Polyglandular endocrine syndrome characterized by the
classic triad of (a) hyperostosis frontalis interna, (b) adipositas, and (c)
virilism and hirsutism. A peculiar, noninflammatory, usually benign
osteopathy with symmetrical thickening of the frontal, parietal, or
occipital bones as a result of deposits on the internal aspects of the
squama frontalis. Other clinical features include menstrual disorders, virilism,
hirsutism, mental disorders, fatigue, somnolence, visual disorders, vertigo,
tinnitus, obesity, polyphagia, polydipsia, polyuria, loss of sense of smell,
decreased glucose tolerance, convulsions, and involvement of the second,
fifth, and seventh cranial nerves with hemiplegia and hemiparesis.
Hyperostosis Frontalis Interna; Morel Syndrome.
The frontal bone lesions associated with obesity and
virilism were first described in 1719 by Giovanni Battista Morgagni, an
Italian anatomist. In 1928, Douglas Hunt Stewart emphasized the association
with obesity. In 1930, Ferdinand Morel emphasized the accompanying menstrual
disturbance, amenorrhea, and impotence. The term Morgagni triad was
introduced by Folke Henschen in 1937, but the term Morgagni-Stewart-Morel syndrome is now commonly used.
Extremely uncommon. Occurs almost exclusively in
females. Age of onset average about 45 years; incidence in females is
Probably autosomal dominant. No reported cases
of male-to-male transmission.
Unknown. The underlying defect causing excess bone
formation appears to be different from normal mechanisms. The biochemical
response to calcitriol and bone biopsy findings (increased number of
osteoblasts) has been shown to be different. Many of the endocrine features
may be related to hyperprolactinemia, which is frequently found in these
Clinical supported by investigations and imaging.
Hyperphosphatasemia is common. Radiologic, CT, and MRI scans confirm bony
overgrowth. Audiology confirms changes in hearing.
May be almost symptomless and is found
incidentally. Skull: Hyperostosis frontalis interna; as a result of progressive
overgrowth, intracranial pressure may increase and lead to brain and nerve
compression, cranial nerve palsies, and seizures; choanal stenosis;
glaucoma. Endocrine: Obesity, hyperphosphatasemia, hyperprolactinemia, galactorrhea,
diabetes mellitus, menstrual irregularity. Neuropsychiatric: “Treatable dementia.” Skin:
Assessment of severity of condition;
exclude raised intracranial pressure and neurologic abnormalities, including
seizures and medications. Assessment of airway and evidence of choanal
stenosis. Assessment of endocrine status and optimization of abnormalities
with endocrine consultation and hormonal manipulation.
Usually minimal. Nasal intubation may be
contraindicated. General considerations for obesity, including problems such
as reflux, intravenous access, monitoring, positioning, and increased risk
for sleep apnea syndrome. The possibility of raised intracranial pressure may
modify the anesthetic technique.
May require intraoperative endocrine
manipulation, such as insulin and glucose monitoring.
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