Impact of gastrointestinal comorbidities in patients with right and left atrial isomerism

Background and aim: Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome. Methods: We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention. Results: Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001). Conclusions: In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

Management and Outcomes of Malformations Associated with Intestinal Malrotations in Newborns: About 5 Cases

Bowel malrotation is an abnormality in the rotation of the primary intestinal loop during embryonic life. It is a severe abnormality exposing the patient to fatal complications. We report here a series of five patients presenting digestive malrotation with various malformative associations. The first three cases are multiple intestinal atresia on incomplete common mesentery. They underwent an intestinal anastomosis: end-lateral duodeno-ileal for one and end-to-end jejuno-jejunal for the other two with Ladd procedure. In the latter two cases, the omphalocele was the associated malformation; complications such as rupture of the omphalocele with intestinal perforation for one and occlusion by strangulation at the level of the neck for the other were recorded. Ileal resection removing the perforation with end-to-end ileo-ileal anastomosis was performed for the first, while reduction of strangulation at the origin of the occlusion was sufficient for the second. The LADD procedure was the complementary gesture in all cases. The outcome was favorable in 3 cases; two of the newborns who had jejunal atresia died, one on day 5 in a picture of acute respiratory distress, the other on day 6 in a picture of disseminated intravascular coagulation.

An Unusual Case of Nausea and Vomiting in Pregnancy: A Case Report

Malrotation of the gut is rare in adults. We discuss the case of a 30-year-old primiparous woman who presented to the acute gynecology ward at 19-weeks’ gestation with ongoing nausea and vomiting throughout pregnancy. She attended on a number of occasions with the same symptoms and was trialed on a number of different antiemetics. Initial biochemical investigations were unremarkable, however, the patient started to develop signs of ‘abdominal obstruction’. A magnetic resonance image (MRI) of the pelvis showed evidence of duodenal obstruction secondary to malrotation which may be secondary to a fibrous (Ladd’s) band. She was treated laparoscopically via a Ladd procedure and had an uneventful recovery. Interestingly, the patient presented again in her second pregnancy with very similar symptoms and underwent another Ladd procedure, but via a laparotomy. This is an interesting, rare and unusual case of nausea and vomiting in pregnancy.

Laparoscopic Ladd Procedure for Malrotation in Newborns and Infants

Background Laparoscopic approach for malrotation has become more popular for neonates and in cases with volvulus, but its safety and efficacy remains controversial. This study reviewed laparoscopy outcomes in neonate/infant malrotation. Methods Medline/PubMed and Lilacs databases were reviewed. Data from studies published in English/Spanish between 1995 and 2019 were collected. Results are presented as percentages and means/medians; logistic regression was used to study possible associations. Results Nineteen papers offered 99 neonates/infants with median age and weight of 10.5 days and 3.5 kg, respectively. Ladd’s procedure was performed in 95 (96%) patients and bands’ division in 4 (4%); appendectomy was not included in 16 (16.2%) patients, and cecopexy was not performed in all cases. Volvulus was reported in 39 (39.4%) patients. There were 11 conversions (11.1%) and 10 recurrences of symptoms (10.1%) that required reintervention. An association was found between volvulus and recurrence ( P = .05) and the need for conversion ( P < .01). There were 10 (10.1%) minor complications and no mortality. The median follow-up was 10 months. Discussion Laparoscopic approach to malrotation is feasible and safe in hemodynamically stable neonates/infants without intestinal necrosis and is associated with 11% conversion rate and 10% reinterventions. The presence of volvulus is associated with recurrence and conversion. Laparoscopic Ladd’s procedure with appendectomy and without cecopexy is the commonly practiced approach that is associated with minor complications.

Impact of gastrointestinal comorbidities in patients with right & left atrial isomerism

