Esophageal Per-Oral Endoscopic Myotomy (E-POEM): Future Directions and Perspectives.

PURPOSE OF REVIEW: POEM is a mature procedure endorsed by societal guidelines as a first line therapy for achalasia and spastic esophageal disorders. Nonetheless, several questions remain, including expanding indications for POEM, periprocedural evaluation and management, and the optimal POEM technique to enhance clinical success while mitigating risk for reflux. RECENT FINDINGS: There is uncertainty regarding several technical aspects of the POEM myotomy; though aggregating evidence supports the use of real-time impedance planimetry to guide the myotomy. While post-POEM reflux remains a concerning long term sequela, there is an increasing focus on the potential role of endoscopic anti-reflux interventions. Lastly, with the widespread adoption of POEM, we continue to witness ongoing efforts to standardize post-procedural care and training in this procedure. POEM is no longer a novel but rather established procedure. Yet, this technique has continued to evolve, with the aim of optimizing treatment success while reducing adverse events and risk for post-procedural reflux.

Short-term results of laparoscopic anti-reflux surgery with the RefluxStop device in patients with gastro-esophageal reflux disease and ineffective esophageal motility.

PURPOSE: In gastro-esophageal reflux disease (GERD) requiring surgical treatment, concomitant ineffective esophageal motility (IEM) is a decisive factor in surgical planning, due to concern regarding dysphagia. Anti-reflux surgery with the RefluxStop device is a promising technique. We assessed initial feasibility and clinical outcomes of RefluxStop surgery in patients with GERD and IEM. METHODS: Retrospective analysis of patients with GERD, hiatal hernia (HH), and IEM, who underwent surgery with RefluxStop at our institution and achieved 12-month follow-up. Technique feasibility was assessed, in addition to symptom resolution (GERD-HRQL questionnaire), adverse events, HH recurrence, dysphagia, and patient satisfaction. Placement of the device was confirmed by video fluoroscopy on postoperative day 1, and at 3 and 12 months. RESULTS: Between June 2020 and November 2022, 20 patients with IEM underwent surgery with RefluxStop and completed 12-month follow-up. All patients reported typical symptoms of GERD, and 12 had preoperative dysphagia. The median HH length was 4.5 cm (IQR, 3.75-5). The median operating time was 59.5 min (IQR, 50.25-64) with no implant-related intra- or postoperative complications. No HH recurrence was observed. One patient reported persistent left-sided thoracic pain at 11 months post-surgery, which required diagnostic laparoscopy and adhesiolysis. Three patients reported severe postoperative dysphagia: balloon dilatation was performed towards resolution. The mean GERD-HRQL scores improved (from 40.7 at baseline to 4.8 at 3 months and 5.7 at 12 months (p <0.001)). CONCLUSION: RefluxStop surgery was feasible and offered effective treatment for this group of patients with GERD and IEM. All patients had complete resolution or significant improvement of GERD symptoms, and 90% of them were satisfied with their quality of life 1 year after surgery.

Relationship Between Esophageal Disease and Pulmonary Fibrosis.

Esophageal disorders are prevalent among patients with chronic lung diseases, including idiopathic pulmonary fibrosis (IPF). Gastroesophageal reflux disease (GERD) has been associated with IPF prevalence, severity, and respiratory decline. The pathophysiologic relationship between GERD and IPF is likely bidirectional, with aspiration of refluxate leading to lung inflammation and fibrosis, while the restrictive pulmonary physiology may contribute to altered transdiaphragmatic pressure gradient and increased reflux. Esophageal symptoms are frequently absent and do not predict esophageal dysfunction or pathologic reflux in patients with IPF, and objective diagnostic tools including upper endoscopy, ambulatory reflux monitoring, and high-resolution manometry are often needed. Impedance-based testing that identifies both weakly/non-acidic and acid reflux may provide important additional diagnostic value beyond pH-based acid testing alone. Novel metrics and maneuvers, including advanced impedance measures on impedance-pH study and provocative testing on HRM, may hold promise to future diagnostic advancements. The main treatment options include medical therapy with acid suppressants and anti-reflux surgery, although their potential benefits in pulmonary outcomes of IPF require further validations. Future directions of research include identifying phenotypes of IPF patients who may benefit from esophageal testing and treatment, determining the optimal testing strategy and protocol, and prospectively assessing the value of different esophageal therapies to improve outcomes while minimizing risks. This review will discuss the pathophysiology, evaluation, and management of esophageal diseases, particularly GERD, in patients with IPF, as informed by the most recent publications in the field, in hopes of identifying targets for future study and research.

