Successful coil embolisation of tortuous portosystemic venous shunts using a steerable microcatheter in a patient with polysplenia after a Fontan-type operation.

Portosystemic venous shunts occur in patients with polysplenia after the Fontan operation. In the long term, these shunts are associated with hyperammonaemia and portal-systemic encephalopathy. Since some shunts are long and tortuous, catheter interventions to close them could be challenging. Instead, a steerable microcatheter could be used for coil embolisation of tortuous portosystemic venous shunts.

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.

Heterotaxy syndrome - An unusual cause for bowel obstruction in an adult.

Heterotaxy syndrome, also known as situs ambiguous, is a spectrum of pathology due to loss of the normal right-to-left asymmetry of the thoraco-abdominal organs. This report describes the case of a 39-year-old female, previously well, who presented with bowel obstruction. CT showed features of left isomerism, with malrotation and volvulus. Contribution: Heterotaxy syndrome consists of cardiac and non-cardiac manifestations. Imaging studies play a crucial role in the individualised management of the patient.

Adult intestinal malrotation presenting as caecal volvulus with incidental findings of duplicate inferior vena cava and other rare abnormalities: case report.

Intestinal malrotation and duplication of the inferior vena cava are rarely diagnosed in adult patients; however, incidence is likely underestimated as they are usually asymptomatic. These congenital malformations have been previously reported in the same patient twice but never with colonic obstruction or ischaemia. A 25-year-old female presented with nausea, vomiting, obstipation, and abdominal pain, and on computed tomography of the abdomen and pelvis was diagnosed with a caecal volvulus and pneumatosis coli associated with intestinal malrotation requiring emergency right hemicolectomy. Incidentally, the patient was noted to have duplication of the inferior vena cava, azygos continuation of the inferior vena cava, and splenic fragmentation. This constellation of symptoms has not been reported in the literature previously. The pattern of malformations follows that of polysplenia syndrome. Although rare, awareness of these malformations can be useful to clinicians.

Polysplenia with situs inversus totalis, azygos continuation of the inferior vena cava, and duplication of the superior vena cava in a healthy adult: A case report.

Polysplenia syndrome is an embryological disorder whereby the usual left-right asymmetry of thoracic and abdominal viscera fails to develop. It is a rare entity, estimated to occur at a frequency of 1 in 40,000, and is often associated with cardiac and biliary abnormalities. More than 75% of patients die before the age of 5 years, and even in the absence of cardiac anomalies, only 5%-10% of patients are expected to survive into adulthood without complications. Although polysplenia syndrome encompasses a wide range of anatomic abnormalities, there is no single pathognomonic feature. Hence, the prognosis of patients with polysplenia depends on their anatomy, thus necessitating radiology in their management. Here we present a case of a 56-year-old man with polysplenia syndrome and situs inversus totalis. This presentation is atypical because polysplenia is usually considered a form of situs ambiguus, and cases with situs inversus totalis are exceedingly rare. Also noted in our patient are variations in the great vessels, including aortic arch branches and the venae cavae which are features not typically associated with either polysplenia syndrome or situs inversus totalis. The patient is healthy and asymptomatic at baseline, with his diagnosis being made incidentally. Our case report is the first to describe this unique combination of cardiothoracic and cardiovascular anatomy. It also emphasizes the importance of radiologists in caring for patients with laterality defects. As these disorders are uncommon, more data on their anatomic variations may help provide better medical care to this patient population.

Heterotaxy pattern associated with sinus node dysfunction in an adult: A case report.

Key Clinical Message: A 26-year-old male patient admitted to the hospital ward with experience of repetitive syncopes for a year. The patient was diagnosed with sick sinus syndrome. The aim of this clinical report is to highlight the variability of anatomical findings associated with polysplenia pattern. Abstract: This case report presents a 26-year-old male patient who presented to the medical ward with a complaint of repeating blackouts for a year. The patient was then diagnosed with sick sinus syndrome, and further investigations revealed left isomerism, polysplenia, and no congenital heart defects. Holter monitoring, ultrasonography, electrocardiography, and computed tomography were used to confirm the diagnosis. The patient underwent DDDR pacemaker implantation for the treatment of SA node dysfunction. The report highlights the variability of anatomical findings associated with polysplenia pattern and the various types of heartbeat disruptions that may occur in the atrial appendages of the left side isomerism.

Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case.

Polysplenia syndrome is a rare congenital anomaly characterized by multiple spleens and associated organ anomalies, which can present with serious complications such as splenic infarction. Diagnosis and management of the disorder can be challenging due to the presence of associated anomalies and the condition is often diagnosed incidentally. We report a case of a six-year-old girl with no significant medical history who presented to the emergency department with fever, abdominal pain, and vomiting. Physical examination and laboratory investigations showed leukocytosis, anemia, and elevated levels of C-reactive protein. A computed tomography scan revealed splenic infarction with polysplenia syndrome. The patient received intravenous antibiotics and pain management and was closely monitored for complications such as sepsis. Early diagnosis and appropriate management are essential to prevent complications, and close monitoring and follow-up are necessary for long-term management.

Splenic Infarct with Polysplenia Syndrome.

A rare case of spontaneous splenic infarction with polysplenia is presented. The diagnosis was made by confirmed by enhanced computed tomography (CT), which showed multiple spleens in the left abdomen and one of the spleen showing low attenuation areas representing infarct. Polysplenia syndrome is a rare entity associated with heterotaxy syndromes. Radiological examinations help the diagnosis by identifying infarcts in the spleen and other abnormal organs in the chest and abdomen. Teaching Point: We report a rare case of polysplenic syndrome with splenic infarction.

Polysplenia syndrome revealed in adulthood by pancreatic and vascular malformations: a case report.

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.

A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study.

Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis.

Laparoscopic sleeve gastrectomy in polysplenia syndrome/left isomerism: A case report.

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/m2. 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.

Polysplenia syndrome with situs ambiguous, common mesentery, and IVC interruption discovered incidentally in an adult.

Polysplenia syndrome associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Polysplenia has been described mainly in childhood owing to critical anatomic malformations related to cardiac defects or biliary atresia. We present a case of polysplenia syndrome found in a 58-year-old man. Only those with mild anatomical abnormalities reach adulthood without a diagnosis. Radiological examinations are helpful to the diagnosis by ascertaining the location and number of spleens, location of other organs in the chest and abdomen, and identification of other associated anomalies.

The Story of Dr Stella (1927-2006).

A life of professional caring, research, teaching, and inspiration-this is the legacy of Dr Stella Zacharioudaki Van Praagh, MD. Among her many outstanding contributions, only a few are recorded here: (1) a new surgical operation for closing apical muscular ventricular septal defects, (2) a newly discovered form of anomalous pulmonary venous drainage and its surgical repair, (3) a new understanding of sinus venosus defects and their surgical repair, (4) the realization that the concept of atrial-level isomerism (mirror-imagery) in the heterotaxy syndromes of asplenia, polysplenia, and single right-sided spleen is erroneous, (5) the understanding that it is possible to diagnose the atrial situs in the majority of cases of the heterotaxy syndromes, and (6) the fact that the concepts of evolution, natural selection, and survival of the fittest were described by Empedocles, an ancient Greek philosopher, in the fifth century bc, and that these concepts were not discovered and published for the first time by Charles Darwin and Alfred Russell Wallace in the 19th century (1858 ad). Dr Stella was conversant with ancient Greek and read it frequently in an ancient Greek study group that she headed. Dr Stella translated from ancient Greek into English a portion of Aristotle's The Physics in which Empedocles' understanding is cited at length. There is no doubt about what Empedocles thought.

Incidental adult polysplenia with situs inversus, interrupted inferior vena cava with azygos continuation, patent ductus arteriosus, and aortic branches variations: a case report.

