Diagnosis of congenital esophageal stenosis in adults and treatment with peroral endoscopic myotomy.
Congenital esophageal stenosis
diagnosis
peroral endoscopic myotomy
Journal
Annals of gastroenterology
ISSN: 1108-7471
Titre abrégé: Ann Gastroenterol
Pays: Greece
ID NLM: 101121847
Informations de publication
Date de publication:
Historique:
received:
19
08
2020
accepted:
28
12
2020
entrez:
19
7
2021
pubmed:
20
7
2021
medline:
20
7
2021
Statut:
ppublish
Résumé
Congenital esophageal stenosis (CES) in adults is a rare disorder that can present as achalasia, particularly in the distal esophagus. We describe the salient features of CES in adults and identify the feasibility and short-term outcomes of peroral endoscopic myotomy (POEM) for CES. In this retrospective, single-center case series, we included 6 patients with a "misdiagnosis" of achalasia established elsewhere, ultimately diagnosed with CES and referred to our institution for POEM. Symptom improvement (clinical success rate), defined as an Eckardt Symptom Score (ESS) of <3 at 2-month follow up was assessed. Six patients (median age: 40 [range: 18-58] years; 4 males) were included. A long-standing history of dysphagia, ring-shaped stenosis on endoscopic examination, "lopsided hourglass" sign on barium esophagogram, and high-resolution manometry findings indicated by a compartmentalized intrabolus pressure pattern with distinction between the stenotic area and the lower esophageal sphincter were the salient features identified. POEM could not be completed in the first 2 cases due to technical challenges. All subsequent 4 patients who underwent successful POEM, exhibited improved ESS of ≤3 (clinical success rate 100%) at 2 months post-POEM. Along with identification of salient features on several diagnostic modalities, a differential diagnosis of CES in adults must be considered in patients presenting with long-standing history of dysphagia arising from childhood and persisting into adulthood. Although favorable short-term outcomes of POEM were achieved, further evaluation is still warranted, and an inexperienced operator should not attempt POEM on CES patients due to its technical difficulties.
Sections du résumé
BACKGROUND
BACKGROUND
Congenital esophageal stenosis (CES) in adults is a rare disorder that can present as achalasia, particularly in the distal esophagus. We describe the salient features of CES in adults and identify the feasibility and short-term outcomes of peroral endoscopic myotomy (POEM) for CES.
METHODS
METHODS
In this retrospective, single-center case series, we included 6 patients with a "misdiagnosis" of achalasia established elsewhere, ultimately diagnosed with CES and referred to our institution for POEM. Symptom improvement (clinical success rate), defined as an Eckardt Symptom Score (ESS) of <3 at 2-month follow up was assessed.
RESULTS
RESULTS
Six patients (median age: 40 [range: 18-58] years; 4 males) were included. A long-standing history of dysphagia, ring-shaped stenosis on endoscopic examination, "lopsided hourglass" sign on barium esophagogram, and high-resolution manometry findings indicated by a compartmentalized intrabolus pressure pattern with distinction between the stenotic area and the lower esophageal sphincter were the salient features identified. POEM could not be completed in the first 2 cases due to technical challenges. All subsequent 4 patients who underwent successful POEM, exhibited improved ESS of ≤3 (clinical success rate 100%) at 2 months post-POEM.
CONCLUSIONS
CONCLUSIONS
Along with identification of salient features on several diagnostic modalities, a differential diagnosis of CES in adults must be considered in patients presenting with long-standing history of dysphagia arising from childhood and persisting into adulthood. Although favorable short-term outcomes of POEM were achieved, further evaluation is still warranted, and an inexperienced operator should not attempt POEM on CES patients due to its technical difficulties.
Identifiants
pubmed: 34276187
doi: 10.20524/aog.2021.0618
pii: AnnGastroenterol-34-493
pmc: PMC8276369
doi:
Types de publication
Journal Article
Langues
eng
Pagination
493-500Informations de copyright
Copyright: © Hellenic Society of Gastroenterology.
Déclaration de conflit d'intérêts
Conflict of Interest: Haruhiro Inoue is an advisor of Olympus Corporation and Top Corporation. He has also received educational grants from Olympus Corp., and Takeda Pharmaceutical Co. Anastasios Manolakis has received grant from the Hellenic Society of Gastroenterology and research fund from Janssen Pharma
Références
J Pediatr Surg. 2002 Feb;37(2):197-201
pubmed: 11819198
Tohoku J Exp Med. 2019;247(1):41-49
pubmed: 30662021
Pediatr Surg Int. 2017 Oct;33(10):1035-1040
pubmed: 28801834
Dig Endosc. 2016 Sep;28(6):645-9
pubmed: 26969481
J Indian Assoc Pediatr Surg. 2016 Jul-Sep;21(3):150-2
pubmed: 27365912
J Indian Assoc Pediatr Surg. 2016 Jul-Sep;21(3):106-9
pubmed: 27365901
Dig Dis. 1999;17(3):172-7
pubmed: 10697666
J Gastroenterol. 2010 Apr;45(4):422-5
pubmed: 20013295
Am J Gastroenterol. 2000 Jan;95(1):32-6
pubmed: 10638555
Br J Hosp Med (Lond). 2010 Mar;71(3):170-1
pubmed: 20220726
J Gastroenterol. 2017 Apr;52(4):401-406
pubmed: 28188367
J Dig Dis. 2015 Aug;16(8):479-82
pubmed: 25989115
Dig Endosc. 2018 Sep;30(5):563-579
pubmed: 30022514
AJR Am J Roentgenol. 1998 Jan;170(1):147-8
pubmed: 9423621
J Pediatr Surg. 2003 Dec;38(12):1716-9
pubmed: 14666450
Clin Radiol. 1985 May;36(3):263-6
pubmed: 4064508
World J Gastrointest Endosc. 2016 Feb 25;8(4):212-9
pubmed: 26962403
J Pediatr Surg. 2020 Nov;55(11):2342-2347
pubmed: 32057439
AJR Am J Roentgenol. 2001 May;176(5):1179-82
pubmed: 11312178
Dig Dis. 2000;18(3):186
pubmed: 11279339
J Chir (Paris). 1992 Jan;129(1):16-9
pubmed: 1560053
World J Gastrointest Endosc. 2015 Mar 16;7(3):183-91
pubmed: 25789088