis, Reuben Lim, Molly Fuchs, Daniel Dajusta, Chris Westgarth Taylor, Ihab Halaweish, Alessandra Gasior, Marc Levitt, John Hutson, Sebastian King

Objective: We aimed to study the impact of anorectal malformation (ARM) type and sacral ratio on continence outcomes in children. We secondarily aimed to compare continence outcomes by age group and determine quality of life (QoL) with different bowel regimens. Background: Children with ARM experience dysfunctional stooling into adulthood. Little is known about how ARM type, sacral ratio, age, and bowel regimen affect continence and QoL. Methods: We administered five validated survey measures on stooling habits and QoL to children aged 2-19 with ARM at two tertiary hospitals. Sacral ratio as a determinant of sacral hypodevelopment was defined as normal (≥0.7), moderate (0.4-0.0.69), or severe (≤0.39). Children not on an enema program were compared to those on an enema program to identify factors associated with achieving voluntary bowel movements defined as continence. All children were compared to identify factors associated with QoL. Results: Of 910 patients, half (52.8%) had mild sacral hypodevelopment. In patients not receiving enemas managed solely at study hospitals, most were continent (69.2%). There were no differences in continence demonstrated by sacral ratio; 79.4% of those who were continent had normal/moderate sacral hypodevelopment and mild ARM.Continence improved with age (50% ≤6 y old, 69.8% 6-12 y old, 82.1% >12 y old; P<0.001). Those on enemas and soiling had a QoL 19.6 points lower than those soiling on laxatives, and 20.7 points lower than those who were clean on enemas or continent with voluntary bowel movements without the need for enemas. Conclusions: In patients on laxatives the type of malformation, but not sacral ratio, is associated with continence in patients with ARM. Continence improves with age; those on enemas with soiling have a worse QoL.

Journal Of Pediatric Gastroenterology And Nutrition • Objective: Esophageal atresia (EA) is a significant congenital anomaly, with most survivors experiencing esophageal dysmotility. Currently, there is no reliable way to predict which patients will develop significant, life-threatening dysmotility. Using high-resolution impedance manometry (HRIM), this study aimed to characterize the common pressure topography patterns in children with repaired EA. Methods: This prospective longitudinal cohort study focused on children (<18 years) with repaired EA. Utilizing HRIM, esophageal motility patterns were studied. Repeat manometric assessments were performed in a selected group. Results: Seventy-five patients with EA (M:F = 43:32, median age 15 months [3 months to 17 years]) completed 133 HRIM studies. The majority (54 out of 75, 85.3%) had EA with distal tracheo-esophageal fistula. Thirty-five out of 75 (46.7%) underwent one study, 24 out of 75 (32.0%) two studies, 14 out of 75 (18.7%) three studies, and 2 out of 75 (2.7%) four studies. Seventy-two patients had analyzable studies. Three common motility patterns were demonstrated: (1) aperistalsis (26 out of 72, 36.1%); (2) distal esophageal contraction (25 out of 72, 34.7%); and (3) pressurization (6 out of 72, 8.3%). A minority demonstrated combination patterns, including aperistalsis with weak distal contraction (10 out of 72, 13.9%) and aperistalsis with pressurization (2 out of 72, 2.8%). Contraction was normal in 3 out of 72 (4.2%). At repeat assessment, the dominant motility pattern persisted in 26 out of 38 (68.4%) of the second studies and 9 out of 15 (60.0%) of the third studies. Conclusions: Utilizing HRIM in children with repaired EA, we have demonstrated objective, distinct, and reproducible motility patterns. In this cohort, the majority of motility patterns were maintained longitudinally, and dysphagia scores remained unchanged, even after dilatation.

