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Abdominal Imaging 1999To present the computed tomographic (CT) features of the abdominal anomalies consistent with polysplenia syndrome in adults. Awareness of these abnormalities may avoid... (Review)
Review
BACKGROUND
To present the computed tomographic (CT) features of the abdominal anomalies consistent with polysplenia syndrome in adults. Awareness of these abnormalities may avoid misdiagnosing characteristic findings as separate pathological processes.
METHODS
Imaging studies, mainly abdominal CT scans, of eight patients were reviewed. Attention was directed to the location of the multiple spleens, stomach, and liver and to the possible presence of a short pancreas, malrotation of the intestine, and venous anomalies. We also reviewed the CT findings of 15 adult patients described in the literature.
RESULTS
Three men and five women underwent CT for various unrelated conditions. The most common findings were multiple spleens along the greater curvature of the stomach, which were located in the right upper quadrant in six patients. The inferior vena cava was seen on the left side in seven subjects, with azygos/hemiazygos continuation in six. A preduodenal portal vein was present in seven subjects. The liver was in the midline in four patients and on the left side in two. A short pancreas was seen in four patients, intestinal nonrotation in five, and dextrocardia in two. The prevalence of these anomalies was similar to that of the reviewed cases.
CONCLUSIONS
CT proved to be an excellent imaging modality in the diagnosis of the abdominal anomalies. Some of these (a short pancreas, multiple spleens, and azygos continuation) can simulate pathological processes. Hence the importance of recognizing these CT findings as part of a syndrome.
Topics: Abnormalities, Multiple; Adult; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Spleen; Syndrome; Tomography, X-Ray Computed
PubMed: 10024407
DOI: 10.1007/s002619900471 -
Journal of Radiology Case Reports Jul 2015The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs... (Review)
Review
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.
Topics: Adult; Aorta, Thoracic; Bronchi; Hepatic Artery; Heterotaxy Syndrome; Humans; Iliac Vein; Kidney Calculi; Lung; Male; Radiography; Renal Artery; Renal Veins; Subclavian Artery; Vena Cava, Inferior
PubMed: 26629295
DOI: 10.3941/jrcr.v9i7.2210 -
Global Pediatric Health 2022Polysplenia syndrome is an uncommon condition associating several splenic nodules (sometimes polylobed spleen and cases of normal spleen have been described) with a...
Polysplenia syndrome is an uncommon condition associating several splenic nodules (sometimes polylobed spleen and cases of normal spleen have been described) with a number of malformations that appear between the fourth and sixth week of embryonic development. Although it has been suggested that genetic, teratogenic, and embryogenic factors may be at fault, the exact etiology remains unclear. Clinically, it is generally asymptomatic or mildly symptomatic. The authors report the case of an 11-months-old infant from a poorly monitored pregnancy. He was admitted to the emergency room for respiratory discomfort in a context of apyrexia. A thoraco-abdominal CT scanner revealed a polysplenia syndrome.
PubMed: 36189184
DOI: 10.1177/2333794X221127640 -
Clinical Imaging Feb 2022Deep venous thrombosis is a hitherto under-recognized complication occurring in patients with polysplenia syndrome, despite the high prevalence of venous anomalies such...
Deep venous thrombosis is a hitherto under-recognized complication occurring in patients with polysplenia syndrome, despite the high prevalence of venous anomalies such as interrupted inferior vena cava (IVC) with azygos/hemiazygos continuation. Here we report the first case of concurrent polysplenia (as evidenced by interrupted IVC with azygos/hemiazygos continuation, multiple left-sided spleens, bowel malrotation with inverted mesenteric veins, preduodenal portal vein, and pancreatic hypoplasia/partial agenesis of the dorsal pancreas) and sickle cell trait, complicated by extensive deep venous thrombosis refractory to medical and interventional radiologic management.
Topics: Heterotaxy Syndrome; Humans; Sickle Cell Trait; Thrombophilia; Vena Cava, Inferior; Venous Thrombosis
PubMed: 34813990
DOI: 10.1016/j.clinimag.2021.11.017 -
Clinical Medicine Insights. Cardiology 2022Heterotaxy syndrome is a rare congenital heart disease with a disarrangement of the heart and abdominal organs. We present a young African female with features of heart...
Heterotaxy syndrome is a rare congenital heart disease with a disarrangement of the heart and abdominal organs. We present a young African female with features of heart failure, diffuse irregular cardiac murmurs, and palpable, tender epigastric mass. A chest and abdominal computed tomography (CT) identified heterotaxy syndrome with left isomerism and fused adrenal glands. This case highlights the feature of fused adrenal glands in a patient with polysplenia.
PubMed: 36187466
DOI: 10.1177/11795468221116851 -
Internal Medicine (Tokyo, Japan) 2016
Topics: Arrhythmia, Sinus; Dextrocardia; Female; Heterotaxy Syndrome; Humans; Pacemaker, Artificial; Radiography; Syncope
PubMed: 26935387
DOI: 10.2169/internalmedicine.55.5840 -
Journal of Computer Assisted Tomography Feb 1981A case of polysplenia associated with total abdominal situs inversus, central localization of the liver, and incomplete development of the inferior vena cava is...
A case of polysplenia associated with total abdominal situs inversus, central localization of the liver, and incomplete development of the inferior vena cava is reported. Various components of the syndrome were easily demonstrated by CT.
Topics: Abnormalities, Multiple; Humans; Male; Middle Aged; Spleen; Tomography, X-Ray Computed
PubMed: 7240483
DOI: 10.1097/00004728-198102000-00020 -
Journal of the Belgian Society of... Oct 2019What to look for in case of polysplenia and/or unusual disposition of several intra-abdominal organs.
What to look for in case of polysplenia and/or unusual disposition of several intra-abdominal organs.
PubMed: 31646265
DOI: 10.5334/jbsr.1903 -
The Indian Journal of Surgery Apr 2014Preduodenal portal vein (PDPV) is a rare developmental anomaly. In infants, this is often associated with duodenal obstruction or biliary atresia. It is generally... (Review)
Review
Preduodenal portal vein (PDPV) is a rare developmental anomaly. In infants, this is often associated with duodenal obstruction or biliary atresia. It is generally asymptomatic in adults (Ooshima et al., Hepato-Biliary-Pancreat Surg 5(4):455-458, 1998). Here, we report a singular case of adult PDPV that was discovered accidentally during emergency laparotomy for peritonitis due to gastric perforation in a 38-year-old lady. A plethora of congenital anomalies was uncovered, which is consistent with the reported cases of classical polysplenia syndrome, viz., multiple spleens of equal volume, visceral heterotaxia, right (Rt.)-sided stomach, a left (Lt.)-sided or a large midline liver, malrotation of intestine, a short pancreas, PDPV and IVC abnormalities (Gayer et al., Abdom Imaging 24:178-184, 1999). In addition, abnormalities like anamolous origin of Lt. gastric and splenic arteries from the abdominal aorta with absent celiac trunk, hepatic artery arising from the superior mesenteric artery, hepatic veins draining directly to Rt. atrium, etc. along with hypertrophic and lipomatous interatrial septum have also been detected during further investigations. As of now, we have come across 29 cases of adult PDPV reported in world literature and we are reporting our unique case with a review of literature on anomalies of visceral organs associated with PDPV.
PubMed: 24891779
DOI: 10.1007/s12262-013-0812-9 -
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