Congenital diaphragmatic hernia and pyloric atresia: a case report

Introduction Congenital pyloric atresia (CPA) is a rare gastrointestinal malformation that can be associated with other anomalies. Case presentation A term baby boy was born with signs of respiratory distress syndrome that required immediate respiratory support. The patient did not have any abnormal sign on prenatal examination, such as polyhydramnion. A chest X-ray revealed shadowing in the left hemithorax with a mediastinal shift to the right suggesting the presence of congenital diaphragmatic hernia (CDH). A CT confirmed it to be left sided CDH complicated by gastrointestinal perforation. An emergency laparotomy was performed and a Bochdalek hernia was revealed. The stomach was ischemic with multiple perforations. Forty-eight hours after the CDH repair and suturing of the perforations, a re-laparotomy was performed due to worsened pneumoperitoneum. During the second operation, blood supply to the stomach had been restored and a repair of partially dehisced gastric lesions was performed. A gasless abdomen, postoperative excessive amounts of swallowed air and non-bilious gastric aspirate raised suspicion of associated gastric outlet obstruction. After two weeks of resuscitation, the diagnosis of CPA was confirmed on gastrointestinal (GI) contrast study. By the next laparotomy inflammation had mostly resolved, allowing creation of a side-to-side pyloroduodenostomy, connecting the stomach to the first part of the duodenum. The postoperative course was uneventful. On one-year follow-up the child had no gastrointestinal disorders. The elective esophagogastroduodenoscopy (EGD) did not show any scarring. Conclusion CPA must be included in the differential diagnosis of newborns with CDH who show signs of gastric outlet obstruction.