Fetal-onset Alexander disease with radiological-neuropathological correlation.

Alexander disease is a leukodystrophy caused by mutations in the GFAP gene, primarily affecting the astrocytes. This report describes the prenatal and post-mortem neuroimaging findings in a case of genetically confirmed, fetal-onset Alexander disease with pathological correlation after termination of pregnancy. The additional value of fetal brain magnetic resonance imaging in the third trimester as a complementary evaluation tool to neurosonography is shown for suspected cases of fetal-onset Alexander disease. Diffuse signal abnormalities of the periventricular white matter in association with thickening of the fornix and optic chiasm can point towards the diagnosis. Furthermore, the presence of atypical imaging findings such as microcephaly and cortical folding abnormalities in this case broadens our understanding of the phenotypic variability of Alexander disease.

Tophaceous Gout of the Middle Ear.

Tophaceous gout can rarely present in the middle ear as a mass-like lesion, causing conductive hearing loss. Noncontrast high-resolution computed tomography (HRCT) of the temporal bone plays a significant role in the diagnosis. Awareness of this condition among radiologists is important since it presents a distinctive appearance on HRCT. We present a case of tophaceous gout of the middle ear diagnosed with photon-counting computed tomography (PCCT). Teaching point: The presence of a partially calcified mass with a semolina-like appearance within the middle ear is highly suggestive of tophaceous gout, even in the presence of normal serum uric acid levels.

Residual Splenic Volume after Main Splenic Artery Embolization is Independent of the Underlying Disease.

PURPOSE: To assess the safety and efficacy of main splenic artery embolization. To assess the potential difference post-embolization of the residual splenic volume in patients embolized for trauma versus those embolized for (pseudo)aneurysms. MATERIALS AND METHODS: A retrospective analysis was performed on a cohort of 65 patients (36 males) who underwent pre- and post-embolization computed tomography. Patients' demographics, pre- and post-interventional medical and radiological data were gathered. Splenic volume calculations were semi-automatically performed via a workstation. Patients with splenic aneurysms or pseudoaneurysms of the main splenic artery (group 1) were compared to those with splenic rupture (group 2) using Wilcoxon rank tests. RESULTS: The main indications for splenic artery embolization were splenic rupture (n = 22; 34%) and splenic pseudoaneurysm (n = 19; 29%). The technical success rate was n = 63; 97%. The procedure-related complication rate was n = 7; 11%, including abscess formation (n = 5; 8%), re-bleeding (n = 1; 1.5 %) and pseudoaneurysm re-opening (n = 1; 1.5%). The overall 30-day mortality was n = 7; 11%.Median follow-up for groups 1 and 2 was 1163 days (61-3946 days) and 702 days (43-2095 days) respectively. When processable (n = 23), the splenic volume in group 1 (n = 7) was 311 cm3 and 257 cm3 (p = 0.1591) before and after embolization respectively, and in group 2 (n = 16) it was 261 cm3 and 215 cm3 (p = 0.4688), respectively. CONCLUSIONS: Main splenic artery embolization is efficacious, with low procedure-related complication and 30-day mortality rates. No significant differences in residual post-embolization splenic volume were found between patients treated for splenic rupture versus those treated for splenic arterial (pseudo)aneurysm.