Wandering accessory paracervical spleen undergoing torsion in pregnancy: A challenging diagnosis and management

Accessory spleen (AS) is a congenital anomaly arising from incomplete fusion of splenic tissue buds, with an incidence of approximately 10% in the general population.1 Accessory spleens are usually located at the splenic hilum or pancreatic tail; ectopic pelvic sites are extremely uncommon. Wandering spleen, in contrast, results from laxity or absence of splenic ligaments and predisposes to torsion. An accessory wandering spleen, in which an ectopic accessory spleen is supplied by a long pedicle that permits abnormal mobility, represents an exceptional combination of both conditions. Its occurrence during pregnancy is extraordinarily rare. Pregnancy might predispose to torsion through hormonal and mechanical factors that increase ligamentous laxity and alter abdominal organ positioning.2 Clinical manifestations are nonspecific, making diagnosis difficult and often delayed until complications occur. While cases of wandering spleen in pregnancy have been described,3 only one prior report has detailed an accessory wandering spleen in the pelvis during gestation.4 We present the first case of torsion of an accessory wandering spleen diagnosed in a primigravida and treated with laparoscopic splenectomy in the third trimester. We emphasize the diagnostic role of multimodal imaging, review the literature, and discuss both surgical and obstetric considerations. A 25-year-old asymptomatic primigravida was referred at 8 weeks of amenorrhea for a paracervical mass. Tumor markers were within normal limits. The patient's medical history was unremarkable, with no prior surgery or abdominal trauma. Pelvic transvaginal and transabdominal ultrasound confirmed an intrauterine pregnancy and revealed a solid, vascularized mass in the Douglas pouch, measuring 55 × 40 × 55 mm (Figure 1a). Pressure from the probe allowed the mass to be moved away from the uterus, adnexa, and rectum. Color Doppler imaging identified a branched vascular axis originating from the left upper abdomen, although the exact source of the vascular pedicle could not be determined (Figure 1b). Subsequent follow-up evaluations showed the mass stable in size and echogenicity. At 19 weeks of gestation, an abdominal MRI without contrast medium was performed, which confirmed the diagnosis of a paracervical accessory wandering spleen. The MRI allowed the visualization of a long vascular pedicle originating from the splenic hilum, traversing the intestinal loops to supply the mass (Figure 1c,d). At 33 weeks, the patient presented with acute onset pelvic pain radiating to the perineal area. A non-contrast computed tomography scan confirmed splenic torsion and ischemia. At urgent diagnostic laparoscopy, a long twisted vascular peduncle running along the omentum and vascularizing an ischemic spleen was observed. A splenectomy was performed (Figure S1a,b), and the patient recovered uneventfully, delivering vaginally at term without complications. This study has received approval from the Institutional Review Board (IRB Approval No. 3629CESC). Additionally, the patient provided written and oral informed consent for the use of anonymized clinical data for research purposes. Accessory wandering spleen is a particularly rare clinical entity, combining features of accessory spleen and wandering spleen. Only one previous case has been reported in pregnancy,4 and to our knowledge, none have required surgical management in the third trimester (Table S1). Our case is novel in several respects: (1) accessory wandering spleen rather than the main wandering spleen, with paracervical location confirmed by MRI; (2) early incidental detection in the first trimester, before complications occurred; and (3) successful laparoscopic splenectomy in the third trimester without need for cesarean section, followed by term vaginal delivery. In contrast, most previously published cases of wandering spleen in pregnancy involved the main spleen, were diagnosed later, and often required urgent laparotomy or cesarean delivery due to complications.5, 6 Our experience therefore broadens the spectrum of presentations and shows that minimally invasive management is feasible even at advanced gestation. Management depends on viability of the spleen. Splenectomy is indicated in infarction, as in our case, whereas splenopexy might be considered if splenic tissue remains viable, particularly in younger patients.7 In pregnancy, however, splenectomy is more commonly performed given the acute presentation and risk of recurrence. From an obstetric perspective, the timing of surgery is critical: intervention too early might expose the fetus to risk of iatrogenic prematurity, while delaying treatment risks rupture or hemodynamic compromise. Our decision to perform laparoscopic splenectomy at 33 weeks was based on acute torsion with maternal pain, but the absence of obstetric indications for cesarean allowed us to avoid preterm birth. Multidisciplinary collaboration and intraoperative fetal monitoring were key to achieving a favorable maternal and neonatal outcome. Torsion of an accessory wandering spleen in pregnancy is an extremely rare condition that might mimic adnexal or pelvic pathology. Multimodal imaging, particularly MRI, is valuable for early and accurate diagnosis. This case demonstrates that laparoscopic splenectomy is feasible and safe in the third trimester, without requiring concomitant cesarean section, and that conservative obstetric management can allow pregnancy to continue to term. Conceptualization: Camilla Caiaffa, Mariachiara Bosco, Stefano Uccella; Data curation: Mariachiara Bosco, Camilla Caiaffa; Writing—Original draft preparation: Camilla Caiaffa; Supervision: Stefano Uccella, Mariachiara Bosco; Validation: Jacopo Weindelmayer, Simone Giacopuzzi, Simone Garzon; Writing—Reviewing and Editing: Mariachiara Bosco, Stefano Uccella, Simone Garzon, Eleonora Pedrazzoli. The authors have nothing to report. The authors declare no conflicts of interest. Data sharing is not applicable to this article as no new data were created or analyzed in this study. Figure S1. (a) A long and twisted peduncle (black arrow) is visible as well as an infarcted mass (ectopic spleen). (b) Laparoscopy: the prolapsed accessory spleen is visible in the pelvis behind the uterus. Table S1. Cases of wandering spleen in pregnancy and postpartum. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.