Digestive tract disorders associated with asplenia/polysplenia syndrome

doi:10.1016/S0022-3468(97)90103-2

Journal of Pediatric Surgery

Volume 32, Issue 1, January 1997, Pages 91-94

https://doi.org/10.1016/S0022-3468(97)90103-2 Get rights and content

Abstract

The authors reviewed experience gained over a 20-year period of asplenia or polysplenia syndrome, focusing on patients with associated digestive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia/polysplenia had associated DTDs. The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus in three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bowel obstruction and on one patient with biliary atresia. One patient with both malrotation and gastric volvulus, and another with only associated malrotation survived. Nine patients died, eight of cardiac insufficiency and one because of hepatic insufficiency. When infants are diagnosed with heterotaxia, they should be examined for other combined DTDs, because they may have a chance for survival if they undergo surgery when their condition is still stable.

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Cited by (85)

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management
2021, American Journal of the Medical Sciences
Citation Excerpt :
However, substantial variation has been reported.2,3,81,84–90 Regardless of the type of isomerism, malrotation of the gut is frequently observed in the presence of heterotaxy and may be characterized by non-rotation, reverse rotation, or mid-gut volvulus3,80,83–89 The Ladd procedure has been recommended as prophylactic therapy to prevent volvulus for infants who survive the neonatal period.61,89,90 Differences in the anatomic location and formation of visceral organs can complicate the evaluation of abdominal pain.
Human laterality disorders comprise a group of diseases characterized by abnormal location (situs) and orientation of thoraco-abdominal organs and vessels across the left-right axis. Situs inversus totalis is mirror image reversal of thoraco-abdominal organs/great vessels. Situs ambiguus, better known as heterotaxy, is abnormal arrangement of thoraco-abdominal organs across the left-right axis excluding situs inversus totalis. Heterotaxy, also referred to as atrial or atrial appendage isomerism, is characterized by abnormal location of left-sided or right-sided organs with loss of asymmetry of normally paired asymmetric organs. It is associated with a variety of anomalies involving the heart, great vessels, lungs and intra-abdominal organs. Right and left atrial isomerism are associated with multiple complex congenital cardiac and vascular anomalies, many of which are lethal when untreated. Isomerism may also affect the lungs, spleen, liver, gall bladder, and intestines. Innovative surgical therapy of heterotaxy/isomerism has reduced early mortality and markedly improved long-term prognosis.
Abnormal Rotation and Fixation of the Intestine
2020, Pediatric Gastrointestinal and Liver Disease, Sixth Edition
Normal intestinal growth and development follows a stereotypical pattern of mesenteric rotation and subsequent intestinal fixation to the body wall during fetal life. Clinically apparent abnormalities of intestinal rotation and fixation are encountered infrequently. The major life-threatening problem associated with abnormal intestinal rotation and fixation is the potential for the intestine to twist on its mesenteric axis, giving rise to a surgical condition known as midgut volvulus. Given the relatively unpredictable nature of midgut volvulus and the catastrophic consequences of subsequent total intestinal necrosis, clinicians must maintain a high index of suspicion for malrotation with volvulus in any infant or child with bilious emesis. In a symptomatic infant or child with midgut volvulus, prompt diagnosis and surgical intervention are essential for prevention of intestinal necrosis. In the incidentally diagnosed asymptomatic infant or child, many pediatric surgeons also recommend prophylactic operative intervention.
Malrotation is not associated with adverse outcomes after cardiac surgery in patients with heterotaxy syndrome
2018, Journal of Pediatric Surgery
Citation Excerpt :
These anatomic abnormalities often include congenital heart disease, ranging from simple septal defects to complex venous and intracardiac anomalies necessitating single ventricle palliation. Intestinal rotational anomalies (IRA) such as malrotation have been reported in 40–90% of patients with HS [2–4]. The feared complication of malrotation is midgut volvulus leading to bowel ischemia.
Patients with heterotaxy syndrome (HS) often have asymptomatic malrotation. There is a lack of consensus regarding the management of these patients, particularly in patients with complex congenital heart disease (CHD). We sought to describe the prevalence of malrotation and incidence of volvulus in a population of patients with complex CHD and to identify the impact of malrotation on morbidity and mortality following cardiac surgery.
We performed a retrospective review of all patients with HS and complex CHD who required cardiac surgery in the first year of life at a single center between October 1995 and September 2015. Malrotation was diagnosed by abdominal imaging or by direct inspection during abdominal surgery. Demographic data was collected along with details of hospitalization following cardiac and GI surgeries. Descriptive analysis along with appropriate hypothesis testing was conducted to evaluate the results.
