title: Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies.
abstract: Polysplenia syndrome is classified as one of the situs ambiguous or heterotaxy syndromes and is classically termed left isomerism or bilateral left-sidedness. However, polysplenia is a controversial and complex entity with no fixed pathognomonic features but rather a broad spectrum of abnormalities. In many published case series, polysplenia was neither associated with viscero-atrial heterotaxy nor with duplication of left-sided structures. The relationship between polysplenia and viscero-atrial situs is not clear. Several recent case reports describe the association of polysplenia with situs inversus totalis or with pancreatic; venous and other anomalies or with several types of malignancy. This article provides the reader with a review of the literature as well as our own experience aiming at better understanding of the polysplenia syndrome, its relationship with viscero-atrial situs and the spectrum of associated extra-cardiac anomalies.
authors.affiliation: Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University, Mansoura, 35112, Egypt, ahm_m_tawfik@hotmail.com.
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: Tawfik
authors.forename: Nihal M
authors.initials: NM
authors.lastname: Batouty
authors.forename: Mona M
authors.initials: MM
authors.lastname: Zaky
authors.forename: Mohamed A
authors.initials: MA
authors.lastname: Eladalany
authors.forename: Ali H
authors.initials: AH
authors.lastname: Elmokadem
mesh_terms: D004065:Digestive System Abnormalities; D005260:Female; D059446:Heterotaxy Syndrome / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D045822:Intestinal Volvulus / Q000151:congenital / Q000209:etiology; D008875:Middle Aged; D010179:Pancreas / Q000002:abnormalities; D013154:Spleen / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed