A 3.5-year-old unimmunized developmentally normal female child presented with vomiting for 6 months, intermittent fever for 5 months, abdominal distention for 4 months and painful abdomen for 1 month. Vomiting was non bilious, non projectile, 4-5 episodes/day, occurring 1-2 h after meal and contained food particles. Fever was on and off, low-grade, not associated with chills and rigor. Pain was localized to upper abdomen, non colicky, aggravated by food intake, relieved by vomiting or medications. The child had significant weight loss (>5 kg) during the past 4 months and had received one unit packed red cell transfusion at some other center. There was no history of loose stools at any point of time, constipation, jaundice, bleeding from any site or contact with a tuberculosis patient. On examination, she was malnourished with her weight only 6.5 kg and height 84 cm, both below the 3rd percentile of WHO standards. She was pale and dehydrated, with sparse and lusterless hair. Abdomen was soft and distended, with tympanic note on percussion, normal bowel sounds and without organomegaly. The rest of the systemic examination did not show any abnormality.
On investigation, her hemogram and chest X-ray were normal, but serum electrolytes were abnormal (hyponatremia with hypokalemia). HIV serology, PPD test and gastric aspirates (n=3) for tuberculosis were negative. Stool examination was negative for ova, cysts or opportunistic pathogens. X-ray abdomen showed no air-fluid level but only gaseous distention of stomach and intestine. Ultrasound of abdomen showed distended stomach with normal pyloric canal. Barium meal showed grossly distended stomach with slow passage of barium from the antero-pyloric region distally (Fig. 1). Suspecting gastric outlet obstruction, exploratory laparotomy was done that revealed hugely distended stomach with normal smooth muscle thickness but no intra-luminal valve, ring or diaphragm or extra-luminal compression was seen (Fig. 2). Heineke-Mikulicz pyloroplasty was done. Pyloric biopsy showed sub-mucosal edema and mild congestion and no inflammatory cells were seen. The diagnosis of primary acquired gastric outlet obstruction during infancy and childhood (Jodhpur disease) with severe malnutrition was made. The child recovered well postoperatively, became asymptomatic gradually and gained 2.5 kg in one month post surgery.