The broad impact of functional lumen imaging probe panometry in addition to high-resolution manometry in an esophageal clinical practice.

High-resolution manometry (HRM) with the Chicago Classification (CC) is the standard paradigm to define esophageal motility disorders. Functional lumen imaging probe (FLIP) panometry utilizes impedance planimetry to characterize esophageal compliance and secondary peristalsis. The aim of this study was to explore the clinical impact of FLIP panometry in addition to HRM. A retrospective chart review was performed on FLIP panometry cases utilizing the 322N catheter. Cases with prior foregut surgeries or botulinum injection within 6 months of FLIP panometry were excluded. EGJ-diameter and distensibility index (DI) and secondary contraction patterns at increasing balloon volumes were recorded. An EGJ-DI of ≥2.8 mm2/mm Hg at 60 mL was considered as a normal EGJ distensibility. CC diagnosis, Eckhardt score, Brief Esophageal Dysphagia Questionnaire, and clinical outcomes were obtained for each FLIP case. A total of 186 cases were included. Absent contractility and achalasia types 1 and 2 showed predominantly absent secondary contraction patterns, while type 3 had a variety of secondary contractile patterns on FLIP panometry. Among 77 cases with EGJ outflow obstruction (EGJOO), 60% had a low EGJ-DI. Among those with no motility disorder or ineffective esophageal motility on HRM, 27% had a low DI and 47% had sustained contractions on FLIP, raising concern for an esophageal dysmotility process along the achalasia and/or spastic spectrum. FLIP panometry often confirmed findings on HRM in achalasia and absent contractility. FLIP panometry is useful in characterizing EGJOO cases. Spastic features on FLIP panometry may raise concern for a motility disorder on the spastic spectrum not captured by HRM. Further studies are needed on FLIP panometry to determine how to proceed with discrepancy with HRM and explore diagnoses beyond the CC.

Elevated average maximum intrabolus pressure on high-resolution manometry is associated with esophageal dysmotility and delayed esophageal emptying on timed barium esophagram.

BACKGROUND: Intrabolus pressure (IBP) recorded by high-resolution manometry (HRM) portrays the compartmentalized force on a bolus during esophageal peristalsis. HRM may be a reliable screening tool for esophageal dysmotility in patients with elevated average maximum IBP (AM-IBP). Timed barium esophagram (TBE) is a validated measure of esophageal emptying disorders, such as esophagogastric junction outflow obstruction and achalasia. This study aimed to determine if an elevated AM-IBP correlates with esophageal dysmotility on HRM and/or delayed esophageal emptying on TBE. METHODS: A retrospective analysis of all HRM (unweighted sample n = 155) performed at a tertiary referral center from 09/2015-03/2017 yielded a case group (n = 114) with abnormal AM-IBP and a control group (n = 41) with a normal AM-IBP (pressure < 17 mmHg) as consistent with Chicago Classification 3. All patients received a standardized TBE, with abnormalities classified as greater than 1 cm of retained residual liquid barium in the esophagus at 1 and 5 min or as tablet retention after 5 min. RESULTS: AM-IBP was significantly related to liquid barium retention (p = 0.003) and tablet arrest on timed barium esophagram (p = 0.011). A logistic regression model correctly predicted tablet arrest in 63% of cases. Tablet arrest on AM-IBP correlated with an optimal prediction point at 20.1 mmHg on HRM. Patients with elevated AM-IBP were more likely to have underlying esophageal dysmotility (95.6% vs. 70.7% respectively; p < 0.001), particularly esophagogastric junction outflow obstruction disorders. Elevated AM-IBP was associated with incomplete liquid bolus transit on impedance analysis (p = 0.002). CONCLUSIONS: Our findings demonstrate that an elevated AM-IBP is associated with abnormal TBE findings of esophageal tablet retention and/or bolus stasis. An abnormal AM-IBP (greater than 20.1 mm Hg) was associated with a higher probability of retaining liquid bolus or barium tablet arrest on TBE and esophageal dysmotility on HRM. This finding supports the recent incorporation of IBP in Chicago Classification v4.0.

