Introduction

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease in which muscle weakness develops insidiously. In advanced stages of the disease dysphagia, weight loss and malnutrition occur in the majority of patients. Thus, the consideration of Percutaneous Endoscopic Gastrostomy (PEG) tube placement is common. However, the optimal timing and setting for the procedure as well desired spirometry results are either are not stated in most guidelines or debated. Previous studies have suggested greater procedural risk for patients whose forced vital capacity (FVC) was less than 50% than predicted

Aims & Methods

The aim of this study was to assess the safety of our tertiary center simplified approach to PEG tube placement in patients with ALS.
The electronical records of patients who underwent percutaneous endoscopic gastrostomy (PEG) placement between 1.1.2020 to 1.1.2023 were retrospectively reviewed. All adult patients diagnosed with ALS who underwent ambulatory PEG tube placement were included. Patients who underwent PEG tube placement during hospitalization due to acute illness were excluded. The common practice of our center regarding PEG placement in patients with ALS is as follows: initial evaluation at the Gastroenterology clinic of patients referred by the neurology clinic, followed by ambulatory PEG placement under sedation by an anesthesiologist. We collected baseline information from the neurology and gastroenterology clinic visits regarding comorbidities and baseline FVC and the functional rating score [ALS-FRS, 0-48 points, lower score indicating a more severe disease). The data concerning the procedure and possible complications were collected from endoscopy reports, emergency department visits and clinic visits after the procedure.

Results

Six hundred and sixty patients had underwent PEG tube placement in our center during study period. Of them, 67 patients had an established diagnosis of ALS (10.2%). One patient was excluded due to PEG placement in the setting of an acute illness during admission, thus 66 patients were included (mean age at PEG placement 63.5±12.7 years and 37.8% were females). Mean ALS-FRS score was 23.6±8.81 and mean FVC was 49.8±19.45% of predicted. More than half (54.7%) of the patients were using non-invasive ventilation support device at home prior to the procedure and 12.1% of patients had underwent endotracheal tube placement prior to PEG tube placement.
The averaged duration of PEG tube placement was 11.96±5.3 minutes. All PEG procedures were completed successfully. Complications were documented during two PEG placements (3%): one patient suffered from aspiration and was hospitalized and later diagnosed with aspiration pneumonia and the second patient suffered from gastric perforation and peritonitis which was diagnosed a few hours after the procedure. During the 30 days following PEG placement, 3 (4.5%) complications were documented: Two patients (3%) suffered from PEG site infections and were treated with antibiotics and one (1.5%) was diagnosed with aspiration pneumonia. No deaths occurred during the procedure or during the 30 days that followed.

Conclusion

The simple straight forward approach to PEG placement in our institution was successful and safe in the vast majority of cases. The complications rate in our cohort is comparable to other studies, even though the patients in our cohort had significantly reduced lung function and functional scores then accepted in the common practice.

References

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2. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis: Andersen P M, Abrahams S, EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force, Eur J Neurol 2012 Mar;19(3):360-75.
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Introduction

Dysphagia is a common condition with consequences surpassing the physical dimension and extending to the patient’s psychological, personal, and professional life. However, when referring to non-obstructive esophageal dysphagia (NOD) and motility disorders, data is still scarce.

Aims & Methods

Our study consists in a prospective and multicentric evaluation of the severity of dysphagia and its impact on quality of life, that was measured with the Eckardt, EuroQol-5D (TTO and VAS) and PROMIS GI Disrupted Swallowing (PDS) scores, in patients with NOD and underlying motility disorders. We included 373 individuals (250 with NOD and 123 without dysphagia).

Results

Among patients with NOD, 97 had esophagogastric junction (EGJ) outflow disorders on manometry according to Chicago V4.0, 24 spastic motility disorders, 38 hypomotile disorders and 91 normal motility. Compared with non-dysphagic individuals, dysphagic patients had significantly worse scores of both EuroQol-5D and PDS (p-values <0.001).
Among esophageal motility disorders, Eckardt score was significantly different between groups (p-value <0.001) as determined by ANOVA. Specifically, it was significantly higher in EGJ outflow disorders (mean of 5.2), when compared to spastic motility disorders (mean of 3.3) or dysphagic patients with normal motility (mean of 3.2).
With respect to Patient-reported outcomes, PDS score was statistically different between groups (p-value 0.003), being higher in the EGJ outflow disorders (mean T-score of 64.1) when compared to hypomotiity disorders (mean of 60.2) and dysphagic patients with normal motility (mean of 60.3).
Regarding patient-reported quality of life, there were no differences between groups in terms of EuroQol-5D TTO or VAS score (p-value of 0.156 and 0.368, respectively).

Conclusion

Our findings highlights the impact of NOD on the quality-of-life of patients. Although physician and patient reported symptoms’ severity were worse on EGJ outflow disorders, overall quality of life seems equally affected among all esophageal motility profiles.