Myxoma Immediately above the Junction of the Inferior Vena Cava and the Right Atrium: A Rare Cause of Budd-Chiari Syndrome.

The Budd-Chiari syndrome (BCS) is a rare disorder caused by the obstruction of the hepatic veins or the inferior vena cava (IVC) at the suprahepatic level. This syndrome is developed by either hepatic vein thrombosis or mechanical venous obstruction and leads to centrilobular hepatic congestion with the subsequent development of fibrosis and cirrhosis. Intracardiac tumors have been rarely reported as a cause of the BCS. These tumors usually originate from the atrial septum. Very rarely, they arise either from the junction of the IVC and the right atrium or from the Eustachian valve. There are a few case reports in the literature where atrial tumors have caused the BCS. In these cases, the tumors were malignant, and the patients died shortly after being diagnosed. We describe a 71-year-old female patient who presented with a 3-month history of abdominal pain and protrusion. On physical examination, blood pressure and pulse rate were normal. Jugular venous pressure was about 10 cm. Cardiac examination revealed a systolic murmur, grade IV/VI, in the left sternal border without radiation. Echocardiography showed a large mass (about 6×4 cm) in the right atrium with close contact to the origin of the IVC, obstructing it. Cardiac magnetic resonance imaging, with and without gadolinium, also confirmed the diagnosis. The patient underwent surgery, and the myxoma was removed. The tumor was a large solid mass, 5×4 cm in size, which originated immediately above the entrance of the IVC. The patient is in good condition 1 year afterward. We emphasize that atrial myxomas should be considered in the differential diagnosis of tumors that cause chronic BCS.

Partial replacement of left hemidiaphragm in dogs by either cryopreserved or decellularized heterograft patch.

OBJECTIVES: Large diaphragmatic defects are still a challenging issue for reconstruction using either synthetic prosthesis or bioprosthesis. To evaluate the possibility of using diaphragm allograft as a natural bioprosthesis in humans, we conducted a two-group study and compared cryopreserved and decellularized diaphragmatic heterograft patched in a canine model. METHODS: At the end of organ harvesting from a human donor, the left hemidiaphragm was taken to the laboratory in phosphate-buffered saline solution. The next step was freezing the grafts at -80°C, and preserving them for up to 2 months in Group 1. It was subjected to a detergent-enzymatic method (containing sodium deoxycholate/DNase lavations) of decellularization for 25 cycles in Group 2. Through left thoracotomy in the eighth intercostal space, cryopreserved patches in six dogs and decellularized patches in five dogs replaced the diaphragm. During the follow-up, sonography was done in all animals, but three and two dogs in Group 1 and 2 underwent computed tomography (CT) scan, respectively. The animals were euthanized after 6 months. RESULTS: There was no mortality. Sonography showed only motion impairment of the patches in all cases. In Group 1, CT scan showed mild atelectasis and scattered infiltration in the left lower lobe, fibrotic bands and minimal fluid collection under the diaphragm. In Group 2, CT scan showed scattered fibrotic bands and mild to moderate elevation of the left hemidiaphragm. There was no evidence of gross disruption and complete healing of the suture line. Necropsy in both groups showed patches were completely replaced with a dense fibrous tissue. In Group 1, focal calcification was noticeable in every case and foreign body-type granulomas were clearly seen all over the grafted tissue. Histology in Group 2 animals showed less inflammatory cell infiltration and scattered foreign body granulomas in comparison with the cryopreserved patch graft. CONCLUSIONS: The gross healing process in the decellularized heterograft is similar to the cryopreserved diaphragm but with fewer inflammatory cells and foreign body granulomas on histology. Both of them can be used instead of bioprostheses with regard to the fact that the decellularized patch technique is more complex and expensive. It is recommended to compare them with commercial bioprostheses.

Congenital hepatic artery aneurysm: a case report in early infancy.

Hepatic artery aneurysm in children is a very unusual pathologic entity. As most patients with this anomaly are asymptomatic, the diagnosis is usually made as an incidental finding on imaging studies performed for other reasons. Here, we report a 35-day-old infant with hepatic artery aneurysm, which was diagnosed accidentally as a liver mass during echocardiography. Considering the age of diagnosis, she most likely had a congenital type of hepatic artery aneurysm. To the best of our knowledge, this is the youngest case to be reported in the literature.

