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Radiology Case Reports May 2021Heterotaxy syndrome is a rare condition characterized by the abnormal arrangement of thoracoabdominal organs across the left-right axis of the body. It is generally...
Heterotaxy syndrome is a rare condition characterized by the abnormal arrangement of thoracoabdominal organs across the left-right axis of the body. It is generally classified as right and left atrial isomerism or asplenia and polysplenia syndrome, even though there are overlaps and uncertainties. The diagnosis of isomerism is typically made by echocardiography. However, multidetector computed tomography and MRI can help in obtaining detailed data on the morphology of the heart, great vessels, the anatomy of the internal organs, and their mutual arrangement that make an accurate diagnosis of heterotaxy syndrome. The authors present here the imaging findings of the heterotaxy polysplenia syndrome in a 21-year-old female with a complete endocardia cushion defect and a duplicated right renal vein.
PubMed: 33717387
DOI: 10.1016/j.radcr.2021.02.015 -
Journal of Arrhythmia Feb 2021As situs ambiguus can cause sinus bradycardia in young patients, the best timing for pacemaker implantation is controversial when the patient is a fertile female.
As situs ambiguus can cause sinus bradycardia in young patients, the best timing for pacemaker implantation is controversial when the patient is a fertile female.
PubMed: 33664913
DOI: 10.1002/joa3.12471 -
Journal of Minimal Access Surgery 2021Heterotaxy syndrome is associated with a plethora of cardiovascular and other multi-system anomalies with a high childhood mortality. A dilated azygos vein as part of...
BACKGROUND
Heterotaxy syndrome is associated with a plethora of cardiovascular and other multi-system anomalies with a high childhood mortality. A dilated azygos vein as part of the polysplenia variant of heterotaxy syndrome may cause oesophageal stenosis owing to a prolonged compression. We describe our technique of extramediastinal oesophago-oesophagostomy in the prone position for this rare congenital syndromic malformation with an excellent outcome.
PATIENTS AND METHODS
A 17-year-old boy with heterotaxy syndrome presented with intermittent dysphagia and postprandial emesis with failure to thrive. Despite the presence of diverse anatomic abnormalities, it was only his symptom of dysphagia due to oesophageal stricture that merited surgical intervention. He underwent an azygos-preserving extramediastinal oesophago-oesophagostomy in the prone position without segmental resection with the establishment of continuity using a modified Collard-type anastomosis.
RESULTS
The patient had an uneventful convalescence, with imaging after 1 year showing no re-stenosis. After a follow-up of 3 years, the patient is free of symptoms and has gained weight.
CONCLUSION
Oesophageal stenosis may result from prolonged compression by anomalous vasculature. An isolated correctable anatomic derangement, young age with good functional reserve, other associated anomalies not causing any symptoms, the physiological advantages of executing the surgery in a prone position and availability of expertise in minimally invasive surgery ensured excellent outcomes. The hitherto unreported technique may open up avenues for further research regarding the behaviour of the oesophageal muscular tube with transection and re-anastomosis for rare benign abnormalities.
PubMed: 33605935
DOI: 10.4103/jmas.JMAS_313_20 -
Scientific Reports Feb 2021Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to...
Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA). We performed a retrospective cohort study of all children who were diagnosed with HPS between 2000 and 2018 at Seoul National University Hospital. The clinical features and outcomes of the 10 patients diagnosed with HPS were reviewed. To clarify the risk factors of HPS in patients with BA, we reviewed 120 patients diagnosed with BA. Underlying liver disease was BA in 8 patients, portal vein agenesis in 1 patient, and portal vein thrombosis in 1 patient. A total of 7 patients underwent liver transplantation (LT). Currently, all seven patients, including 3 patients with severe HPS, survived after LT. The prevalence of HPS in children with BA was 7%. Polysplenia/interrupted inferior vena was the only risk factor for HPS in BA patients in multivariate analysis. The Pediatric End-Stage Liver Disease score was not associated with the development of HPS. Children with severe HPS undergoing LT had excellent outcomes. Screening for HPS in children with BA is required regardless of the severity of liver diseases.
Topics: Biliary Atresia; Child; End Stage Liver Disease; Hepatopulmonary Syndrome; Humans; Liver Transplantation; Prevalence; Retrospective Studies; Risk Factors; Seoul; Severity of Illness Index
PubMed: 33603173
DOI: 10.1038/s41598-021-83785-x -
Journal of Cardiology Cases Jan 2021A 28-year-old woman with polysplenia was referred to our hospital for atrial lead failure. She had undergone an intracardiac repair (ICR) for incomplete atrioventricular...
