Lindsay M. Ryerson, MD,​a Scott Pharis, MD,b​ Charissa Pockett, MD,​b Reeni Soni, MD,c​ Deborah Fruitman, MD,​d Kristine J.Heterotaxy Guleserian, MD,​e Melissa Nater, MD, Syndromef​ Stephen C Raynor, MD,​g Andrew and S. Mackie, MD, Intestinal SM,h​ Bryan Dicken, MDi Rotation Abnormalities BACKGROUND: abstract

Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a METHODS: cohort of infants≤ with HS and determine the incidence and natural history of IRA. Infants 6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at RESULTS: the discretion of each participating center. Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants – were evaluated routinely. The median age at symptom presentation was 46 days (range: 5 171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a – combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a CONCLUSIONS: median follow-up of 1.6 years (range: 0.06 4.93 years). IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

WHAT’S KNOWN ON THIS SUBJECT: Intestinal a b Pediatric Cardiac Intensive Care Unit, Stollery Children’s Hospital, Edmonton, Alberta, Canada; Department rotation abnormalities (IRAs) are commonly of Pediatrics, University of Saskatchewan, Saskatoon, Saskatchewan, Canada; cDepartment of Pediatrics, University of Manitoba, Winnipeg, Manitoba, Canada; dDepartment of Pediatrics, University of Calgary, Calgary, associated with heterotaxy syndrome (HS) and Alberta, Canada; eDivision of Cardiovascular Surgery, Nicklaus Children’s Hospital, Miami, Florida; fAdvocate may be an important source of morbidity. There is Children’s Heart Institute, Advocate Children’s Hospital, Oak Lawn, Illinois; gDivision of Pediatric Surgery, disagreement as to whether an asymptomatic infant Children’s Hospital and Medical Center, University of Nebraska Medical Center, Omaha, Nebraska; and with HS and IRA should have a prophylactic Ladd h i Departments of Pediatrics and Surgery, University of Alberta, Edmonton, Alberta, Canada procedure. Dr Ryerson conceptualized and designed the study, collected data, coordinated and supervised WHAT THIS STUDY ADDS: This was the first multi- data collection, drafted the initial manuscript, and reviewed and revised the manuscript; Drs institutional study in which a cohort of infants Pharis, Pockett, Fruitman, Soni, Guleserian, Nater, and Raynor collected data and reviewed and revised the manuscript; Dr Mackie performed statistical analysis and critically reviewed with HS was prospectively observed. Children with the manuscript; Dr Dicken conceptualized and designed the study and reviewed and revised symptomatic IRAs presented by 6 months of age, but the manuscript; and all authors approved the final manuscript as submitted and agree to be not all children required a Ladd procedure. No child accountable for all aspects of the work. developed midgut volvulus or complications from the This trial has been registered at www.​clinicaltrials.​gov (identifier NCT01591928). Ladd procedure. DOI: https://​doi.​org/​10.​1542/​peds.​2017-​4267 Accepted for publication May 15, 2018 Address correspondence to Lindsay M. Ryerson, MD, Pediatric Cardiac Intensive Care Unit, MAHI 6A7, 11220 83rd Ave, Edmonton, AB T6G 2B7, Canada. E-mail: [email protected] To cite: Ryerson LM, Pharis S, Pockett C, et al. Heterotaxy Syndrome and Intestinal Rotation Abnormalities. Pediatrics. 2018;142(2):e20174267