Effects of Pyridostigmine on Esophageal and Pharyngeal Motility in Dysphagic Patients Undergoing High-Resolution Manometry.

Weak or absent peristalsis of the esophageal musculature is a common finding in ambulatory patients suffering from dysphagia and frequently associated with gastroesophageal reflux. There is currently no pharmacologic intervention that reliably improves esophageal contractility in patients suffering from various esophageal motility disorders. Our objective was to evaluate the acute effects of pyridostigmine on high-resolution manometry parameters in patients suffering from dysphagia with evidence of esophageal dysmotility. Pyridostigmine is an acetylcholinesterase inhibitor which increases effective concentrations of acetylcholine at the neuromuscular junction of both striated and smooth muscle cells. We conducted a prospective crossover study of five patients with dysphagia and proven esophageal dysmotility. Three patients had baseline ineffective esophageal motility and two had achalasia. Patients underwent pharyngeal and esophageal manometry before and after pyridostigmine administration. The median distal contractile integral (DCI), a marker of esophageal contractile vigor, was significantly higher post pyridostigmine administration 3001 (1950.3-3703.2) mmHg × s × cm compared to pre-pyridostigmine DCI of 1229.9 (956.2-2100) mmHg × s × cm; P < 0.001. Pre-pyridostigmine 18/25 (72%) of the patient's swallows was peristaltic compared to 25/25 (100%) post-pyridostigmine; P < 0.005. No other pharyngeal or esophageal high-resolution manometry parameter differed significantly after pyridostigmine administration. The results of this pilot study demonstrate that pyridostigmine acutely improves esophageal contractile vigor in patients suffering from dysphagia with esophageal dysmotility. Further investigation with larger sample size, longer follow-up, side effect profile, and patient-reported outcome measures is still needed to determine the clinical usefulness of pyridostigmine in specific disorders of esophageal motility.

Foregut Dysmotility in the Lung Transplant Patient.

PURPOSE OF REVIEW: To explore the role of upper gastrointestinal disease in the clinical course of lung transplant patients - including its pathophysiology, diagnostic testing, and treatment options. RECENT FINDINGS: Gastroesophageal reflux disease (GERD) and foregut motility disorders are more prevalent among end-stage lung disease patients and are associated with poorer outcomes in lung transplant recipients. A proposed mechanism is the exposure of the lung allograft to aspirated contents, resulting in inflammation and rejection. Diagnostic tools to assess for these disorders include multichannel intraluminal impedance and pH (MII-pH) testing, high resolution esophageal manometry (HREM), and gastric emptying scintigraphy. The main treatment options are medical management with acid suppressants and/or prokinetic agents and anti-reflux surgery. In particular, data support the use of early anti-reflux surgery to improve outcomes. Newer diagnostic tools such as MII-pH testing and HREM allow for the identification of both acid and non-acid reflux and esophageal motility disorders, respectively. Recent studies have demonstrated that early anti-reflux surgery within six months post-transplant better protects against allograft injury and pulmonary function decline when compared to late surgery. However, further prospective research is needed to evaluate the short and long-term outcomes of these diagnostic approaches and interventions.

Endoscopic ultrasound: a powerful tool to modify treatment algorithms in opioid-induced achalasia.

