Multidisciplinary care of fetal vein of Galen arteriovenous malformation diagnosed via Doppler ultrasound and magnetic resonance imaging: A case report.

Fetal vein of Galen malformation (VOGM) represents a rare congenital anomaly affecting the fetal cerebral vasculature. A 27-year-old Middle Eastern woman was referred due to intrauterine growth restriction (IUGR) and fetal cardiac anomalies identified at 35 weeks of gestation. The diagnosis of fetal VOGM with dilated neck vessels was established through a combination of color Doppler ultrasonography and magnetic resonance imaging. A multidisciplinary approach involving maternal-fetal medicine, neonatology, pediatric cardiology, and interventional radiology was implemented. Given the grave prognosis for the baby, the patient received comprehensive counseling. Subsequent monitoring revealed a non-reassuring fetal heart trace, prompting the decision to perform a cesarean section. The newborn, a girl, was admitted to the neonatal intensive care unit for further management but she died shortly thereafter, with heart failure and intracranial hemorrhage identified as the probable causes of death. In summary, the diagnosis and management of VOGMs demand specialized expertise and a collaborative, multidisciplinary approach to optimize patient care and outcomes.

Primary anterior visual pathway germinoma in a 13-year-old boy: A case report.

Background: Primary optic nerve and chiasmal germinomas are very rare. These lesions can commonly be mistaken for optic pathway gliomas based on imaging alone. It is radiosensitive and cured in most of the cases. Case Description: We report a rare case of a 13-year-old boy with primary bilateral optic nerves and chiasmal germinoma who underwent partial surgical resection followed by radiotherapy. Follow-up brain imaging after two months post-radiotherapy showed interval regression of the tumor. Our literature review identified that 12 reported cases of primary anterior visual pathway germinoma had been reported to regress significantly post-radiotherapy alone or with chemotherapy. Conclusion: Histologic correlation is essential for appropriate treatment, alleviating symptoms, and avoiding irreversible vision loss.

Development and Validation of a Pediatric MRI-Based Perianal Crohn Disease (PEMPAC) Index-A Report from the ImageKids Study.

BACKGROUND: As part of the prospective multicenter ImageKids study, we aimed to develop and validate the pediatric MRI-based perianal Crohn disease (PEMPAC) index. METHODS: Children with Crohn disease with any clinical perianal findings underwent pelvic magnetic resonance imaging at 21 sites globally. The site radiologist and 2 central radiologists provided a radiologist global assessment (RGA) on a 100 mm visual analog scale and scored the items selected by a Delphi group of 35 international radiologists and a review of the literature. Two weighted multivariable statistical models were constructed against the RGA. RESULTS: Eighty children underwent 95 pelvic magnetic resonance imaging scans; 64 were used for derivation and 31 for validation. The following items were included: fistula number, location, length and T2 hyperintensity; abscesses; rectal wall involvement; and fistula branching. The last 2 items had negative beta scores and thus were excluded in a contending basic model. In the validation cohort, the full and the basic models had the same strong correlation with the RGA (r = 0.75; P < 0.01) and with the adult Van Assche index (VAI; r = 0.93 and 0.92; P < 0.001). The correlation of the VAI with the RGA was similar (r = 0.77; P < 0.01). The 2 models and the VAI had a similar ability to differentiate remission from active disease (area under the receiver operating characteristic curve, 0.91-0.94). The PEMPAC index had good responsiveness to change (area under the receiver operating characteristic curve, 0.89; 95% confidence interval, 0.69-1.00). CONCLUSIONS: Using a blended judgmental and mathematical approach, we developed and validated an index for quantifying the severity of perianal disease in children with CD. The adult VAI may also be used with confidence in children.

Can MR enterography screen for perianal disease in pediatric inflammatory bowel disease?

