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Congenital hypertrophic pyloric stenosis(IHPS)

MedGen UID:
196010
•Concept ID:
C0700639
•
Congenital Abnormality
Synonyms: Inherited hypertrophic pyloric stenosis; PYLORIC STENOSIS, INFANTILE HYPERTROPHIC
SNOMED CT: Congenital hypertrophic pyloric stenosis (48644003)
 
Monarch Initiative: MONDO:0100239
OMIM®: 179010
OMIM® Phenotypic series: PS179010

Definition

An abnormality characterized by thickening of the muscle in the wall of the pylorus. It results in the narrowing of the pyloric channel. The overlying mucosa may appear hypertrophic as well. Clinical signs and symptoms appear early in life and include projectile vomiting and dehydration. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital hypertrophic pyloric stenosis

Professional guidelines

PubMed

The diagnosis and management of congenital hypertrophic pyloric stenosis.
RHEINLANDER HF, SWENSON O
J Pediatr 1952 Sep;41(3):314-9. doi: 10.1016/s0022-3476(52)80008-3. PMID: 12991156

Recent clinical studies

Etiology

Single-Site Umbilical Laparoscopic Pyloromyotomy Using a Pyloric Electrocoagulation Chisel Combined with a Left-Handed Main Operation for Congenital Hypertrophic Pyloric Stenosis.
Yu LJ, Su BL, Shan WY, Luo CF, Shu Q
J Laparoendosc Adv Surg Tech A 2020 Nov;30(11):1248-1252. Epub 2020 Aug 20 doi: 10.1089/lap.2020.0478. PMID: 32833592
The Safety and Effectiveness of Laparoscopic versus Open Surgery for Congenital Hypertrophic Pyloric Stenosis in Infants.
Huang WH, Zhang QL, Chen L, Cui X, Wang YJ, Zhou CM
Med Sci Monit 2020 May 14;26:e921555. doi: 10.12659/MSM.921555. PMID: 32407297Free PMC Article
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis.
Ma S, Liu J, Zhang Y, Yang Y, Jin H, Ma X, Wei H
Sci Rep 2017 Aug 25;7(1):9527. doi: 10.1038/s41598-017-10264-7. PMID: 28842652Free PMC Article
Congenital hypertrophic pyloric stenosis and associated anomalies in the genitourinary tract.
Atwell JD, Levick P
J Pediatr Surg 1981 Dec;16(6):1029-35. doi: 10.1016/s0022-3468(81)80870-6. PMID: 6279815
Congenital hypertrophic pyloric stenosis.
Owens MP, Beljan JR
Calif Med 1973 May;118(5):33-7. PMID: 4700031Free PMC Article

Diagnosis

Gastric Peroral Endoscopic Pyloromyotomy for Infants With Congenital Hypertrophic Pyloric Stenosis.
Zhang H, Liu Z, Ma L, Li Q, Huang Y, Dong K, Ye H, Liu J, Liu H, Ren X, Yang H, Hou C, Ge K, Wang H, Zhou P, Fang Y
Am J Gastroenterol 2023 Mar 1;118(3):465-474. Epub 2022 Aug 23 doi: 10.14309/ajg.0000000000001973. PMID: 36002919
Laparoscopic pyloromyotomy for congenital hypertrophic pyloric stenosis: Our experience with twenty cases.
Ramji J, Joshi RS
Afr J Paediatr Surg 2021 Jan-Mar;18(1):14-17. doi: 10.4103/ajps.AJPS_119_20. PMID: 33595535Free PMC Article
Congenital hypertrophic pyloric stenosis.
Singh UK, Kumar R
Indian J Pediatr 2002 Aug;69(8):713-5. doi: 10.1007/BF02722710. PMID: 12356225
Congenital hypertrophic pyloric stenosis. Surgical experience.
Gibbs MK, Van Herrden JA, Lynn HB
Mayo Clin Proc 1975 Jun;50(6):312-6. PMID: 1127996
Congenital hypertrophic pyloric stenosis.
Owens MP, Beljan JR
Calif Med 1973 May;118(5):33-7. PMID: 4700031Free PMC Article

