Splenic infarction following torsion of wandering spleen involving pancreatic tail successfully managed in resource limited setting: a case report

Abstract

Introduction and importance

Wandering spleen is a rare condition characterized by the extreme laxity or absence of ligaments that normally secure the organ in its anatomical position in the left upper quadrant. Torsion of a wandering spleen is an uncommon cause of acute abdominal pain and is rarely diagnosed preoperatively. Torsion involving the distal pancreas is even more unusual. Although patient presentations can be atypical, early diagnosis and prompt intervention can lead to successful outcomes.

Case presentation

A 40-year-old Black female patient of Amhara ethnicity, from Ethiopia, presented with dull, aching abdominal pain and episodes of vomiting. After initial physiological stabilization and radiologic evaluation, laparotomy was performed, revealing torsion of a wandering spleen with involvement of the pancreatic tail, accompanied by adhesions of the small bowel and omentum. Following the release of adhesions, a splenectomy was performed. The patient was discharged in stable condition and continued to do well during follow-up visits.

Conclusion

Despite its rarity and potential for atypical presentations, a high index of suspicion for torsion of a wandering spleen is essential, particularly in patients with risk factors. With prompt diagnosis and timely intervention, patients with this condition can achieve favorable outcomes.

Introduction

Torsion of the spleen is a rare cause of acute abdominal pain. A “wandering spleen” is characterized by laxity or absence of the supporting splenic ligaments and where a long pedicle facilitates abnormal positioning of the spleen outside its native left subdiaphragmatic location. Wandering spleen predisposes the spleen to torsion, blood-flow impairment, and ischemia, and can cause a variety of symptoms from mild intermittent abdominal pain to acute abdominal crisis [1]. The first detailed description of this clinical entity was by Van Horne in 1667 as an incidental finding during autopsy [2].

Wandering spleen is a rare clinical entity with a less than 0.2% reporting incidence rate [1, 3]. It is a condition characterized by the absence or underdevelopment of one or all the ligaments that hold the spleen in its normal position. The lienorenal ligament attaches the hilum of the spleen to the left kidney, and the gastrosplenic ligament attaches the hilum of the spleen to the greater curvature of the stomach. Inferiorly, the spleen is supported with the phrenicocolic ligament [4]. It affects children, who make up one-third of all cases, with a female predominance after the age of 1 year. In pediatric cases, its presentation includes acute abdomen and nonspecific symptoms, such as nausea, vomiting, and fever. When wandering spleen is combined with splenic torsion, it may cause venous congestion and enlargement at the beginning. As the pathological course progresses, it may end up in splenic ischemia and infarction; finally, splenic necrosis and rupture could occur [5]. At adult age it most frequently affects women of reproductive age, in whom acquired laxity of the splenic ligaments is usually the cause. The hypothesized cause is found to be hormonal changes during pregnancy leading to ligamentous laxity [6]. Apart from its normal position, the spleen can be found anywhere in the abdomen, from the bottom of the left diaphragm to the pelvis [7]. Another extremely rare presentation is the finding of splenic torsion in its normal anatomical position [8].

The clinical presentation of wandering spleen is variable, but the main symptom is abdominal pain. It ranges from asymptomatic abdominal mass to intestinal obstruction or acute abdominal, which requires urgent surgical intervention [9]. Its major complication is acute torsion with subsequent infarction, which is a potentially fatal emergency [10]. Recognition of this medical condition can help avoid any confusion with acute abdomen of other etiologies. The diagnosis can be confirmed by imaging techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI) [11].

In general, a multidisciplinary approach should be considered, using history and physical examination to guide appropriate consultation with radiology and general surgery. Aggressive treatment is usually needed, and for most patients surgical intervention is required as a definitive measure [12].

Here we present a case of a 40-year-old female patient presenting with acute abdomen following 1080° counterclockwise torsion of splenic pedicle involving pancreatic tail with splenic infarction, which was successfully managed with open splenectomy in a setting where minimal invasive surgery is not available and well practiced. This work has been reported in line with CARE guidelines.

Case presentation

A 40-year-old Black, multiparous female patient of Amhara ethnicity, who is a housewife from a low socioeconomic background, referred from a rural area in Ethiopia, presented with an acute onset of dull, aching abdominal pain. She also experienced episodes of vomiting of ingested material and abdominal distension. The patient has no history of trauma, previous interventions, or significant medical, family, or social history. Physical examination revealed a palpable mass in the periumbilical and right lower quadrant areas with accompanying regional tenderness. Laboratory results disclosed a high normal leukocyte count whereas urine human chorionic gonadotropin (HCG) and organ function tests were nonrevealing.