BACKGROUND: Opioid use in the U.S. has increased dramatically over the last 15 years, recently being declared a public health emergency. Opioid use is associated with esophageal dysmotility lending to a confusing clinical picture compared to true achalasia. Patients exhibit symptoms and elicit diagnostic results consistent with esophageal motility disorders, in particular type III achalasia. Modified therapeutic strategies and outcomes become challenging. Differentiating true achalasia from opioid-induced achalasia is critical. Conventional surgical interventions, i.e., myotomy, are ineffective in the absence of true achalasia. We assess the utility of esophageal muscle layer mapping with endoscopic ultrasound (EUS) in distinguishing primary from opioid-induced achalasia. METHODS: From 2016 to 2019, patients with abnormal manometry and suspected achalasia underwent esophagogastroduodenoscopy and EUS mapping of esophageal round muscle layer thickness. Maximum round layer thickness and length of round muscle layer thickness > 1.8 mm were collected and compared between opioid users and non-opioid users using Wilcoxon Rank sum test. RESULTS: 45 patients were included: 12 opioid users, 33 non-opioid users. Mean age 56.8 years (range 24-93), 53.3% male patients. Mean BMI in the opioid-induced achalasia group was 30.2 kg/m2, mean BMI in the primary achalasia group 26.8 kg/m2 (p = 0.11). In comparing endoscopic maximum round layer thickness between groups, non-opioid patients had a thicker round muscle layer (2.7 mm vs 1.8 mm, p = 0.05). Length of abnormally thickened esophageal muscle (greater than 1.8 mm) also differed between the two groups; patients on opioids had a shorter length of thickening (4.0 cm vs 0.0 cm, p = 0.04). Intervention rate was higher in the non-opioid group (p = 0.79). Of the patients that underwent therapeutic intervention, symptom resolution was higher in the non-opioid group (p = 0.002), while re-intervention post-procedure for persistent symptomatology was elevated in the opioid subset (p = 0.06). Patients in the opioid group were less likely to undergo invasive treatment (Heller). As of 2017 all interventions in the opioid group have been endoscopic. CONCLUSION: Endoscopic ultrasound is an essential tool that has improved our treatment algorithm for suspected achalasia in patients with chronic opioid usage. Incorporation of EUS findings into treatment approach may prevent unnecessary surgery in opioid users.

Hirschsprung disease and other gastrointestinal motility disorders in patients with CCHS.

Congenital central hypoventilation syndrome (CCHS) is an autonomic nervous system dysfunction due to PHOX2B gene mutation. Little is known about gastrointestinal motility disorders in CCHS patients. This study aims to describe the spectrum of gastrointestinal motility disorders in CCHS and provide PHOX2B genotype-phenotype correlation with Hirschsprung Disease (HD). We reviewed the records of 72 CCHS patients seen at Children's Hospital Los Angeles from 1999 to 2019. Data collected included demographics, PHOX2B genotype, ventilator dependence, medical and surgical history, and gastrointestinal motility studies. Of the 72 patients, 31% had HD, 50% females, and 60% had 20/27 PARM. Rectosigmoid HD formed 73% of the cases whereas long segment (up to splenic flexure involvement) forms represented 23%. Four patients had total colonic aganglionosis, including one patient with 20/25 PARM genotype. One HD patient was identified with colonic myopathy in the residual segment. One patient was found to have achalasia type 1.Conclusion: Nearly one third of our CCHS patients had HD. Although most had 20/27 PARM, 2 patients had 20/25 PARM. Thus, CCHS patients with constipation are at risk for HD regardless of genotype. Colonic myopathy may coexist in treated HD with refractory constipation. Achalasia may occur in patients with CCHS. What is Known: • Patients with CCHS have motility disorders and present with esophageal dysmotility and constipation as a manifestation of their autonomic nervous system dysfunction. • About 20% of patients with CCHS have Hirschsprung disease and previously described to be associated with NPARM and 20/27 PARM genotype. What is New: • Thirty-one percent of CCHS patients in our series have Hirschsprung disease (HD). • HD, including the more severe total colonic aganglionosis was found in a patient with 20/25 PARM genotype suggesting that CCHS patients with constipation should be screened for HD regardless of genotype.

Esophageal dysmotility and lung disease in patients with systemic sclerosis: is there a possible association or correlation? A retrospective chart review.