Downloaded from www.aappublications.org/news by guest on October 4, 2021 PEDIATRICS Volume 142, number 2, August 2018:e20174267 ARTICLE Children with heterotaxy syndrome infants with HS and determine the the vast majority of infants, this was (HS) have an abnormal lateralization incidence, spectrum of disease, and immediately after birth. No infant of the abdominal and thoracic organs natural history of IRA and secondary was enrolled after symptomatic as well as isomerism1 of their atrial midgut volvulus. We hypothesized presentation. Exclusion criteria were appendages. They often also have1 that asymptomatic infants with HS other congenital abnormalities that complex congenital heart disease. and IRA would not benefit from necessitated abdominal surgery. Children with HS may have intestinal a prophylactic Ladd procedure Data were collected from each rotation abnormalities (IRAs), because the risks of a prophylactic institution and entered into a secure which put2 them at risk for midgut intervention outweighed its potential Web application (research electronic volvulus. IRAs are defined as any benefit. data capture) that was designed and deviation3 from normal midgut METHODS managed in Canada (University of rotation. Advances in cardiac Alberta, Edmonton, Canada). surgery have improved survival in children with HS such that there is This study was approved by the Data were collected from local increasing attention to IRAs and their local ethics research board of each hospital medical records, including management. participating institution. Infants operative, radiologic, and Historically, a diagnosis of IRA were recruited from 7 institutions echocardiographic records. Data ’ ’ across North America: Stollery collection included demographic was followed by a Ladd procedure ’ irrespective of the child s symptoms. Children s Hospital in Edmonton, data, cardiac and medical diagnoses, There is agreement that any Alberta; Alberta Children s Hospital diagnostic imaging results, cardiac symptomatic child with IRA needs in Calgary, Alberta; Royal University catheterization procedures, and Hospital in Saskatoon, Saskatchewan; immediate surgical evaluation. ’ cardiac and gastrointestinal Health Sciences Center in Winnipeg, The role of a prophylactic Ladd ’ surgeries. Infants were evaluated for procedure in an asymptomatic child Manitoba; Children s Medical Center IRA at the discretion of their treating in Dallas, Texas; Advocate Children s is unresolved. Recently, there has ’ medical team. IRAs were defined as been a paradigm shift away from Hospital in Oak Lawn, Illinois; and any deviation from normal midgut Children s Hospital and Medical prophylactic Ladd procedures in ’ rotation. Data revealed an indication infants with HS given the anesthetic Center in Omaha, Nebraska. Stollery for IRA evaluation, methods of Children s Hospital is the cardiac risk associated with shunted ’ evaluation, and evaluation results. single ventricle physiology, the surgical referral center for 3 smaller Results were classified as positive low prevalence of volvulus in this children s hospitals staffed by fully – (malrotation); negative (normal group, and the higher prevalence of – trained pediatric cardiologists. The intestinal rotation); nonrotation (the post Ladd procedure complications,4 7 other 3 enrolling institutions are all small intestine is located primarily including small-bowel obstruction. ‍ ‍ congenital cardiac surgical centers. on the right side of the abdomen, There is no published literature HS was defined as any arrangement and the large intestine is located detailing the natural history of IRA of internal thoracoabdominal organs primarily on the left side of the in older patients with HS. IRAs in that was not situs solitus or situs abdomen); or indeterminate (unable the non-HS population may present – inversus with associated congenital to determine intestinal rotation). beyond infancy but tend to have an 10 12 heart disease. ‍ ‍ HS was separated IRA investigations were interpreted atypical presentation with chronic 8,9​ into isomerism of the right atrial by local pediatric radiologists. symptoms. ‍ appendages, typically associated Data in which the clinical course The morbidity of an elective Ladd with asplenia, which we term right of the children was detailed were procedure in all asymptomatic isomerism (RI), and isomerism of collected, including the performance infants needs to be balanced against the left atrial appendages, usually of a Ladd procedure or other the morbidity in the occasional infant associated with polysplenia, gastrointestinal surgeries, indications who develops midgut volvulus and which we term left isomerism (LI). for a Ladd procedure (prophylactic, ≤ requires an emergency laparotomy. Study inclusion criteria included a including those that were part As this population continues to diagnosis of HS, an age of 6 months, of a combined procedure or an increase as a result of continuing and written informed consent emergent procedure), complications advances in cardiovascular care, it from a parent or legal guardian. after a Ladd procedure, and the is vital to devise an evidence-based Families of infants with a prenatal development of a symptomatic treatment model. For this multi- diagnosis of HS were consented intestinal obstruction in those institutional study, our objective was and enrolled into the study at their children with IRA who did not have a to prospectively observe a cohort of first presentation to hospital; for Ladd procedure. Downloaded from www.aappublications.org/news by guest on October 4, 2021 2 RYERSON et al TABLE 1 Cardiac Anatomy and IRAs of 38 Children With HS Variable RI (n = 22), n (%) LI (n = 16), n (%) P Continuous variables are presented as mean (SD) and median Single ventricle physiology 21 (96) 9 (56) .01 Symptoms of IRA 5 (23) 3 (19) 0.99 (interquartile range or range) Investigated for IRA 15 (68) 14 (88) .25 as appropriate, and categorical a b ’ IRA 12 (80) 9 (64) .43 variables are presentedt