Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Refractory Gastroesophageal Reflux Disease: Diagnosis and Management.

Gastroesophageal reflux disease (GERD) is common, with increasing worldwide disease prevalence and high economic burden. A significant number of patients will remain symptomatic following an empiric proton pump inhibitor (PPI) trial. Persistent symptoms despite PPI therapy are often mislabeled as refractory GERD. For patients with no prior GERD evidence (unproven GERD), testing is performed off antisecretory therapy to identify objective evidence of pathologic reflux using criteria outlined by the Lyon consensus. In proven GERD, differentiation between refractory symptoms (persisting symptoms despite optimized antisecretory therapy) and refractory GERD (abnormal reflux metrics on ambulatory pH impedance monitoring and/or persistent erosive esophagitis on endoscopy while on optimized PPI therapy) can direct subsequent management. While refractory symptoms may arise from esophageal hypersensitivity or functional heartburn, proven refractory GERD requires personalization of the management approach, tapping from an array of non-pharmacologic, pharmacologic, endoscopic, and surgical interventions. Proper diagnosis and management of refractory GERD is critical to mitigate undesirable long-term complications such as strictures, Barrett's esophagus, and esophageal adenocarcinoma. This review outlines the diagnostic workup of patients presenting with refractory GERD symptoms, describes the distinction between unproven and proven GERD, and provides a comprehensive review of the current treatment strategies available for the management of refractory GERD.

The current state of gastrointestinal motility evaluation in cystic fibrosis: a comprehensive literature review.

Background and Objective: As life expectancy in cystic fibrosis (CF) has increased over the years, a shift in focus toward extra-pulmonary comorbidities such as gastrointestinal (GI) disease has become a topic of particular importance. Although not well-defined in the current literature, GI dysmotility is thought to significantly contribute to GI symptomatology in the CF population. The objective of this article was to provide a comprehensive review of diagnostic modalities at the disposal of the clinician in the evaluation of patients with CF (pwCF) presenting with GI complaints. Furthermore, we aimed to highlight the available literature regarding utilization of these modalities in CF, in addition to their shortcomings, and emphasize areas within the motility literature where further research is essential. Methods: A comprehensive review of all available literature in the English language through December 1, 2022 utilizing PubMed was conducted. Our search was limited to GI motility/transit and dysmotility in pwCF. Two researchers independently screened references for applicable articles and extracted pertinent data. Key Content and Findings: Several diagnostic imaging and manometry options exist in the evaluation of dysmotility; however, the literature is lacking in high-quality, prospective studies to validate such testing in pwCF. Common symptoms experienced and diagnostic motility tools available based on segment of the GI tract as related to pwCF are explored in the current review. Shortcomings in the current literature are identified and future direction to enhance research efforts within the field of CF-related dysmotility is provided. Conclusions: The influence of CF on GI integrity and motility is far-reaching. Despite improvements in longevity and advancement of pulmonary-specific treatment strategies, further high-quality research targeting the evaluation and management of GI dysmotility in pwCF is needed.

Analyzing Outcomes of Nuss and Ravitch Repair for Primary and Recurrent Pectus Excavatum in Adults.

BACKGROUND: Limited data exist for pectus excavatum repair in adults. We reviewed outcomes in adult patients undergoing Ravitch or Nuss pectus excavatum repairs to determine whether there was a statistical difference in postoperative complications and recurrence between primary and redo operations. METHODS: Patients undergoing pectus excavatum repair between 2001 and 2018 were excluded if they were receiving a concurrent unrelated operation, aged younger than 18 years, or had less than 1 year of follow-up (for recurrence analysis). Postoperative complications were recorded based on procedure type (Ravitch/Nuss) and iteration of repair (primary/redo). Continuous patient data were compared using Student t tests for variables such as age, length of stay, estimated blood loss, body mass index, and number of bars inserted. Fisher exact or χ2 tests were performed for postoperative complications and recurrence rates between groups. RESULTS: Of 290 patients, there were no significant differences in postoperative complications or recurrence rates between all Nuss repairs (n = 237; P = .59) and all Ravitch repairs (n = 53; P = .48), redo Nuss repairs (n = 53; P = .26) and Ravitch repairs (n=26; P = .99), and primary (P = .26) and redo Nuss (P = .10) repairs or primary (P = .99) and redo Ravitch (P = .99) repairs. There were significant differences in age, length of stay, follow-up, bars inserted, and estimated blood loss between all Nuss and Ravitch repairs (P < .05). CONCLUSIONS: Postoperative complication and recurrence rates were not statistically different between Nuss and Ravitch procedures of all types, suggesting either procedure may have utility in recurrent pectus excavatum. Further research may look to expand sample size and a prospective study investigating long-term outcomes.

Where Do We Begin? Building Blocks to Establish a Cardiothoracic Surgery Interest Group.

