Preoperative splenic artery embolization in klippel-Trenaunay syndrome with massive splenomegaly: A case report
A 29-year-old woman with a history of KTS with vascular malformations involving both legs and pelvis was admitted to the hospital for open splenectomy. She had lymphedema of the lower extremities and cutaneous port-wine stains. Seven years prior to the admission, the patient had noncirrhotic portal hypertension of unknown etiology, complicated by portal vein thrombosis, and bleeding esophageal varices. Since then, she has undergone endoscopic variceal banding and percutaneous transhepatic portal vein recanalization with stent placement. Subsequently, she developed massive splenomegaly with thrombocytopenia and a 30-pound weight loss over six months. She had left-sided abdominal pain associated with massive splenomegaly.\nHematocrit was 25.3%, hemoglobin 8.5 g/dL, and platelet count 38,000/mm3. Computed tomography (CT) of the abdomen demonstrated massive splenomegaly with innumerable, less than 1 cm, low attenuation lesions in the spleen. Magnetic resonance imaging (MRI) of the abdomen depicted a markedly enlarged spleen with similar innumerable abnormal signal structures within the spleen []. Splenomegaly and splenic involvement of KTS were delineated on both CT and MRI images. Because of the risk of bleeding, the patient underwent transcatheter splenic artery embolization, immediately prior to open splenectomy.\nAfter obtaining written informed consent, the procedure was performed on the morning of surgery. Digital subtraction angiogram of celiac trunk demonstrated a dilated and tortuous splenic artery, and an enlarged spleen with inhomogeneous parenchymal staining. Innumerable avascular lesions were seen in the splenic parenchymal phase. There was no arteriovenous shunting []. After placing a catheter distal to the origins of the caudal pancreatic and left gastroepiploic arteries, distal splenic artery embolization was performed using Gelfoam slurry (Ferrosan, Somerville, NJ), achieving occlusion of the segmental arteries. The proximal splenic artery (immediately distal to its origin at the celiac axis), measuring 8 mm in diameter was occluded using a 14 mm diameter Amplatzer Vascular Plug II (AVP II; St. Jude Medical Inc. St. Paul, MN). Follow-up arteriography confirmed optimal deployment of AVP II in splenic artery. No persistent flow, through the deployed AVP II was demonstrated on the completion celiac digital subtraction angiography (DSA) []. Selective left gastric arteriography demonstrated no evidence of collateral blood flow to the spleen.\nAfter splenic artery embolization, the patient was transferred to the operating room for open splenectomy. After careful dissection, the spleen was isolated on its hilum. The spleen was soft, secondary to the prior splenic artery embolization. Surgical ligation of the splenic artery before dissection for surgical exposure was not necessary due to prior embolization. The estimated blood loss was 100 cc during surgical splenectomy. The patient received pneumococcal and hemophilus vaccines, immediately after surgery.\nOn pathology examinations, the removed spleen measured 27.5 × 21 × 6.5 cm in size and weighed 2,935 g []. Grossly, the spleen was diffusely enlarged without any discrete lesions. Large quantities of blood exuded from its cut surfaces. Histologically, the spleen had mixed arteriovenous and lymphatic components []. Thrombocytopenia was successfully corrected after splenectomy with platelet count of 160,000/mm3 on postoperative day 5. Postoperative recovery was smooth without complications. Patient was discharged 5 days after the surgery. The patient was closely followed by the surgical and hematological services. During 6 months after the surgery, abdominal pain has improved with weaning off narcotics. The platelet count of 184,000/mm3was also noted at her 6-month follow-up visit.