OBJECTIVE: Our aim was to evaluate the relationship between esophageal dysmotility and lung disease by correlating the results of Echocardiogram and Pulmonary Function Test (PFT) with Esophageal Transit Study (ETT). METHODS: Charts of Systemic Sclerosis (SSc) patients fulfilling 2013 ACR/EULAR classification criteria seen in Rheumatology clinics were reviewed and their demographics, ETT result, PFT, and echocardiogram data were collected at baseline, years 1, 3, 5, and 10. Patients were divided based on their ETT status and were compared with respect to each variable using a two-sided two-sample t test for continuous variables and a Fisher's exact test for categorical variables. RESULTS: 130 patients were identified with either limited cutaneous SSc (109) or diffuse cutaneous SSc (21) with a mean age of 52.65 years. The mean DLCO was statistically worse in abnormal ETT patients [p value = 0.0004] as were the progression rates per year for DLCO at - 2.25 (p value = 0.019). Progression rate of FVC per year was statistically significant in the abnormal ETT group, although the mean value was not. The number of patients with abnormal PASP was not statistically different between the two groups (p values 0.104, 0.178, 0.653 at baseline, years 3 and 5, respectively). CONCLUSION: The presence of esophageal dysmotility was associated with increased pulmonary involvement in the form of abnormal DLCO with worsening progression rates per year. There was no statistically significant difference in PASP and FVC between the two groups; however, the progression rate for FVC was worse in adjusted models.

The Challenges of Esaphagogastric Junction Outflow Obstruction, Is It Really a Diagnosis? Creating a Systematic Clinical Approach for EGJOO.

Esophagogastric junction outflow obstruction (EGJOO) is currently diagnosed according to the Chicago Classification V3 by an elevated median integrated relaxation pressure on high resolution manometry. However, EGJOO may not be an accurate diagnosis, as it may be based on abnormal IRP from an artifact, affected by narcotics, an achalasia variant, or a mechanical cause of obstruction. This heterogenous diagnosis can often lead to unnecessary testing and treatment. The purpose of this study is to develop a stepwise clinical management protocol on how to evaluate EGJOO. Motility studies were reviewed for the last 2 years and 39 patients were diagnosed with EGJOO. Clinical information was reviewed, and patients were classified into six stepwise categories to explain an elevated IRP resulting in EGJOO diagnosis: (1) underlying catheter artifact (2) opioid use (3) achalasia variant (4) jackhammer esophagus with obstruction (5) missed esophageal lesion (ex. Schatzki ring, EOE) and (6) extrinsic compression. 40% (n = 14) of patients with elevated IRP were due to an underlying catheter artifact. 8.6% (n = 3) were due to opioid use. 8.6% (n = 3) were due to achalasia variant. 31.4% (n = 11) were due to jackhammer esophagus with obstruction. 5.7% (n = 2) were due to missed esophageal lesion. 5.7% (n = 2) were due to external compression by cardiomegaly and aortic aneurism. EGJOO is not a diagnostic end point, but a heterogenous category with multiple underlying etiologies. We believe the use of a stepwise approach to these patients can help avoid further unnecessary testing.

Patterns of esophageal dysmotility elicited by multiple rapid swallows.

BACKGROUND: High-resolution manometry (HRM) is a gastrointestinal motility diagnostic system that measures intraluminal pressures using closely aligned sensors. Multiple rapid swallows (MRS) are used in conjunction with HRM to assess esophageal physiology prior to anti-reflux and hiatal hernia procedures. METHODS: A retrospective, qualitative study was conducted on 90 patients who underwent HRM with MRS in a single community clinic. 80 patients met the inclusion criteria. MRS testing consisted of rapid 2 mL swallows in 2-3 s intervals with patients in a seated, upright position. Clinical information was reviewed including indications for HRM, prior diagnostic workup, manometry, distal contractile integral (DCI), and integrated residual pressure (IRP). HRM studies were visualized using Manoview Analysis Software v3.0 (Medtronic). RESULTS: Certain esophageal dysmotility and pressurization manometry patterns were previously undetected on HRM alone. In our study, the addition of MRS was clinically helpful in (1) assessing contraction reserve, (2) highlighting features of jackhammer, (3) stimulating esophageal spasm, and (4) visualizing distal esophageal pressurization pattern with mechanical obstruction. Additionally, abnormal pathophysiology such as (5) paradoxical LES contraction (achalasia) and (6) loss of deglutition inhibition were identified. MRS had a diagnostic utility of 21.25% (n = 17) among the 80 patients. An intolerance rate of 7.7% (n = 7) was observed in patients unable to complete the protocol. CONCLUSIONS: Augmentation of HRM with MRS produces unique manometric features that have clinical utility in uncovering esophageal disorders. MRS provocation testing is a practical, inexpensive, well-tolerated addition to HRM that may yield useful clinical information to guide complicated diagnoses and medical management.

