Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Apr 17;8(2):64-67.
doi: 10.1080/20009666.2018.1444905. eCollection 2018.

Adult idiopathic hypertrophic pyloric stenosis - a common presentation with an uncommon diagnosis

Syed Moin Hassan  1 , Ateeq Mubarik  2 , Salman Muddassir  2 , Furqan Haq  3
Affiliations
Case Reports

Adult idiopathic hypertrophic pyloric stenosis - a common presentation with an uncommon diagnosis

Syed Moin Hassan et al. J Community Hosp Intern Med Perspect. .

Abstract

Background and Objectives: Adult Idiopathic hypertrophic pyloric stenosis (AIHPS) is a rare but well-defined entity in adults with only 200-300 cases reported so far in the literature.We describe a case of AIHPS and the relevant literature review. Methods and Results: The patient presented with acute onset upper abdominal pain associated with nausea, vomiting, foul-smelling black tarry stools, and anorexia. On the Esophagogastroduodenoscopy (EGD), pylorus demonstrated a unique "cervix sign." The patient had multiple endoscopic dilations with minimal relief. She then underwent a distal partial gastrectomy with a Billroth 1 gastroduodenostomy with considerable improvement in her symptoms on follow up. Conclusion: Adult Idiopathic hypertrophic pyloric stenosis (AIHPS) is a rare disease which is also underreported due to a difficulty in diagnosis. The most common symptoms of AIHPS are postprandial nausea, vomiting, early satiety, and epigastric pain as seen in our patient. Endoscopy usually shows ?Cervix sign? a unique sign showing a fixed, markedly narrowed pylorus with a smooth border. Multiple treatments have been proposed for AIHPS, including endoscopic dilation, pyloromyotomy with or without pyloroplasty, gastrectomy with a Billroth 1 gastroduodenostomy. Currently, there is no evidence of one surgical technique being superior to another. Further research needs to be done on AIHPS before one technique can be standardized as the standard of care.

Keywords: Adult idiopathic hypertrophic pyloric stenosis (AIHPS); cervix sign; gastric outlet obstruction; pyloric stenosis.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
CT abdomen pelvis showing thickening of the distal gastric antrum (yellow arrow). Full color available online.
Figure 2.
Figure 2.
Endoscopy showing (a) 300 ml of residual oatmeal (b) & (c) Pylorus demonstrating ‘cervix sign’ (d) Serial Balloon Dilator being inserted into the small pylorus opening.
Figure 3.
Figure 3.
Endoscopy showing (a) Serial Balloon Dilator serially expanding the stenotic pyloric opening from 12 mm to 15 mm with bloody response; (b) on withdrawal after dilation a split defect of at least 5 mm is seen.
Figure 4.
Figure 4.
Cross section of the pylorus showing (a) increased thickness of the pylorus muscle, (b) focally hyperplastic gastric muscularis propria (c) 10× magnification and hyperplastic muscularis layer reaching up to the lamina propria.
See this image and copyright information in PMC

References

    1. Taliaferro L, Barnett DA, Mann NS.. Adult hypertrophic pyloric stenosis: case report. Tex Med. 1986;82:27–28. - PubMed
    1. Thielemann H, Anders S, Naveke R, Diermann J. Primary hypertrophic pyloric stenosis. An uncommon condition of pyloric stenosis in adults. Zentralbl Chir. 1999;124(10):947–949. - PubMed
    1. Simson JN, Thomas AJ, Stoker TA. Adult hypertrophic pyloric stenosis and gastric carcinoma. Br J Surg. 1986;73:379–380. - PubMed
    1. Christiansen KH, Grantham A. Idiopathic hypertrophic pyloric stenosis in the adult: a review of the literature and the report of two cases. Arch Surg. 1962;85:207–214. - PubMed
    1. Cruveilhier J. Anatomie pathologique du corps humain, ou descriptions, avec figures lithographiées et coloriées, des diverses altérations morbides dont le corps humain est susceptible. Chez J B Baillière. 1835.

Publication types