Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children

Hypertrophic pyloric stenosis (HPS) is the most important cause of non-bilious vomiting during infancy and it is usually idiopathic. It is very rare in older children and adolescents, in which gastric outlet obstruction (GOO) is more typically secondary to other conditions. Gastrointestinal malforma...

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Main Authors: C. Plessi, M. Sica, F. Molinaro, G. Fusi, F. Rossi, M. Costantini, F. Roviello, L. Marano, A. D'ignazio, C. Spinelli, R. Angotti
Format: Article
Language:English
Published: Elsevier 2021-06-01
Series:Journal of Pediatric Surgery Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213576621000828
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author C. Plessi
M. Sica
F. Molinaro
G. Fusi
F. Rossi
M. Costantini
F. Roviello
L. Marano
A. D'ignazio
C. Spinelli
R. Angotti
author_facet C. Plessi
M. Sica
F. Molinaro
G. Fusi
F. Rossi
M. Costantini
F. Roviello
L. Marano
A. D'ignazio
C. Spinelli
R. Angotti
author_sort C. Plessi
collection DOAJ
description Hypertrophic pyloric stenosis (HPS) is the most important cause of non-bilious vomiting during infancy and it is usually idiopathic. It is very rare in older children and adolescents, in which gastric outlet obstruction (GOO) is more typically secondary to other conditions. Gastrointestinal malformation are frequently associated with Down syndrome and some of them (especially duodenal abnormalities) can be detected quite late. In infants the standard treatment of HPS is represented by a Ramstedt pyloromyotomy, while in adults a distal gastric resection is more indicated and it is usually associated to a Billroth I or II reconstruction. Case report: We present an unusual case of a 12-year-old girl with Down syndrome, diagnosed with late-onset HPS. She came to our attention with periodic non-bilious vomiting and growth delay. She underwent multiple imaging examinations, which highlighted the presence of an HPS. Based on patient's age and the thickness of the pyloric muscle, we decided to perform a partial gastrectomy with a Roux-en-y reconstruction. Patient was discharged on the X day. She is now on follow up, gains weight and presents good conditions. Discussion: HPS is rare but possible during childhood and adolescence. Diagnosis is usually performed through ultrasounds (US) and upper gastrointestinal radiological series (UGI), but sometimes further examinations are required (abdominal MRI, esophagogastroduodenoscopy). In older children since less invasive techniques are not effective, we have to consider partial gastrectomy. Based on our experience, Roux-en-y reconstruction is a valid option, associated with poor complication and good quality of life.
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spelling doaj.art-8728947e60294560afc5cc27b484f0f62022-12-21T22:10:53ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662021-06-0169101860Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older childrenC. Plessi0M. Sica1F. Molinaro2G. Fusi3F. Rossi4M. Costantini5F. Roviello6L. Marano7A. D'ignazio8C. Spinelli9R. Angotti10Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy; Corresponding author.Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, ItalyDivision of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, ItalyDivision of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, ItalyDepartment of Medical Biotechnology, University of Siena, Siena, ItalyDepartment of Medical Biotechnology, University of Siena, Siena, ItalyDepartment of Medicine, Surgery and Neurosciences, Unit of General Surgery and Surgical Oncology, University of Siena, Siena, ItalyDepartment of Medicine, Surgery and Neurosciences, Unit of General Surgery and Surgical Oncology, University of Siena, Siena, ItalyDepartment of Medicine, Surgery and Neurosciences, Unit of General Surgery and Surgical Oncology, University of Siena, Siena, ItalyDivision of Pediatric and Adolescent Surgery, Department of Surgery, University of Pisa, Pisa, ItalyDivision of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, ItalyHypertrophic pyloric stenosis (HPS) is the most important cause of non-bilious vomiting during infancy and it is usually idiopathic. It is very rare in older children and adolescents, in which gastric outlet obstruction (GOO) is more typically secondary to other conditions. Gastrointestinal malformation are frequently associated with Down syndrome and some of them (especially duodenal abnormalities) can be detected quite late. In infants the standard treatment of HPS is represented by a Ramstedt pyloromyotomy, while in adults a distal gastric resection is more indicated and it is usually associated to a Billroth I or II reconstruction. Case report: We present an unusual case of a 12-year-old girl with Down syndrome, diagnosed with late-onset HPS. She came to our attention with periodic non-bilious vomiting and growth delay. She underwent multiple imaging examinations, which highlighted the presence of an HPS. Based on patient's age and the thickness of the pyloric muscle, we decided to perform a partial gastrectomy with a Roux-en-y reconstruction. Patient was discharged on the X day. She is now on follow up, gains weight and presents good conditions. Discussion: HPS is rare but possible during childhood and adolescence. Diagnosis is usually performed through ultrasounds (US) and upper gastrointestinal radiological series (UGI), but sometimes further examinations are required (abdominal MRI, esophagogastroduodenoscopy). In older children since less invasive techniques are not effective, we have to consider partial gastrectomy. Based on our experience, Roux-en-y reconstruction is a valid option, associated with poor complication and good quality of life.http://www.sciencedirect.com/science/article/pii/S2213576621000828Hypertrophic pyloric stenosisRoux-en-y reconstructionDown syndrome
spellingShingle C. Plessi
M. Sica
F. Molinaro
G. Fusi
F. Rossi
M. Costantini
F. Roviello
L. Marano
A. D'ignazio
C. Spinelli
R. Angotti
Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
Journal of Pediatric Surgery Case Reports
Hypertrophic pyloric stenosis
Roux-en-y reconstruction
Down syndrome
title Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
title_full Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
title_fullStr Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
title_full_unstemmed Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
title_short Diagnosis and treatment of primary hypertrophic pyloric stenosis (HPS) in older children
title_sort diagnosis and treatment of primary hypertrophic pyloric stenosis hps in older children
topic Hypertrophic pyloric stenosis
Roux-en-y reconstruction
Down syndrome
url http://www.sciencedirect.com/science/article/pii/S2213576621000828
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