An 18-year-old female with recurrent esophageal variceal bleeding due to extrahepatic portal venous obstruction was referred to us for surgical management. On general physical examination she was pale. There was no jaundice or lymphadenopathy. Abdominal examination disclosed a massive splenomegaly (12 cm below the costal margin). Laboratory tests revealed a hemoglobin of 85 g/L, a total leucocyte count of 4.2x109/L and the platelets were 30x109/L. Liver function tests and other biochemical investigations were within normal limits. Color Doppler sonography revealed multiple collateral channels replacing the portal vein (portal cavernoma) and massive splenomegaly. Furthermore, it demonstrated multiple, small foci of echogenic nodules with no acoustic shadowing in the spleen. Non-enhanced CT divulged massive splenomagaly with a pressure effect on the ipsilateral kidney and multiple, discrete, millimetric hyperdense spots in the spleen (Figure 1). A contrast-enhanced splenoportovenogram confirmed the presence of portal cavernoma, esophageal varices, multiple perisplenic collaterals and extensive thrombosis of the splenoportal venous axis. A diagnosis of extrahepatic portal vein obstruction leading to portal hypertension was made.
Pneumococcal and Haemophilus influenzae vaccines were given and the patient underwent a modified Suguira procedure consisting of extensive esophagogastric devascularization combined with esophageal transaction, reanastomosis and splenectomy. The postoperative hospital period was uneventful and the patient was discharged on the tenth postoperative day. Histopathological examination of the spleen disclosed an abnormality.
What is the preoperative diagnosis of the splenic lesions?
What is the differential diagnosis of small, discrete, hyperdense splenic lesions?
This patient had ultrasound evidence of splenomegaly with discrete, multiple, small echogenic foci in the splenic parenchyma. Both color Doppler and CT splenoportovenography revealed evidence of extrahepatic portal hypertension. In addition, CT confirmed the presence of multiple hyperdense foci in the splenic parenchyma (Figure 1). In this clinical setting, the splenic lesions represent Gamna-Gandy bodies (GGB).
The differential diagnosis of small, discrete, hyperdense splenic lesions apart from GGB would also include sarcoidosis, miliary tuberculosis, histoplasmosis and Pneumocystitis carinii infection.
Microscopic examination of the splenic lesions revealed typical spheroid, bamboo-shaped fibers resembling mycelial structures (Figure 2, 3). There was also evidence of infiltration of the macrophages and foreign body giant cells. Hence, a histopathological diagnosis of GGB in the spleen was made.