Open peroral endoscopic myotomy for the treatment of achalasia: a case series of 82 cases.

During peroral endoscopic myotomy (POEM), creation of the tunnel is highly technically demanding and mucosal injury is one of the most common potential complications. We explored a method without a submucosal tunnel, which we call open peroral endoscopic myotomy (O-POEM). This study aimed to assess the feasibility and safety of O-POEM. O-POEM was performed on 82 patients with achalasia. Treatment success was defined as an Eckardt score of less than or equal to 3 after the myotomy. Adverse events including operative and postoperative adverse events were recorded. Treatment success and procedure-related adverse events were analyzed. After a median follow-up of 18 months (range: 6-26 months), the treatment success (Eckhart score ≤3) was achieved in 96.3% of cases (mean score pre- vs. post-treatment (7.4 vs. 1.8); P < 0.001) with a recurrence of 3 cases. Ten patients (12.2%) had adverse events consisting of 2 cases of mediastinitis, 1 case of post-O-POEM bleeding, 1 case of subcutaneous emphysema, 6 cases of pleural effusion. Two cases of mediastinitis required intraprocedural drainage, and other patients were managed by endoscopy and conservative medical treatment. There were no deaths. No patients required surgical conversion. Clinical reflux occurred in 15.9% of patients (13/82). O-POEM was reliable and effective for the treatment of achalasia. In addition, O-POEM might be a better option for patients with severe submucosal fibrosis.

The impact of gastroesophageal reflux disease symptoms in scleroderma: effects on sleep quality.

Systemic scleroderma/sclerosis (SSc) is an autoimmune connective tissue disease, which can lead to esophageal motor dysfunction and gastroesophageal reflux disease (GERD). Nocturnal GERD symptoms may be associated with sleep disturbances, which in turn can drastically affect well-being and fatigue levels. We hypothesized that GERD symptoms would be associated with poorer sleep in patients with SSc. Rheumatologist established SSc patients completed the following questionnaires: the UCLA scleroderma clinical trial consortium gastrointestinal tract instrument (GIT) 2.0 questionnaire; the Pittsburgh sleep quality index (PSQI); the fatigue severity scale (FSS); the multidimensional gastrointestinal symptom severity index (GSSI). Poor sleep quality was defined by a PSQI total score >5. Questionnaires were completed by 287 patients [mean (SD) age = 59 (14) years; female = 243]. Poor sleep quality was identified in 194 (68%) patients. Patients with poor sleep quality reported less sleep time and increased fatigue compared to those with normal sleep scores. SSc patients with poor sleep had significantly higher GIT Reflux scores (P < .001), and poor sleep was more frequent in those with moderate/severe versus mild/no heartburn on GISSI (P < .001). Narcotic and antidepressant use was significantly more frequent in SSc patients with poor sleep quality. Multivariable logistic regression supported the association between GERD symptoms and poor sleep after controlling for age, sex, and body mass index (BMI) (2.53, 95% confidence interval (CI) 1.52-4.25; P < .001). The association remained after controlling for narcotic and antidepressant use (2.20, 95% CI 1.29-3.73; P < .001). SSc patients who reported GERD symptoms were also more likely to report poor sleep quality. Future studies should examine mechanisms underlying nocturnal GERD symptoms in SSc patients, and the impact of improved GERD symptom control on sleep quality.

Impact of Toupet Versus Nissen Fundoplication on Dysphagia in Patients With Gastroesophageal Reflux Disease and Associated Preoperative Esophageal Dysmotility: A Systematic Review and Meta-Analysis.