as counts Ladd performed 4 (18) 3 (19) 0.99 Deceased 7 (31) 2 (13) .25 (percentages).P Fisher s exact test and unpaired tests were used to a n = 15. P ≤ b calculate values when applicable. n = 14. values of .05 were considered statistically significant. Analyses were conducted by using SAS n of concerning symptoms, which by using UGI; 14 (67%) had IRA software (SAS Institute, Inc, Cary, n included not tolerating feeds ( = 3), (11 with malrotation and 3 with NC). n n vomiting ( = 2), bilious vomiting nonrotation). One infant with RESULTS (n = 1), hematochezia ( = 1), and malrotation had an immediate abdominal distention and tenderness prophylactic Ladd procedure. One ( = 1) (Table 2). The median (range) infant with nonrotation by UGI had – Infants were enrolled from January age at symptom presentation was a prophylactic Ladd procedure as 2012 to December 2016. Thirty-eight 46 days (5 171 days). The oldest part of a combined procedure with infants (53% boys) were included; symptomatic infant presented at a gastrostomy tube at 966 days of 22 infants had RI, and 16 infants 171 days of age with nonbilious age. One infant with a UGI diagnosis had LI (Table 1). Two infants with vomiting; the UGI demonstrated of malrotation developed possible LI who also had duodenal atresia malrotation. A Ladd procedure was symptoms of intestinal obstruction, were excluded because they had not performed, and the infant was including irritability and episodic open abdominal surgery in which managed expectantly with no further increases in his lactate, at 18 days a Ladd procedure was performed gastrointestinal symptoms (follow-up of age after initial cardiac palliation. incidentally. Thirty-four infants of 483 days). He had an emergent laparotomy (90%) had an antenatal diagnosis and Ladd procedure that revealed of HS; of the 30 infants (79%) Of the 8 infants who weren incomplete rotation without bowel with single ventricle physiology, investigated becausen of symptoms, wall congestion, suggesting acute or 24 infants had an unbalanced 7 had IRA (malrotation [ = 3] intermittent volvulus. atrioventricular septal defect with and nonrotation [ = 4]; Table 2). either the hypoplastic left or right One infant had discrepant findings Nine infants were not evaluated for ventricle, and 4 infants had atresia between the UGI and the operative IRA; 5 were too hemodynamically of an atrioventricular valve. Two findings. Three infants had a Ladd unstable to investigate, including infants (both with RI) had a balanced procedure: 2 infants had an emergent 2 infants who died before the atrioventricular septal defect and Ladd procedure at 5 days and 2 IRA investigation and 1 infant were initially palliated with a months of age, respectively, and who presented with abdominal systemic-to-pulmonary artery shunt 1 infant had a prophylactic Ladd distention and a radiograph of free for pulmonary atresia or severe procedure as part of a combined intraperitoneal air at 20 days of pulmonary stenosis. The median procedure with a peritoneal dialysis age. At the time of the emergent – laparotomy and incidental Ladd (range) length of follow-up was 1.6 catheter insertion 45 days after ’ years (0.06 4.93 years). the initial symptom presentation. procedure, she had nonrotation No infant had findings of volvulus without volvulus or Ladd s bands. Twenty-nine infants (76%) at their Ladd procedure. Of the 4 The remaining infants were not were evaluated for IRA; 8 were symptomatic infants with IRA who evaluated at the discretion of their symptomatic, and 21 were did not have a Ladd procedure (2 referral institution. No families asymptomatic. All infants were had symptoms of vomiting, and 2 refused an investigation for IRA. investigated by using upper were not tolerating their feeds), gastrointestinal imaging (UGI); 94% none presented with symptoms of In total, 7 children had a Ladd had a UGI with a small bowel follow intestinal obstruction. These children procedure (4 had a prophylactic – through. Twenty-one of the 29 were managed for a median (range) Ladd procedure, and 3 had an infants who were evaluated (72%) of 1.6 years (1.32 3.1 years). emergent Ladd procedure; Table had IRA; 14 infants had malrotation, 3). Five had single ventricle and 7 had nonrotation. Eight Twenty-one asymptomatic physiology at the time of their Ladd infants were investigated because infants were routinely evaluated procedure; the Ladd procedure Downloaded from www.aappublications.org/news by guest on October 4, 2021 PEDIATRICS Volume 142, number 2, August 2018 3 TABLE 2 Description of 8 Symptomatic Infants With HS Patient Heterotaxy Primary Cardiac Symptoms Age at UGI Ladd Age at Ladd Findings at Ladd Procedure No. Type Diagnosis Suggesting IRA Symptoms, d Results Procedure Procedure, d Performed A4 RI UAVSD, DORV, PS, Not tolerating 121 Positive No — — TAPVR feeds C1 RI Dextrocardia, UAVSD, Vomiting 61 Positive No — — DORV, PS, TAPVR D1 LI UAVSD, hypoplastic Vomiting 171 Positive No — — arch, interrupted IVC D2 LI Dextrocardia, UAVSD, Hematochezia 38 Negative No — — PA, interrupted IVC E2 RI UAVSD, PS, TAPVR Abdominal 5 Nonrotation Yes 5 Nonrotation with wide distention mesentery and no volvulus; and chylous ascites tenderness E7 RI AVSD, DORV, aorta Not tolerating 46 Nonrotation Yes with PDC 95 Incomplete rotation without anterior and to the feeds volvulus, inflammation, right of pulmonary multiple jejunal perforations artery, PS, TAPVR E8 RI Dextrocardia, Not tolerating 46 Nonrotation No — — L-looped ventricles, feeds UAVSD, DORV, PS S3 LI DORV, LAVV atresia, Bilious vomiting 23 Nonrotation Yes 64 Nonrotation with Ladd’s bands interrupted IVC adhering duodenum to cecum and right colon AVSD, atrioventricular septal defect; DORV, double outlet right ventricle; IVC, inferior vena cava; LAVV, left atrioventricular valve; PA, pulmonary atresia; PDC, peritoneal dialysis catheter; PS, pulmonary stenosis; TAPVR, total anomalous pulmonary venous return; UAVSD, unbalanced atrioventricular septal defect; —, not applicable.

