Ruptured intracranial aneurysm in an adolescent with Alport's syndrome--a new expression of type IV collagenopathy: case report.

BACKGROUND: Aneurysmal subarachnoid hemorrhage is uncommon in the pediatric and adolescent age groups. Collagenopathies, particularly of collagen type III and IV, have been recognized in the pathogenesis of intracranial (i.c.) aneurysms in recent studies of cerebrovascular immunoreactivity and the molecular architecture of the walls of cerebral blood vessels. Alport's syndrome is a genetic disease characterized by imperfect synthesis of type IV collagen leading to sensorineural hearing loss and hematuria. The hematuria results from an abnormal synthesis of the type IV collagen of the glomerular basement membrane. CASE DESCRIPTION: After a motor vehicle accident, this 14-year-old male driver presented with diffuse subarachnoid hemorrhage confirmed by computerized tomography. Subsequent cerebral angiography revealed a left carotid artery bifurcation aneurysm. A retrospective review of the patient's history disclosed renal biopsy-proven hereditary Alport's syndrome. The patient underwent left pterional craniotomy and clipping of the aneurysm, which had clearly ruptured. CONCLUSIONS: The authors present the first clinical observation of a ruptured cerebral aneurysm in an adolescent male with Alport's syndrome. In this case the type IV collagenopathy of Alport's syndrome may have contributed to the genesis of this adolescent's aneurysm. Intracranial aneurysms may be more common in the population of collagenopathies than previously suspected and non-invasive screening may be appropriate.

Transient hyponatriemia complicated by seizures after endoscopic third ventriculostomy.

We present an infant who underwent endoscopic third ventriculostomy due to symptomatic hydrocephalus secondary to aqueductal stenosis. This is the first reported case of inappropriate secretion of antidiuretic hormone complicated by hyponatriemia and seizures following endoscopic surgery. The possibility of such a neuroendoscopic complication should alert neurosurgeons and close observation of serum electrolytes is highly recommended in the acute postoperative period, particularly in infants.

Falcotentorial plasmacytoma. Case report.

Intracranial solitary plasmacytomas are extremely rare tumors and are often misdiagnosed preoperatively. The authors report the successful treatment of a patient who harbored such a tumor involving both the falx and tentorium; this is the second case reported. A 59-year-old woman suffered from a seizure disorder due to a falcotentorial lesion, which had been identified 3 years earlier and was thought at the time to be an en plaque meningioma. Most recently, the patient presented with symptoms of increased intracranial pressure and hemiparesis. Computerized tomography and magnetic resonance imaging of her head revealed progressive growth of the tumor. The patient underwent partial resection of the tumor and chemo- and radiation therapies. Intracranial plasmacytomas must always be included in a differential diagnosis because potential complete cure can be achieved using fairly conservative treatment modalities.

Failure of hydroxyapatite cement to set in repair of a cranial defect: case report.

OBJECTIVE AND IMPORTANCE: Hydroxyapatite cement, a new biomaterial that is being marketed as a method for reconstructing cranial defects, offers many advantages. We document, herein, the complete dissolution and failure of this material to set in a surgically dry field, under optimal conditions, an occurrence that has not been previously reported. CLINICAL PRESENTATION: Hydroxyapatite cement was used for reconstruction of a frontal bone defect secondary to a traumatic depressed cranial fracture in a 9-year-old male patient. At the time of suture removal on postoperative Day 6, we observed serous discharge from the wound, a reappearance of the cranial defect, and brain pulsations visible subcutaneously. INTERVENTION: The patient was returned to the operating room, at which time we learned that the hydroxyapatite cement had migrated out of the defect; small concretions of the cement were scattered throughout the subgaleal space. The concretions of cement in the subgaleal space and the small amount of cement remaining in the defect were removed, and titanium mesh was used. An excellent cosmetic result was achieved. CONCLUSION: Although offering many advantages, hydroxyapatite cement does carry a risk of failure to set, despite optimal technique. Causes for failure to set, as well as possible modifications in the use of material and technique, are discussed.