Therapy

Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis.
Mata-Gómez J, Guerrero-Domínguez R, García-Santigosa M, Ontanilla A
Braz J Anesthesiol 2015 Jul-Aug;65(4):302-5. Epub 2015 Apr 28 doi: 10.1016/j.bjane.2014.03.012. PMID: 26123148
Congenital hypertrophic pyloric stenosis.
Singh UK, Kumar R
Indian J Pediatr 2002 Aug;69(8):713-5. doi: 10.1007/BF02722710. PMID: 12356225
Folic acid for the prevention of congenital anomalies.
Hall J, Solehdin F
Eur J Pediatr 1998 Jun;157(6):445-50. doi: 10.1007/s004310050850. PMID: 9667396
Periconceptional folic acid containing multivitamin supplementation.
Czeizel AE
Eur J Obstet Gynecol Reprod Biol 1998 Jun;78(2):151-61. doi: 10.1016/s0301-2115(98)00061-x. PMID: 9622312
Postoperative hypoglycemia in congenital hypertrophic pyloric stenosis.
Shumake LB
South Med J 1975 Feb;68(2):223-4. doi: 10.1097/00007611-197502000-00024. PMID: 1118756

Prognosis

Pyloric stenosis secondary to peptic ulcer disease in pediatric patients: A case report and review of the literature.
Zhou J, Liu G, Song X, Liu H, Wang D, Kang Q
Medicine (Baltimore) 2023 Mar 24;102(12):e33404. doi: 10.1097/MD.0000000000033404. PMID: 36961135Free PMC Article
Endoscopic pyloromyotomy for congenital pyloric stenosis.
Ibarguen-Secchia E
Gastrointest Endosc 2005 Apr;61(4):598-600. doi: 10.1016/s0016-5107(05)00075-1. PMID: 15812419
Extramucosal pyloromyotomy by laparoscopy.
Alain JL, Grousseau D, Longis B, Ugazzi M, Terrier G
Eur J Pediatr Surg 1996 Feb;6(1):10-2. doi: 10.1055/s-2008-1066457. PMID: 8721169
Congenital hypertrophic pyloric stenosis--a 20-year review of 282 surgically treated infants.
Eckstein HB, Agrawal M
Z Kinderchir 1982 Jun;36(2):50-2. doi: 10.1055/s-2008-1059860. PMID: 7136296
Congenital hypertrophic pyloric stenosis: a long-term follow-up of 41 cases.
Solowiejczyk M, Holtzman M, Michowitz M
Am Surg 1980 Oct;46(10):567-71. PMID: 7425431

Clinical prediction guides

The Safety and Effectiveness of Laparoscopic versus Open Surgery for Congenital Hypertrophic Pyloric Stenosis in Infants.
Huang WH, Zhang QL, Chen L, Cui X, Wang YJ, Zhou CM
Med Sci Monit 2020 May 14;26:e921555. doi: 10.12659/MSM.921555. PMID: 32407297Free PMC Article
Gastric Peroral Endoscopic Myotomy for Treatment of Congenital Pyloric Stenosis-First Clinical Experience.
Kozlov Y, Kovalkov K, Smirnov A
J Laparoendosc Adv Surg Tech A 2019 Jun;29(6):860-864. Epub 2019 Apr 23 doi: 10.1089/lap.2018.0803. PMID: 31017541
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis.
Ma S, Liu J, Zhang Y, Yang Y, Jin H, Ma X, Wei H
Sci Rep 2017 Aug 25;7(1):9527. doi: 10.1038/s41598-017-10264-7. PMID: 28842652Free PMC Article
Etiopathogenic analysis of the caries on three patients with Noonan Syndrome.
Barberia Leache E, Saavedra Ontiveros D, Maroto Edo M
Med Oral 2003 Mar-Apr;8(2):136-42. PMID: 12618674
Does dehydration affect thickness of the pyloric muscle? An experimental study.
Starinsky R, Klin B, Siman-Tov Y, Barr J
Ultrasound Med Biol 2002 Apr;28(4):421-3. doi: 10.1016/s0301-5629(01)00524-5. PMID: 12049953

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