Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy

Santosh Sanagapalli, Sabine Roman, Audrey Hastier, Rupert W. Leong, Kalp Patel, Amanda Raeburn, Matthew Banks, Rehan Haidry, Laurence Lovat, David Graham, Sarmed S. Sami, Rami Sweis

Research output: Contribution to journalArticleResearchpeer-review

Abstract

Background: Achalasia diagnosis requires elevated integrated relaxation pressure (IRP; manometric marker of lower esophageal sphincter [LES] relaxation). Yet, some patients exhibit clinical features of achalasia despite normal IRP and have LES dysfunction demonstrable by other means. We hypothesized these patients to exhibit equivalent therapeutic response compared to standard achalasia patients. Methods: Symptomatic achalasia-like cases, despite normal IRP, displayed evidence of impaired LES relaxation using rapid drink challenge (RDC), solid swallows during high-resolution manometry, and/or barium esophagogram; were treated with achalasia therapies and compared to standard achalasia patients with raised IRP. Outcomes included equivalence for short- and long-term symptom response and stasis on barium esophagogram. Key Results: Twenty-nine normal IRP achalasia cases (14 males, median age 50 year, median Eckardt 6, barium stasis 12 ± 7 cm) and 29 consecutive standard achalasia controls underwent therapy. Among cases, LES dysfunction was most often identified by RDC and/or barium esophagogram. Short-term symptomatic success was equivalent in cases vs controls (90% vs 93%; 95% CI for difference: −19% to 13%). Median short-term (1 vs 1; 95% CI for difference: 0-1) and long-term Eckardt scores (2 vs 1; 95% CI for difference: 0-2) were similar in cases and controls, respectively. Adequate clearance was observed in 67% of cases vs 81% of controls on post-therapy esophagogram. Conclusions and Inferences: We described a subset of achalasia patients with normal IRP, but impaired LES relaxation identifiable only on additional provocative tests. These patients benefited from treatment, suggesting that such tests should be performed to increase the number of clinically relevant diagnoses.

LanguageEnglish
Article numbere13586
Pages1-9
Number of pages9
JournalNeurogastroenterology and Motility
Volume31
Issue number6
DOIs
Publication statusPublished - Jun 2019
Externally publishedYes

Fingerprint

Esophageal Achalasia
Lower Esophageal Sphincter
Pressure
Barium
Therapeutics
Swallows
Manometry

Keywords

  • achalasia
  • high resolution manometry
  • timed barium swallow

Cite this

Sanagapalli, S., Roman, S., Hastier, A., Leong, R. W., Patel, K., Raeburn, A., ... Sweis, R. (2019). Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy. Neurogastroenterology and Motility, 31(6), 1-9. [e13586]. https://doi.org/10.1111/nmo.13586
Sanagapalli, Santosh ; Roman, Sabine ; Hastier, Audrey ; Leong, Rupert W. ; Patel, Kalp ; Raeburn, Amanda ; Banks, Matthew ; Haidry, Rehan ; Lovat, Laurence ; Graham, David ; Sami, Sarmed S. ; Sweis, Rami. / Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy. In: Neurogastroenterology and Motility. 2019 ; Vol. 31, No. 6. pp. 1-9.
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abstract = "Background: Achalasia diagnosis requires elevated integrated relaxation pressure (IRP; manometric marker of lower esophageal sphincter [LES] relaxation). Yet, some patients exhibit clinical features of achalasia despite normal IRP and have LES dysfunction demonstrable by other means. We hypothesized these patients to exhibit equivalent therapeutic response compared to standard achalasia patients. Methods: Symptomatic achalasia-like cases, despite normal IRP, displayed evidence of impaired LES relaxation using rapid drink challenge (RDC), solid swallows during high-resolution manometry, and/or barium esophagogram; were treated with achalasia therapies and compared to standard achalasia patients with raised IRP. Outcomes included equivalence for short- and long-term symptom response and stasis on barium esophagogram. Key Results: Twenty-nine normal IRP achalasia cases (14 males, median age 50 year, median Eckardt 6, barium stasis 12 ± 7 cm) and 29 consecutive standard achalasia controls underwent therapy. Among cases, LES dysfunction was most often identified by RDC and/or barium esophagogram. Short-term symptomatic success was equivalent in cases vs controls (90{\%} vs 93{\%}; 95{\%} CI for difference: −19{\%} to 13{\%}). Median short-term (1 vs 1; 95{\%} CI for difference: 0-1) and long-term Eckardt scores (2 vs 1; 95{\%} CI for difference: 0-2) were similar in cases and controls, respectively. Adequate clearance was observed in 67{\%} of cases vs 81{\%} of controls on post-therapy esophagogram. Conclusions and Inferences: We described a subset of achalasia patients with normal IRP, but impaired LES relaxation identifiable only on additional provocative tests. These patients benefited from treatment, suggesting that such tests should be performed to increase the number of clinically relevant diagnoses.",
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author = "Santosh Sanagapalli and Sabine Roman and Audrey Hastier and Leong, {Rupert W.} and Kalp Patel and Amanda Raeburn and Matthew Banks and Rehan Haidry and Laurence Lovat and David Graham and Sami, {Sarmed S.} and Rami Sweis",
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Sanagapalli, S, Roman, S, Hastier, A, Leong, RW, Patel, K, Raeburn, A, Banks, M, Haidry, R, Lovat, L, Graham, D, Sami, SS & Sweis, R 2019, 'Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy', Neurogastroenterology and Motility, vol. 31, no. 6, e13586, pp. 1-9. https://doi.org/10.1111/nmo.13586

Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy. / Sanagapalli, Santosh; Roman, Sabine; Hastier, Audrey; Leong, Rupert W.; Patel, Kalp; Raeburn, Amanda; Banks, Matthew; Haidry, Rehan; Lovat, Laurence; Graham, David; Sami, Sarmed S.; Sweis, Rami.

In: Neurogastroenterology and Motility, Vol. 31, No. 6, e13586, 06.2019, p. 1-9.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Achalasia diagnosed despite normal integrated relaxation pressure responds favorably to therapy

AU - Sanagapalli, Santosh

AU - Roman, Sabine

AU - Hastier, Audrey

AU - Leong, Rupert W.

AU - Patel, Kalp

AU - Raeburn, Amanda

AU - Banks, Matthew

AU - Haidry, Rehan

AU - Lovat, Laurence

AU - Graham, David

AU - Sami, Sarmed S.

AU - Sweis, Rami

PY - 2019/6

Y1 - 2019/6

N2 - Background: Achalasia diagnosis requires elevated integrated relaxation pressure (IRP; manometric marker of lower esophageal sphincter [LES] relaxation). Yet, some patients exhibit clinical features of achalasia despite normal IRP and have LES dysfunction demonstrable by other means. We hypothesized these patients to exhibit equivalent therapeutic response compared to standard achalasia patients. Methods: Symptomatic achalasia-like cases, despite normal IRP, displayed evidence of impaired LES relaxation using rapid drink challenge (RDC), solid swallows during high-resolution manometry, and/or barium esophagogram; were treated with achalasia therapies and compared to standard achalasia patients with raised IRP. Outcomes included equivalence for short- and long-term symptom response and stasis on barium esophagogram. Key Results: Twenty-nine normal IRP achalasia cases (14 males, median age 50 year, median Eckardt 6, barium stasis 12 ± 7 cm) and 29 consecutive standard achalasia controls underwent therapy. Among cases, LES dysfunction was most often identified by RDC and/or barium esophagogram. Short-term symptomatic success was equivalent in cases vs controls (90% vs 93%; 95% CI for difference: −19% to 13%). Median short-term (1 vs 1; 95% CI for difference: 0-1) and long-term Eckardt scores (2 vs 1; 95% CI for difference: 0-2) were similar in cases and controls, respectively. Adequate clearance was observed in 67% of cases vs 81% of controls on post-therapy esophagogram. Conclusions and Inferences: We described a subset of achalasia patients with normal IRP, but impaired LES relaxation identifiable only on additional provocative tests. These patients benefited from treatment, suggesting that such tests should be performed to increase the number of clinically relevant diagnoses.

AB - Background: Achalasia diagnosis requires elevated integrated relaxation pressure (IRP; manometric marker of lower esophageal sphincter [LES] relaxation). Yet, some patients exhibit clinical features of achalasia despite normal IRP and have LES dysfunction demonstrable by other means. We hypothesized these patients to exhibit equivalent therapeutic response compared to standard achalasia patients. Methods: Symptomatic achalasia-like cases, despite normal IRP, displayed evidence of impaired LES relaxation using rapid drink challenge (RDC), solid swallows during high-resolution manometry, and/or barium esophagogram; were treated with achalasia therapies and compared to standard achalasia patients with raised IRP. Outcomes included equivalence for short- and long-term symptom response and stasis on barium esophagogram. Key Results: Twenty-nine normal IRP achalasia cases (14 males, median age 50 year, median Eckardt 6, barium stasis 12 ± 7 cm) and 29 consecutive standard achalasia controls underwent therapy. Among cases, LES dysfunction was most often identified by RDC and/or barium esophagogram. Short-term symptomatic success was equivalent in cases vs controls (90% vs 93%; 95% CI for difference: −19% to 13%). Median short-term (1 vs 1; 95% CI for difference: 0-1) and long-term Eckardt scores (2 vs 1; 95% CI for difference: 0-2) were similar in cases and controls, respectively. Adequate clearance was observed in 67% of cases vs 81% of controls on post-therapy esophagogram. Conclusions and Inferences: We described a subset of achalasia patients with normal IRP, but impaired LES relaxation identifiable only on additional provocative tests. These patients benefited from treatment, suggesting that such tests should be performed to increase the number of clinically relevant diagnoses.

KW - achalasia

KW - high resolution manometry

KW - timed barium swallow

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U2 - 10.1111/nmo.13586

DO - 10.1111/nmo.13586

M3 - Article

VL - 31

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