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

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.

Gastroptosis due to Gastric Outlet Obstruction Secondary to Duodenal Tumor: Glenard’s Disease Revisited

“…The retained gastric content drained from the nasogastric tube may be caused by the accumulation of undigested food and blood that leaked from the pylorus, which created a foul-smelling and dark-colored material. Hassan, et al [ 8 ] reported similar findings from patient with GOO due to adult idiopathic hypertrophic pyloric stenosis. In GOO, a large amount of food debris is retained because only gastric juice promotes digestion in contrast with small-bowel obstruction, in which food is exposed to pancreatic enzymes that play an important role in digesting food debris [ 9 ].…”

Gastric outlet obstruction secondary to adult hypertrophic pyloric stenosis: case report and literature review

“…9,10 Zarineh et al divided AHPS into primary, which has no underlying cause and under which idiopathic AHPS would be classified, and secondary, caused by an underlying disorder such as excessive healing of gastric or duodenal ulcers, malignant tumors, GISTs, postoperative intraabdominal adhesions, bezoars, and increased vagal activity, causing hypertrophy of the pylorus. 2 The secondary type of AHPS usually demonstrates predominantly localized replacement by fibrous tissue, with little or no smooth muscle hypertrophy. 5 Secondary AHPS often does not present any symptoms.…”

“…1 There are various classifications, however the most common type of AHPS is secondary to underlying gastrointestinal pathologies, such as peptic ulcer, malignant tumors, and certain inflammatory diseases. 2 Chronic peptic ulcer resulting in gastric outlet obstruction is the least common complication, occurring in approximately 2 percent of cases. 3 Patients with secondary AHPS often do not present any symptoms.…”

Gastric Outlet Obstruction in the Current Era–A Pictorial Review on Computed Tomography Imaging

“…CT imaging may also help to identify secondary causes such as hernia, inflammatory changes, or malignancy. A more reliable sign [16][17][18] to suggest hypertrophic pyloric stenosis is the "cervix sign," which is seen on endoscopy and refers to a fixed smooth narrowing of the pylorus that persists even after anticholinergic administration (►Fig. 10).…”

“…It has been classified into three types by Danikas et al as follows: Type 1: infantile adult hypertrophic pyloric stenosis (HPS) diagnosed at a late stage wherein history of symptoms in infancy is present, Type 2: most common type which occurs secondary to other underlying gastrointestinal pathologies, such as hiatus hernia, malignancy, and certain inflammatory diseases, and Type 3: primary idiopathic variety. 16 A more widely accepted classification is into primary and secondary types as reported by Zarineh et al 17 When no underlying pathology can be found, it is classified as primary/idiopathic, and in the presence of an underlying disease such as malignancy, postoperative adhesions, fibrosis consequent to healing of ulcers, etc., it is called as secondary HPS. Patients may present with epigastric pain and distension, which is relieved by vomiting.…”