WS is an uncommon clinical finding wherein the spleen is located at a position other than its usual anatomical position. In addition, it may be attributed to the absence or laxity of the supporting structures which could be either congenital or acquired [3, 4]. Although WS can be diagnosed in all age groups, it is 10 times more common among women in adulthood and is seen more often in women of reproductive age.5 Patients with WS may be asymptomatic, and the condition is only detected as a mobile mass during physical examination or on radiological studies carried out for other irrelevant reasons [1, 6]. Although the majority of the cases are asymptomatic, torsion of a WS is seen in approximately 0.2–0.3% of splenectomies[3]. Further, correct preoperative diagnosis is complex because the condition can be confused with other acute abdominal conditions such as acute appendicitis, ovarian torsion, or cholecystitis[3]. Furthermore, various imaging modalities can be used to detect a suspected WS. Among these imaging modalities, ultrasonographic evaluation is an inexpensive and noninvasive method used to characterize a WS with ischemia in an emergency room setting [7]. Additional contrast-enhanced CT would further complement the diagnosis by demonstrating signs indicating splenic pedicle torsion [8]. In this study, CT images of the patient showed an intraparenchymal hypodense area with poor contrast enhancement, leaving a residual rim of enhancing capsule supplied by small capsular vessels (rim sign), which is suggestive of parenchymal infarction [9].
The exact cause of the spleen drifting from its usual position in our patient remained uncertain. The patient did not have a family history of WS, nor did she have specific laboratory or clinical findings to conclude the diagnosis. ARS has been associated with abdominal wall anomalies from redundant umbilical skin, umbilical hernia, or omphalocele [1, 2]. Several cases with a WS accompanied with omphalocele have been reported previously [10, 11]. In addition, herniation of intra-abdominal organs through the abdominal wall defect may cause rotation anomaly of the stomach and the spleen or an inadequate fusion of the peritoneum. Because duplication of the peritoneum makes up ligaments that suspend the spleen, failure of this embryological process may later contribute to irregular ligamental development. Meanwhile, an absence of ligamentous attachment would eventually result in a WS and torsion around its pedicles. Moreover, mutations in the transcription factors PITX2 and FOXC1 are associated with the pathogenesis of ARS [1, 2, 12]; earlier studies have reported that PITX2 is necessary in order to induce cellular behavior within the dorsal mesentery for leftward tilt during the gut morphogenesis [13]. Therefore, PITX2 mutation linked to ARS can promote gut malrotation and in turn, abnormal ligamentous development.
In patients presenting with an acute abdominal pain, detorsion and splenopexy would be feasible surgical options when there are no signs of infarction, hypersplenism, or thrombosis [3]. Further, results of splenopexy have been reported to be positive in preventing complications of WS while preserving the splenic function [4, 6]. For this case, it did not regain its normal vascularity after detorsion; therefore, laparoscopic splenectomy was performed. This validates any delay in diagnosis that can lead to severe consequences. Hence, laparoscopic splenectomy would be an optimal method for WS because it is free from attachments and other organs. Nonetheless, partial splenectomy of the totally strangulated spleen is generally impractical because the torsion involves the vascular pedicle, although a partial splenectomy with splenopexy for a partial infarcted WS has been reported in the literature [14].