was performed before or after limitations on their care, 2 children no failure of expectant management their initial palliative cardiac died of respiratory failure, 1 child in asymptomatic infants (specifically, procedure (none were delayed died of chronic renal failure, 1 no infant developed volvulus); and until after a bidirectional superior child died of a severe bleeding (3) no difference in symptomatic cavopulmonary anastomosis). There complication while on extracorporeal presentation nor incidence of IRA were no findings of volvulus at the life support, and 1 child died after between infants with RI and infants time of the Ladd procedure. There cardiac arrest with severe hypoxic- with LI. were no complications after the Ladd ischemic injury to the central nervous procedure. There was no statistical system. The authors of a recent single-center series report an incidence of IRA of difference in the incidenceP of IRA DISCUSSION between infants with RI and infants 60% to 83%5,6,​ 13,​ in14​ screened patients with LI (80% vs 64%; = .43). with HS. ‍ ‍ ‍ The authors of two There was no failure of expectant recent systematic15,16​ reviews report management of an abdominal IRAs are not a distinct entity but a similar results. ‍ The first included catastrophe in those children continuum of abnormalities of the 11 heterogeneous retrospective15 with IRA who did not have a Ladd position and peritoneal attachment studies of 649 patients with HS. procedure. of the large and small bowel, which Of the 44% of patients who were reflects a failure that occurs at any screened, 47% had IRA. Of the entire Nine children died (7 had single time in midgut development. In cohort, there was a 1.2% prevalence – ventricle physiology) at a median this study, we aimed to determine of volvulus, with no studies (range) age of 3.5 months (0.6 29 the incidence, spectrum of disease, describing failure of observation. The months). Survival in this series was and natural history of IRA in infants second meta-analysis (24 studies, higher in the cohort with LI (87%) with HS as well as the incidence of including 1433 patients with HS) compared with the cohortP with RI secondary midgut volvulus in a multi- suffers from similar15 limitations as (68%) although this did not reach institutional prospective cohort of Landisch et al with heterogeneity statistical significance ( = .25). infants with HS. The main findings in reporting overall incidence of The cause of death was known in of this study include (1) a 72% IRA, detection rate of IRA after

