2025 Volume 61 Issue 2 Pages 404-408
Hirschsprung’s disease（HD）is characterized by functional intestinal obstruction due to the absence of ganglion cells in the intestine. The prevalence of preterm infants with HD has increased in recent years. While Hirschsprung-associated enterocolitis, bowel perforation and ileus are well-documented complication, small intestinal volvulus without malrotation has not been previously reported. We report a case of an extremely low birth weight infant with HD, complicated by intestinal volvulus without malrotation. The patient was a girl born at 25 weeks of gestation with a birth weight of 711 g and had a family history of HD. HD was suspected due to abdominal distension. She tolerated enteral feeding with frequent enemas and bougie insertion. At 70 days of age, abdominal distension worsened following sedation and ventilatory management for photocoagulation for retinopathy of prematurity. Although bowel decompression was initiated, abdominal distention did not fully resolve. At 83 days of age, the distention further exacerbated, accompanied by respiratory deterioration and elevated inflammatory markers. Given the suspicion of HD-associated ileus or enteritis, bowel decompression and antibiotic therapy were administered. However, her condition did not improve, and laparotomy was performed. Intestinal volvulus without malrotation was identified, and a portion of the ileum subsequently progressed to gangrene, necessitating resection. Biopsy of the sigmoid colon during laparotomy and rectal biopsy at 145 days of age confirmed the diagnosis of HD. This case suggests that preterm infants with HD may develop small intestinal volvulus, a complication that should not delay the decision for laparotomy.
