Polysplenia syndrome with situs ambiguous, common mesentery, and IVC interruption discovered incidentally in an adult

Abstract

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.

A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study

“…The splenic tissue was divided into one large spleen subdivided into two distinct lobes, a smaller, more rounded spleen, situated inferior from the first, and several smaller spleens situated among the greater curvature of the stomach and on the anterior wall ( Table 1 ). The topography of the multiple spleens found in our case is consistent with polysplenia syndrome [ 15 , 23 , 24 ]. The macroscopic aspect of the spleens is of normal morphology and they did not present a health issue and did not contribute to the thanatogenesis in our case.…”

Wandering spleen torsion in a patient with polysplenia syndrome

“…PSS was first described by Helwig in 1929. It is an extremely rare syndrome, with a reported incidence of just 1 per 250,000 live births [2] . In addition to having 2 or more spleens, PSS patients frequently have cardiac, pulmonary, gastrointestinal, and gentiourinary anomalies [5] .…”

“…Heterotaxy is a very rare condition characterized by the abnormal arrangement of the thoracoabdominal organs resulting from a disruption in the normal left-right axis during early embryonic development [1] . Polysplenia syndrome (PSS) is a subtype of heterotaxy and refers to the presence of two or more spleens in association with other intra-abdominal and intra-thoracic abnormalities [2] . A wandering spleen is also a rare condition caused by an absence or underdevelopment of one or more of the ligaments that hold the spleen in its normal anatomical position in the left upper quadrant of the abdomen, increasing the risk of splenic torsion [3] .…”

Un caso raro de <i>situs</i> ambiguo con poliesplenia y pulmones trilobulados en una mujer anciana

“…Dentro de la literatura los casos reportados en la etapa adulta encontrados fueron: mujer de 23 años de forma incidental, mujer de 31 años, hombre de 65 con dolor abdominal y mujer de 67 años con síndrome aórtico agudo por Corral et al 14 , mujer de 28 años con obstrucción intestinal en el caso de Ali et al 15 , y hombre de 56 años con anemia y dolor abdominal generalizado por Mortaji et al 16 . Siendo así nuestro caso en particular interesante por la edad de la paciente, ya que como se mencionó el diagnóstico por imagen se integró a la edad de 93 años.…”