An unusual case of cutaneous and hypothalamic histiocytosis X (HX) is reported. The hypothalamic involvement occurred as a tumor that mimicked a chiasm glioma on computed tomography angioscanning. Magnetic resonance imaging after gadolinium injection localized the tumor within the third ventricle floor. The HX origin of the tumor was confirmed by histological examination of hypothalamic biopsies obtained by transventricular endoscopy. The results of endocrine evaluation were consistent with anterior panhypopituitarism resulting from a multiple releasing-hormone secretory defect, but there was no diabetes insipidus. This unusual endocrine aspect has not been previously described in the field of hypothalamic HX. Lastly, the tumor was insensitive to low dose megavoltage radiation therapy. This unusual case stresses the superiority of magnetic resonance imaging over computed tomography scanning in the assessment of suprasellar tumors and emphasizes the usefulness of transventricular endoscopy in these cases.
Objectives
To quantify and model normal foetal lung and liver elasticities between 24 and 39 weeks of gestation (WG) using two-dimensional shear wave elastography (2D-SWE). To assess the impact of the distance between the probe and the target organ on the estimation of elasticity values.
Methods
Measurements of normal foetal lungs and liver elasticity were prospectively repeated monthly between 24 and 39 WG in 72 foetuses using 2D-SWE. Elasticity was quantified in the proximal lung and in the region inside the hepatic portal sinus. The distance between the probe and the target organ was recorded. Trajectories representing foetal lung and liver maturation from at least 3 measurements over time were modelled.
Results
The average elasticity for the lung and liver was significantly different from 24 WG to 36 WG (p < 0.01). Liver elasticity increased during gestation (3.86 kPa at 24 WG versus 4.45 kPa at 39 WG). From 24 WG to 32 WG, lung elasticity gradually increased (4.12kPa at 24 WG, 4.91kPa at 28 WG, 5.03kPa at 32 WG, p < 0.002). After 32 WG, lung elasticity decreased to 4.54kPa at 36 WG and 3.94kPa at 39 WG. The dispersion of the average elasticity values was greater for the lung than for the liver (p < 0.0001). Variation in the elasticity values was less important for the liver than for the lung. The values were considered valid and repeatable except for a probe-lung distance above 8cm.
Conclusion
Foetal lung and liver elasticities evolve differently through gestation. This could reflect the tissue maturation of both organs during gestation.
Trial registration
clinicaltrials.gov identifier: NCT03834805
Key Points
• Prenatal quantification of foetal lung elasticity using 2D shear wave elastography could be a new prenatal parameter for exploring foetal lung maturity.
• Liver elasticity increased progressively from 24 weeks of gestation (WG) to 39 WG, while lung elasticity increased first between 24 and 32 WG and then decreased after 32 WG.
• The values of elasticity are considered valid and repeatable except for a probe-lung distance above 8cm.
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