Wandering Spleen with Splenic Torsion: Unusual Cause of Acute Abdomen

Background

The term “wandering spleen” refers to splenic hypermobility and loss of fixation brought on by the laxity or underdevelopment of the suspensory ligaments in the spleen.^1,2 The ligaments stretching and, frequently, the spleen’s axial rotation is linked to this loss of fixation.³ This propensity can be amplified by additional acquired and congenital factors.¹ Congenital causes such as abnormal mesogastrium development have been proposed.^4,5 However, pregnancy is mentioned as an acquired cause.⁶

It is a rare disease in children that has only sometimes been documented. It can be asymptomatic or can cause chronic symptoms from an abdominal mass in addition to sudden surgical emergencies with splenic infarction due to torsion.^7,8 About 0.2–0.3% of individuals who need a splenectomy had wandering spleens detected.⁸ Due to the symptoms’ lack of specificity, diagnostic modalities like ultrasound and Computed Tomography scan are crucial for the diagnosis.^2,9,10 Splenic torsion may be misdiagnosed as acute cholecystitis, appendicitis, gastric hemorrhage, intestinal obstruction, colon cancer, urine retention, and torsed ovarian cyst.^3,8 Surgery is one of the treatment choices for wandering spleen, and laparotomy or laparoscopy methods can be used to perform splenectomy, or splenopexy.²

The purpose of this case report is to discuss a 12-year-old male child who appeared with an acute abdomen caused by a wandering spleen, along with acute appendicitis and splenic torsion.

Case Presentation

A 12-year-old male boy complained of crampy, lower abdominal pain that had been going on for four days. Additionally, he had a history of appetite loss, constipation, and mucoid diarrhoea. He also expressed abdominal distension, however, he had never vomited. No family history of similar symptoms was identified. He resides with his parents, three siblings, and grandparents. There were no recognized medical conditions in the child or his family.

On evaluation, the patient was acutely sick looking, appeared to be in severe agony. His vital sign was remarkable for tachycardia (pulse rate of 134 beats per minute), a respiratory rate of 22 breaths per minute, a temperature of 36.40°C, and a blood pressure of 110/75 millimetre of mercury. He lacked a depressed eyeball and had moist buccal mucosa. His abdomen was distended, moves with respiration, and tender everywhere else but the epigastrium. An 8 cm by 5 cm firm, tender mass across the left lower quadrant (Figure 1) with the lowest boundary out of reach, and no evidence of fluid accumulation. The assessment of other systems was normal.

Figure 1 Visible lower abdominal mass on abdominal examination.

Investigations

Blood tests were conducted which revealed a white blood cell count 10,300/micL with a neutrophilia of 78.5%, with moderate anemia (Hg 8.6 g/dl, Hct 28%), MCV 86 fl and platelet 775x10³/micL. Ultrasound investigation identified a whirlpool sign at the splenic vessel, and the spleen is held in place in the lower abdomen with change in echogenicity. Wandering spleen with focal area infarction was the ultrasound diagnosis.

Treatment and Outcome

The patient was kept nil by mouth and was put on maintenance fluid. He was started on broad-spectrum antibiotics and analgesia. After stabilizing the patient an emergency laparotomy was done. Intraoporately slightly enlarged spleen located intraperitoneally below the level of umbilicus. Vascular pedicle torsioned 720° was found unusually entering via the convexity of the spleen (Figure 2). Areas of necrosis apparent as soft pale patchy areas were noted (Figure 3). There was adhesion to the tip to appendix and loop of terminal ileum (Figure 4). There was no accessory spleen. Appendix tip was erythematous with petechial bleeding up on releasing from its adhesion to the spleen but the ileum was released from the adhesion uneventfully. With an intraoperative diagnosis of wandering spleen with multifocal infarction secondary to splenic volvulus plus secondarily inflammed appendix, splenectomy and appendectomy was done. Post-operative period is uneventful. He stayed for 2 hours in recovery and was then transferred to a surgical ward and stayed for seven days. After seven days stay, the patient was discharged with PO amoxicillin prophylaxis and appointed after a week for pneumococcal conjugate vaccine.

Figure 2 Intra operative finding shows torsioned spleen along mesentery.

Figure 3 Intra operative finding shows focal infarct of spleen.

Figure 4 Intra operative finding shows adherence of ileum and appendix with spleen.

