An 18-year-old female with recurrent esophageal variceal bleeding

Ann Saudi Med 29(1) January-February 2009 www.saudiannals.net 61 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 4.2×109/L and the platelets were 30×109/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 An 18-year-old female with recurrent esophageal variceal bleeding

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 vac-cines were given and the patient underwent a modified Suguira procedure consisting of extensive esopha-gogastric devascularization combined with esopha-geal 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. T his patient had ultrasound evidence of splenom m megaly with discrete, multiple, small echogenic foci in the splenic parenchyma. Both color Doppler and CT splenoportovenography revealed evim m dence of extrahepatic portal hypertension. In addition, CT confirmed the presence of multiple hyperdense foci in the splenic parenchyma ( Figure 1). In this clinim m cal setting, the splenic lesions represent GamnamGandy bodies (GGB).
The differential diagnosis of small, discrete, hyperm m dense splenic lesions apart from GGB would also inm m clude sarcoidosis, miliary tuberculosis, histoplasmosis and Pneumocystitis carinii infection.
Microscopic examination of the splenic lesions rem m vealed typical spheroid, bamboomshaped fibers resemm m bling 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.

DISCUSSION
GamnamGandy bodies (GGB), also known as siderotic nodules or tobacco flecks, are minute, firm nodules of fibrous tissue impregnated with iron pigments (hemom m siderin) and calcium salts. They occur as a result of fom m cal hemorrhages and necrosis followed by accumulation of hemosiderin. The most common cause of GGB in the spleen is portal hypertension; it is observed in 9% to 12% of these patients. 1,2 They have been also seen in conditions like paroxysmal nocturnal hemoglobinm m uria, 3 hemolytic anemia, sickle cell anemia, leukemia, 4 lymphoma, 5 angiosarcoma, 6 in patients receiving blood transfusions and in acquired hemosiderosis. 7 However, it is unclear whether in such conditions their occurm m rence is a result of primary disease or associated portal hypertension.
Rarely GGB may be seen in extrasplenic sites like in cardiac myxoma, 8 renal cell carcinoma, 9 ovary, 10 liver, 11 thymoma, 12 follicular adenoma of thyroid, 13 retroperim m   toneal lymph nodes 12 and in central and peripheral nervous system neoplasms. 14 Alhough Tedeschi et al 13 described the various histological features of GGB, the presence of spheroid bamboomshaped or articulated fim m bers that resemble mycelial structures are considered pathognomic of GGB.
In portal hypertension these lesions should be dism m tinguished from sclerotic venous branches within the spleen, which shows the presence of blood flow within them on Doppler imaging. On sonography, multiple hyperechoic foci within the spleen may also be seen in sarcoidosis, 15 histoplasmoisis, tuberculosis, 16 and disseminated Pneumocystitis carinii infection. 17 Such conditions can be excluded by proper clinical history and other classical features. A nonmenhanced CT scan detects GGB as multiple faint high attenutation spots within the spleen; however, their detection depends on the amount of calcium deposition. MRI is described as the most sensitive method for detection of GGB. Due to their hemosiderin content, such lesions are characm m terized by low signal intensities on all pulse sequences. The gradient echo sequence is considered to be highly sensitive for detection of hemosiderin and typically shows the blooming effect. 18 Thus in patients of portal hypertension, detection of such lesions on various imaging modalities not only strengthens the diagnosis of portal hypertension, but also excludes other causes like miliary tuberculosis, histoplasmosis and disseminated Pneumocystitis carin n nii infections. This report also provides a correlation of various imaging modalities with histopathological diagnosis.