Polysplenia syndrome in adulthood: a case report

Abstract

Polysplenia syndrome mainly described in pediatrics; rarely and incidentally in adulthood. Most patients had their diagnosis done during childhood due to the frequent association to cardiac anomalies that speak for themselves earlier in life. Multiple spleens, cardiac defect and vascular malformation of the inferior vena cava with azygos or hemiazygos continuation are the most frequent observed malformations. Our patient was one this rarest adulthood incidental diagnosis, who presented in the emergency department for nephritic colic, and while imaging for this, multiples spleens and other visceral malformations were diagnosed. Hopefully, cardiac ultrasound hadn't showed any cardiac malformation and the patient was discharged aware of this condition. Through this publication we report the possible incidental diagnosis of polysplenia condition and highlight the fact that people with such important malformation can lead a normal life, and only awareness should be given for future surgeries, instrumental treatment or else.

Adult intestinal malrotation presenting as caecal volvulus with incidental findings of duplicate inferior vena cava and other rare abnormalities: case report

“…IVC abnormalities and intestinal malrotation are known abnormalities associated with polysplenia syndrome [ 14 ]. However, polysplenia is rare in adulthood as most cases also have severe congenital cardiac malformations, which result in 75% mortality by 5 years old [ 15 ]. Our case had no known cardiac malformations and had not required cardiac interventions as a child.…”

Polysplenia with situs inversus totalis, azygos continuation of the inferior vena cava, and duplication of the superior vena cava in a healthy adult: A case report

“…PS occurs in approximately 1 in 40,000 live births and characteristically presents with 2 or more splenules in the left upper quadrant, instead of a single large spleen [ 7 , 8 ]. Another anatomic abnormality almost as commonly associated with PS is azygos interruption of the abdominal inferior vena cava in the thorax [ 9 ]. Several other anatomic features that are variably associated with PS include dextrocardia, abnormal pulmonary and portal venous return, various congenital heart diseases, bilateral bilobed lungs, midgut malrotation, dorsal pancreatic agenesis, gallbladder agenesis or atresia, and a large midline liver [ 6 ].…”

“…Likewise, upper GI series can rule-out malrotation of the intestines, which predisposes patients to volvulus. Patients also commonly receive contrast-enhanced CT imaging, which provides information about vascular anatomy [ 6 , 9 ]. This was particularly useful in our patient, who received a CT pulmonary angiogram, since it highlighted his unique vascular anatomy, including the suspected hepatic vein drainage into the right atrium.…”