Case Presentation
A 22-year-old unmarried Pakistani woman visited the Emergency Room (ER), complaining of severe lower abdominal pain that had been occurring intermittently for the past five days. She described the pain as throbbing and non-radiating. Initially, she sought medical advice from her local practitioner, who recommended an abdominal and pelvic ultrasound. The ultrasound revealed an oval hypoechoic area in her right lower abdomen. It measured 15.9 × 8.8 × 5.9 cm, with no blood flow observed on color Doppler imaging. Based on these findings, her local practitioner suspected a right ovarian cyst. He referred her to our facility for further evaluation and possible drainage of the cyst.
Upon presentation at our facility, the patient reported mild pain in her lower abdomen but was otherwise stable. Her medical, surgical, and menstrual histories were unremarkable. Physical examination revealed a soft, non-tender abdomen with a palpable hard mass in the right hypochondrium.
Investigations
Upon admission, laboratory tests showed anemia with a hemoglobin count of 9 g/dl, leukocytosis with predominant neutrophilia, and deranged liver function tests, including elevated direct bilirubin, alkaline phosphatase, and LDH levels. Serum tumor marker tests indicated an increased serum CA-125 level, while other tumor markers were within normal ranges.
A pelvic ultrasound performed at the facility revealed a solid cystic heterogeneous mass with specks of calcification in the right periumbilical region. Subsequent MRI findings showed a comma-shaped mass in the lower abdomen/pelvic region. This was suggestive of an enlarged wandering spleen with torsion of its pedicle and infarction. Based on these findings, doctors performed exploratory laparotomy with possible splenectomy.
Management
During the laparotomy, doctors discovered an enlarged wandering spleen with torsion of the splenic vein. Alongwith thrombosed arterial supply from the omentum wrapped over the mass. Histopathological examination of the tissue confirmed hemorrhagic infarction and congestion of the spleen, along with separate omental tissue. The patient recovered well postoperatively and they discharged him two days later.
Ectopic Spleen
Wandering spleen, also known as ectopic spleen, is a rare condition characterized by the displacement of the spleen from its normal anatomical position. In this condition, it is shifted from the left hypochondrium to an ectopic site in the lower abdomen or pelvic cavity. Most literature on wandering spleen focuses on young females, particularly multiparous individuals. However, it can occur in individuals of any age and gender.
Symptoms of wandering spleen can vary widely, with some patients presenting asymptomatically while others experience abdominal discomfort or palpable abdominal masses. Complications of wandering spleen include splenic rupture, hemoperitoneum, and peritonitis, which are more likely to occur in cases of acute torsion. However, this patient’s presentation was initially misdiagnosed as an ovarian cyst, highlighting the challenge of differentiating between the two conditions based on imaging alone.
The diminished blood flow to the spleen in cases of wandering spleen can lead to anemia and thrombocytopenia. However, our patient exhibited neutrophilic leukocytosis, likely due to tissue necrosis and possible secondary bacterial infection. Deranged liver enzymes observed in our patient may have been caused by portal vein congestion secondary to splenic vessel obstruction.
Splenic Torsion
Torsion of splenic pedicles can result in partial or complete splenic infarction, with symptoms ranging from acute abdominal pain to chronic abdominal masses. In this case, the degree of splenic torsion was significant, with torsion of both splenic veins and thrombosis of the arterial supply.
While the patient primarily complained of intermittent abdominal pain, other symptoms such as vomiting, constipation, or abdominal distension were absent. Pregnancy and multiparity are known predisposing factors for wandering spleen due to hormonal changes and weakened abdominal wall structures. However, this patient was nulliparous, emphasizing that wandering spleen can occur in individuals without these risk factors.
Thrombocytopenia can occur in cases of splenic torsion, but our patient exhibited normal to high platelet counts, likely due to post-splenectomy reactive thrombocytosis. Additionally, an elevated serum CA-125 level was observed, along with a right adrenal neoplastic mass. However, histopathological examination revealed no malignancy, confirming the dislocated spleen as the source of the elevated CA-125 level.
While doctors use ultrasound as the initial imaging modality for suspected wandering spleen, multi-slice spiral CT is more accurate, especially for early detection. CT imaging provides clear visualization of the spleen parenchyma and vascularization, aiding in differential diagnosis from other abdominal masses.
In addition to the wandering spleen diagnosis, CT contrast findings revealed bilateral dense breast parenchyma with well-defined lesions in the left breast. This prompted a referral to a breast surgeon for further evaluation. However, the patient did not follow up on this recommendation.
Surgery, including splenectomy or splenopexy, is the standard treatment for wandering spleen. In this patient’s case, splenectomy was performed due to pedicle torsion and splenic infarction. Post-splenectomy, doctors advise patients to receive vaccinations against specific pathogens to prevent post-splenectomy sepsis.
Conclusion: Ectopic Spleen
In conclusion, ectopic spleen is a rare condition that can present with a variety of symptoms, often mimicking other abdominal masses. Accurate diagnosis requires careful evaluation, and imaging modalities such as CT can aid in distinguishing wandering spleen from other conditions. Prompt surgical intervention is essential to prevent complications and ensure optimal patient outcomes.
