Understanding the Operative Experience of the Practicing Pediatric Surgeon: Implications for Training and Maintaining Competency

Importance The number of practicing pediatric surgeons has increased rapidly in the past 4 decades, without a significant increase in the incidence of rare diseases specific to the field. Maintenance of competency in the index procedures for these rare diseases is essential to the future of the profession. Objective To describe the demographic characteristics and operative experiences of practicing pediatric surgeons using Pediatric Surgery Board recertification case log data. Design, Setting, and Participants We performed a retrospective review of 5 years of pediatric surgery certification renewal applications submitted to the Pediatric Surgery Board between 2009 and 2013. A surgeon’s location was defined by population as urban, large rural, small rural, or isolated. Case log data were examined to determine case volume by category and type of procedures. Surgeons were categorized according to recertification at 10, 20, or 30 years. Main Outcome and Measure Number of index cases during the preceding year. Results Of 308 recertifying pediatric surgeons, 249 (80.8%) were men, and 143 (46.4%) were 46 to 55 years of age. Most of the pediatric surgeons (304 of 308 [98.7%]) practiced in urban areas (ie, with a population >50 000 people). All recertifying applicants were clinically active. An appendectomy was the most commonly performed procedure (with a mean [SD] number of 49.3 [35.0] procedures per year), nonoperative trauma management came in second (with 20.0 [33.0] procedures per year), and inguinal hernia repair for children younger than 6 months of age came in third (with 14.7 [13.8] procedures per year). In 6 of 10 “rare” pediatric surgery cases, the mean number of procedures was less than 2. Of 308 surgeons, 193 (62.7%) had performed a neuroblastoma resection, 170 (55.2%) a kidney tumor resection, and 123 (39.9%) an operation to treat biliary atresia or choledochal cyst in the preceding year. Laparoscopy was more frequently performed in the 10-year recertification group for Nissen fundoplication, appendectomy, splenectomy, gastrostomy/jejunostomy, orchidopexy, and cholecystectomy (P < .05) but not lung resection (P = .70). It was more frequently used by surgeons recertifying in the 10-year group (used in 11 375 of 14 456 procedures [78.7%]) than by surgeons recertifying in the 20-year (used in 6214 of 8712 procedures [71.3%]) or 30-year group (used in 2022 of 3805 procedures [53.1%]). Conclusions and Relevance Practicing pediatric surgeons receive limited exposure to index cases after training. With regard to maintaining competency in an era in which health care outcomes have become increasingly important, these results are concerning

Management of Anorectal Malformations and Hirschsprung Disease.

Anorectal malformations (ARM) and Hirschsprung disease (HD) are managed with placement of normal intestine within the anal sphincter complex. Long-term complications specific to ARM include fistula remnants, recurrence, urinary reflux with associated chronic renal insufficiency, sexual dysfunction, and fertility difficulties. Complications specific to HD include enterocolitis, persistent or acquired aganglionosis, and internal sphincter achalasia. ARM and HD patients are both at risk of stricture, bowel dysfunction and incontinence, which can have a severe impact on quality of life. Bowel management strategies should be tailored to the patient\u27s specific category of bowel dysfunction

Temporary Retrograde Occlusion of High-Flow Tracheo-Esophageal Fistula

This report describes a temporary retrograde occlusion technique for control of a high-flow tracheo-esophageal fistula in a critically ill, premature infant born at 29 weeks’ gestational age, with a diagnosis of type C (Gross) esophageal atresia and tetralogy of Fallot (TOF). This procedure is a useful bridging maneuver before definitive surgical correction for extremely low birth weight, unstable neonates with tracheo-esophageal fistula who are suffering from associated malformations.</jats:p

Minimizing variance in pediatric patients after repair of anorectal malformations.

PURPOSE: A standardized anorectal malformation (ARM) perioperative protocol was implemented across two pediatric tertiary hospitals. The protocol addressed antibiotic duration, feeding advancement, VACTERL workup, and wound management. We aim to evaluate complications, length of stay (LOS), and protocol compliance. METHODS: Introduced in June 2020, data from a 24-month protocol cohort (after a 6-month transition) were compared to a 24-month control cohort. Outcomes included demographics, compliance, complications and LOS. RESULTS: Forty-five patients were included (control: 26, protocol: 19). Most were diagnosed with rectoperineal fistula (53%), 42% had significant cardiac disease, and 44% underwent prior colostomy. VACTERL workup was completed in 89%. Thirty-five patients underwent posterior sagittal anorectoplasty, and 10 cutback anoplasty. Median postoperative LOS was unchanged (control: 2 days; protocol: 2 days, p=0.80). Total LOS showed no difference (control: 3 days, protocol: 2 days, p = 0.51). No wound infections occurred. Wound dehiscence occurred in 1 control and 2 protocol patients (p=0.57). Compliance was 53% with deviations attributed to early diet initiation (n=4), incomplete VACTERL workup (n=2), and wound care (n=2). CONCLUSION: A standardized ARM perioperative protocol is feasible across institutions. It maintained excellent outcomes, including short LOS and few complications, though the small sample limited statistical power

Genetic and functional studies of a missense variant in a glutamate transporter, SLC1A3, in Tourette syndrome

OBJECTIVE: Abnormalities in neurotransmission within the cortico-striatal-thalamo-cortical circuitry are implicated in the pathogenesis of Tourette syndrome. Glutamate is a major excitatory neurotransmitter and an important member in the cortico-striatal-thalamo-cortical circuitry. To explore the role of glutamatergic neurotransmission in genetic susceptibility of Tourette syndrome, we carried out the genetic and functional characterization of sequence variants in SLC1A3 gene, which encodes the main glutamate transporter in astrocytes in individuals with well-characterized Tourette syndrome (n=256) and normal controls (n=224). METHODS: Exon-containing regions of SLC1A3 gene were screened using capillary electrophoresis-single strand conformation polymorphism followed by direct sequencing. Sequence variants were genotyped by restriction enzyme digestion and studied using glutamate uptake assay and membrane protein pull-down for transporter function. RESULTS: A missense variant involving a highly conserved residue, E219D, was identified in 11 heterozygous individuals with Tourette syndrome and four in the controls. The allele frequency for E219D was 2.4 folds higher in the Tourette syndrome (0.022) compared with the control cohort (0.009) although the difference did not reach statistical significance in the current cohorts (P=0.09). A H-glutamate-uptake assay showed that E219D conveys a significant increase (1.66 fold) in the SLC1A3-mediated glutamate uptake in HEK293 cells. A biotin-mediated membrane pull-down analysis showed a similar increase (1.5 fold) of mutant SLC1A3 protein in the membrane fraction of transfected HEK293 cells compared with that in the wild type controls. CONCLUSION: These results indicate that E219D is a functional SLC1A3 variant that is presented in a small number of individuals with Tourette syndrome. Further studies on possible changes in glutamate transport in the pathogenesis of Tourette syndrome are warranted