Torsion of a wandering spleen (WS) is a rare cause of acute abdomen in the pediatric patient.1 Laxity or absence of the supporting ligaments predispose to spleen’s migration in the abdominal cavity and torsion of its vascular pedicle.2 Patients with WS can be asymptomatic or experience mild abdominal discomfort; in some cases splenic infarction occurs, representing thus a surgical emergency.3 Hence, an accurate diagnosis is not deferrable. We describe the case of a patient with a 10-time twisted vascular pedicle of the spleen, located in an anomalous position.
A 8-year-old child presents at our Department with a 4-day history of crampy abdominal pain in the lower quadrants, worsened in the last hours and associated with vomit and fever. Parents refer chronic constipation and no history of abdominal trauma. Urological symptoms are absent. On physical examination, palpation of the right iliac fossa is painful; Blumberg, Rovsing and Giordano signs are negative and peristalsis is normal. No abdominal mass is palpable. Blood tests show a white blood cell count of 29.9x109/L and a C-reactive protein of 179 mg/L; hemoglobin level is 13.8 g/dL and platelet count is 249x109/L. Acute appendicitis is the initial suspicion. Abdominal radiographs find modest bowel distension. An urgent ultrasonography (US) of the abdomen, performed at the Emergency Department, detects a 13x6x8 cm solid tubular mass, located medially in the pelvis. A second US examination is performed to obtain further information about this radiological finding. Limited by meteorism, US reports a normal size spleen in the left hypocondrium and a parenchymatous pelvic mass, poorly vascularized at the colour-Doppler control. A computed tomography (CT) scan reveals an empty splenic fossa and a median unenhanced spleen with a twisted vascular pedicle (Figure 1). Therefore, splenic infarction is suspected. An urgent laparoscopy shows an enlarged ischemic spleen in meso-hypogastrium. Its 15 cm-long vascular pedicle is 10-time twisted and no spleen’s supporting ligaments are documented. Because of the increased size of the spleen an open approach is preferred (Figure 2). After detorsion of the twisted pedicle, a splenectomy is performed since the spleen appears necrotic. Histological examination documents thrombosis of splenic vessels. The patient’s post-operative course is uneventful and she is discharged 5 days later with a long-term antibiotic prophylaxis.
Torsion of a WS is an uncommon clinical condition, characterized by twisted vascular pedicle of a hypermobile spleen, an anomaly due to the absence or laxity of spleen’s supporting ligaments.1,2 WS accounts for less than 0.2% of splenectomies and its incidence shows a bimodal distribution: children below 10 years old and women of childbearing age.4 In children, WS represents more likely a congenital defect, resulted from an abnormal development of the dorsal mesogastrium.5 The clinical presentation of WS is variable. It can be an incidental finding in asymptomatic patients or it can cause intermittent pain or mild abdominal discomfort, due to its torsion and spontaneous detorsion. In some cases, persistent torsion leads to splenic infarction, which requires emergency surgical intervention. In our case, the child presented a 4-day history of abdominal pain in the lower quadrants, vomit and fever. On physical examination, an abdominal mass may be palpable in any abdominal quadrant, but this feature is absent in 33% of patients,6 as in our case. Imaging represents a useful adjunct in diagnosing such an uncommon condition. Pre-operative evaluation includes plain abdominal radiographs, US with colour-Doppler control and enhanced CT scan. US can reveal absence of the spleen in its normal location and detect a parenchimatous mass, usually located in the pelvis. Moreover, colour-Doppler control evaluates the vascularization of WS.3 However, the exam depends on the operator’s skills and it is often limited by meteorism.1 In our case, the second US misdiagnosed the condition. The most common CT findings are evidence of an empty splenic fossa and an ectopic spleen, located in the left mid-abdomen, and the whirl sign, a tomographic sign of pedicle torsion.6 Contrast-enhanced CT provides crucial information concerning the viability of the spleen. The above-mentioned findings were all documented in the CT scan performed in our case, which suspected the presence of a twisted spleen in the pelvis. Therapeutic options of torsion of WS are splenopexy and splenectomy, by open or laparoscopic approach. In our case, an urgent laparoscopy showed a 10-time twisted spleen, but at that time our laparoscopic skills were improper to complete the procedure.