Wandering spleen is an exceedingly rare condition; normally asymptomatic unless splenic torsion has occurred, which is an emergency. There have been fewer than 500 cases reported worldwide with an incidence… Click to show full abstract
Wandering spleen is an exceedingly rare condition; normally asymptomatic unless splenic torsion has occurred, which is an emergency. There have been fewer than 500 cases reported worldwide with an incidence of less than 0.5%. The spleen is located in the left hypochondrium and lies between the fundus of the stomach and the diaphragm. It is held in place by two folds of peritoneum: the phrenicolienal and the gastrolienal ligaments, the latter containing the short gastric vessels. Awandering spleen can be congenital in origin as a result of laxity of support ligaments, such as in Ehlers–Danlos or Marfan syndromes, or acquired secondary to Nissen fundoplication, which involves takedown of the short gastric vessels and results in increased mobility of the spleen. The aim of this report is to present a rare case of splenic torsion. A 48-year-old Caucasian female, transferred from another hospital to our unit, presented with constant generalized abdominal pain of increasing severity over the last seven days, associated with nausea and vomiting. The patient was afebrile and haemodynamically stable, with a blood pressure of 134/87 mmHg and a heart rate of 96 bpm. Abdominal examination revealed generalized tenderness, worst in the paraumbilical region, without signs of peritonism or overt abdominal masses. Her past medical history included laparoscopic paraoesophageal hernia repair without fundoplication six years prior. Unfortunately, operative notes were not available to clarify the details of that operation. Haematological and biochemical investigations revealed leukocytosis with white cell count 22.8 × 10/L, haemoglobin 94 g/L, C-reactive protein 358 g/L and normal lactate, renal and liver function tests. Chest X-ray showed a large gastric bubble (Fig. 1). She underwent a computed tomography (CT) scan, which showed a grossly distended stomach, with transition point at the level of the proximal duodenum, the gastric antrum lying in an abnormally high position and a wandering spleen occupying the lower abdomen with associated whirling of the splenic vessels (Fig. 2). There was also free fluid, possible blood, intraperitoneally. These features were consistent with gastric volvulus and associated splenic torsion. After initial resuscitation, she needed emergency laparotomy on the basis of the clinical, biochemical and CT scan findings. There was approximately 800 mL of intra-abdominal haemorrhagic fluid, with a distended enlarged stomach without signs of volvulus or ischaemia. The spleen was enlarged, and located in the pelvis without ligamentous attachments apart from elongated vascular pedicle. The spleen was congested with multiple capsule tears due to volvulus around its pedicle, with 540° twisting of the pedicle (Fig. 3). The spleen itself was not viable and required splenectomy. The pancreas was in its retroperitoneal anatomical position, far away from splenic pedicle. Pathological examination of the spleen showed red pulp and the splenic hilar blood vessels congestion, consistent with effects of splenic volvulus. Post-operative course was uneventful. Wandering spleen is a rare clinical entity, which may present with chronic nonspecific abdominal pain, an acute abdomen, or with symptoms related to the spleen’s abnormal position and often increased size. When presenting in an acute setting, it is often with symptoms from splenic ischaemia due to torsion of the vessels. This torsion may lead to strangulation, infarction and rupture of the spleen. Clinical diagnosis of wandering spleen and its complications is difficult. Our patient had generalized abdominal tenderness, likely related to haemoperitoneum. Definitive diagnosis is only possible with imaging or abdominal exploration. CT scan has been the mainstay for imaging, although ultrasound and magnetic resonance imaging may be helpful. A ‘whorled’ appearance of the splenic vessels is characteristic of splenic torsion, and changes in attenuation of the organ may be helpful in determining the extent of the ischaemia. This was the mainstay in the diagnosis of this patient and concluded our decision for emergency surgery. Torsion of the spleen may also be associated with volvulus of adjacent organs, particularly the stomach, tail of the pancreas and occasionally the liver. In our case the spleen was completely separated from the stomach, probably during the previous surgery, and therefore other organs were spared.
               
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