8 children; 3 children died after a incidence of IRA, which is5,6,​ consistent13,​ 14​ screening asymptomatic cases, cardiac arrest because of previous with previous literature ‍ ‍ ‍ ; (2) prevalence of volvulus, and surgical Downloaded from www.aappublications.org/news by guest on October 4, 2021 4 RYERSON et al 16 complications. It reports a 58% incidence of IRA in screened patients. After Ladd Procedure Complications Both reviews report higher risks of

perioperative complications15,16​ than that of volvulus. ‍ The reported of Volvulus

Presence risk of small bowel obstruction after No None No None No None

a Ladd5,17,​ procedure18​ is as high as 27%. ‍ ‍ The incidence of postoperative complications after the rotation rotation rotation Degree of

Malrotation Ladd 5,procedure17,​ 19​ may be as high as Nonrotation No None Nonrotation No None Incomplete Incomplete Nonrotation No None Nonrotation No None 61%. ‍ ‍

There is controversy in the literature on whether every asymptomatic

Procedure infant with HS needs to be combined procedure with GT combined procedure with PDC combined procedure with emergent laparotomy for query NEC Reason for Ladd investigated for IRA and, if present, whether an elective Ladd procedure should be offered. There is agreement

20 Prophylactic, that any symptomatic infant needs an immediate surgical evaluation. Procedure, d We believe that evaluating for IRA gives both the care team and the

— family additional information about UGI Results Age at Ladd Nonrotation 966 Prophylactic, Positive 22 Prophylactic Incomplete patient risks, particularly if they live far from tertiary care. Heightened awareness to the complexity of this 5 Nonrotation 5 Emergent 46 Nonrotation 95 Prophylactic, 18 Positive 18 Emergent 20 — — patient population is crucial to its

Symptoms, d management. The absence of IRA is useful information for the medical team, especially in those patients — — — with single ventricle physiology who

may have an20 atypical presentation distention feeds free intraperitoneal air

AXR suggested of volvulus. Families and primary care physicians need to be educated about the potential implications of

— abdominal or nonspecific symptoms screening screening screening and the risk of a possible abdominal Reason for UGI Symptoms Age at catastrophe. Patients with single ventricle physiology may remain in No Yes Routine Yes Symptoms Not tolerating Yes Symptoms Bilious vomiting 23 Nonrotation 64 Emergent Yes Routine the hospital beyond their first month for IRA Screened of life. The natural history of IRA in the general population suggests that 80% of symptomatic patients

present in the first month,21, 22​and 90% present in the first year. ‍ If they Diagnosis interrupted IVC anterior and to the right of pulmonary artery, PS, TAPVR interrupted IVC obstructed TAPVR hypoplastic arch, interrupted IVC

