ABSTRACT
Vanishing bile duct syndrome (VBDS) is an acquired condition characterized by the destruction and loss of intrahepatic bile ducts resulting in cholestasis. VBDS has been described in various conditions including neoplastic and immunologic disorders, infections, hepatic ischemia, and drug toxicity. The diagnosis is confirmed by liver biopsy revealing the loss of interlobular bile ducts in greater than 50% of portal tracts. Prognosis is variable and often unpredictable but appears to be influenced by the etiology of bile duct destruction and overall patient health. VBDS has been described as a rare paraneoplastic process in patients with Hodgkin lymphoma. This case describes a 26-year-old female who presented with a neck mass, jaundice, and pruritus. Initial workup revealed direct hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and elevated international normalized ratio. She went on to receive a diagnosis of stage II classical Hodgkin lymphoma, nodular sclerosing subtype, and biopsy-proven VBDS. Over the course of chemotherapy, complete metabolic resolution of Hodgkin lymphoma and complete normalization of bilirubin were achieved. She was given gemcitabine and cyclophosphamide as a liver sparing regimen initially with some improvement in liver function tests and a reduction in lymph node volumes. She received six cycles of adriamycin/bleomycin/vinblastine/dacarbazine (ABVD) with complete remission attained after four cycles by positron emission tomography/computed tomography criteria. This report illustrates asafe chemotherapy regimen in the presence of marked liver dysfunction. Workup for VBDS including liver biopsy should be pursued in Hodgkin lymphoma patients with evidence of cholestasis in the absence of extrahepatic bile duct damage or other known etiology of liver injury.
ABSTRACT
BACKGROUND: The identification of anatomic landmarks in endoscopic skull base or revision sinus surgery can be challenging. Normal anatomy is significantly altered with many paranasal tumors. Traditional endoscopic surgical landmarks extrapolated from inflammatory disease, such as the superior turbinate, may have been previously removed or involved in pathology. A frequently used rule to enter the sphenoid, "stay below or at the level of the orbital floor as dissection proceeds posteriorly and one will avoid the skull base," is assessed anatomically. METHODS: The maxillary sinus roof height, relative to the nasal floor, was assessed as an operative landmark. Computed tomography (CT) performed on paranasal sinuses was studied. The relative height, ratio, and proportions of the maxillary sinus, ethmoid roof, cribriform fossa, and sphenoid planum were measured using computerized assessments. RESULTS: Three hundred paranasal sinus systems were evaluated. The roof of the maxillary sinus was below the level of the skull base in 100% relative to the cribriform and 100% relative to the sphenoid planum. The mean distance of the maxillary roof below the skull base was 10.1 +/- 2.7 mm for the cribriform and 11.0 +/- 2.9 mm for the sphenoid. CONCLUSION: The maxillary sinus roof can be used as a robust landmark to allow safe dissection and debulking of pathology. Pathology removal can proceed posterior with this landmark to enable a safe entry to the sphenoid sinus, and thus the true skull base, when normal structures such as the superior turbinate and ostium are not available.
