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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 approximately 90%.

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 cases.

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: Hypertrichosis.

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.

Richards A, Brain C, Dillon MJ, et al: Craniometaphyseal and craniodiaphyseal dysplasia, head and neck manifestations and management. J Laryngol Otol 110:328, 1996.  [PubMed: 8733453]
Thurnau GR, Stein SA, Schaefer GB, et al: Management and outcome of two pregnancies in a woman with craniodiaphyseal dysplasia. Am J Perinatol 8:56, 1991.  [PubMed: 1987972]

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