BACKGROUND: Controversy exists regarding the best surgical approach for the management of gastroesophageal reflux disease (GORD) and associated preoperative esophageal dysmotility. Our aim was to conduct a systematic review and meta-analysis to compare the outcomes of Toupet fundoplication (TF) and Nissen fundoplication (NF) in patients with GORD and coexistent preoperative esophageal dysmotility. METHODS: We conducted a systematic search of electronic information sources, including MEDLINE, EMBASE, CINAHL, CENTRAL, ClinicalTrials.gov , and bibliographic reference lists. We applied a combination of free text search and controlled vocabulary search adapted to thesaurus headings, search operators, and limits in each of the above-mentioned databases. Postoperative dysphagia and improvement in dysphagia were primary outcome parameters. RESULTS: We identified 3 randomized controlled trials and 1 observational study reporting a total of 220 patients, of whom 126 underwent TF and the remaining 94 patients had NF. Despite the existence of significantly higher preoperative dysphagia in the TF group (29.3% vs 4.2%, P = .05), TF was associated with significantly lower postoperative dysphagia (odds ratio [OR] = 0.31, P = .002) with low between-study heterogeneity ( I2 = 11%, P = .34), and significantly higher improved dysphagia (OR = 10.32, P < .0001) with moderate between-study heterogeneity ( I2 = 31%, P = .23) compared with NF. CONCLUSION: TF may be associated with significantly lower postoperative dysphagia than NF in patients with GORD and associated preoperative esophageal dysmotility. However, no definite conclusions can be drawn as the best available evidence comes mainly from a limited number of heterogeneous randomized controlled trials. Future studies are encouraged to include patients with similar preoperative dysphagia status and report the outcomes with respect to recurrence of acid reflux symptoms.

Subclinical dysphagia in persons with Prader-Willi syndrome.

Prader-Willi Syndrome (PWS) is caused by a genetic imprinting abnormality resulting from the lack of expression of the paternal genes at 15q11-q13. Intellectual disability, low muscle tone, and life-threatening hyperphagia are hallmarks of the phenotype. The need for the Heimlich maneuver, death from choking, and pulmonary infection occur in a disproportionally high number of persons with PWS. The widely held belief is that eating behaviors are responsible for choking and aspiration; yet, no investigation had sought to determine if swallowing impairments were present in persons with PWS. To address this research and clinical gap, simultaneous videofluoroscopy and nasal respiratory signals were used to record swallowing function and breathing/swallowing coordination in 30 participants with PWS. Subjects consumed thin liquid and barium cookies under two randomized conditions as follows: (i) controlled (cues to swallow and standardized bolus sizes); (ii) spontaneous (no cues or bolus size control). Under videofluoroscopy, the cohort showed disordered pharyngeal and esophageal swallowing in both conditions with disturbances in timing, clearance, and coordination of swallowing with the respiratory cycle. No participant showed a sensory response such as attempting to clear residue or coughing; thereby supporting the lack of overt symptoms. We conclude that the high death rate from choking and pulmonary infection in children and adults with PWS may be related, in part, to underlying, asymptomatic dysphagia. The combination of rapid eating and dysphagia would increase the risk of aspiration-related morbidity and mortality. © 2016 Wiley Periodicals, Inc.

Clinical presentation and disease course of patients with esophagogastric junction outflow obstruction.

Esophagogastric junction outflow obstruction, characterized by preserved peristalsis in conjunction with an elevated integrated relaxation pressure, can result from specific anatomic variants or may represent achalasia in evolution. There is limited information on the clinical significance of this diagnosis. The aim of this study is to describe the clinical characteristics and outcomes in our cohort of patients with esophagogastric junction outflow obstruction.Consecutive adult patients who had undergone high-resolution esophageal manometry between February 2013 and November 2015 with a diagnosis of esophagogastric junction outflow obstruction were identified. Electronic medical records were reviewed to determine: (1) secondary causes of esophagogastric junction outflow obstruction; (2) treatment; and (3) natural history. Improvement in symptoms noted during follow-up evaluation was considered to be a favorable outcome. Worsening of symptoms or no change in symptoms was considered to be an unfavorable outcome.Of 874 manometries performed during this time period, 83 met the criteria for esophagogastric junction outflow obstruction. Of these patients, 11 had secondary causes: paraesophageal hernia (4), Nissen fundoplication (2), esophageal stricture (3), prior laparoscopic band placement (1), and diverticulum (1). All of these secondary causes were identified by barium esophagram. The remaining 72 patients were categorized as idiopathic esophagogastric junction outflow obstruction. Two patients developed type II achalasia on follow-up. An additional two patients had no symptoms as testing was performed for preoperative evaluation prior to bariatric surgery, leaving 68 patients for symptom follow-up analysis. Of these, 19 had a favorable outcome, 18 had an unfavorable outcome, and 31 were lost to follow-up. Of those with a favorable outcome, 6 patients underwent treatment: medication (3), botulinum toxin injection followed by laparoscopic Heller myotomy (1), botulinum toxin injection and medication (1), and bougie dilation (1). Of the 18 patients with an unfavorable outcome, 6 patients underwent treatment: botulinum toxin injection (5) and medication (1). Computed tomography scan or endoscopic ultrasound was performed in 40% of patients with available follow-up and none of these studies revealed secondary causes. The overall median follow-up time was 5 months.Esophagogastric outflow obstruction is a manometric finding of unclear significance. Secondary causes should first be excluded with structural studies. The evolution of esophagogastric junction outflow obstruction to achalasia is rare. Symptoms in patients with esophagogastric junction outflow obstruction do not always require treatment and treatment response is variable. The challenge in managing these patients lies in distinguishing which patients will need intervention. Further studies are needed for consideration of subgrouping this disease or modifying the categorization into clinically relevant entities.