Primary Cardiac remain asymptomatic and have a close follow-up at discharge, they can be discharged from the hospital LI UAVSD, d-TGA, PA, LI LAVV atresia, DORV, LI Dextrocardia, AVSD, RI UAVSD, PS, TAPVR Yes Symptoms Abdominal RI PA UAVSD, DORV, Yes Routine RI aorta AVSD, DORV, RI PA, UAVSD, DORV, without receiving a prophylactic Type

Heterotaxy Ladd procedure. Similarly, if they  7 Children Who Had a Ladd Procedure remain asymptomatic at the time of – their bidirectional cavopulmonary Patient No. E2 A1 E4 E7 S3 S6 S5 AVSD, atrioventricular septal defect; AXR, abdominal x-ray; DORV, double outlet right ventricle; d-TGA, dextro-transposition of the great arteries; GT, gastrostomy tube; IVC, inferior vena cava; LAVV; left atrioventricular valve; NEC, necrotizing double outlet right ventricle; d-TGA, dextro-transposition of the great arteries; GT, AVSD, atrioventricular septal defect; AXR, abdominal x-ray; DORV, total anomalous pulmonary venous return; UAVSD, unbalanced atrioventricular septal defect; — , not applicable. enterocolitis; PA, pulmonary atresia; PDC, peritoneal dialysis catheter; PS, stenosis; TAPVR, TABLE 3 anastomosis (typically at 4 6 months of age), they are unlikely to require a Ladd procedure because the natural Downloaded from www.aappublications.org/news by guest on October 4, 2021 PEDIATRICS Volume 142, number 2, August 2018 5 ’ history of IRA suggests a low risk of the severity of malrotation has26 describing the presence of Ladd s subsequent volvulus. yielded inconsistent26,27​ findings. They bands and a narrow mesentery. and others have ‍ separated IRA This narrow operative definition It is important to avoid overtreating into distinct anatomic categories: misses the wide spectrum of IRA. those infants with HS who do not (1) true malrotation with a narrow There was no difference in IRA have a narrow mesentery given mesenteric stalk, (2) nonrotation between infants with RI and infants their increased perioperative risk 23,24​ with a broad mesentery, and with LI in the current study, which of complications. ‍ These risks 14 (3) atypical rotation defined as is similar to others. Children with continue into adulthood when malposition of the ligament of LI have a better cardiac prognosis presentation of IRA may occur with 8,9​ Treitz or duodenal malposition. with typically less severe congenital vague gastrointestinal symptoms. ‍ 31,32​ Unfortunately, there is often Little is known about the natural heart disease. ‍ These data do discordance between the imaging and history of IRA in patients with 28 not support differing expectant operative findings,​ which is also HS after infancy. This may be management in asymptomatic infants noted in this study. In this study, the because patients die either of their with HS on the basis of their variety cohort was divided into categories cardiac disease or other morbidity of isomerism. of malrotation and nonrotation on secondary to HS (including IRA), the assumption that the nonrotated they remain asymptomatic, or they Although a multi-institutional study, bowel would have a wide mesenteric are subject to publication bias. In the study size was small, reflecting base. That assumption is likely a recent study, authors who used the rarity of HS, which is estimated incorrect because our cohort includes 33 statistical analysis to assess the at a 1:10000 ratio. Symptomatic infants with nonrotated intestines need for surgery in asymptomatic infants consistently presented who developed symptoms suggestive children and adults with IRA before 6 months of age although of volvulus. Unfortunately, we have (but without HS) found that an eligibility for the study included an subsequently discovered infants with observation without a prophylactic age <6 months; it is possible that nonrotation both by UGI and direct Ladd procedure was preferred 25 inspection at the laparotomy who older children with HS not included among older patients. Any had a narrow mesentery. Even in the in this study may develop midgut increased surgical risk, as would be absence of a narrow mesenteric stalk, volvulus beyond 6 months of age. expected for any child with cardiac ’ infants with IRA remain at risk for Because the majority of enrolling disease, further lowered the age volvulus secondary to Ladd s bands. institutions