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Clinical Case Reports Jun 2023A 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...
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.
PubMed: 37334338
DOI: 10.1002/ccr3.7511 -
Cureus May 2023Polysplenia syndrome is a rare congenital anomaly characterized by multiple spleens and associated organ anomalies, which can present with serious complications such as...
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.
PubMed: 37284391
DOI: 10.7759/cureus.38589 -
Cardiology in the Young Sep 2023Portosystemic venous shunts occur in patients with polysplenia after the Fontan operation. In the long term, these shunts are associated with hyperammonaemia and...
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.
Topics: Humans; Fontan Procedure; Portal Vein; Cardiovascular Abnormalities; Hepatic Encephalopathy
PubMed: 37042614
DOI: 10.1017/S1047951123000859 -
Journal of Pediatric Surgery Jun 2023Intestinal malrotation may be asymptomatic in patients with heterotaxy syndrome (HS), and whether these newborns benefit from prophylactic Ladd procedures is unknown....
PURPOSE
Intestinal malrotation may be asymptomatic in patients with heterotaxy syndrome (HS), and whether these newborns benefit from prophylactic Ladd procedures is unknown. This study sought to uncover nationwide outcomes of newborns with HS receiving Ladd procedures.
METHODS
Newborns with malrotation were identified from the Nationwide Readmission Database (2010-2014) and stratified into those with and without HS utilizing ICD-9CM codes for situs inversus (759.3), asplenia or polysplenia (759.0), and/or dextrocardia (746.87). Outcomes were analyzed using standard statistical tests.
RESULTS
4797 newborns with malrotation were identified, of which 16% had HS. Ladd procedures were performed in 70% overall and more common in those without heterotaxy (73% vs. 56% HS). Ladd procedures in newborns with heterotaxy were associated with higher complications compared to those without HS including surgical site reopening (8% vs. 1%), sepsis (9% vs. 2%), infections (19% vs. 11%), venous thrombosis (9% vs. 1%), and prolonged mechanical ventilation (39% vs. 22%), all p < 0.001. HS newborns were less frequently readmitted with bowel obstructions (0% vs. 4% without HS, p < 0.001) with no readmissions for volvulus in either group.
CONCLUSION
Ladd procedures in newborns with heterotaxy were associated with increased complications and cost without differences in rates of volvulus and bowel obstruction on readmission.
TYPE OF STUDY
Retrospective Comparative.
LEVEL OF EVIDENCE
III.
Topics: Humans; Infant, Newborn; Heterotaxy Syndrome; Intestinal Volvulus; Retrospective Studies; Intestinal Obstruction; Cardiovascular Abnormalities; Digestive System Abnormalities
PubMed: 36941169
DOI: 10.1016/j.jpedsurg.2023.02.013 -
Journal of the West African College of... 2023Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with...
Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with various congenital anomalies and non-syndromic (isolated anomaly). We present a case of syndromic BA associated with polysplenia and intestinal malrotation, discovered incidentally during the Kasai procedure. The small intestine was found to be non-rotated with the duodenojejunal junction to the right of the vertebral column. The presence of accessory spleens was noted. Kasai portoenterostomy and Ladd's procedure were performed. The patient had an uneventful postoperative course with the passage of cholic stool from the third postoperative day. At the seventh-month follow-up, the stool remained cholic. A multidisciplinary approach in the care of babies with BA and long-term follow-up is crucial for a successful outcome.
PubMed: 36923807
DOI: 10.4103/jwas.jwas_195_22 -
Cureus Feb 2023Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional...
Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date.
PubMed: 36909095
DOI: 10.7759/cureus.34689 -
Radiology Case Reports May 2023The heterotaxy syndrome is a type of syndrome that involves multiple visceral abnormalities, vascular ones and associated with left isomerism. Malformation of...
The heterotaxy syndrome is a type of syndrome that involves multiple visceral abnormalities, vascular ones and associated with left isomerism. Malformation of gastroenterologic system includes polysplenia (segmented spleen or multiple splenules), agenesis (partial or complete) of the dorsal pancreas and anomalous of the inferior vena cava implantation. Here, we describe and show the anatomy of a patient with left side inferior vena cava, situs ambiguous (complete common mesentery), polysplenia, and short pancreas. We also discuss about the embryologic process and the implications of these anomalies during gynecologic, digestive, and liver surgeries.
PubMed: 36895889
DOI: 10.1016/j.radcr.2023.02.005 -
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 -
Journal of Surgical Case Reports Dec 2022Ectopic splenic tissue can be classified as accessory spleen, polysplenia or a phenomenon termed as splenosis. Once believed a rare occurrence, the incidence of...
Ectopic splenic tissue can be classified as accessory spleen, polysplenia or a phenomenon termed as splenosis. Once believed a rare occurrence, the incidence of splenosis is now thought to be significantly higher. Generally, splenosis is asymptomatic and discovered incidentally during operation, imaging or at autopsy. The case presented herein describes an incidental finding of an intraabdominal splenosis, which was subsequently biopsied to investigate for peritoneal metastatic disease. The biopsied tissue subsequently caused significant post-operative haemorrhage. Past medical history and specific pre-operative imaging modalities for patients presenting with asymptomatic peritoneal or intra-abdominal nodules are discussed. Splenosis is highlighted as a common condition to consider prior to invasive investigations.
PubMed: 36589686
DOI: 10.1093/jscr/rjac540 -
Problemy Radiatsiinoi Medytsyny Ta... Dec 2022With limited time for analysing computed tomography (CT) data in medical emergencies, radiologists and clinicians are not always aware of congenital pathologies,...
With limited time for analysing computed tomography (CT) data in medical emergencies, radiologists and clinicians are not always aware of congenital pathologies, especially in asymptomatic cases. We present a case report of heterotaxy syndrome detected incidentally in a 62-year-old female with massive pulmonary embolism, local saccular aneurysm of the aortic arch and COVID-19. The presented case describes an unusual combination of life-threatening conditions and congenital anomalies. Using the CT angiography, we reported the preserved topography and morphology of the heart chambers. The superior and inferior venae cavae were located on the left, draining into a significantly dilated coronary sinus. Disordered abdominal organ arrangement included midline liver, right-sided stomach, the central location of the short pancreas, malrotation of the intestine, polysplenia, and atypical rise of visceral branches of the abdominal aorta. A comprehensive assessment of CT images promoted verification of undiagnosed congenital anomalies. Prompt recognition of heterotaxy syndrome in life-threatening conditions significantly impacts treatment tactics and should be the course of diagnostic and cautious interventions to reduce further complications.
Topics: Female; Humans; Middle Aged; Heterotaxy Syndrome; COVID-19; Pancreas; Aortic Aneurysm; Pulmonary Embolism
PubMed: 36582111
DOI: 10.33145/2304-8336-2022-27-495-503