-
Gastrointestinal Radiology 1991The report of a 29-year-old woman with polysplenia syndrome, Crohn's disease, and bilateral cataracts is presented. The patient was noted to have a right-sided stomach... (Review)
Review
The report of a 29-year-old woman with polysplenia syndrome, Crohn's disease, and bilateral cataracts is presented. The patient was noted to have a right-sided stomach and small bowel, Crohn's ileitis, and a left-sided colon. Results of roentgenography of the chest and echocardiography were consistent with a diagnosis of hypoplasia of the inferior vena cava with azygos continuation. The patient underwent laparotomy with cholecystectomy, exploration of the common bile duct, and choledochoscopy for cholelithiasis, choledocholithiasis, and chronic cholecystitis. Laparotomy revealed a liver that had two lobes, each with the morphologic appearance of the left lobe. The gallbladder was centrally located. T-tube cholangiography revealed a quadruplication of the intrahepatic biliary ducts. To our knowledge, this patient is the only known adult with this syndrome in whom cholangiography demonstrated isomerism of the biliary tree. A review of the literature on this subject is given with emphasis on biliary anomalies.
Topics: Abnormalities, Multiple; Adult; Biliary Tract; Female; Humans; Intestines; Liver; Radiography; Spleen; Syndrome
PubMed: 2016033
DOI: 10.1007/BF01887336 -
Surgical and Radiologic Anatomy : SRA Apr 2021Heterotaxy syndrome with polysplenia is an extremely rare congenital disorder caused by a disruption in the embryonic development that results in an abnormal arrangement...
Heterotaxy syndrome with polysplenia is an extremely rare congenital disorder caused by a disruption in the embryonic development that results in an abnormal arrangement of the abdominal and thoracic organs. We present the case of a 59-year-old female patient with invasive ductal carcinoma of the right breast (luminal A type) and CT findings of heterotaxy syndrome with polysplenia. The most remarkable anomalies identified were a left inferior vena cava draining into the hemiazygos vein, absent inferior vena cava at the thoracic level, and hepatic veins directly draining into the right atrium. Moreover, an atrial septal defect was identified, explaining the pulmonary hypertension of unknown cause previously detected in the patient. The relevance of this case lies in the unusual anatomical abnormalities found and the large patient survival, having in to account the great rate of heterotaxy syndrome mortality in the first years of life.
Topics: Bone Neoplasms; Breast Neoplasms; Carcinoma, Ductal, Breast; Chemoradiotherapy, Adjuvant; Contrast Media; Female; Heterotaxy Syndrome; Humans; Incidental Findings; Liver Neoplasms; Mastectomy, Segmental; Middle Aged; Spleen; Time Factors; Tomography, X-Ray Computed
PubMed: 32995936
DOI: 10.1007/s00276-020-02586-5 -
Indian Pediatrics Mar 1991
Topics: Abnormalities, Multiple; Child, Preschool; Humans; Male; Meckel Diverticulum; Spleen; Syndrome; Thalassemia
PubMed: 1937710
DOI: No ID Found -
Pediatrics International : Official... Feb 2019
Topics: Child; Female; Heterotaxy Syndrome; Humans; Spleen; Tomography, X-Ray Computed; Torsion Abnormality
PubMed: 30809877
DOI: 10.1111/ped.13760 -
Digestive and Liver Disease : Official... Oct 2005A rare case of double cancer with situs ambiguus with polysplenia is presented. A 58-year-old patient was initially diagnosed with an early gastric cancer. On... (Review)
Review
A rare case of double cancer with situs ambiguus with polysplenia is presented. A 58-year-old patient was initially diagnosed with an early gastric cancer. On evaluation, the computed tomography of the abdomen demonstrated situs ambiguus with polysplenia. We performed a subtotal gastrectomy with the stomach being reconstructed in a Billroth-II fashion. Three months after the operation, he again visited our department complaining nausea and dysphagia. Examinations confirmed the other oesophageal malignancy with advanced stage. Because of unfamiliarity to situs anomaly and rarity of double cancer, we missed the other coexistent cancer. This is the first case presentation of a double carcinoma occurring in a patient with situs ambiguus with polysplenia. The literature is reviewed and the importance of preoperative evaluation is discussed.
