Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary? : A Systematic Review in Search of the Evidence

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 Conference proceedings and unpublished data were screened additionally.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

Variations in the detection of anorectal anomalies at birth amongst European cities

Aim: The diagnosis of anorectal malformations (ARM) is made at birth by perineal examination of the newborn, yet small series reported late diagnosis in almost 13%. No large series to date have looked into the magnitude of missed ARM cases in the neonatal period across Europe. This study aimed to define the rate of missed ARM at birth across four UK and EU centers. Methods: All ARM cases treated at two UK tertiary centers in the past 15 years were compared to two tertiary European centers. Demographic and relevant clinical data were collected. Late diagnosis was defined as any diagnosis made after discharge from the birth unit. Factors associated with late diagnosis were explored with descriptive statistics. Results: Across the four centers (117/1350, 8.7%) were sent home from the birth unit without recognizing the anorectal anomaly. Missed cases showed a slight female predominance (1.3:1), and the majority (113/117, 96.5%) were of the low anomaly with a fistula to the perineum. The rate of missed ARM cases was significantly higher in the UK centers combined (74/415, 17.8%) compared to those in the EU (43/935, 4.6%), (p<0.00001), and this was independent of individual center and year of birth. Conclusion: Significant variation exist between the UK and other European countries in the detection of ARM at birth. We recommend raising the awareness of accurate perineal examination at time of newborn physical examination. We feel this highlights an urgent need for a national initiative to assess and address the timely diagnosis of ARM in the UK

Efficacy of antireflux surgery in children with or without neurological impairment: a systematic review

Abstract Background Antireflux surgery is commonly performed in children, yet evidence for its efficacy is limited. The aim of this review was to determine the effect of antireflux surgery with regard to objective measures of quality of life (QoL) and value of upper gastrointestinal investigations in neurologically normal (NN) and neurologically impaired (NI) children. Methods A systematic review was conducted of articles reporting children undergoing antireflux surgery in whom preoperative and postoperative objective testing was performed. Primarily, Embase, CINAHL, MEDLINE and PubMed were searched from inception to April 2019. Methodological Index for Non-randomized Studies (MINORS) criteria were used to assess article quality. Results Of 789 articles, 14 met the eligibility criteria, 12 prospective observational and 2 retrospective studies. The median MINORS score was 59·4 (i.q.r. 39 to 62·5) per cent. Seven studies reported assessment of validated QoL measures before and after antireflux surgery in 148 children. Follow-up ranged from 1 to 180 months. All studies confirmed significant improvements in QoL measures among NN and NI children at all follow-up points. Eleven studies reported on preoperative and postoperative investigations in between 416 and 440 children children. Follow-up ranged from 0·5 to 180 months. Nine studies confirmed improvements in gastro-oesophageal reflux using 24-h oesophageal pH monitoring with or without manometry, but conflicting results were identified for four studies reporting gastric emptying. No studies reported fluoroscopy or endoscopy adequately. Conclusion Based on the results of studies of low-to-moderate quality, antireflux surgery improved QoL and reduced oesophageal acid exposure in NN and NI children in the short and medium term. Although antireflux surgery is a common elective operation, the lack of rigorous preoperative and postoperative evaluation(s) in the majority of patient-reported studies is striking. </jats:sec