Assessment of pediatric cricopharyngeal achalasia with high resolution manometry

Cricopharyngeal achalasia (CPA) is an uncommon cause of oropharyngeal dysphagia (OPD) which is the failure of upper esophageal sphincter (UES) to relax during bolus passage. The diagnostic challenges in OPD have been overcome with the use of high resolution manometry (HRM) in children where a cathe...

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Main Authors: Özlem Boybeyi Türer, Numan Demir, Thomas Ciecieraga, Rıza Önder Günaydın, Tutku Soyer
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2019-10-01
Series:The Turkish Journal of Pediatrics
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Online Access:https://turkjpediatr.org/article/view/774
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author Özlem Boybeyi Türer
Numan Demir
Thomas Ciecieraga
Rıza Önder Günaydın
Tutku Soyer
author_facet Özlem Boybeyi Türer
Numan Demir
Thomas Ciecieraga
Rıza Önder Günaydın
Tutku Soyer
author_sort Özlem Boybeyi Türer
collection DOAJ
description Cricopharyngeal achalasia (CPA) is an uncommon cause of oropharyngeal dysphagia (OPD) which is the failure of upper esophageal sphincter (UES) to relax during bolus passage. The diagnostic challenges in OPD have been overcome with the use of high resolution manometry (HRM) in children where a catheter based biomechanical evaluation testing of the oropharyngeal swallowing is performed. Herein, we present a case with severe dysphagia diagnosed as CPA utilizing HRM testing. An 8-year-old boy was seen in our clinic with a two-year history of difficult swallowing, recurrent respiratory tract infections, hypoxia and seizure secondary to aspiration. Esophagography revealed an indentation of the cricopharangeal muscle (CPM) in the cervical part of the esophagus. Videofluroscopic swallow studies (VFSS) revealed cricopharyngeal bar at level of C5-6 and diffuse dysmotility in esophagus. Conventional esophageal manometry revealed absence of peristaltic activity throughout the esophagus. Esophagogastroduodenoscopy revealed narrowing in upper esophagus that with applied force allowed passage of the endoscope. The patient underwent UES dilatation 6 times. He had temporary relief of symptoms. Since he did not have sustained response to dilatation, a botulinium toxin (5IU/each quadrant) injection (BTI) to CPM was performed twice. His symptoms recurred at the end of 3 months. HRM was performed revealing weakness of CPM and uncoordinated contractions and relaxations in UES. We could not reliably differentiate if HRM findings were a sign of primary illness or secondary to BTI. Swallowing rehabilitation was adjusted based on HRM findings. He is still under follow-up with mild dysphagia to certain solids. CPA is an uncommon cause of dysphagia with limited therapeutic options. HRM should be performed in OPD before considering any treatment modality including esophageal dilatation and BTI. HRM has become gold standard diagnostic tool in OPD that provides objective evaluation of pharyngeal and UES motility in children.
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spelling doaj-art-7d49f85f4c4d487e9ae1c044ab40d6262025-08-20T02:01:51ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212019-10-0161510.24953/turkjped.2019.05.025Assessment of pediatric cricopharyngeal achalasia with high resolution manometryÖzlem Boybeyi Türer0Numan Demir1Thomas Ciecieraga2Rıza Önder Günaydın3Tutku Soyer4Departments of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara.Department Physiotherapy and Rehabilitation, Hacettepe University Faculty of Health Sciences, Ankara.New York Presbyterian - Weill Cornell Medical College, Department of Pediatric Gastroenterology, New York, USA.Departments of Otorhinolaryngology, Hacettepe University Faculty of Medicine, Ankara.Departments of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara. Cricopharyngeal achalasia (CPA) is an uncommon cause of oropharyngeal dysphagia (OPD) which is the failure of upper esophageal sphincter (UES) to relax during bolus passage. The diagnostic challenges in OPD have been overcome with the use of high resolution manometry (HRM) in children where a catheter based biomechanical evaluation testing of the oropharyngeal swallowing is performed. Herein, we present a case with severe dysphagia diagnosed as CPA utilizing HRM testing. An 8-year-old boy was seen in our clinic with a two-year history of difficult swallowing, recurrent respiratory tract infections, hypoxia and seizure secondary to aspiration. Esophagography revealed an indentation of the cricopharangeal muscle (CPM) in the cervical part of the esophagus. Videofluroscopic swallow studies (VFSS) revealed cricopharyngeal bar at level of C5-6 and diffuse dysmotility in esophagus. Conventional esophageal manometry revealed absence of peristaltic activity throughout the esophagus. Esophagogastroduodenoscopy revealed narrowing in upper esophagus that with applied force allowed passage of the endoscope. The patient underwent UES dilatation 6 times. He had temporary relief of symptoms. Since he did not have sustained response to dilatation, a botulinium toxin (5IU/each quadrant) injection (BTI) to CPM was performed twice. His symptoms recurred at the end of 3 months. HRM was performed revealing weakness of CPM and uncoordinated contractions and relaxations in UES. We could not reliably differentiate if HRM findings were a sign of primary illness or secondary to BTI. Swallowing rehabilitation was adjusted based on HRM findings. He is still under follow-up with mild dysphagia to certain solids. CPA is an uncommon cause of dysphagia with limited therapeutic options. HRM should be performed in OPD before considering any treatment modality including esophageal dilatation and BTI. HRM has become gold standard diagnostic tool in OPD that provides objective evaluation of pharyngeal and UES motility in children. https://turkjpediatr.org/article/view/774cricopharyngeal achalasiadysphagiahigh resolution manometry
spellingShingle Özlem Boybeyi Türer
Numan Demir
Thomas Ciecieraga
Rıza Önder Günaydın
Tutku Soyer
Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
The Turkish Journal of Pediatrics
cricopharyngeal achalasia
dysphagia
high resolution manometry
title Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
title_full Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
title_fullStr Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
title_full_unstemmed Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
title_short Assessment of pediatric cricopharyngeal achalasia with high resolution manometry
title_sort assessment of pediatric cricopharyngeal achalasia with high resolution manometry
topic cricopharyngeal achalasia
dysphagia
high resolution manometry
url https://turkjpediatr.org/article/view/774
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AT rızaondergunaydın assessmentofpediatriccricopharyngealachalasiawithhighresolutionmanometry
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