Adult idiopathic hypertrophic pyloric stenosis presenting with gastroduodenal intussusception: A rare case report.

Adult idiopathic hypertrophic pyloric stenosis (AIHPS) is a rare entity first described by Cruveilhier in 1835. There are only approximately 200 cases reported in the English literature to date. Histologically, it may be mistaken for spindle cell neoplasms such as gastrointestinal stromal tumour (GIST). Patients with AIHPS usually present with early satiety, abdominal fullness, postprandial vomiting, epigastric pain, and eructations. Adult intussusception is rare and only accounts for 5% of all intussusceptions. Gastroduodenal intussusception is one of the rare types of adult intussusception. This is more likely to occur when a benign or malignant stomach lesion acts as a lead point. We report a case of AIHPS in a 70-year-old lady presenting with gastroduodenal intussusception. An oesophagogastroduodenoscopy (OGDS) was performed, and it revealed a diffusely thickened and narrowed pyloric antrum. A contrasted computed tomography (CECT) of the thorax and abdomen showed a distended stomach with circumferential thickening of the pylorus. The pre-pyloric antrum was intussuscepting into the pylorus, and the apex is seen within the first part of duodenum. She underwent distal gastrectomy with a Roux-en-y reconstruction via laparoscopic approach and was discharged well. AIHPS is a rare condition and should be a differential in adults presenting with gastric outlet obstruction. We believe in cases of AIHPS presenting with gastroduodenal intussusception, a distal gastrectomy with reconstruction is a reasonable approach. A multidisciplinary approach is essential to obtain the best outcome.

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

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.