We identified 49 patients with HS, 42 with single ventricle anatomy and 7 with biventricular anatomy. Of the 49 patients, 29 (59%) were diagnosed with malrotation, 6 (12%) had normal intestinal rotation, and 14 (29%) had no evaluation of intestinal rotation. The prevalence of malrotation in the population who underwent abdominal imaging was 29 out of 35 (83%). There was no difference in survival following cardiac surgery between patients with malrotation and those with unknown or normal intestinal anatomy. Comparing patients with malrotation and patients with normal or unknown intestinal rotation, there was also no difference in surrogate markers of morbidity. Of the 29 patients with known malrotation, only 2 patients (7%) underwent therapeutic Ladd procedures and 19 (65%) underwent prophylactic Ladd procedures.
We conclude that the outcomes following cardiac surgery for patients with HS are not impacted by the presence of malrotation. Furthermore, we also found that the incidence of volvulus in the studied group is low. Given these findings, and the understanding that patients with HS and significant CHD are frequently tenuous and high risk surgical candidates, we do not believe performing prophylactic Ladd procedures is warranted.
III.
Organ system response to cardiac function-splanchnic
2018, Critical Heart Disease in Infants and Children
The splanchnic circulation receives over 25% of the cardiac output and contains a similar percentage of the total blood volume under normal conditions. Thus the splanchnic circulation can act as a site of regulation of distribution of cardiac output and also as a blood reservoir. Multiple regulatory pathways are involved in the distribution of the splanchnic circulation. This chapter focuses on the intestinal and hepatic circulations because of their functional importance and in particular, because they are sites of dysregulation during pathologic conditions and surgical stresses, such as cardiopulmonary bypass. A review of the anatomy and physiology of both the intestinal and hepatic circulation is presented followed by the effects of cardiopulmonary bypass on this circulation. Splanchnic complications associated with cardiac surgical procedures will be reviewed. These include postcoarctectomy syndrome, protein-losing enteropathy, intestinal ischemia, necrotizing enterocolitis, ischemic hepatitis, acute hepatic failure, chronic hepatic dysfunction and cirrhosis, and pancreatitis.
Manifestations of bodily isomerism
2016, Cardiovascular Pathology
Citation Excerpt :
Our findings also fail to show that the risk of volvulus is high in either group. Previous studies, nonetheless, have demonstrated a risk of volvulus among those with heterotaxy and malrotation to be minimal, although a study by Yu and colleagues demonstrated a 10% risk [10–19]. There is now emerging evidence that at least two fifths of those with isomerism lie on the ciliopathy spectrum, although it is abnormal ciliary motion and not ultrastructure that is the underlying defect [27].
We report the findings present in 49 postmortem specimens from patients with so-called heterotaxy, concentrating on those found in the extracardiac systems of organs. Also known as bodily isomerism, we suggest that it is important to segregate the syndromes into their isomeric subtypes to be able to make inferences regarding likely extracardiac and intracardiac findings to allow for proper surveillance. We demonstrate that this is best done on the basis of the atrial appendages, which were isomeric in all the hearts obtained from the specimens available for our inspection. The abdominal organs do not demonstrate isomerism, and they show variable features when compared to the isomeric atrial appendages.
Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis
2016, Journal of Pediatrics
Citation Excerpt :
The incidence of IRA after surgery (and autopsy) was lower than suggested by radiology, which would also explain the heterogeneity among the studies. First, there was variation in inter- and intracenter practice in screening and treating asymptomatic IRA, hence the pathway leading to a diagnosis of IRA varied.10,26,27,29,31,37,40,41 Some centers, despite having identified IRA on screening, had treated a subgroup of patients conservatively based on the mesenteric base assessment on radiology to be wide enough (eg, abdominal situs inversus without malrotation, duodenojejunal malposition without malrotation) to assume that those patients were at no increased risk of midgut volvulus compared with the general population.10,29,31,37,40
To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy.
PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I² statistic.
From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Postoperative mortality after Ladd procedure mainly was associated with cardiac insufficiency, and overall it was significantly greater in the emergency group compared with the prophylactic group (18% vs 5.6%). The complication rate also was greater in case of emergency vs prophylactic abdominal surgery (27% vs 16%); adhesional small bowel obstruction was the most common complication overall (6%).
The screen-detected incidences of IRA and acute midgut volvulus were significantly greater in heterotaxy than the normal population. Prophylactic Ladd procedure was associated with less morbidity and mortality compared with emergency surgery. A long-term prospective randomized trial is needed to define the indication for screening and prophylactic treatment of IRA in heterotaxy.

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^☆: Presented at the 29th Annual Meeting of the Pacific Association of Pediatric Surgeons, Singapore, May 12–15, 1996.

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Digestive tract disorders associated with asplenia/polysplenia syndrome☆

Abstract

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

Smith's recognizable patterns of human malformation