Triangular cord sign in detection of biliary atresia: is it a valuable sign?

BACKGROUND: Early detection of biliary atresia (BA) has a vital role in prevention of liver cirrhosis in these patients. There are some evidences that triangular cord (TC) sign, i.e., triangular structure located cranial to the portal vein bifurcation on ultrasonographic examination, is suggestive of BA in suspected cases. The aim of this study is to evaluate and compare the sensitivity, specificity, and accuracy of TC sign with other methods of diagnosis such as hepatobiliary scan. METHODS: Fifty-eight infants referred to pediatric gastroenterology ward with diagnosis of infantile cholestasis from March 2004 to March 2008 were evaluated to find the cause of cholestasis. Diagnosis was made by means of history, clinical examination, hepatobiliary scan, and liver biopsy. Ultrasonographic examination was focused on presence of TC sign in patients. If the diagnosis was in favor of BA, patient was sent for direct cholangiography as a gold-standard test for confirmation of the diagnosis. The sensitivity, specificity, and accuracy of the tests were compared with golden standard. RESULTS: Among 58 infants with infantile cholestasis, BA was diagnosed and confirmed in 10 infants (17.2%). Hepatobiliary scintigraphy had 80% sensitivity, 72.9% specificity, and 74.1% accuracy. TC sign had 70% sensitivity, 95.8% specificity, and 91.3% accuracy. CONCLUSION: TC sign is more accurate than hepatobiliary scan and has acceptable sensitivity and specificity for diagnosis of BA.

Sinonasal clear cell adenocarcinoma: a case report.

BACKGROUND: Nonsalivary adenocarcinomas are the most interesting tumors found in the sinonasal area. They are rare tumors arising from surface epithelium. The clear cell type of this tumor is even more rare. We present cytologic findings of clear cell sinonasal adenocarcinoma and related pitfalls. CASE: A 52-year-old woman presented with a left-cheek facial mass of 3-4 years' duration, with progressive enlargement, nasal discharge and discoloration of the lateral side of her left eye. Computed tomography was performed, revealing an expansile mass involving the nasal cavity, left maxillary sinus, ethmoid sinus with extension to sphenoid sinus, left side of oral cavity and left orbit. Fine needle aspiration performed through the upper buccogingival canine fossa showed clusters of epithelial cells with clear cytoplasm, round nuclei, inconspicuous nucleoli and slight pleomorphism. Some normal ciliated columnar epithelial cells are identified in the vicinity of neoplastic cells. The mass was reported to be a clear cell neoplasm, and excision of the whole mass was performed. CONCLUSION: Cytologic findings of this rare tumor overlap with those of salivary gland-type tumors with clear cell change and should be added to the list of head and neck tumors with clear cell change.

Cytologic differentiation of struma ovarii from other ovarian neoplasms.

OBJECTIVE: To present the cytologic findings of struma ovarii and value of cytology and immunocytochemistry (ICC) using thyroglobulin (TGB) and thyroid transcription factor-1 (TTF-1) in evaluation of this unusual ovarian neoplasm, together with the diagnostic pitfalls. STUDY DESIGN: Intraoperative cytologic findings of 7 patients with struma ovarii are reviewed. Cytologic material of both cystic and solid components was collected intraoperatively. ICC staining was done, and cell block preparation performed on selected cases. RESULTS: The cases were divided in to 3 groups: group 1--diagnosis of struma ovarii was made by cytology and confirmed by ICC (1 case); group 2--diagnosis was suggestive on cytology or cell block and confirmed by ICC staining (4 cases); group 3--on cytologic diagnosis indistinguishable from other cystic ovarian neoplasms (2 cases). Cytologic findings were typically colloid with mosaic pattern, follicles, follicular cells only, sheets of follicular cells, both colloid and follicular cells, proteinaceous background or degenerated epithelial cells indistinguishable from other cystic ovarian neoplasms. CONCLUSION: Cytologic findings of struma ovarii are distinct enough to be suggested intraoperatively, and ICC for TGB or TTF-1 is a valuable tool for preoperative fine needle aspiration biopsy and intraoperative diagnosis of this benign ovarian neoplasm.