Most cases with chronic subdural hematoma (CSDH) are treated by simple irrigation and drainage, then more than eighty percent of them result in good recovery. But we sometimes encounter intractable cases with hematoma re-collection, which is considered of repeated bleeding from macrocapillary in the hematoma capsule. Embolization of the middle meningeal artery (MMA) is considered to be useful to eliminate the blood supply to this structure. The authors experienced seven cases of intractable CSDH treated by MMA embolization and no recurrence took place in all cases for up to 15 months. Endovascular treatment may be a good alternative modality for recurrent CSDH.
Hajdu-Cheney syndrome is a rare congenital disease with acro-osteolysis, osteoporotic changes of the spine and long bones of extremities and marked basilar invagination with an unusually deformed skull. Magnetic resonance imaging of a 32-year-old male revealed the deformed skull and almost horizontal basal angle and the elongated and upwardly shifted brain stem caused by the tip of the odontoid process of the second cervical vertebra invaginating the base of the skull. In addition there were atrophic pituitary gland, widely open sella turcica and symmetrical fluid collections along the optic nerve sheath.
Morphometric studies at autopsy of a patient with sensory neuropathy associated with small-cell lung carcinoma showed preferential loss of large-diameter sensory nerve cell bodies (fifth lumbar dorsal root ganglion), marked decrease of large myelinated fibers in the dorsal root and sural nerve, and almost total loss of myelinated fibers in the fasciculus gracilis.
The case of a 24-year-old man with Hadju-Cheney syndrome is reported. No similar disease occurred in his family. Inverted and bullous tips of fingers were noted at age 9 and protruded occipital bone at age 10. He complained on left facial spasm and facial tics for 1 year. Examination revealed a man of short stature, with a brachycephalic skull and hypertelorism. Radiologically there was an extreme degree of basilar impression with the basal angle of 180 degrees, demineralized spinal vertebrae, and acro-osteolysis. Both bone and CT scans demonstrated the abnormalities of the skull clearly. The basilar artery ran almost horizontally on angiography. He gradually deteriorated neurologically with bulbar, pyramidal and cerebellar signs and symptoms. Review of the literature revealed at least ten non-familial and nine familial cases of this disorder. It is considered that this syndrome is a genetically determined generalized dysplastic bone disorder.
Background: Hepatic inflammatory pseudotumor (IPT) is considered to be benign in biological behavior, and its malignant transformation is extremely rare. There has only been one published case of primary hepatic lymphoma complicated by hepatic IPT.Case presentation: A 73-year-old man presented with obstructive jaundice and a pancreatic head mass. Histology of the mass revealed chronic pancreatitis with lymphoid follicle formation, leading to a diagnosis of a suspicion of follicular pancreatitis. After a choledochojejunostomy, a hepatic tumor was detected, and a biopsy revealed lymphoplasmacytic infiltration. Immunohistochemistry confirmed the polyclonal nature of lymphoplasma cells, indicative of an IPT. The hepatic tumor disappeared during follow-up, but the patient exhibited a high fever related to tumor recurrence. A biopsy revealed the co-existence of a diffuse large B-cell lymphoma and an IPT. IgG4-related disease was excluded because storiform fibrosis, obliterative phlebitis, and a significant increase in IgG4-immunoreactive cells were absent in all investigated tissues. The tumor completely disappeared after chemotherapy.Conclusion: Careful observation is necessary in this kind of situation because the presence of a hepatic IPT may represent an increased risk of malignant transformation.Abbreviations: IPT: inflammatory pseudotumor; DLBCL: diffuse large B-cell lymphoma; CT: computed tomography; LDH: lactate dehydrogenase; sIL-2R: soluble interleukin-2 receptor; EBER: Epstein-Barr virus-encoded small RNA; R-CHOP: rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone
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