Objective: (1) Is screening of intestinal rotational anatomy obligatory in "asymptomatic" patients with heterotaxy? (2) Does detection of an anomaly warrant surgical correction?
Summary of background data: Heterotaxy is an abnormal arrangement of thoraco-abdominal viscera across a left-to-right axis. Intestinal rotational anomalies are frequent among patients with heterotaxy, but debate exists as to whether they are benign in nature, requiring careful observation alone, or if surgical correction is warranted to prevent obstruction or midgut volvulus.
Methods: A systematic review [according to PRISMA guidelines] was conducted using CINAHL, EMBASE, Medline, and Cochrane Databases. Article quality was assessed using MINORS criteria. Conference proceedings and unpublished data were screened additionally.
Results: Nineteen studies met the eligibility criteria but reporting was adequate for 9. All were observational studies. These included a total of 414 patients managed expectantly, that is, "asymptomatic patients" in whom no intestinal rotation screening was undertaken (group A), 191 cases in whom screening was performed routinely (group B), and 92 patients considered "symptomatic" of potential rotational anomalies and therefore underwent imaging or laparotomy (group C). In group A, 1 patient developed symptoms attributable to malrotation in whom laparotomy confirmed the diagnosis (0.24%). Among groups B and C, 151 had Ladd's operations (53%) and 14 cases of malrotation with obstruction or volvulus were described (4.9%), of which 2 "symptomatic patients" died before laparotomy. Overall surgical complication rate was 17% with 30-day mortality rate of 2.6% to 4.6%.
Conclusion: The evidence base for screening "asymptomatic" patients is weak especially considering the life-limiting comorbidities.