Discussion

The spleen is a vital organ in both newborns and prepubescent children. It is a pulpy organ about the size of a fist that holds the body’s largest single collection of lymphoid tissue. It is located obliquely in the left hypochondrium and consists of ligaments, which are peritoneal attachments named after the organs to which they attach. These ligaments, which include the lienorenal, splenocolic, splenophrenic, gastrosplenic, and phrenicocolic ligaments, keep the spleen in the left hypochondrium where it normally occupies an anatomical position.^11,12 Consequently, the lack or laxity of the aforementioned ligaments enables the spleen to be freely mobile in the abdominal cavity and to be mostly found in the pelvis, causing a condition called wandering spleen.¹³ Dorsal mesogastrium is also responsible for the formation of the peritoneum, the greater omentum and several peritoneal folds. Embryologically, Congenital defects of the mesogastrium are the cause of the spleen’s excessive motility.^7,14 All cases of wandering spleen have been found associated with long splenic pedicle which contains splenic vessels and pancreas.^15–17

This uncommon condition also known as splenoptosis, is most frequently found in children and women of childbearing age.⁶ Children account for one-third of instances, with most (70%) being older than 10 years.⁵ Fewer than 500 cases are reported with incidence of less than 0.2%.¹⁷

The clinical presentation of a wandering spleen is variable. It may pass asymptomatically throughout life.¹⁸ Despite the fact that the majority are probably asymptomatic, some require surgical intervention because to acute or chronic torsion, which can result in symptoms including abdominal discomfort, a mass in the abdomen, symptoms of splenic infarction, or left side portal hypertension.^14,19 Wandering spleen can also cause intestinal obstruction which can be the initial presentation.²⁰ Potential complications of wandering spleen includes sepsis, splenic rupture, and acute pancreatitis.²¹ Patients with splenic torsion may appear with an acute abdomen from an infarction. Reports showed that Spleen weight larger than 500gm and prolonged twisting greater than 180° predispose to splenic infarction.^6,15 Similarly, in our case, the spleen had a 720° twist and was grossly enlarged.

Absence of spleen in its location and finding of spleen in other site is confirmatory sonographic diagnostic modality for wandering spleen. Doppler and color flow sonography were also used in the diagnostic evaluation because of their capacity to noninvasively evaluate splenic parenchymal flow and flow in major splenic vessels; however, Contrast-Enhanced Tomography (CECT) scan may be required if bowel gas obscures visualization of spleen.^1,3,22 Other diagnostic modalities are Magnetic Resonance Imaging (MRI), splenic angiogram and scintigraphy.²⁰

Splenectomy, which involves removing the entire spleen, and splenopexy, which involves preserving the spleen, are two surgical treatments for managing a wandering spleen.⁴ One of the main dangers of having the spleen removed is post-splenectomy septicemia, and patients who have had the procedure are more susceptible to infection, particularly from encapsulated pathogens such Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae.²³ Overwhelmingly,post-splenectomy sepsis has a mortality rate between 30 and 60%.²⁴ Therefore in patients with a viable spleen, splenopexy is the surgical procedure of choice in order to preserve the spleen, regardless of the presence of symptoms.^25,26 Only splenic torsion patients with significant infarction and thrombosis of the splenic vessels should undergo splenectomy.^1,19

Even though it has a strong immune function, the absence of a functioning spleen very slightly increases the risk of fulminant infection in otherwise healthy adults. However, the spleen’s capacity to phagocytose and destroy organisms is crucial for the host’s resistance against severe bacterial sepsis in the very young and in people with very major underlying illnesses.¹¹ The use of combination vaccines against Hemophilus influenzae, polyvalent pneumococcal, and Neisseria meningitidis is advised at least three days prior to an elective splenectomy if performing one is necessary.²⁷ Prophylactic penicillin in addition to pneumococcal and meningococcal vaccines should be administered to all patients who have had splenectomy.^4,28

Conclusion

Clinically, wandering spleen is quite challenging to diagnose, thus it’s essential to use diagnostic tools like ultrasound or CECT scan to identify the condition early. If the spleen is viable and there is no thrombosis, splenopexy is advised. Splenic torsion with infarction should be treated with a splenectomy, and with provision prophylactic antibiotics, and a pneumococcal and meningococcal vaccine combination to prevent post splenectomy sepsis.