were tertiary congenital threshold at which an observation There are no specific tests that will cardiac surgical centers, there is was preferred over a prophylactic accurately predict the width of the the possibility of referral bias in surgery. Another retrospective vascular pedicle. The addition of a this study. Seventy-nine percent of review of 170 patients with IRA contrast enema that reveals a mobile infants had complex single ventricle described that 48% of patients 8 cecum and right colon may identify physiology and needed neonatal presented as adults. Of that infants at a higher risk of volvulus palliation. In this study, we may have group, the incidence of volvulus secondary to a narrow vascular missed children with HS who had a at the time of diagnosis was 12% pedicle. Attempting to characterize less severe congenital heart disease (operative findings confirming IRA on the basis of the position of the that did not require cardiac surgery volvulus that required an intestinal duodenal-jejunal junction relative to in infancy, or alternatively, we may resection were not reported). This the pylorus is less compelling than have missed neonates who died of suggests that the natural history the duodenal-jejunal flexure that fails either volvulus or severe congenital of IRA progressing to acute midgut to cross vertebral pedicles and may heart disease before a surgical volvulus is low. Although it is signify a narrow vascular pedicle. referral. Unfortunately, we were not uncertain if we can extrapolate the UGI has been shown to be a sensitive able to evaluate 100% of infants, non-HS experience to the population imaging modality for the presence the majority because they were too that has HS, health care providers 29 of IRA ; the presence or absence unstable. Eighty percent of infants should be suspicious of chronic ’ of symptoms should guide surgical were cared for at a single cardiac abdominal symptoms that may be decision-making. surgical institute (Stollery Children s related to an undiagnosed IRA in Hospital), where we have an ongoing older children and adults. 26 It has been suggested that infants dialogue with an engaged general Papillon et al suggest that the with LI have a lower incidence of 30 surgical team, and as such, even rotation variant predicts the width IRA compared with infants with RI. symptomatic infants were carefully of the mesentery and the risk of However, malrotation was defined observed, and not all had a Ladd volvulus. The intent to characterize on the basis of operative findings procedure. We cannot extrapolate Downloaded from www.aappublications.org/news by guest on October 4, 2021 6 RYERSON et al ABBREVIATIONS our experience of careful observation in diagnosing the presence of to other institutions. IRA and informing the family and HS: heterotaxy syndrome CONCLUSIONS medical team. The presence or absence of symptoms should guide IRA: intestinal rotation surgical decision-making. Expectant abnormality LI: left isomerism IRAs are present in the majority of management for the asymptomatic RI: right isomerism screened infants with HS. Infants infant is reasonable because no infant UGI: upper gastrointestinal with symptomatic IRA presented managed as such developed midgut imaging by 6 months of age. UGI is useful volvulus. PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2018 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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Downloaded from www.aappublications.org/news by guest on October 4, 2021 8 RYERSON et al Heterotaxy Syndrome and Intestinal Rotation Abnormalities Lindsay M. Ryerson, Scott Pharis, Charissa Pockett, Reeni Soni, Deborah Fruitman, Kristine J. Guleserian, Melissa Nater, Stephen C Raynor, Andrew S. Mackie and Bryan Dicken Pediatrics originally published online July 26, 2018;

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Downloaded from www.aappublications.org/news by guest on October 4, 2021 Heterotaxy Syndrome and Intestinal Rotation Abnormalities Lindsay M. Ryerson, Scott Pharis, Charissa Pockett, Reeni Soni, Deborah Fruitman, Kristine J. Guleserian, Melissa Nater, Stephen C Raynor, Andrew S. Mackie and Bryan Dicken Pediatrics originally published online July 26, 2018;

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