Wandering spleen leading to splenic torsion with gastric and pancreatic volvulus

Background

Wandering spleen is a rare condition that occurs when the spleen has increased laxity or lacks one or more of the ligaments holding the spleen in its normal position. We defined a wandering spleen as the anatomical abnormality where the spleen is not in its normal, anatomic position in the left upper quadrant.1 The first description of this phenomenon was by Van Horne over 350 years ago, who described a wandering spleen during an autopsy.2 Cases of wandering spleen are uncommon with fewer than 350 cases reported and an incidence of less than 0.2%.3 It most frequently affects children under the age of 10 and women of childbearing age. In women of childbearing age, it is thought that the acquired laxity of splenic ligaments is the culprit. Treatment is typically surgical, including either splenopexy versus splenectomy. However, patient characteristics, presentation and intraoperative findings may influence treatment options.

Case presentation

A 37-year-old woman with a complicated medical history, including ulcerative colitis status post total abdominal colectomy with J-pouch anastomosis, primary sclerosing cholangitis with a transjugular intrahepatic portosystemic shunt awaiting liver transplantation (Model for End-Stage Liver Disease (MELD) score of 14 and Child’s B staging) and a known wandering spleen (figure 1) presented to the emergency department.

The patient reported increasing left upper quadrant pain over 6 days, which had acutely worsened in the last 24 hours. The patient also endorsed significant nausea, but no emesis, along with subjective fever and chills. She also mentioned a recent episode of pancreatitis which had been resolving.

On discussion, she also mentioned having recurrent episodes of vague abdominal pain requiring hospitalisations and visits to the emergency department over the last several years that was likely related to her wandering spleen.

On physical examination, the patient had severe tenderness to palpation in the left upper quadrant and flank. Her abdomen was moderately distended.

Investigations

Her laboratory examination revealed a decreased white cell count of 2.5×10⁹/L and platelets of 48 x 10⁹/L, but a normal lactate of 1.2 mmol/L. Her liver function tests were elevated with an aspartate aminotransferase of 277 IU/L, alanine aminotransferase of 206 IU/L, alkaline phosphatase of 574 IU/L and total bilirubin of 4.9 mg/dL. Her international normalised ratio was normal at 1.2 with an albumin of 3.6 g/dL. Her lipase level had decreased from 498 to 293 since her last admission for pancreatitis. Her MELD Score was noted to be 14 on presentation.

CT of her abdomen and pelvis showed findings consistent with wandering spleen with swirling of the splenic vascular pedicle concerning for splenic torsion. In addition, this splenic torsion involved a portion of the stomach and distal pancreas, along with a non-occlusive thrombus of the splenic vein. There was also noted to be small bowel dilatation to 6 cm (figure 2).

Differential diagnosis

This patient had several different aetiologies for her pain that were raised prior to the imaging. Prior to CT imaging, our differential diagnosis included pancreatitis, small bowel obstruction and a volvulus related to her wandering spleen.

Given her recent episode of pancreatitis and lipase of 215, pancreatitis was in the differential diagnosis. However, her pain came on acutely and was severely painful. Furthermore, her lipase had been decreasing, which was 488 from her previous admission 10 days prior and once her imaging was obtained, her pancreas did not appear acutely inflamed compared with her previous films.

Given her numerous previous abdominal surgeries, small bowel obstruction was also possible. On the CT scan, dilatation of the small bowel to the distal anastomosis was described, increasing the possibility that her pain was from a small bowel obstruction with transition point. However, given the acute onset of her pain and the focal nature of the pain, small bowel obstruction seemed less likely.

Obtaining the CT scan aided in the diagnosis of this patient. Given her known history of a wandering spleen, the patient was at high risk of potential sequalae including splenic torsion and volvulus. The CT revealed impressive swirling/torsion of the spleen along with involvement of the stomach and pancreas (figure 2). Because of the involvement of the spleen, stomach and pancreas, ischaemia to any of these organs could potentially be responsible for her symptoms. However, the significant degree of splenic torsion, which correlated with the location of her pain, was thought to be the most likely cause of her symptoms.

Treatment

The likelihood of spontaneous detorsion was thought to be low and given the risk of potential gastric, pancreatic or splenic ischemia, thus emergent surgical intervention was recommended to the patient. The risks and benefits of operative treatment versus medical management were explained to the patient, and consent was obtained prior to taking the patient to the operating room. The patient received 1 5-pack of pooled platelets preoperatively. She then underwent exploratory laparotomy via left subcostal incision with midline extension, which confirmed a highly mobile, volvulised spleen underneath the liver with significant torsion of the splenic vascular pedicle (figure 3A). A left subcostal incision was chosen, as opposed to a midline incision, as CT imaging showed the torsion of the vascular pedicle itself occurring in the left upper quadrant. This volvulus involved the greater curvature of the stomach without obstruction, as well as the tail of the pancreas. The spleen appeared congested, but on detorsing the spleen, it did not appear to be less hyperemic. Of note, the spleen was without irreversible ischemia. The stomach and tail of the pancreas appeared viable and undisturbed. Given the splenic congestion and patient’s recurrent episodes of abdominal pain involving hospitalisations and visits to the emergency department likely related to her wandering spleen, we decided to proceed with splenectomy over splenopexy. After reduction of the torsion (figure 3B), a splenectomy was performed with distal ligation of the splenic artery and vein. Transection of the hilum was completed with Endo-GIA tan load stapler with two clips to control the artery proximally and remain in case of bleed to aid with identification and splenic artery embolisation. The spleen was sent to pathology. Several adhesions were also lysed, which were anchoring her small bowel in the pelvis, possibly causing the dilated loops of bowel seen on CT scan.

Outcome and follow-up

The patient’s postoperative course was uncomplicated. Her pain was well-controlled with oral pain medication. Her diet was advanced in a stepwise fashion. She was able to ambulate independently and void spontaneously on discharge.

Final pathology report showed the following:

Diagnosis:

A. Spleen, resection: congested spleen with capsular adhesions. Congested hilar lymph node.

History:

A 37-year-old woman diagnosed with splenic ischemia.

Operative procedure/tissue submitted: exploratory laparotomy, splenectomy, possible detorsion and all indicated procedures.

Gross description:

A. ‘Spleen’ received in formalin in a large container is a 878 g, 19.3×15.5×8.5 cm intact spleen. The external capsule is intact. There is a moderate amount of adherent purple membranous tissue adjacent to the hilum and along the anti-hilar aspect. Sectioning reveals red-brown, diffusely bloody cut surfaces. The vessels at the hilum are diffusely engorged containing a moderate amount of black-red clotted blood. Additionally identified at the hilum is a 0.7×0.5×0.4 cm possible splenial.

A1. Dilated vessel with clotted blood. Two adhesions along serosal surface.

A2–A3. Capsular adhesions.

A4. Possible splenial.