SummaryA case of a very low birthweight premature infant with a clinical presentation of necrotising enterocolitis that was found to have malrotation and midgut volvulus at autopsy is presented. Consultation with the Pediatric Surgery was sought and, on arrival at the bedside, the surgeon placed a Penrose drain in the right lower quadrant of the abdomen. Foul smelling gas escaped through the surgical wound and feculent, bloody material drained from the site. Ventilation improved after the drain was placed and the abdomen decompressed, but the infant remained gravely ill. BMJ Case Reports OUTCOME AND FOLLOW-UPDespite all efforts, the infant's acidaemia and coagulopathy continued. Florid pulmonary haemorrhage ensued and she became bradycardic. After counselling the family, life support was withdrawn. The time from initial grossly bloody stool to death was 12 h. A premortem blood culture and a peritoneal swab at the time of abdominal decompression and Penrose drain placement grew Enterobacter sakazakii within 24 h.An autopsy examination using the standard Rokitansky evisceration method was performed. There was incomplete intestinal rotation with Ladd's bands at the third portion of the duodenum and caecum in midabdomen ( fi gure 2 ). Histologically, there were massively dilated lymphatics, veins and arteries within the small bowel submucosa consistent with gross fi ndings of incomplete rotation and midgut volvulus. Of a total of 161 cm of bowel, approximately 75% demonstrated necrosis with the exception of the proximal duodenum and distal rectum.Histologically, there was extensive transmural ischaemic necrosis but no evidence of perforation. Additional fi ndings included bilateral haemorrhagic adrenal glands (Waterhouse-Friderichsen syndrome; fi gure 3 ). Postmortem blood cultures were positive for E sakazakii . DISCUSSIONThe classic description of intestinal malrotation is that of the term infant who presents with bilious emesis and then has the diagnosis confi rmed with an upper gastrointestinal contrast study (UGI) that demonstrates the right-sided position of the duodenal-jejunal junction or evidence of a midgut volvulus. 4 There exists a number of case reports of this phenomenon in term infants, but the fi nding has rarely been described in the very low birthweight (VLBW) preterm infant. 5 6 Intestinal malrotation occurs in approximately 1% of the general population. During fetal development, midgut rotation is a multistep process requiring herniation of the intestinal contents, rotation 270° around the superior mesenteric artery and return to the abdominal cavity. Nonrotation, malrotation, reverse rotation and incomplete rotation are variations of this phenomenon. With malrotation, the gut has 'fl oppy' attachments and can lose its blood supply through torsion or restrictive Ladd's bands. Midgut volvulus and ischaemia can occur at any age with no apparent warning or trigger, but often presents in the pediatric population. The typical early symptoms are irritability, poor feeding and bilious emesis, which ar...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.