Torsion of a wandering spleen with infarction and portal vein thrombosis

A 26-year-old male presented to emergency department with a 15-day history of abdominal pain intensified in the last hours. Physical examination revealed abdominal involuntary guarding, and fever. Laboratory tests disclosed high fibrinogen and C-reactive protein levels (685mg/dL, 79.6mg/dL). An ultrasound examination was performed.

Abdominal ultrasound revealed a capsulated “comma-shape” solid mass of 18x10x14 cm in the right lumbar-iliac region (Fig.1a). Absence of spleen in its usual location was detected, so the mass was considered to be the enlarged spleen. Torsion of its vascular pedicle,  with a thrombosis of the splenic vein, was seen (Fig. 1b). The right portal vein was thrombosed (Fig. 1c). There was a small amount of freed fluid in pelvis.

An abdominal CT was performed confirming the abnormal location of the spleen and the whorled appearance of the twisted splenic pedicle (1800 degree) and the pancreatic tail, with a thrombosis of the splenic and collateral veins. Secondary, the spleen demonstrated heterogeneous enhancement with a marked lower hypo density area of infarction. Abnormal hepatic perfusion secondary to right portal vein thrombosis/embolism was detected. (Fig. 2 to 6).

All these findings were consistent with a torsion of a wandering spleen and splenic vein thrombosis with partial infarction and secondary involvement of the liver. A splenectomy was performed. (Fig. 7).

Wandering spleen is a very uncommon clinical entity in which the spleen is not found in its usual location or orientation, but moves to any part of the abdomen or the pelvis [1].

The origin of this disorder is an abnormal laxity or absence of the supporting splenic ligaments. It may be congenital or acquired, due to conditions like splenomegaly or pregnancy [2].

Secondary to ligamentous laxity, the spleen is attached by a long pedicle containing the splenic vessels and, often, the pancreatic tail, and consequently, the spleen is prone to torsion and infarction [1].

Clinical diagnosis of wandering spleen is difficult due to its rarity and nonspecific presenting symptoms. It is usually asymptomatic, but if a torsion exists, patients will suffer from abdominal pain, which can be chronic (mild torsion), intermittent (due to torsion-detorsion) or acute abdomen (due to severe torsion and splenic infarction) [2].

That's why radiologists play an essential role in its diagnosis.

CT and ultrasonography techniques can detect the absence of the spleen in its normal position and the presence of a solid spleen-like mass located somewhere else in the abdomen or pelvis, which are the clues to the diagnosis [3]. However, contrast-enhanced CT is considered as the best diagnostic imaging technique, especially in the detection of complications [1, 3, 4]. MRI can be used as an alternative to CT, but it’s unusually performed [2].

If splenic torsion exists, the whorled appearance of the twisted pedicle by CT is a specific sign [3].

The torsion may result in splenic vein thrombosis, splenic infarction or both [1], as in our patient, which also showed thrombosis of the collateral veins that had previously developed. The spleen may also be enlarged due to accompanying congestion [2].

Splenic infarction is represented by a wedge-shaped non-enhancing hypodensities (partial) or a total non-enhanced spleen, with a capsular rim enhancement [1, 3, 4].

Free abdominal fluid and marked stranding of the perihilar fat may be seen [3].

Associated portal venous thrombosis has been reported, too, as in our case [4, 5]. Other rare complications are related to the injury to adjoining organs secondary to torsion (gastric volvulus, gastrointestinal obstruction, celiac axis occlusion, acute pancreatitis, pancreatic necrosis) [4].

Treatment is usually surgical with splenopexy or splenectomy, depending on the degree of torsion and spleen viability [1, 2, 3, 4]. In the current case, splenectomy was required because of the development of splenic ischemia, with excellent results.

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