Sharman Tan Tanny, Assia Comella, Lisa Mccall, John Hutson, Sue Finch, Mark Safe, Warwick Teague, Taher Omari, Sebastian King

Journal Of Pediatric Gastroenterology And Nutrition • July 06, 2023

Sharman Tan Tanny, Nicholas Senior, Assia Comella, Lisa Mccall, John Hutson, Sue Finch, Mark Safe, Warwick Teague, Taher Omari, Sebastian King

Objective: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). Methods: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. Results: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. Conclusions: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

Journal Of Pediatric Surgery • May 17, 2023

Hannah M Evans Barns, Marnie Hall, Misel Trajanovska, John Hutson, Frank Muscara, Sebastian King

Objective: The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health-related outcomes in children with chronic conditions. We performed a cross-sectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood. Methods: Parents/caregivers of children with HD, aged 4-14 years, managed at a tertiary pediatric surgical center were surveyed. Parent psychosocial outcomes, including adjustment to illness and family response, were assessed using four validated measures: Family Management Measure (FaMM); Parent Experience of Child Illness (PECI); Patient Reported Outcomes Measurement Information System (PROMISR) anxiety; and PROMISR depression. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess child quality of life (proxy-report). Results: Forty parents (mean age 38.7 ± 5.6 years) of children with HD (mean age 8.0 ± 2.5) participated. Parents expressed greater long-term uncertainty (PECI) and poorer perceived condition management ability (FaMM) than comparator chronic disease cohorts. Other scores for parental adjustment to their child's condition (PECI) and family response (FaMM) were comparable to reference cohorts. Symptoms of anxiety and depression were prevalent in our cohort (52.5 % and 42.5 % respectively); however, the proportion with moderate - severe PROMISR anxiety (χ2 = 2.50, p = 0.114) and depression (χ2 = 0.156, p = 0.693) scores did not significantly differ from the expected population distribution. Proxy-reported child quality of life (PedsQL) was significantly reduced relative to healthy children (p = 0.0003), but comparable to those with physical health problems with special healthcare needs (p = 0.624). Conclusions: Parents of children with HD experience long-term uncertainty and have poorer perceived condition management ability than parents of children with other chronic childhood illnesses. This work highlights the importance of targeted parental education and support beyond primary surgical management, and provides a benchmark for this cohort, against which subsequent intervention-based studies may be assessed. Methods: II.

Journal Of Pediatric Surgery • January 01, 2023

Hannah M Evans Barns, Liesel Porrett, Penelope Hartmann, Jessica Taranto, Suzie Jackson Fleurus, Phil Dinning, John Hutson, Warwick Teague, Sebastian King

Objective: The majority of patients with an anorectal malformation (ARM) have associated congenital anomalies. It is well established that all patients diagnosed with an ARM should undergo systematic screening, including renal, spinal, and cardiac imaging. This study aimed to evaluate the findings and completeness of screening, following local implementation of standardized protocols. Methods: A retrospective cohort study was performed assessing all patients with an ARM managed at our tertiary pediatric surgical center, following a standardized protocol implementation for VACTERL screening (January 2016-December 2021). Cohort demographics, medical characteristics, and screening investigations were analyzed. Findings were compared with our previously published data (2000-2015), conducted prior to protocol implementation. Results: One hundred twenty-seven (64 male, 50.4%) children were eligible for inclusion. Complete screening was performed in 107/127 (84.3%) children. Of these, one or more associated anomalies were diagnosed in 85/107 (79.4%), whilst the VACTERL association was demonstrated in 57/107 (53.3%). The proportion of children that underwent complete screening increased significantly in comparison with those assessed prior to protocol implementation (RR 0.43 [CI 0.27-0.66]; p < 0.001). Children with less complex ARM types were significantly less likely to receive complete screening (p = 0.028). Neither presence of an associated anomaly, nor prevalence of the VACTERL association, differed significantly by ARM type complexity. Conclusions: Screening for associated VACTERL anomalies in children with ARM was significantly improved following standardized protocol implementation. The prevalence of associated anomalies in our cohort supports the value of routine VACTERL screening in all children with ARM, regardless of malformation type. Methods: II.