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Pyloric stenosis, infantile hypertrophic, 1(IHPS1)

MedGen UID:
357978
Concept ID:
C1867403
Disease or Syndrome
Synonyms: IHPS1; PYLORIC STENOSIS, INFANTILE
 
Monarch Initiative: MONDO:0008355
OMIM®: 179010

Definition

Infantile pyloric stenosis is the most common condition requiring surgical intervention in the first year of life. It typically presents in infants 2 to 6 weeks after birth. Clinically the disorder is characterized by projectile vomiting, visible gastric peristalsis, and a palpable pyloric tumor (summary by Everett et al., 2008). Mortality was high until successful treatment by pyloromyotomy was developed by Ramstedt (1912). Genetic Heterogeneity of Infantile Hypertrophic Pyloric Stenosis Multiple susceptibility loci have been implicated in IHPS including IHPS1 on chromosome 12q, IHPS2 (610260) on chromosome 16p13-p12, IHPS3 (612017) on chromosome 11q14-q22, IHPS4 (300711) on chromosome Xq23, and IHPS5 (612525) on chromosome 16q24. [from OMIM]

Clinical features

From HPO
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Projectile vomiting
MedGen UID:
67442
Concept ID:
C0221151
Sign or Symptom
Vomiting that ejects the gastric contents with great force.
Congenital hypertrophic pyloric stenosis
MedGen UID:
196010
Concept ID:
C0700639
Congenital Abnormality
Pyloric stenosis, also known as infantile hypertrophic pyloric stenosis, is an uncommon condition in infants characterized by abnormal thickening of the pylorus muscles in the stomach leading to gastric outlet obstruction. Clinically infants are well at birth. Then, at 3 to 6 weeks of age, the infants present with projectile vomiting, potentially leading to dehydration and weight loss.
Hypochloremic metabolic alkalosis
MedGen UID:
152850
Concept ID:
C0740895
Disease or Syndrome

Professional guidelines

PubMed

Lascano D, Kelley-Quon LI
Surg Clin North Am 2021 Oct;101(5):799-812. Epub 2021 Jul 30 doi: 10.1016/j.suc.2021.05.021. PMID: 34537144
Iwanaka T, Yamataka A, Uemura S, Okuyama H, Segawa O, Nio M, Yoshizawa J, Yagi M, Ieiri S, Uchida H, Koga H, Sato M, Soh H, Take H, Hirose R, Fukuzawa H, Mizuno M, Watanabe T
Asian J Endosc Surg 2015 Nov;8(4):390-407. doi: 10.1111/ases.12263. PMID: 26708583
Corner B
Arch Dis Child 2003 Jan;88(1):87; author reply 87. doi: 10.1136/adc.88.1.87. PMID: 12495978Free PMC Article

Recent clinical studies

Etiology

Choi G, Je BK, Kim YJ
Korean J Radiol 2022 Jan;23(1):124-138. doi: 10.3348/kjr.2021.0111. PMID: 34983099Free PMC Article
Kandasamy D, Sharma R, Gupta AK
Indian J Pediatr 2019 Sep;86(9):805-816. Epub 2019 Feb 14 doi: 10.1007/s12098-019-02877-9. PMID: 30767163
Hendricks CM, Edgerton CA, Lesher AP
Am Surg 2018 Sep 1;84(9):e354-e356. PMID: 30269705
Hajiran CJ, Hobbs GR, Vona-Davis LC, Nakayama DK
Am Surg 2016 Jan;82(1):E3-5. PMID: 26802838Free PMC Article
Ranells JD, Carver JD, Kirby RS
Adv Pediatr 2011;58(1):195-206. doi: 10.1016/j.yapd.2011.03.005. PMID: 21736982

Diagnosis

Choi G, Je BK, Kim YJ
Korean J Radiol 2022 Jan;23(1):124-138. doi: 10.3348/kjr.2021.0111. PMID: 34983099Free PMC Article
Rich BS, Dolgin SE
Pediatr Rev 2021 Oct;42(10):539-545. doi: 10.1542/pir.2020-003277. PMID: 34599053
Hendricks CM, Edgerton CA, Lesher AP
Am Surg 2018 Sep 1;84(9):e354-e356. PMID: 30269705
Peters B, Oomen MW, Bakx R, Benninga MA
Expert Rev Gastroenterol Hepatol 2014 Jul;8(5):533-41. Epub 2014 Apr 10 doi: 10.1586/17474124.2014.903799. PMID: 24716658
Gasseling J
Radiol Technol 2004 Mar-Apr;75(4):314-6. PMID: 15085639