BACKGROUND: With the impending shortage of cardiothoracic (CT) surgeons, much focus has been on increasing trainee interest, particularly at the medical student level. We aimed to determine the effect of participation in our Cardiothoracic Surgery Interest Group (CTSIG) on medical student attitudes regarding surgery, and especially CT surgery. METHODS: An anonymous survey was administered at two time points to the current member list of our 2015 to 2016 CTSIG and repeated for the 2016 to 2017 academic year. A set CTSIG event schedule was developed with current CT faculty and included events every 1 to 2 months. Total CTSIG membership was 101 students. Descriptive analysis was performed on quantitative data. RESULTS: Of 73 preclinical students completing both pre/postsurveys, 62% (45) were male. Postsurveys demonstrated increased interest in surgery and CT surgery as a career, which was significantly greater than those reporting no change (p < 0.01 for both). Common factors mentioned as deterrents for a CT surgery career included lifestyle, length of training, and associated personality/culture. Events hosted by the CTSIG most frequently reported as increasing interest included lunches/dinners with CT surgery faculty (89%), a "Leadership in Surgery" event (58%), and an "Intro to CT Surgery and Q/A Session" hosted by a CT surgeon (51%). CONCLUSIONS: Establishing a CTSIG that includes preset events with exposure to CT surgery by shadowing and interaction with faculty in a relaxed setting has the ability to increase preclinical interest in CT surgery. More emphasis on advocacy is needed to clear up misconceptions about CT surgery to maintain recruitment of outstanding students in our CT surgery heritage.

Unmatched Integrated Cardiothoracic Surgery Program Applicants: Where Do They End Up?

BACKGROUND: With the recent introduction of Integrated Cardiothoracic Surgery Residency Programs (IPs), limited data exist on unmatched applicants. We aimed to determine the dropout rate in individuals who applied for IP but did not match. METHODS: An online Institutional Review Board-approved survey was sent to current residents (n = 409), provided by the Thoracic Surgery Directors Association, to evaluate preferences and pathway to cardiothoracic surgery (CTS). Descriptive analysis was performed on quantitative data. Main Residency Match Data from 2008 to 2017 were collected. RESULTS: Of 250 respondents, 89 (36%) were in IPs. In the cohort, 110 (44%) applied for at least one IP, whereas only 10 of the remaining 140 CTS residents had contemplated applying. From written comments of those 140 residents, the most common reasons against applying for IP programs were (1) uncertainty of IP training/belief that general surgery offered more well-rounded training and (2) uncommitted to CTS as a medical student. Interestingly, 96% of IP residents were set on a cardiac career, whereas a larger proportion of 4/3 and traditional residents were interested in general thoracic (36%). According to the National Resident Matching Program, 147 individuals applied to IP programs from 2008 to 2011 and were unmatched. Only 20 of those individuals (14%), from our results, ended up in a CTS residency program. CONCLUSIONS: Only a small percentage of applicants that did not match to an IP from 2008 to 2011 have ended up in CTS. As IPs continue to develop and improve, the concerns brought about by current CTS residents must be addressed to attract the next generation of exceptional surgeons.

An unusual etiology of hyperparathyroidism: robotic-assisted resection of a giant functional intrathymic parathyroid cyst.

Parathyroid cysts (PCs) are relatively rare entities, with an even smaller proportion that functionally produce parathyroid hormone (PTH). Given associated hypercalcemia, often symptomatic, as well as potentially related osteoporosis and/or nephrolithiasis, resection of these functional cysts is often indicated. This case report details the management course for a patient who presented with primary hyperparathyroidism and was ultimately found to have a functional intrathymic PC. During initial workup, 4-dimensional computed tomography (4D-CT) of the neck demonstrated enlarged left upper and right lower parathyroid glands; however, the patient's hyperparathyroid state persisted even after bilateral neck exploration and resection of these two glands. Subsequent postoperative imaging of the mediastinum revealed a large (11 cm) thymic cyst. The patient consequently underwent uneventful robotic-assisted thoracoscopic excision of the mediastinal cyst. Intraoperative blood PTH levels dropped from 734 pg/mL preoperatively to 86 pg/nL 10 minutes following resection, consistent with surgical cure by the Miami Criteria. At two months postoperatively, the patient's serum total calcium (STC) was normal at 9.2 mg/dL. Final surgical pathology noted a 15-gram parathyroid gland, with cystic degeneration. As the robot becomes further integrated into the everyday practice of thoracic surgery, we believe this approach offers advantages over conventional video-assisted thoracoscopic surgery (VATS) for mediastinal resections. Advantages include better visualization and finer, more precise dissection, especially important in this case, given the proximity of vital structures and the small, but real, risk of parathyromatosis associated with intraoperative cyst rupture.