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Medicine Jan 2016Polysplenia syndrome (PSS) is a rare congenital abnormality. Metastases to spleen and skeletal muscle from differentiated thyroid cancer (DTC) are also extremely rare.... (Review)
Review
Polysplenia Syndrome With Splenic and Skeletal Muscle Metastases From Thyroid Carcinoma Evaluated by FDG PET/CT: Case Report and Literature Review: A Care-Compliant Article.
Polysplenia syndrome (PSS) is a rare congenital abnormality. Metastases to spleen and skeletal muscle from differentiated thyroid cancer (DTC) are also extremely rare. Our case report aims to present an interesting case of PSS associated with splenic metastasis (SM) and skeletal muscle metastasis (SMM) from advanced papillary thyroid carcinoma which was evaluated on fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT). An 84-year-old Chinese man was admitted with the history of multiple enlarged masses in bilateral neck, right axillary, and inguinal areas for >2 months. The results of ultrasonography examination were highly suggestive of malignancy. The histological results of the following biopsy were consistent with papillary thyroid carcinoma with involvement of multiple regional lymph nodes. He was referred for an FDG PET/CT imaging to evaluate the situation. FDG PET/CT showed that an intense FDG-avid thyroid mass with widespread regional lymph node involvement and distant metastases in the body. Unexpected sites of metastases were detected in the spleens and skeletal muscles. Most interestingly, FDG PET/CT imaging also described the typical imaging findings of PSS including the 2 right-sided spleens, azygos and hemiazygos continuation of inferior vena cava (IVC), right-sided stomach, middle line liver, a short pancreas, preduodenal portal vein (PPV), and malrotation of gut. Whole body FDG PET/CT imaging can accurately evaluate the situation of DTC by detecting regional lymph node involvement, common and rare sites of distant metastases which are closely related to staging, management, and prognosis of this disease. Whole-body FDG PET/CT is also valuable in demonstrating the typical imaging features of PSS.
Topics: Aged, 80 and over; Carcinoma; Fluorodeoxyglucose F18; Heterotaxy Syndrome; Humans; Male; Multimodal Imaging; Muscle Neoplasms; Muscle, Skeletal; Positron-Emission Tomography; Splenic Neoplasms; Thyroid Neoplasms; Tomography, X-Ray Computed
PubMed: 26825891
DOI: 10.1097/MD.0000000000002532 -
Circulation. Cardiovascular Imaging Feb 2018It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often...
BACKGROUND
It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often based on the pattern of associated findings rather than on atrial morphology itself, which can be difficult to define. The objective was to assess how often concordant patterns of isomerism classified by atrial appendage morphology, bronchopulmonary pattern, and splenic status are breached using cardiac magnetic resonance or computed tomography. The secondary objective was to determine the feasibility of defining atrial appendage morphology using cardiac magnetic resonance or computed tomography.
METHODS AND RESULTS
Retrospective review of 114 pediatric patients (median, 2.4 years; range, 1 day-17.9 years) with heterotaxy who underwent cardiac magnetic resonance or computed tomography was performed to evaluate atrial appendage, bronchopulmonary, and visceral organ arrangements. Atrial appendage and splenic anatomy were not definable in 17 of 114 (15%) and 4 of 114 (3.5%) patients, respectively. In the remaining 93 patients, 39% had classic right isomerism (bilateral right atrial appendages, right bronchopulmonary pattern, and asplenia) and 40% had classic left isomerism (bilateral left atrial appendages, left bronchopulmonary pattern, and polysplenia). Classic pattern of isomerism was breached in 20 of 93 (21.5%) patients: 13 (65%) displayed incongruent abdominal situs, 5 (25%) incongruent bronchial situs, 1 (5%) had discrepant appendage morphology, and 1 (5%) incongruent situs at all levels.
CONCLUSIONS
Atrial appendage morphology is difficult to assess and not always indicative of bronchopulmonary or abdominal situs. Discordance between bronchopulmonary branching, atrial appendage arrangement, and splenic status was identified in >20% patients with heterotaxy. Independent description of each organ system is required when arrangements are disharmonious among different organ systems.
Topics: Abnormalities, Multiple; Adolescent; Atrial Appendage; Bronchi; Child; Child, Preschool; Feasibility Studies; Female; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Infant; Infant, Newborn; Isomerism; Magnetic Resonance Imaging, Cine; Male; Reproducibility of Results; Retrospective Studies; Spleen; Tomography, X-Ray Computed
PubMed: 29444810
DOI: 10.1161/CIRCIMAGING.117.006917 -
Surgical and Radiologic Anatomy : SRA Nov 2016To analyze the vascular structure of the liver in patients with a right-sided round ligament.
PURPOSE
To analyze the vascular structure of the liver in patients with a right-sided round ligament.
METHODS
We reviewed 16 patients with a right-sided round ligament and 3 polysplenia and situs inversus patients with a left-sided round ligament who underwent multidetector row CT with contrast media. The patient population consisted of 13 men and 6 women (mean 62 years). We analyzed the axial and volume-rendered images for the location of the round ligament, gallbladder, portal veins, hepatic veins, and hepatic artery. The following imaging findings for the patients with polysplenia and situs inversus were horizontally reversed.
RESULTS
The prevalence of a right-sided round ligament with and without polysplenia was 75 and 0.11 %, respectively. The gallbladder was located to the right, below, and left of the round ligament in 27.7, 38.8 and 33.3 %, respectively. Independent branching of the right posterior portal vein was noted in 57.8 %. PV4 was difficult to identify in 36.8 %. The middle hepatic vein was located to the left of the round ligament. Two branching patterns for the lateral and medial branches of the right anterior hepatic artery were noted: the common (44.4 %) and separated types (55.5 %). Both of the right anterior hepatic artery and portal vein ramified into two segments; the lateral segment with many branches and the medial segment with a few branches.
