Late-onset volvulus without malrotation in extremely preterm infants--a case-control-study.

BACKGROUND: Volvulus without malrotation in preterm infants is a rare but potentially life-threatening event of unknown aetiology. Confusion with necrotising enterocolitis may delay surgical intervention thereby aggravating morbidity and mortality. We aimed at elucidating potential risk factors for, and characteristic clinical signs of, volvulus without malrotation in preterm infants. METHODS: Retrospective, single-centre case-control study (2007-2011). For every index patient, five infants of similar gestational age, birth weight and birth year were evaluated. Additionally, all 9 cases of necrotising enterocolitis occurring during the above period were evaluated. Data are presented as median (interquartile range). RESULTS: Five extremely premature infants suffering from volvulus without malrotation were identified (gestational age at birth 24.4 (23.6-25.5) weeks, birth weight 480 (370-530) g). All were small for gestational age and female; three out of five died. Volvulus occurred several weeks after birth, whereas necrotising enterocolitis occurred significantly earlier. Beyond that, no striking differences in clinical or laboratory presentation of volvulus without malrotation and necrotising enterocolitis were found. Infants with volvulus had significantly more frequent manipulations with rectal tubes for flatulence, but there were no differences in the frequency of enemas, abdominal massage or defecation. In infants with volvulus, nasal high-frequency oscillation was used more frequently for respiratory support, and PEEP-level tended to be higher. CONCLUSIONS: In extremely premature infants volvulus without malrotation represents a life-threatening event that occurs typically several weeks after birth with an acute abdomen and seems to affect predominantly girls. Infants requiring intensive non-invasive respiratory support might be at highest risk.

Epidemiological study on intestinal volvulus without malrotation in VLBW infants.

BACKGROUND: We conducted a monthly epidemiological survey in Germany to detect the prevalence of volvulus without malrotation (VWM) in very low birthweight (VLBW) infants and to identify factors for a better distinction between this rare and life-threatening event and other acute abdominal diseases in preterm infants. METHODS: Throughout 2014 and 2015, every paediatric department in Germany was asked to report cases of VWM in infants with birth weights <1500 g to the Surveillance Unit for Rare Paediatric Conditions in Germany. Hospitals reporting a case were asked to return an anonymised questionnaire and discharge letter. RESULTS: Of 36 cases reported, detailed information was submitted on 29, with 26 meeting entry criteria. With 9896 and 10 140 VLBW infants born in Germany in 2014 and 2015, respectively, we estimated a prevalence of 1.52/1000 VLBW infants for 2014 and 1.08/1000 in 2015. No specific early symptom could be determined. 10% died, and 21% of infants developed short bowel syndrome. Calculated from all verified VWM arise death in 8% and a short bowel syndrome in 15%. CONCLUSION: VWM is a rare source of acute abdomen in VLBW infants. No specific signs and symptoms potentially facilitating an early recognition of VWM could be found from this survey. Because the rates of death and short bowel syndrome are high, VWM should always be considered early in an acute abdomen in a VLBW infant.