A 28-year-old woman with polysplenia was referred to our hospital for atrial lead failure. She had undergone an intracardiac repair (ICR) for incomplete atrioventricular septal defect and the implantation of epicardial pacing leads due to complete atrioventricular block at the age of 1 year. When she was 13 years old, an endocardial dual-chamber pacemaker was implanted via the right subclavian vein because of epicardial lead failure. The contrast-enhanced computed tomography scan revealed an inferior vena cava defect with an azygos vein connection to the superior vena cava, occlusion of the right brachiocephalic vein, a defect of the left brachiocephalic vein, and a persistent left superior vena cava ligated at the ICR. Therefore, lead exchange was indicated. During the operation, the temporary pacing lead and the guidewire for emergent deployment of the Bridge Occlusion Balloon® were advanced through the azygos vein and placed at the right ventricle and the hepatic vein, respectively. Both 11-Fr and 13-Fr mechanical rotational dilator sheaths were needed for the lead extraction owing to dense calcification and tight adhesions. The atrial lead was successfully extracted without any complications despite extremely restricted venous access. A new atrial lead was inserted through the space created by the 13-Fr sheath. < Transvenous lead extraction in patients with polysplenia is technically challenging. These patients often undergo pacemaker implantation in childhood, which results in tight adhesions and dense calcifications on the leads, and venous access is extremely restricted. It may be impossible to use a snare and deploy the endovascular balloon to prevent a catastrophic complication from the right femoral vein to the superior vena cava in cases of the inferior vena cava defect.>.
PubMed: 33437340
DOI: 10.1016/j.jccase.2020.09.004 -
Clinical Journal of Gastroenterology Apr 2021Situs inversus abdominus is a congenital abnormality characterized by the mirror image positioning of the abdominal organs, making it difficult to diagnosis and...
Situs inversus abdominus is a congenital abnormality characterized by the mirror image positioning of the abdominal organs, making it difficult to diagnosis and treatment of many diseases. Here, we report a rare case of gastric varices in situs inversus abdominus patient. A 55-year-old man was diagnosed with F2-shaped gastric varices associated with alcoholic cirrhosis. Abdominal dynamic computed tomography showed situs inversus abdominus, and gastric varices with a gastrorenal shunt. Due to the complex anatomy, emergency interventional radiology during bleeding was extremely difficult. Therefore, after thorough consultation, we decided to treat gastric varices prophylactically by balloon-occluded retrograde transvenous obliteration. After treatment, the patient's clinical course was generally good, with no adverse events, and the gastric varices disappeared. It is important to assess anatomical positioning using three-dimensional reconstruction computed tomography images before treatment, to adequately prepare, including selecting appropriate devices (i.e., catheters and sheath). Although preventive treatment of gastric varices is controversial, in cases with anatomical complexity, preventive treatment with sufficient preparation would be recommended. To the best of our knowledge, there are no reports of treatment for gastric varices in situs inversus abdominus patient. This case will serve as a reference for successful treatment in future cases.
Topics: Balloon Occlusion; Embolization, Therapeutic; Esophageal and Gastric Varices; Humans; Male; Middle Aged; Situs Inversus; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 33403531
DOI: 10.1007/s12328-020-01332-z -
Korean Journal of Transplantation Dec 2020Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully...
Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully performed in a pediatric recipient with BA-PS. The recipient was 29-month-old boy who underwent Kasai procedure. The coexisting malformations included agenesis of the inferior vena cava with azygos vein continuation, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. With pediatric end-stage liver disease score of 33, the patient was allocated for split LT. Under this condition, the left lateral section graft was equivalent to a graft-recipient weight ratio of 2.6%. The recipient surgery was performed according to the standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Portal collateral veins were embolized intraoperatively to secure portal vein inflow. No surgical complications were developed. Currently, the patient has been doing well for 4 years after transplantation. Our pediatric patient with BA-PS had various anatomical malformations. Thorough preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring are necessary for successful LT.
PubMed: 35770105
DOI: 10.4285/kjt.20.0023 -
International Journal of Surgery Case... 2020This is a case report that presents an unusual case of Polysplenia Syndrome (PSP) undergoing laparoscopic sleeve gastrectomy (LSG). This kind of presentation would be of...
INTRODUCTION
This is a case report that presents an unusual case of Polysplenia Syndrome (PSP) undergoing laparoscopic sleeve gastrectomy (LSG). This kind of presentation would be of interest to general surgeons who perform bariatric surgery.
