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Abstract Background Congenital diaphragmatic hernia (CDH) is sometimes associated with complications involving herniation of intrathoracic organs, which further increase mortality rate. We encountered a case of postoperative gastric and splenic volvulus shortly after left CDH repair in a female neonate who was treated with gastropexy. Case presentation At 39 weeks gestation, a female patient with left Bochdalek CDH was delivered (birth weight: 3748 g, Apgar score: 3/4). The patient was provided ventilator support with nitric oxide. After pulmonary hypertension improved, CDH repair was performed via the abdominal approach on day 7. The stomach, small intestine, large intestine, and spleen were herniated through a diaphragmatic defect of 4 × 2 cm. Although the diaphragm was directly closed, it was tight and the reconstructed diaphragm “dome” was shallow, restricting space for the spleen and stomach. Nonetheless, the spleen was positioned in the left upper abdomen and the stomach was positioned medially. The postoperative course was complicated by organo-axial gastric volvulus, and laparotomy was performed on day 14. In addition to the gastric volvulus, we confirmed a wandering splenic volvulus. The spleen was easily detorted and returned to the left upper abdomen. However, the patient experienced relapse of gastric volvulus without splenic volvulus. Gastropexy was performed electively on day 47. Postoperatively, the patient could be fed orally, and the patient’s development was satisfactory 6 years after surgery. Conclusions The cause of these rare complications appeared to be tight direct diaphragmatic closure, which reduced space for the spleen and stomach beneath the left diaphragm.

Congenital diaphragmatic hernia (CDH) is a common cause of neonatal mortality. If CDH is associated with non-immune fetal hydrops (FH), the mortality rate is even higher. However, the pathogenesis and management of FH associated with CDH are still under discussion. We present here a case of left-sided CDH accompanied by prolonged FH, where unexpected ileal perforation developed after CDH repair.The female patient was diagnosed with left-sided CDH with right-sided pleural effusion (PE) at 26 weeks' gestation. At 35 weeks' gestation, FH progressed with bilateral PE and the patient was delivered by cesarean section with a body weight of 2068 g and Apgar 3/7. Subcutaneous edema progressed, but PE attenuated with improvement of pulmonary hypertension, and CDH repair was performed on Day 4. Postoperatively, the patient's pulmonary hypertension worsened, and intestinal perforation was observed on Day 11. In the emergency laparotomy, there was a single small bowel perforation of 5 mm in diameter in the terminal ileum. A 5 cm of the ileum was resected and the bowel was anastomosed in a single stage. The patient was weaned from the ventilator on Day 28. Although bilateral periventricular leukomalacia was confirmed, the patient was discharged without significant neurological symptoms on Day 76.