Intestinal malrotation in an extremely preterm very low birthweight infant

Abstract

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 After counselling the family, life support was withdrawn.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...

Intestinal malrotation in extremely premature infants: a potential trap

“…Intestinal pneumatosis, a common radiological finding in NEC, was seen in three of our patients and has been reported previously in malrotation and volvulus with ischemic bowel [14,17]. Necrotizing enterocolitis without intestinal perforation is frequently managed medically but the possibility of midgut volvulus must be considered in the differential diagnosis of the extremely premature infant with abdominal distension and intestinal pneumatosis.…”

“…No previous study has systematically investigated symptomatic intestinal malrotation in extremely premature infants. Costner et al [14] reported a 26-week gestation female infant who developed a distended tender abdomen with blood stained stools at 23 days of age and died 12 h later from midgut volvulus secondary to intestinal malrotation. She had no antecedent vomiting, feed intolerance or bilious gastric aspirates.…”

Volvulus in term and preterm infants ‐ clinical presentation and outcome

“…However, in our view the most likely explanation for the high rate of partial bowel resection was that a timely diagnosis that prevents intestinal necrosis is very challenging in preterm infants. Intestinal pneumatosis that is considered highly suggestive for NEC has also been described in infants with volvulus (12,19). 2).…”

“…Therefore, it is difficult to distinguish volvulus from necrotising enterocolitis (NEC) or paralytic ileus during sepsis. Intestinal pneumatosis that is considered highly suggestive for NEC has also been described in infants with volvulus (12,19). Both entities, NEC and paralytic ileus during sepsis, are more common in extremely preterm infants than volvulus and an immediate surgical intervention after the onset of symptoms would be detrimental in both cases.…”