The intraoperative findings were distended, thick walled stomach, no gastric volvulus, mild eventration of the left diaphragm, and pyloric olive (Fig. 3). Pyloromyotomy was performed.
An intraoperative fluoroscopy with installation of Omnipaque contrast (GE Healthcare, USA) through the nasogastric tube showed the passage of the contrast from the stomach to the duodenum and upper jejunum, and the possibility of other associated anomalies such as intestinal malrotation was ruled out.
The eventration of the left diaphragm was left alone for conservative management.
The postoperative period was uneventful. The patient was discharged on third postoperative day. After 6 months of follow-up, patient was doing well with no vomiting and gain of weight.
HPS usually appears at 2–8 weeks of life as nonbilious projectile emesis. The mean age at diagnosis is 3 weeks of age. The emesis is progressive and can lead to gastritis 2.
The initial emesis may appear to be reflex and, with progress of the disease, becoming forceful 1.
In our case, the vomiting was nonprojectile that can be explained by early presentation.
The bilious emesis does not rule out HPS. It reported to be present in 1.4% of the affected infants and hematemesis because of gastritis present in 5% of the infants with HPS 8.
Diagnosis is made typically by history and physical examination augmented by radiographic imaging such as ultrasound or UGI contrast study 1,2.
The previously described ‘classical’ features of HPS such as hypochloremic, hypokalemia, metabolic alkalosis, palpable pyloric mass, and emaciation are now reported less frequently.
The reason for this change is likely due to HPS being diagnosed earlier because of the use of ultrasound 8,9. The association of HPS with chronic gastric volvulus and diaphragmatic eventration has been reported 5,6. In addition, the association of diaphragmatic hernia with gastric volvulus has been reported 10,11. Kotobi et al. found congenital diaphragmatic hernia in 65% of the children with acute gastric volvulus, and 84% of those aged less than 1 month 10.
Generally, gastric distention, regardless of the cause, can cause rotation of the stomach in neonates and infants with lax or immature ligaments, with the possibility of gastric volvulus increased in patients with abnormal large amounts of subdiaphragmatic space 5. Because the main symptoms of chronic gastric volvulus are recurrent projectile vomiting, abdominal distention, and failure to thrive overlap those of HPS, the differential diagnosis can be difficult 5. In our case, the patient presented earlier, at 10 days old with nonprojectile milky vomiting since birth. The coexistence left diaphragmatic eventration with gastric outlet obstruction and abnormal position and configuration of the stomach, which masked the HPS and raised the diagnosis of acute gastric volvulus that required emergency surgical exploration.
Although the association between HPS and other anomaly is rare, it should be kept in mind when facing similar conditions.
We report a 10-day-old boy who presented with nonprojectile milky vomiting since birth. Radiological study showed stomach rotation and lying below the eventrated left diaphragm with gastric outlet obstruction, which raises a range of differential diagnosis at the top gastric volvulus, HPS, intestinal malrotation, and pyloric web. The diagnosis of HPS was established during surgery. We recommend intaoperative UGI in such cases to rule out unexpected associated other anomalies.
Conflicts of interest
There are no conflicts of interest.
1. Curt SK, Mark WGeorge W, Patrick J. Lesions of the stomach. Ashcraft’s pediatric surgery 2010: 5th ed.. USA: Elsevier Inc.; 391–395.
2. Ward E, Easley D, Pohl J. Previously unsuspected infantile hypertrophic pyloric stenosis diagnosed by endoscopy. Dig Dis Sci 2008; 53:946–948.
3. Huang IF, Tiao MM, Chiou CC, Shih HH, Hu HH, Ruiz JP. Infantile hypertrophic pyloric stenosis before 3 weeks of age in infants and preterm babies. Pediatr Int 2011; 53:18–23.
4. Pandey A, Gangopadhyay AN, Upadhyay VD. Malrotation associated with infantile hypertrophic pyloric stenosis in a neonate. J Paediatr Child Health 2009; 45:71–74.
5. Anagnostara A, Koumanidou C, Vakaki M, Manoli E, Kakavakis K. Chronic gastric volvulus and hypertrophic pyloric stenosis in an infant. J Clin Ultrasound 2003; 31:383–386.
6. Oğuzkurt P, Senocak ME, Hiçsönmez A. A rare coexistence of two gastric outlet obstructive lesions: infantile hypertrophic pyloric stenosis and organoaxial gastric volvulus. Turk J Pediatr 2000; 42:87–89.
7. Velaoras K, Bitsori M, Galanakis E, Charissis G. Hypertrophic pyloric stenosis in twins: same genes or same environments? Pediatr Surg Int 2005; 21:669–671.
8. Piroutek MJ, Brown L, Thorp AW. Bilious vomiting does not rule out infantile hypertrophic pyloric stenosis. Clin Pediatr (Phila) 2012; 51:214–218.
9. Glatstein M, Carbell G, Boddu SK, Bernardini A, Scolnik D. The changing clinical presentation of hypertrophic pyloric stenosis: the experience of a large, tertiary care pediatric hospital. Clin Pediatr (Phila) 2011; 50:192–195.
10. Kotobi H, Auber F, Otta E, Meyer N, Audry G, Hélardot PG. Acute mesenteroaxial gastric volvulus and congenital diaphragmatic hernia. Pediatr Surg Int 2005; 21:674–676.
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11. Karabulut R, Türkyilmaz Z, Sönmez K, Karakus SC, Basaklar AC. Delayed presentation of congenital diaphragmatic hernia with intrathoracic gastric volvulus. World J Pediatr 2009; 5:226–228.