Topics: Adenocarcinoma; Biomarkers, Tumor; CA-19-9 Antigen; Carcinoembryonic Antigen; Endoscopy, Gastrointestinal; Esophageal Neoplasms; Gastrectomy; Gastroenterostomy; Humans; Male; Middle Aged; Neoplasm Staging; Neoplasms, Multiple Primary; Preoperative Care; Situs Inversus; Spleen; Splenic Diseases; Stomach Neoplasms; Tomography, X-Ray Computed
PubMed: 16023901
DOI: 10.1016/j.dld.2005.02.013 -
Clinical Case Reports May 2020Polysplenia is heterotaxy syndrome or bilateral left-sidedness. We report a case of polysplenia syndrome in order to draw attention to this rare syndrome that must be...
Polysplenia is heterotaxy syndrome or bilateral left-sidedness. We report a case of polysplenia syndrome in order to draw attention to this rare syndrome that must be excluded in an infant presenting with congenital heart disease and intestinal malformations.
PubMed: 32477531
DOI: 10.1002/ccr3.2768 -
Surgical and Radiologic Anatomy : SRA Mar 2012Anatomical anomalies of visceral organs associated with gastric cancer are extremely rare. Here, we report a case of preduodenal portal vein (PDPV), intestinal... (Review)
Review
PURPOSE
Anatomical anomalies of visceral organs associated with gastric cancer are extremely rare. Here, we report a case of preduodenal portal vein (PDPV), intestinal malrotation, interruption of the inferior vena cava (IVC), and polysplenia associated with gastric cancer in an adult patient, together with a review of the literature on the anomalies of visceral organs associated with gastric cancer.
METHODS
We describe the diagnosis and surgical treatment in a 63 year-old man who had a preoperative diagnosis of PDPV, intestinal malrotation, interruption of the IVC with azygos continuation, and polysplenia associated with gastric cancer. Fifteen reports, in the English literature up to 2011, on visceral organ anomalies detected in gastric cancer patients were identified by searching Medline.
RESULTS
All of the 15 cases of anomalies associated with gastric cancer, including the present case, were correctly diagnosed by preoperative imaging. Situs anomaly was the most frequent anatomical anomaly detected, and PDPV was observed in only four cases. In 12 cases, gastrectomy was performed, and gastrojejunostomy was done in 1 case.
CONCLUSIONS
Although embryological anomalies such as PDPV, intestinal malrotation, interruption of the IVC, and polysplenia are rarely encountered in abdominal surgery, surgeons must be aware of their possible existence and be able to recognize them to avoid major intraoperative injuries.
Topics: Abnormalities, Multiple; Adenocarcinoma; Contrast Media; Digestive System Abnormalities; Follow-Up Studies; Gastrectomy; Humans; Incidental Findings; Lymph Node Excision; Male; Middle Aged; Neoplasm Staging; Portal Vein; Risk Assessment; Spleen; Stomach Neoplasms; Tomography, X-Ray Computed; Treatment Outcome; Vena Cava, Inferior
PubMed: 22080108
DOI: 10.1007/s00276-011-0894-7 -
AJR. American Journal of Roentgenology Oct 1992
Topics: Adolescent; Arteriovenous Malformations; Choristoma; Humans; Male; Radiography; Spleen; Stomach; Stomach Neoplasms
PubMed: 1529867
DOI: 10.2214/ajr.159.4.1529867 -
Biology of the Neonate 2005Heterotaxy results from failure of the developing embryo to establish normal left-right asymmetry. Typical manifestations include abnormal symmetry and malposition of... (Review)
Review
Heterotaxy results from failure of the developing embryo to establish normal left-right asymmetry. Typical manifestations include abnormal symmetry and malposition of the thoraco-abdominal organs and vessels, complex congenital heart disease and extracardiac defects involving midline-associated structures. The spleen is almost always affected, and there is syndromic clustering of the malformations corresponding to the type of splenic abnormality present. This review outlines the embryologic and genetic background of the heterotaxy syndrome as well as the characteristic anatomic features, clinical manifestations, and diagnostic clues of its two main presentations with asplenia or polysplenia.
Topics: Activin Receptors, Type II; Animals; Electrocardiography; Heart Defects, Congenital; Humans; Infant, Newborn; Lung; Mice; Situs Inversus; Spleen; Syndrome; Viscera
PubMed: 16113522
DOI: 10.1159/000087625 -
La Revue de Medecine Interne Sep 2021
Topics: Abdomen; Humans; Spleen
PubMed: 33446395
DOI: 10.1016/j.revmed.2020.12.003