BACKGROUND: Pediatric Crohn's disease is associated with perianal disease (PAD). Magnetic resonance enterography (MRE) assesses small bowel involvement in pediatric inflammatory bowel disease (PIBD). Pelvic MRI (P-MRI) is the gold standard for assessing PAD. PURPOSE: To determine if MRE can accurately detect PAD in PIBD, distinguishing perianal fistulae (PAF) from perianal abscesses (PAA), referenced against P-MRI. STUDY TYPE: Retrospective. POPULATION: Seventy-seven PIBD patients, 27 females (mean age 14.1 years), with P-MRI and MRE within 6 months. FIELD STRENGTH/SEQUENCE: 1.5T and 3T; P-MRI: sagittal fat suppressed (FS) T2 fast spin-echo (FSE), coronal short tau inversion recovery, axial T1 FSE, coronal and axial postcontrast FS T1 FSE; MRE: coronal balanced steady-state free-precession (SSFP), coronal cine SSFP, coronal and axial single-shot T2 FS, axial SSFP, coronal ultrafast 3D T1 -weighted gradient echo FS (3D T1 GE), axial diffusion-weighted imaging, coronal and axial postcontrast 3D T1 GE FS. ASSESSMENT: Two radiologists independently, then by consensus, assessed randomized MRI exams, recording PAF number, location, and length; and PAA number, location, length, and volume. Sensitivity analysis used clinical disease as the gold standard, calculated separately for P-MRI and MRE. STATISTICAL TESTS: Comparing MRE and P-MRI consensus data, sensitivity, specificity, positive, and negative predictive values (P/NPV) were calculated. Inter- and intrareader reliability were assessed using kappa statistics. RESULTS: P-MRI and MRE were paired, detecting PAD in 73 patients, PAF in 63, and PAA in 31 P-MRI. MRE sensitivities, specificities, PPV, and NPV were: PAD 82%, 100%, 100%, 23%; PAF 74%, 71%, 92%, 38%; PAA 51%, 85%, 69%, 72%; clinical 82%, 22%, 37%, 69%; clinical P-MRI 96%, 8%, 37%, 80%. MRE interreader agreement for PAD was moderate (kappa = 0.51 [0.29-0.73]), fair for PAF and PAA. DATA CONCLUSION: Using a standard technique, MRE can detect PAD with high specificity and moderate sensitivity in PIBD, missing some PAF and small PAA. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1638-1645.

Accuracy and interobserver agreement of diffusion-weighted imaging in pediatric inflammatory bowel disease.

PURPOSE: To determine interobserver agreement (IOA) and accuracy of conventional MR enterography (MRE), qualitative diffusion, and apparent diffusion coefficient (ADC) values for detecting clinically active inflammation. METHODS: MREs in 57 consecutive children with suspected inflammatory bowel disease were retrospectively reviewed. RESULTS: Substantial IOA for conventional MRE (kappa=0.65) and qualitative diffusion (kappa=0.64), but fair to good IOA for ADC, (intra-class coefficient=0.63) were seen. Conventional MRE detected active clinical inflammation well (area under curve [AUC] 0.725), while qualitative diffusion and ADC did not perform well (AUC=0.572 and 0.461, respectively). CONCLUSION: DWI can be helpful in diagnosing inflammatory bowel disease but does not perform well in identifying those with active inflammation.

Diffusion-weighted imaging in pediatric body MR imaging: principles, technique, and emerging applications.

Diffusion-weighted (DW) imaging is an emerging technique in body imaging that provides indirect information about the microenvironment of tissues and lesions and helps detect, characterize, and follow up abnormalities. Two main challenges in the application of DW imaging to body imaging are the decreased signal-to-noise ratio of body tissues compared with neuronal tissues due to their shorter T2 relaxation time, and image degradation related to physiologic motion (eg, respiratory motion). Use of smaller b values and newer motion compensation techniques allow the evaluation of anatomic structures with DW imaging. DW imaging can be performed as a breath-hold sequence or a free-breathing sequence with or without respiratory triggering. Depending on the mobility of water molecules in their microenvironment, different normal tissues have different signals at DW imaging. Some normal tissues (eg, lymph nodes, spleen, ovarian and testicular parenchyma) are diffusion restricted, whereas others (eg, gallbladder, corpora cavernosa, endometrium, cartilage) show T2 shine-through. Epiphyses that contain fatty marrow and bone cortex appear dark on both DW images and apparent diffusion coefficient maps. Current and emerging applications of DW imaging in pediatric body imaging include tumor detection and characterization, assessment of therapy response and monitoring of tumors, noninvasive detection and grading of liver fibrosis and cirrhosis, detection of abscesses, and evaluation of inflammatory bowel disease.