After initial physiological stabilization, radiologic examinations, including an abdominal ultrasound and contrast-enhanced computed tomography (CT) of the abdomen, were performed. The abdominal ultrasound with color Doppler revealed a 14 × 10 cm mass in the left lower quadrant, showing echodensity similar to splenic parenchyma, with no color Doppler flow and no visible spleen in its usual anatomical location. On the basis of the radiology department’s recommendation, a contrast-enhanced CT of the abdomen was carried out, which revealed a spleen measuring approximately 15 cm in size, located in the lower abdomen. The CT also showed twisting of the splenic vessels at the hilum, resembling a “whirlpool” appearance, with heterogeneous density and no post-contrast enhancement. Additionally, part of the pancreatic tail appeared twisted, though without any change in density. Free peritoneal fluid was also noted. Financial constraints and cultural beliefs posed significant challenges during the diagnostic evaluation and intervention of our patient. After obtaining informed written consent, the patient was taken to the operating room with a preoperative diagnosis of splenic infarction secondary to torsion of a wandering spleen, involving the tail of the pancreas. Laparotomy revealed the spleen located in the right lower quadrant, with an elongated vascular pedicle twisted 1080° counterclockwise, involving the tail of the pancreas. The omentum and small bowel were adherent to the spleen, and minimal peritoneal fluid was present, as shown in Fig. 1. Splenectomy was performed after gently dissecting the omentum and small bowel from the spleen, with the pancreatic tail preserved (Fig. 2). The patient was discharged on postoperative day 4 following an uneventful hospital course, with instructions provided according to the postsplenectomy protocol. An appointment for immunization against encapsulated organisms was scheduled after 2 weeks postoperatively. During follow-up appointments, the patient was found to be in good health, with no complications from the procedure (Table 1).

Figure. 1

Intraoperative picture showing adhesion of the small bowel and omentum to the splenic hilum and twisted vascular pedicle

Figure. 2

Intraoperative picture showing small bowel, omentum, and splenic hilum after adhesions released

Table 1 Summary of patient’s condition during follow-up visits

Discussion

The anatomic position of the spleen is constant. The lienogastric, lienorenal, and phrenocolic ligaments provide support and attachment. Wandering spleen occurs when there is a failure of development or laxity of these supporting structures. Several causes have been proposed. A congenital defect has been implicated in some cases. With incomplete fusion of the embryonic mesogastrium posteriorly, the lienorenal ligament fails to develop, and the pancreas is not completely retroperitoneal, so that its distal portion is included in the intraperitoneal splenic hilum. Acquired factors suggested have been splenomegaly, trauma, and abdominal laxity and hormonal effects of pregnancy, which may account for the increased incidence of this condition in women of childbearing age [13]. The first description of a wandering spleen is attributed to Von Horne, in 1667, as an autopsy finding on an adult [2, 14].

The most commonly described clinical picture is the presence of a palpable mass associated with abdominal discomfort, and torsion is the most common complication [15]. Clinical symptomatology is variable and may range from the patient being completely asymptomatic, presenting with a mobile abdominal lump, or having intermittent abdominal pain because of partial torsion and spontaneous detorsion of the splenic pedicle. Often, patients may also present with an acute abdomen due to complete torsion and infarction. On examination, a firm, mobile abdominal mass with characteristic “notched borders” may be felt, but this is always not the case because splenic engorgement may obliterate the splenic notch, and therefore a clinical diagnosis is usually tricky [16].

Sonography and CT permit visualization of the spleen and it will be seen in an unusual location and may be enlarged. Enlargement of the distal pancreas with heterogeneous sonographic appearance or low attenuation on CT scanning should suggest that the tail of the pancreas has also undergone torsion and ischemic change [13]. In our patient, the abnormal position of the spleen was demonstrated on both while twisting of the splenic vascular pedicle and involvement of the pancreatic tail was revealed on contrast-enhanced CT of the abdomen. Angiography is the most definitive diagnostic modality in the diagnostic evaluation of wandering spleen [17]. The course and length of the splenic artery and the exact location of its torsion may be seen.

Treatment of choice is splenopexy if the spleen is viable, despite the high rate of recurrence. When it is not possible to preserve the spleen, the standard treatment is splenectomy followed by prophylactic antibiotic therapy and vaccination for encapsulated bacteria [18]. The best surgical approach is said to be minimally invasive surgery in a setup where it is available and well practiced, unlike in resource-limited setting such as ours.

Conclusion

Torsion and infarction of a wandering spleen is a rare abdominal emergency. Characteristic imaging features on radiological modalities such as abdominal sonography with color Doppler and CT scan are crucial in making an accurate and timely diagnosis. A fair degree of accurate assessment about viability and thrombosis of the splenic vessels can be made on CT scan, which may help the surgeon decide the right mode of treatment.

Despite its rare prevalence and atypical presentations, a high index of suspicion for torsion of wandering spleen is very important in patients with acute abdomen, especially in those with risk factors or initial abdominal sonographic evaluation revealing absence of spleen in its normal anatomic position. With rapid diagnosis, timely surgical intervention, and appropriate subsequent follow-up, patients can survive this clinical condition.