ABSTRACTObjectiveHeterotaxy syndrome (HS), being right atrial isomerism (RAI) or left atrial isomerism (LAI) often presents with congenital heart disease (CHD). Intestinal abnormalities including malrotation are common. We aim to assess the impact of gut abnormalities on outcome in HS.MethodsWe reviewed cardiology records of HS patients regarding presence of CHD, time for cardiac intervention, presence of gastrointestinal (GI) abnormalities and type/time of surgery. A questionnaire about GI status was sent to patients <18 years old. Kaplan-Meier curves were derived for survival data.ResultsData were available for 195 patients (49 RAI, 146 LAI) of 247 identified. Questionnaires were sent to 77 families, 47 replied. CHD was present in all RAI and 63.7% of LAI cases. Thirty-eight patients had abdominal surgery (19.5%), similar rate in RAI and LAI (20.4% vs 19.1%, p=0.92). Ladd procedure was performed in 17 (44.7%), non-Ladd in 12 (31.5%) and both procedures in nine patients (23.7%). Ten-year freedom from Ladd procedure for all was 86% (RAI=87%; LAI=85%, p=0.82). Freedom from any GI surgery at one year was 86% (RAI=86%; LAI=86%, p=0.98) and at ten years was 80% (RAI=77%; LAI=81%, p=0.65). Ten-year freedom from cardiac surgery was 34% (RAI=7%; LAI=42%, p<0.0001).ConclusionsIn our cohort, one in five patients required abdominal surgery, mostly in their first year, similar in RAI and LAI. Between one and ten years of follow up the impact of GI abnormalities on outcome was minimal. Medium term survival was related to CHD.ARTICLE SUMMARYSTRENGTHS AND LIMITATIONS OF THIS STUDYThis study is the largest cohort study investigating the impact of gastrointestinal abnormalities in cardiology patients with heterotaxy syndrome.It is the first clinical study to show that HS patients suffer from a wider spectrum of abdominal abnormalities, other than typical malrotation, varying in severity from asymptomatic malrotation to complete non-rotation, namely atresia at multiple intestinal levels. This is relevant in prenatal family counseling but also raises questions regarding the indication of elective Ladd procedure in all heterotaxy syndrome patients, as a different procedure might be indicated.Patient morbidity was investigated from both the cardiology and gastrointestinal point 12 of view.Its main limitations in design is that is not a prospective study. Cardiology records have been reviewed with regards to GI symptomatology, screening investigations and procedures.It involved both reviewing the medical records and directly contacting the family for the subcohort that was below 18 years old and lived in the UK, in an attempt to minimise any data errors. Our records were consistent with the information provided by parents/guardians in all cases but one.

Reversed Bowel Rotation with Midgut Volvulus and Internal Hernia

Congenital intestinal malrotation is a group of intestinal rotational anomalies occurring during embryogenesis. Reversed rotation is considered the rarest type of malrotation and often presents with symptoms of bowel obstruction. We present a rare case of a 27-year-old woman who presented with acute abdomen. The patient’s preoperative computed tomography (CT) scan and operative findings confirmed reverse malrotation, internal hernia of foramen of Winslow, and midgut volvulus. The transverse colon, duodenum, small bowel, cecum, and appendix were abnormally located, with the presence of Ladd bands. The patient underwent an emergency laparotomy (Ladd procedure) with an uneventful postoperative recovery and an unremarkable follow-up CT scan of the abdomen. A review of the literature for intestinal reverse malrotation is also presented to provide an understanding of the expected clinical picture and imaging findings for this rare anomaly.

Biliary Atresia Splenic Malformation Syndrome: A Single Center Experience

Introduction: The cause of biliary atresia (BA) is not understood exactly as well as biliary atresia splenic malformation (BASM) syndrome. BA is destructive biliary fibrosis; the etiology may be multifactorial. Association of cytomegalovirus (CMV) and BA have been shown in many reports but CMV and BASM have not been mentioned in the literature. So we aimed to report BASM experiences, an association of CMV infection and need of duodenoduodenostomy if preduodenal portal vein exists. Materials and Methods: The data were collected retrospectively from Cukurova University which is one of the largest tertiary hospitals in Turkey between 2005-2017. The patients of sex, age, blood chemistry counts, TORCH infections blood parameters, BA types, operational findings and mortality were noted. Results: In total, 59 BA patients were diagnosed between 2005- 2017. Seven of them were classified as BASM. The median age of them was 60 days (45-90 days). Three of them were girl and 4 of them were male in gender. The main complaint of whole patients was jaundice. The jaundice of 6 patients began since birth. One of them began at 20 days-age. Median total / direct blood bilirubin levels were 9.6 / 5.4 mg/dl. Median values of liver function tests; ALT, AST and GGT were 77 IU/L, 201 IU/L and 607 IU/L respectively. Five of the patients showed positive results for anti-CMV immunoglobulin M. All had positive anti-CMV Ig G and anti-toxoplasmosis Ig G. Evaluation of the types of BA revealed that one patient had type 2, while all others had type 3. Four of BASM patients had polysplenia and one had asplenia. Five of them had a preduodenal portal vein. All of them had midgut malrotation. One had inferior vena cava interruption. One had hepatic artery anomaly which was originated from SMA. The median time of follow-up was 4 years (1-5 years). All of them are alive and 1 required liver transplantation. Conclusion: BASM should be kept in mind by the surgeon for the requirement of additional surgical procedures such as Ladd procedure, duodenoduodenostomy with Kasai Porto-enterostomy. Duodenoduodenostomy may be performed when the existence of preduodenal portal vein. Further research is recommended for CMV infection and BASM.