Polysplenia syndrome is a rare condition (about 2.5:100,000 live births) wherein a person has two or more spleens with accompanying various thoracic and abdominal organ abnormalities. Polysplenia is usually associated with situs ambiguous, while polysplenia with situs inversus is very rare. Herein, we report a rare case of incidentally found polysplenia syndrome combined with situs inversus in a 59-year-old female who also had an interrupted inferior vena cava (IVC) with azygos continuation, patent ductus arteriosus (PDA), and variations in the aortic arch branches. An aberrant left subclavian artery with Kommerell's diverticulum and a large aneurysm in the non-aberrant, proximal-right subclavian artery were also noted.

Síndrome de heterotaxia: hallazgos tomográficos / Heterotaxy syndrome: tomographic findings

Resumen El objetivo de este trabajo es caracterizar los aspectos tomográficos relevantes en el síndrome de heterotaxia, mediante cuatro pacientes que ejemplifican los hallazgos más frecuentes en esta patología. Situs solitus es la disposición habitual de los órganos y vasos sanguíneos y situs inversus se refiere a la imagen en espejo del situs solitus. Cuando la disposición de los órganos es indeterminada e impredecible y no se corresponde con el situs solitus ni el situs inversus, estamos frente al situs ambiguus o síndrome de heterotaxia, espectro de anomalías poco frecuente en las relaciones de los órganos toracoabdominales. Puede acompañarse de isomerismo derecho o isomerismo izquierdo. Clasificarlo en dos subgrupos es habitualmente difícil, ya que ninguno de estos tiene hallazgos únicos y patognomónicos, sino que existe amplia superposición. Ambos son de mal pronóstico, en los casos de isomerismo izquierdo un 5-10% llegan a la edad adulta, siendo de peor pronóstico los casos de isomerismo derecho, debido a que presentan inmunodepresión secundaria a la asplenia y cardiopatías congénitas más severas. Se debe analizar cada caso de forma individualizada y detallada para establecer el diagnóstico, determinar la asociación lesional y establecer aquellos pacientes que presenten mayor riesgo de complicaciones.

Abstract The objective of this brief communication is to characterize the relevant tomographic aspects in the heterotaxy syndrome, by means of 4 patients that exemplify the most frequent findings in this pathology. Situs solitus is the usual arrangement of organs and blood vessels and situs inversus refers to the mirror image of situs solitus. When the arrangement of the organs is indeterminate and unpredictable and does not correspond to situs solitus or the situs inversus, we are facing the situs ambiguus or heterotaxy syndrome, abnormal spectrum of anomalies in the relations of the thoracoabdominal organs. It may be accompanied by right isomerism or left isomerism. Attempts to classify it into two subgroups are usually difficult since none of these has unique and pathognomonic findings, but rather there is broad overlap. Both are of poor prognosis, in the cases of left isomerism 5-10% reach adulthood, with a worse prognosis being the cases of right isomerism due to the fact that they have immunodepression secondary to asplenia and more severe congenital cardiopathies. Each case should be analyzed in an individualized and detailed manner to establish the diagnosis, determine the lesional association and establish those patients that present a higher risk of complications.

Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature.

The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with unique vascular anomalies in a 35 year old male patient evaluated initially for nephrolithiasis by ultrasonography, and intravenous pyelography. Further evaluation by multidetector row computed tomography showed bilateral bilobed lungs with hyparterial bronchi, cardiac apex to the left, five branches from left-sided aortic arch with retroesophageal right subclavian artery, interrupted inferior vena cava with azygos continuation, left renal vein continuing as hemiazygos vein and replaced common hepatic artery arising from the superior mesenteric artery. Other vascular anomalies include right internal iliac vein joining the left common iliac vein and precaval course of the single main right renal artery. Anomalies involving abdominal organs include right-sided stomach, midline liver, multiple splenules (polysplenia) in right upper quadrant of abdomen, short truncated pancreas, intestinal malrotation, inversion of superior mesenteric vessels and a preduodenal portal vein. To the best of our knowledge this is the first report of association of left renal vein continuing as hemiazygos vein, precaval right renal artery and anomalous branching pattern of aortic arch with heterotaxy syndrome.