Therapy

Lascano D, Kelley-Quon LI
Surg Clin North Am 2021 Oct;101(5):799-812. Epub 2021 Jul 30 doi: 10.1016/j.suc.2021.05.021. PMID: 34537144
Liu ZQ, Li QL, Liu JB, Liu HF, Ye H, Fang Y, Zhou PH
Endoscopy 2020 Apr;52(4):E122-E123. Epub 2019 Oct 25 doi: 10.1055/a-1022-4274. PMID: 31652470
El-Gohary Y, Abdelhafeez A, Paton E, Gosain A, Murphy AJ
Pediatr Surg Int 2018 Jan;34(1):21-27. Epub 2017 Oct 13 doi: 10.1007/s00383-017-4196-y. PMID: 29030700
Peters B, Oomen MW, Bakx R, Benninga MA
Expert Rev Gastroenterol Hepatol 2014 Jul;8(5):533-41. Epub 2014 Apr 10 doi: 10.1586/17474124.2014.903799. PMID: 24716658
Albanese A, Bentivoglio AR, Cassetta E, Viggiano A, Maria G, Gui D
Aliment Pharmacol Ther 1995 Dec;9(6):599-604. doi: 10.1111/j.1365-2036.1995.tb00428.x. PMID: 8824646

Prognosis

Grassia R, Gusolfino MD, Conti CB
Gastroenterology 2020 Mar;158(4):e9-e10. Epub 2019 Sep 24 doi: 10.1053/j.gastro.2019.09.020. PMID: 31560889
Hendricks CM, Edgerton CA, Lesher AP
Am Surg 2018 Sep 1;84(9):e354-e356. PMID: 30269705
Yan XQ, Zheng NN, Xing FZ, Yu L, Lu W, Duan XF, Yang J, Bian HQ
Chin Med J (Engl) 2015 Apr 5;128(7):982-4. doi: 10.4103/0366-6999.154327. PMID: 25836622Free PMC Article
Peters B, Oomen MW, Bakx R, Benninga MA
Expert Rev Gastroenterol Hepatol 2014 Jul;8(5):533-41. Epub 2014 Apr 10 doi: 10.1586/17474124.2014.903799. PMID: 24716658
Ranells JD, Carver JD, Kirby RS
Adv Pediatr 2011;58(1):195-206. doi: 10.1016/j.yapd.2011.03.005. PMID: 21736982

Clinical prediction guides

Lovasik BP, Srinivasan JK, Pettitt BJ
Am Surg 2023 Sep;89(9):3716-3720. Epub 2023 May 5 doi: 10.1177/00031348231175108. PMID: 37144475
Gagey AC, de Queiroz Siqueira M, Desgranges FP, Combet S, Naulin C, Chassard D, Bouvet L
Br J Anaesth 2016 May;116(5):649-54. doi: 10.1093/bja/aew070. PMID: 27106968
Sullivan KJ, Chan E, Vincent J, Iqbal M, Wayne C, Nasr A; Canadian Association of Paediatric Surgeons Evidence-Based Resource
Pediatrics 2016 Jan;137(1) Epub 2015 Dec 30 doi: 10.1542/peds.2015-2550. PMID: 26719292
Peters B, Oomen MW, Bakx R, Benninga MA
Expert Rev Gastroenterol Hepatol 2014 Jul;8(5):533-41. Epub 2014 Apr 10 doi: 10.1586/17474124.2014.903799. PMID: 24716658
Abu-Kishk I, Stolero S, Klin B, Lotan G
Surg Laparosc Endosc Percutan Tech 2010 Apr;20(2):e47-9. doi: 10.1097/SLE.0b013e3181d838aa. PMID: 20393318

Recent systematic reviews

Obaid YY, Toubasi AA, Albustanji FH, Al-Qawasmeh AR
J Pediatr Surg 2023 Mar;58(3):458-466. Epub 2022 Aug 24 doi: 10.1016/j.jpedsurg.2022.08.016. PMID: 36137827
Boybeyi-Turer O, Celik HT, Arslan UE, Soyer T, Tanyel FC, Kiran S
Pediatr Surg Int 2022 Jul;38(7):951-961. Epub 2022 Apr 20 doi: 10.1007/s00383-022-05128-9. PMID: 35441884
Lunger F, Staerkle RF, Muff JL, Fink L, Holland-Cunz SG, Vuille-Dit-Bille RN
J Surg Res 2022 Jun;274:1-8. Epub 2022 Feb 2 doi: 10.1016/j.jss.2021.12.042. PMID: 35104694
van den Bunder FAIM, van Wijk L, van Woensel JBM, Stevens MF, van Heurn LWE, Derikx JPM
Paediatr Anaesth 2020 Jul;30(7):749-758. Epub 2020 Jun 18 doi: 10.1111/pan.13879. PMID: 32298502Free PMC Article
Lauriti G, Cascini V, Chiesa PL, Pierro A, Zani A
Eur J Pediatr Surg 2018 Oct;28(5):393-399. Epub 2017 Jul 12 doi: 10.1055/s-0037-1604116. PMID: 28701002

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