Symptom association latency during ambulatory reflux monitoring: a review of 1445 symptoms.

To determine the optimal time window of symptom association in GERD patient, a 2-minute time window is nonapplicable for all gastroesophageal reflux (GERD) symptoms and 5 minutes remains an arbitrary choice. Symptom association analysis is a critical component in pH-impedance (imp) testing. Symptom index (SI) and symptom association probability (SAP) are the two widely accepted methods. Both were introduced in the preimpedance era and initially tested in patients with typical GERD symptoms. We reviewed ambulatory pH-imp studies of (294) patients referred for evaluation of possible GERD symptoms from January 2012 to August 2015. Patients with heartburn (HB), regurgitation (Reg), cough, and throat clearing (TC) alone or in combination were reviewed. The analysis time windows were separated into five intervals (0-1, 1-2, 2-3, 3-4, 4-5 minutes) to explore the frequency of symptom occurrence in each window. The SI was then calculated and contrasted to the usual 5-minute window; similar calculations were made to SAP 2-minute windows. Secondary analysis was performed to test whether symptoms due to acid reflux have a different time perception than non-acid. Overall, there were 1445 total symptoms preceded by impedance detected reflux. Frequency analysis showed that (34.7%- 86.7%) of symptoms occurred in the first minute after onset of the reflux. χ2 showed a significant association between SI with 1 minute and SAP for heartburn (P ≤ 0.0001) and regurgitation (P = 0.0003). There was also a significant association between cough with 2-minute window and SAP (P = 0.025). There was no significant association between TC at 1-, 2-, 3-, and 4-minute time windows and SAP. There was no evidence of increased frequency of positive reflux to symptoms over time. There was also no significant difference in time window in relation to acid or nonacid reflux. It seems doubtful that a universal time window can be applied to all GERD symptoms to calculate symptom association.

How to Optimally Apply Impedance in the Evaluation of Esophageal Dysmotility.

The utilization of impedance technology has enhanced our understanding and assessment of esophageal dysmotility. Esophageal high-resolution manometry (HRM) catheters incorporated with multiple impedance electrodes help assess esophageal bolus transit, and the combination is termed high-resolution impedance manometry (HRIM). Novel metrics have been developed with HRIM-including esophageal impedance integral ratio, bolus flow time, nadir impedance pressure, and impedance bolus height-that augments the assessment of esophageal bolus transit. Automated impedance-manometry (AIM) analysis has enhanced understanding of the relationship between bolus transit and pressure phenomena. Impedance-based metrics have improved understanding of the dynamics of esophageal bolus transit into four distinct phases, may correlate with symptomatic burden, and can assess the adequacy of therapy for achalasia. An extension of the use of impedance involves impedance planimetry and the functional lumen imaging probe (FLIP), which assesses esophageal biophysical properties and distensibility, and could detect patterns of esophageal contractility not seen on HRM. Impedance technology, therefore, has a significant impact on esophageal function testing in the present day.

Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.