CONCLUSIONS
The right-sided round ligament divided the right anterior section into the lateral and medial segments based on the portal vein and hepatic artery anatomy.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anatomic Variation; Child; Female; Gallbladder; Humans; Liver; Male; Middle Aged; Radiography, Abdominal; Round Ligament of Liver; Spleen; Young Adult
PubMed: 27068289
DOI: 10.1007/s00276-016-1674-1 -
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 -
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 -
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 -
Fetal and Pediatric Pathology 2015Limb body wall complex (LBWC) is characterized by multiple severe congenital malformations including an abdominal and/or thoracic wall defect covered by amnion, a short...
Limb body wall complex (LBWC) is characterized by multiple severe congenital malformations including an abdominal and/or thoracic wall defect covered by amnion, a short or absent umbilical cord with the placenta almost attached to the anterior fetal wall, intestinal malrotation, scoliosis, and lower extremity anomalies. There is no consensus about the etiology of LBWC and many cases with abnormal facial cleft do not meet the requirements for the true complex. We describe a series of four patients with LBWC and other malformations in an attempt to explain their etiology. There are several reports of fetuses with LBWC and absent gallbladder and one of our patients also had polysplenia. Absent gallbladder and polysplenia are associated with laterality genes including HOX, bFGF, transforming growth factor beta/activins/BMP4, WNT 1-8, and SHH. We postulate that this severe malformation may be due to abnormal genes involved in laterality and caudal development.
Topics: Abdominal Wall; Abnormalities, Multiple; Abortion, Spontaneous; Adult; Body Patterning; Cloaca; Diseases in Twins; Female; Fetal Death; Gallbladder; Hernia, Umbilical; Heterotaxy Syndrome; Humans; Kyphosis; Lower Extremity Deformities, Congenital; Male; Pregnancy; Retrospective Studies; Scoliosis; Spine; Thoracic Wall; Umbilical Cord
PubMed: 26111189
DOI: 10.3109/15513815.2015.1055021 -
Europace : European Pacing,... Jul 2017We sought to establish the technical feasibility of transseptal puncture and left atrial (LA) ablation through the right internal jugular vein via the superior vena cava...
Transseptal puncture and catheter ablation via the superior vena cava approach for persistent atrial fibrillation in a patient with polysplenia syndrome and interruption of the inferior vena cava: contact force-guided pulmonary vein isolation.
AIMS
We sought to establish the technical feasibility of transseptal puncture and left atrial (LA) ablation through the right internal jugular vein via the superior vena cava (SVC) approach in patients with an interrupted inferior vena cava (IVC).
METHODS AND RESULTS
A 34-year-old man with persistent atrial fibrillation (AF) and polysplenia syndrome (hypoplasia of the left kidney, aplasia of the pancreas tail, bilaterally bilobed lungs, and an interrupted IVC) was referred to our hospital for radiofrequency ablation. Because transseptal puncture and LA ablation would be impossible by a standard IVC approach via the femoral vein, we performed transseptal puncture and LA ablation through the right internal jugular vein via the SVC approach using a manually curved Brockenbrough needle and intracardiac echocardiographic guidance. We accomplished pulmonary vein (PV) isolation using a deflectable guiding sheath and a contact force-sensing ablation catheter to monitor the contact force and the force-time integral of the tip of the ablation catheter. No complications occurred during or after the procedure. The patient was discharged home without recurrence of AF 3 days after the procedure. He had no recurrence of AF and was taking no medication 5 months after ablation.
CONCLUSIONS
We successfully performed transseptal puncture in a patient with persistent AF, polysplenia syndrome, and complete interruption of the IVC using the superior route through the internal jugular vein. We also accomplished PV isolation using a deflectable guiding sheath and real-time monitoring of the contact force of the ablation catheter.
Topics: Abnormalities, Multiple; Adult; Atrial Fibrillation; Cardiac Catheterization; Catheter Ablation; Catheterization, Central Venous; Echocardiography; Heart Septum; Heterotaxy Syndrome; Humans; Magnetic Resonance Imaging; Male; Punctures; Tomography, X-Ray Computed; Treatment Outcome; Vena Cava, Inferior; Vena Cava, Superior
PubMed: 27174901
DOI: 10.1093/europace/euw095 -
The Pan African Medical Journal 2022Polysplenia syndrome (PSS) is a rare congenital disease that associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary...
Polysplenia syndrome (PSS) is a rare congenital disease that associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Most patients with PSS die in the early neonatal period because the disease is often accompanied by serve cardiac and biliary abnormalities. However, some patients have only mild cardiovascular malformations or anomalies in the abdominal organs, which are typically diagnosed incidentally in adulthood. We report the case of a 54-year-old woman who consulted for chronic atypical diffuse abdominal pain. The clinical examination was normal. Abdominal computed tomography showed a total of 5 spleens with vascular and pancreatic malformations as part of polysplenia syndrome. Symptomatic treatment was instituted with good evolution. No specific therapeutic indication was indicated in our case discovered incidentally in adulthood.
Topics: Female; Infant, Newborn; Humans; Adult; Middle Aged; Heterotaxy Syndrome; Pancreas; Abdomen; Vascular Malformations
PubMed: 36590997
DOI: 10.11604/pamj.2022.43.77.31496 -
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