PRESENTATION OF CASE
The case presented is a 28-year-old female patient who was not a known case of Polysplenia Syndrome. During the patients' pre-operative assessment, physical examination was done and a type of heterotaxy syndrome was suspected upon palpation of the stomach. Therefore, Computed Tomography (CT) imaging was performed which confirmed PSP. She underwent laparoscopic sleeve gastrectomy due to her morbid obesity, as her Body Mass Index (BMI) was 40.8 kg/m. Post-operatively, water soluble contrast study was performed to rule out leak and the patient had uneventful follow-up.
DISCUSSION AND CONCLUSION
The surgical approach of such patients is somewhat similar to those with situs solitus; however, the performing surgeon must take into consideration the anatomical orientation. Proper assessment of such patients prior to surgery with history, full physical examination, and fitting imaging modalities is essential.
PubMed: 33076202
DOI: 10.1016/j.ijscr.2020.09.079 -
International Journal of Surgery Case... 2020Polysplenia is a complex polymalformative syndrome that is frequently accompanied by an inconsistent spectrum of visceral and vascular anatomical abnormalities and is...
PURPOSE OF THE STUDY
Polysplenia is a complex polymalformative syndrome that is frequently accompanied by an inconsistent spectrum of visceral and vascular anatomical abnormalities and is extremely undiscovered in adulthood. The objective of this article is to limit the intraoperative risks generated by the lack of knowledge of these anatomical variations by insisting on the inconstancy of all these variations and the perfect knowledge of the reference anatomy and the exploration of the anatomical variations in imaging before the surgery.
PATIENT AND METHODS
The patient was 50-year-old who was hospitalized in our department for gastric adenocarcinoma. During her extension assessment, a polysplenia syndrome was accidentally discovered on the scan images, which showed an exceptional association between preduodenal portal vein, agenesis of the dorsal pancreas, and polysplenia.
CONCLUSION
There are neither specific clinical symptoms of the polysplenia syndrome, nor any biological sign; hence, the interest of recognizing this pathology is to avoid diagnostic errors, but also to guide the surgeon during the surgical act performed.
PubMed: 33074136
DOI: 10.1016/j.ijscr.2020.10.005 -
Surgical Case Reports Sep 2020Discordant atrioventricular connection with concordant ventriculoarterial connection, otherwise known as isolated ventricular inversion (IVI), is an extremely rare...
BACKGROUND
Discordant atrioventricular connection with concordant ventriculoarterial connection, otherwise known as isolated ventricular inversion (IVI), is an extremely rare congenital cardiac malformation. Reports on the corrective surgery for this anomaly in neonates are few, and the procedure is difficult and complicated. Herein, we report our use of atrial septostomy as a palliative procedure followed by corrective surgery for the repair of neonatal IVI with situs ambiguous(inversus) morphology.
CASE PRESENTATION
A 2-day-old girl weighing 3.5 kg was admitted to our hospital with a low oxygen saturation (SpO) of 70% She was diagnosed with IVI [situs ambiguous(inversus), D-loop, and D-Spiral], atrial septal defect, patent ductus arteriosus (PDA), interrupted inferior vena cava with azygos continuation to the left superior vena cava (SVC), and polysplenia by transthoracic echocardiography and cardiac computed tomography. We planned to perform corrective surgery and decided to first increase interatrial mixing by performing surgical atrial septostomy and PDA ligation 7 days after birth. However, despite the surgical septostomy, pulmonary venous blood flowed toward the right ventricle via the tricuspid valve rather than toward the left-sided atrium and hypoxemia persisted. We decided to perform the intra-atrial switch procedure at the age of 17 days via a re-median sternotomy. The cardiopulmonary bypass (CPB) circuit was established with ascending aorta and venous drainage through the SVC and hepatic veins. Utilizing a left-sided atrium(l-A) approach, a bovine pericardial patch was used for the intra-atrial baffle, which was trimmed into a trouser-shaped patch. Continuous suture using the patch was lying from the front of the right-sided upper pulmonary vein and rerouted SVC, hepatic vein, and coronary sinus to the tricuspid valve. Overall, CPB weaning proceeded smoothly; however, direct current cardioversion was performed for junctional ectopic tachycardia. The postoperative course was uneventful. Her postoperative SpO improved (approximately 99-100%); overall, the patient showed clinical improvement. Discharge echocardiography showed normal biventricular function and an intact atrial baffle with no venoatrial or atrioventricular obstruction.
CONCLUSION
We successfully performed an intra-atrial switch procedure for isolated ventricular inversion in a neonate. Long-term follow-up will be necessary to ensure the maintenance of optimal cardiac function.
PubMed: 32990806
DOI: 10.1186/s40792-020-01016-3