Ruptured cerebral artery aneurysm and bacterial meningitis in a man with osteogenesis imperfecta.

This report describes a 38-year-old man with osteogenesis imperfecta who died of a ruptured cerebral artery aneurysm and bacterial meningitis. He had multiple long bone fractures in the past, and approximately 4 months before death, he had surgery to relieve symptoms of basilar impression. The surgery was complicated by a postoperative wound infection. For the next 4 months, he had intermittent headaches and vomiting. He was found dead in his bed at home. At autopsy, he had a ruptured anterior communicating artery aneurysm and bacterial meningitis. Cerebrospinal fluid and blood cultures had growth of Staphylococcus aureus. Osteogenesis imperfecta is a disorder of type I collagen. Type I collagen is present in many tissues, including blood vessels. The etiology of cerebral artery aneurysm formation is multifactorial. Some patients with cerebral artery aneurysms have been shown to have abnormalities in type III collagen. There has not been a reported relationship made between abnormalities in type I collagen and aneurysms. Meningitis can also result in cerebral artery aneurysms, but they are usually due to Aspergillus or Mycobacterium species. The case we report is unique; cerebral artery aneurysm formation may have been due to osteogenesis imperfecta and/or bacterial meningitis.

Sudden death in an infant resulting from torsion of the uterine adnexa.

A 2-month-old infant girl died suddenly as a result of torsion of the uterine adnexa. The infant was found unresponsive in bed and was pronounced dead shortly after her arrival at a hospital. There were no antecedent signs of illness. At autopsy, the right ovary and right fallopian tube were twisted and were dark purple, swollen, and necrotic. The right ovary was enlarged by a follicle cyst 4 cm in diameter, which likely precipitated the torsion. The mechanism of death was unclear but may have resulted from the release of cytokines produced in response to necrotic adnexal tissue. Fatal uterine adnexal torsion has been reported rarely in infants; in all those cases there were antecedent symptoms. Torsion of the uterine adnexa should be included in the differential diagnosis of sudden death in infancy.

July 2001: 58-year-old man with a temporal bone mass.

The July 2001 Case of the Month (COM). A 58-year-old man with right ear hearing loss since childhood presented with a two year history of dizziness and vertigo. Neuroradiological studies showed a large mass arising from the petrous portion of the temporal bone. The lesion was resected and microscopic examination revealed a cholesterol granuloma with a small component of cholesteatoma. It is important to distinguish between cholesterol granuloma and cholesteatoma because of treatment differences. However, these two entities can occasionally be seen together and rare giant variants have been described.