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Erschienen in:
02.05.2023 | Paediatric Neuroradiology
Increased pituitary volumes in patients with Sanfilippo syndrome (mucopolysaccharidosis type 3, MPS III)
Erschienen in: Neuroradiology | Ausgabe 9/2023
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Purpose
To evaluate apparent pituitary gland enlargement in
patients with Sanfilippo syndrome observed at our institution.
Methods
Twelve
patients with Sanfilippo syndrome with brain MRI were studied. Anterior,
posterior, and whole pituitary volumes were estimated using the prolate
ellipsoid volume calculation method (π/6 × L × W × H). Convexity along the upper
pituitary margin (Elster’s grade) was also measured. These values were compared
to two age- and sex-matched groups (normal controls and patients with Hurler
syndrome) using one-way ANOVA followed by Tukey’s post hoc analysis for multiple
comparisons.
Results
In the
Sanfilippo cohort, the mean whole pituitary volume was 529.9 mm, the mean
anterior pituitary volume was 333.4 mm, and the mean posterior pituitary volume
was 59.1 mm with Elster’s grade of 4.2. In the control cohort, the mean whole
pituitary volume was 217.4 mm, the mean anterior pituitary volume was 154.8 mm,
and the mean posterior pituitary volume was 28.4 mm with Elster’s grade of 2.5.
In the Hurler syndrome cohort, the mean whole pituitary volume was 310.0 mm,
the mean anterior pituitary volume was 178.2 mm, and the mean posterior
pituitary volume was 35.4 mm with Elster’s grade of 3.5.
Conclusion
In our
cohort of patients with Sanfilippo syndrome, whole, anterior, and posterior
pituitary volumes and degree of convexity along the upper pituitary border were
all significantly greater than controls. The cause of these morphological
changes is unclear, as is clinical correlation of the findings.