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Radiology Case Reports Jul 2022Polysplenia Syndrome is a rare condition that refers to the presence of 2 or more spleens in association with other thoracoabdominal abnormalities. Here, we report a...
Polysplenia Syndrome is a rare condition that refers to the presence of 2 or more spleens in association with other thoracoabdominal abnormalities. Here, we report a case of a 13-year-old girl who presented with acute lower abdominal pain and was diagnosed with polysplenia syndrome after obtaining a CT scan of her chest, abdomen and pelvis. Diagnostic imaging also revealed the presence of a wandering spleen hanging in the lower abdomen and upper pelvic cavity and showing signs of infarction. The patient underwent splenectomy afterward and splenic torsion was confirmed intraoperatively. To the best of our knowledge, this was the first reported case of wandering spleen torsion in a patient with polysplenia syndrome. Physicians should keep in mind the possibility of a wandering spleen torsion presenting in various locations when dealing with polysplenia syndrome patients complaining of abdominal pain.
PubMed: 35570876
DOI: 10.1016/j.radcr.2022.04.009 -
Surgical Case Reports Feb 2019Polysplenia refers to the presence of two or more equal-sized spleens. Very rarely, one of the multiple spleens may develop torsion and infarction.
BACKGROUND
Polysplenia refers to the presence of two or more equal-sized spleens. Very rarely, one of the multiple spleens may develop torsion and infarction.
CASE PRESENTATION
A 21-year-old woman presented with left upper quadrant pain, the cause of which could not be diagnosed. She returned to our hospital, 2 days later, without any pain improvement. Enhanced computed tomography showed splenic infarction and polysplenia. Initially, we could not identify the cause of the infarction and started conservative therapy, which did not result in any improvement. Hence, we performed a splenectomy, after securing informed consent. Because the patient was a young woman, we opted for a laparoscopic approach. During surgery, we identified the cause of the infarction as spleen pedicle torsion; the infarcted spleen was excised using an automated suturing device. We completed the laparoscopic surgery without converting it to an open laparotomy, and the patient was discharged 4 days later. This was a rare case of polysplenia with splenic torsion.
CONCLUSION
Laparoscopic splenectomy is minimally invasive and has cosmetic advantages. Thus, this approach may be considered as a treatment option for this condition.
PubMed: 30778694
DOI: 10.1186/s40792-019-0582-0 -
Annals of Gastroenterology 2013
PubMed: 24714827
DOI: No ID Found -
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 -
Archives of Medical Science : AMS 2020
PubMed: 32864021
DOI: 10.5114/aoms.2020.97972 -
American Journal of Medical Genetics Oct 1988A 19-week gestation female fetus had multiple anomalies suggesting Meckel syndrome with polysplenia. In over 200 reported cases of Meckel syndrome, only 2 similar cases... (Review)
Review
A 19-week gestation female fetus had multiple anomalies suggesting Meckel syndrome with polysplenia. In over 200 reported cases of Meckel syndrome, only 2 similar cases are documented. Both were male infants, one with bilateral left-sidedness (polysplenia), the other with bilateral right-sidedness (M-anisosplenia). Meckel syndrome is a complex MCA (multiple congenital anomaly) syndrome. Thus, the finding of polysplenia is to be interpreted as another such midline anomaly, rather than as a causally independent malformation.
Topics: Abnormalities, Multiple; Female; Functional Laterality; Humans; Infant, Newborn; Male; Syndrome
PubMed: 3068988
DOI: 10.1002/ajmg.1320310212 -
Radiology Case Reports Sep 2019Polysplenia syndrome associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Polysplenia has been...
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.
PubMed: 31320964
DOI: 10.1016/j.radcr.2019.05.032 -
La Revue de Medecine Interne Jun 2018
Topics: Aged; Diaphragmatic Eventration; Heterotaxy Syndrome; Humans; Lung; Male; Radiography, Thoracic; Thoracic Diseases; Wandering Spleen
PubMed: 28277264
DOI: 10.1016/j.revmed.2016.09.008 -
JOP : Journal of the Pancreas Jul 2007Agenesis of the dorsal pancreas is very rare and may be associated with other congenital disease states. It has a rare association with polysplenia/heterotaxy syndrome.... (Review)
Review
CONTEXT
Agenesis of the dorsal pancreas is very rare and may be associated with other congenital disease states. It has a rare association with polysplenia/heterotaxy syndrome. Most commonly, these states occur due to errors in development of the asymmetric organs and may be associated with benign to severe congenital cardiac malformations.
CASE REPORT
We report a case of a 25-year-old male with known coarctation of the aorta who was otherwise asymptomatic. Following a routine cardiac examination, he was incidentally discovered to have an absent body and tail of the pancreas on imaging. Further testing demonstrated findings consistent with a diagnosis of polysplenia/heterotaxy syndrome with agenesis of the dorsal pancreas.
CONCLUSIONS
In patients with congenital heart disease, there is increased likelihood for the presence of other congenital malformations. In particular, polysplenia/heterotaxy syndrome, while very rare, has been shown to be associated with cardiac abnormalities. The importance in diagnosis lies in the potential clinical consequences of polysplenia/heterotaxy syndrome and agenesis of the dorsal pancreas, including late-onset diabetes mellitus, pancreatitis, and intestinal volvulus.
Topics: Abnormalities, Multiple; Adult; Aortic Coarctation; Humans; Male; Pancreas; Radiography; Spleen; Syndrome
PubMed: 17625295
DOI: No ID Found -
Congenital Heart Disease 2011Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially... (Review)
Review
Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review.
OBJECTIVE
Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies.
DESIGN
Retrospective cohort study and literature review were used.
RESULTS
Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right-to-left shunting.
CONCLUSIONS
PSVC and other abdominal venous anomalies may be clinically important but under-recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis-associated pulmonary arteriovenous malformations.
Topics: Abnormalities, Multiple; Arteriovenous Malformations; Boston; Fontan Procedure; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans; Phlebography; Portal Vein; Pulmonary Artery; Pulmonary Veins; Renal Veins; Retrospective Studies; Spleen; Treatment Outcome; Vascular Malformations; Vena Cava, Inferior
PubMed: 21269410
DOI: 10.1111/j.1747-0803.2010.00478.x