A 23-year-old Saudi male, known case of sickle cell disease, presented to the medical OPD in June, 2013 with one-week history of mild vague abdominal pain in the left upper quadrant associated with early satiety. On examination, the patient was conscious. His vital signs were as follows: blood pressure 110/60 mmHg, pulse rate 84/minute, and temperature 37.1 C. He had mild pallor but not jaundiced. Abdominal examination revealed massive splenomegaly (10 cm below costal margin). His investigations were as follows: WBC: 4.32, Hb: 11.3, PLT: 184, LDH: 807, TBIL: 31, Conj.Bil: 0, ALT: 39, AST: 32, and ALKP: 66. Abdominal ultrasound showed homogenous echo pattern with slightly enlarged liver (16 cm at the right midclavicular line) and smooth hepatic boarders. There were no focal lesions; spleen was confirmed to be enlarged (20 cm) with dilated portal vein.
Ultrasound portal-splenic Doppler showed the diameter of the portal vein measuring 12.5 mm in caliber. It also showed normal patent biphasic flow pattern with average velocities and normal hepatopedal flow direction with no evidence of portal vein thrombosis. The hepatic veins also displayed average normal diameter measuring 10.5 mm in caliber. It appeared patent on color Doppler interrogation displaying average velocities with no evidence of portal-systemic collateral vessels. Hematology consultation recommended splenectomy which was carried out laparoscopically with uneventful postoperative recovery.
In July 2015, the patient presented to the OPD with obstructive jaundice. On examination, he was conscious, alerted, and oriented. His vital signs were as follows: blood pressure 112/58 mmHg, pulse rate 81/minute, and temperature 36.9 C. He looked jaundiced. Abdomen (after splenectomy) was soft, lax, and nontender. His investigations were as follows: WBC 5.9, Hb: 11.2, platelet: 251, BUN: 1.2, creatinine: 52, sodium: 138, potassium 4.2, chloride 105, bicarbonate 22. TBIL: 187.7, ALP: 425 AST: 84, ALT: 54, and Direct Bil: 42.5. Abdominal ultrasound showed gallstone with signs of cholecystitis, biliary dilatation, and common bile duct (CBD) stone. Abdominal Doppler ultrasound showed patent portal vein. The patient was admitted to the hospital where ERCP was performed; sphincterotomy and CBD stone extraction were done. He developed mild pancreatitis which resolved in two days.
In October 2015, the patient presented again with abdominal pain and obstructive jaundice. His full investigations can be seen in Table 1. Abdominal ultrasound showed gallstones, cholecystitis, and dilated bile ducts (Figure 1). He underwent ERCP; cholangiogram showed a CBD stone difficult to be extracted. Balloon sphincteroplasty up to 10 mm was performed which was complicated by massive bleeding from the ampulla. The blood was just oozing and pouring from the orifice. There was no arterial bleeding. The bleeding was massive and could not be controlled. The patient went into acute hypovolemic shock (Hb dropped to 5.8 g/dl), and he was then intubated and transferred to the ICU where he was resuscitated and received six units of packed RBCs and FFPs. His condition improved after two days. Later on, he was evaluated for the cause of massive bleeding. Abdominal CT showed cavernous transformation of portal vein with splenic and portal veins thrombosis, in addition to extensive collaterals at porta hepatis and around the biliary tree (Figure 2). Upper endoscopy showed grade II esophageal varices. ERCP was successfully performed after 2 days to assess the area of bleeding and put stent to assure free drainage of the bile duct. The patient's condition improved after ERCP. He was then transferred to the medical ward where his condition was stable with no bleeding or hemoglobin drop. His LFT results were as follows: TBIL: 58.1, ALP: 156, AST: 19, ALT: 13, TP: 72, INR: 1.3, and albumin: 33. The patient was seen by general surgery and planned for laparoscopic cholecystectomy.