Cerebral cortex maldevelopment in syndromic craniosynostosis.

Aim: To assess the relationship of surface area of the cerebral cortex to intracranial volume (ICV) in syndromic craniosynostosis.

Method: Records of 140 patients (64 males, 76 females; mean age 8y 6mo [SD 5y 6mo], range 1y 2mo-24y 2mo) with syndromic craniosynostosis were reviewed to include clinical and imaging data. Two hundred and three total magnetic resonance imaging (MRI) scans were evaluated in this study (148 patients with fibroblast growth factor receptor [FGFR], 19 patients with TWIST1, and 36 controls).

Saethre-Chotzen syndrome: long-term outcome of a syndrome-specific management protocol.

Aim: To assess the long-term outcomes of our management protocol for Saethre-Chotzen syndrome, which includes one-stage fronto-orbital advancement.

Method: All patients born with Saethre-Chotzen syndrome between January 1992 and March 2017 were included. Evaluated parameters included occipital frontal head circumference (OFC), fundoscopy, neuroimaging (ventricular size, tonsillar position, and the presence of collaterals/an abnormal transverse sinus), polysomnography, and ophthalmological outcomes.

The effect of early fusion of the spheno-occipital synchondrosis on midface hypoplasia and obstructive sleep apnea in patients with Crouzon syndrome.

Introduction: The investigators hypothesized that patients with Crouzon syndrome and premature fusion of the spheno-occipital synchondrosis (SOS) more often have, or have more severe midface hypoplasia and obstructive sleep apnea (OSA).

Methods: A prospective cohort study was performed among patients with Crouzon syndrome to analyze SOS closure, midface hypoplasia represented by the sella-nasion angle (SNA) and OSA.

Results: Forty patients were included in whom the OSA-prevalence was 65%.

Imaging of the Sella and Parasellar Region in the Pediatric Population.

Masses in the sella and parasellar region comprise about 10% of all pediatric brain tumors but type and frequency differs from those in adults. Imaging is critical for diagnosis and characterization of these lesions. By assessing the site of origin, signal and contrast enhancement characteristics, and the presence or absence of characteristic patterns, differential diagnosis can narrow the possibilities.

Lumps and Bumps of the Neck in Children-Neuroimaging of Congenital and Acquired Lesions.

Neck masses present as palpable lumps and bumps in children with acquired lesions more common than congenital ones. Assessment of the anatomical site of origin, signal, and contrast enhancement characteristics may help define the etiology of the lesions, eg, developmental, inflammatory, vascular, or neoplastic. The age of the patient along with detailed clinical history and physical exam findings are important element to narrow down the differential diagnosis.

Skeletal maturity of children with multiple osteochondromas: is diminished stature due to a systemic influence?

Background: Multiple ostechondromas (MO) is an autosomal dominant inherited disease caused by mutated exostosin genes. It mostly affects the long bones and can lead to growth disturbances, especially disproportionate short stature. Both the local effect on growth plates and the systemic influence of the gene disorder on growth mechanisms might explain the diminished stature.

Dynamic contrast-enhanced MRI assessment of hyperemic fractional microvascular blood plasma volume in peripheral arterial disease: initial findings.

Objectives: The aim of the current study was to describe a method that assesses the hyperemic microvascular blood plasma volume of the calf musculature. The reversibly albumin binding contrast agent gadofosveset was used in dynamic contrast-enhanced magnetic resonance imaging (DCE MRI) to assess the microvascular status in patients with peripheral arterial disease (PAD) and healthy controls. In addition, the reproducibility of this method in healthy controls was determined.

MRI of arterial flow reserve in patients with intermittent claudication: feasibility and initial experience.

Objectives: The aim of this work was to develop a MRI method to determine arterial flow reserve in patients with intermittent claudication and to investigate whether this method can discriminate between patients and healthy control subjects.

Methods: Ten consecutive patients with intermittent claudication and 10 healthy control subjects were included. All subjects underwent vector cardiography triggered quantitative 2D cine MR phase-contrast imaging to obtain flow waveforms of the popliteal artery at rest and during reactive hyperemia.