Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.

Kaposiform lymphangiomatosis (KLA) is a life-threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease. KLA is a lymphatic anomaly and significant advancements have been made in understanding its pathogenesis and etiology since its first description in 2014. This review provides multidisciplinary, comprehensive, and state-of-the-art information on KLA patient presentation, diagnostic imaging, pathology, organ involvement, genetics, and pathogenesis. Finally, we describe current therapeutic approaches, important areas for research, and challenges faced by patients and their families. Further insights into the pathogenesis of KLA may advance our understanding of other vascular anomalies given that similar signaling pathways may be involved.

Utilization of 3-T fetal magnetic resonance imaging in clinical practice: a single-institution experience.

BACKGROUND: As the safety and efficacy of fetal magnetic resonance imaging (MRI) at 3 tesla (T) continues to evolve, understanding its potential benefits and limitations is becoming increasingly important. OBJECTIVE: We aim to compare the image quality of fetal MRI between 1.5 T and 3 T in routine clinical practice. MATERIALS AND METHODS: Fetal MRIs performed at 3 T between Jan. 1, 2019, and Dec. 31, 2019, at our institution were retrospectively reviewed by four fellowship-trained subspecialty radiologists. Imaging quality by system, sequence and artifacts were compared with matched controls at 1.5 T and rated using a modified Likert scale. RESULTS: Thirty-three fetal MRIs at 3 T were reviewed, and a control group of studies for the same clinical indication and equivalent gestational age were selected for comparison. Two of the four radiologists preferred 3-T image quality of the brain with slight agreement among the four reviewers (k=0.19, P=0.01). Three of the four radiologists had no preference for 1.5 T vs. 3 T in the majority of cases in evaluating the chest and abdomen. In the overall assessment, 3 T was preferred in less than half of cases by all four radiologists (k=0.07, P=0.26). In the evaluation of standing wave, moire fringe and magnetic susceptibility artifacts, 3 T was not preferred in the majority of studies by all four radiologists. Total exam time was significantly longer in the 3-T fetal MRIs (75.0±15.1 min) compared to the 1.5-T fetal MRIs (55.5±13.3 min, P<0.001). CONCLUSION: While 3 T is a feasible alternative to 1.5 T for fetal MRI, the increased artifacts and longer exam times observed at 3 T without clear improvement in overall image quality make 1.5 T preferable for fetal MRI in routine clinical practice.