Organ histopathological associations with hyperostosis frontalis interna in humans / Asociaciones histopatológicas de órganos con hiperostosis frontal interna en humanos

SUMMARY: An increased thickening of the frontal bone by irregular laminar additions on the inner surface just deep to the dura mater have been known in the archaeological and medical record as hyperostosis frontalis interna (HFI). The distribution of this is idiosyncratically restricted to the frontal and has no known etiology. The prevalence among post-menopausal females and rarity in males suggests that it is hormonally driven. Here we report histopathological findings of particularly hormonally active organs (pituitaries, gonads and liver) from a geriatric cadaveric sample in which HFI is assessed. HFI was present in 50 % of males (7/14) and 95 % (21/22) of females. All males with HFI had testicular atrophy or had testes absent. Males with HFI category C or D had moderate to severe testicular atrophy. Decreased numbers of interstitial cells (Leydig cells) were present in 83.3 % of males with HFI. All but one female (21/22) from this study exhibited evidence of HFI, and ovarian pathologies were unevenly distributed (fibromas in two) and most exhibited signs of being healthy and post-menopausal. Liver pathologies had opposite patterns between the sexes, with more liver pathologies occurring among males without HFI (particularly passive congestion and bile stasis). The only exceptions were that the one case of liver neoplasia was found in a male with HFI and steotosis was found in two cases with HFI and one case without HFI. In females all cases of liver pathologies (steotosis, hepatitis, passive congestion, fibrosis, and bile stasis) were associated with HFI. It appears that gonadal pathology is most closely associated with HFI in males but not females, suggesting that the role of estradiol in this unusual growth of bone in geriatric humans may be worth investigating further.

RESUMEN: Un aumento del engrosamiento del hueso frontal, por adiciones laminares irregulares en la superficie interna, justo en la profundidad de la duramadre, se conoce en los registros arqueológicos y médicos como hiperostosis frontal interna (HFI). La distribución de ésta, se restringe idiosincráticamente al hueso frontal y no tiene etiología conocida. La prevalencia entre las mujeres posmenopáusicas y la rareza en los hombres sugiere que se debe a las hormonas. Aquí informamos los hallazgos histopatológicos de órganos hormonalmente activos (hipófisis, gónadas e hí- gado) de una muestra de cadáveres geriátricos en la que se evaluó HFI. La HFI estuvo presente en el 50 % de los hombres (7/14) y el 95 % (21/22) de las mujeres. Todos los hombres con HFI tenían atrofia testicular o no tenían testículos. En los hombres con HFI categoría C o D se observó atrofia testicular de moderada a grave. Hubo una disminución en el número de células intersticiales (células de Leydig) en el 83,3 % de los hombres con HFI. En 21de 22 mujeres se observó evidencia de HFI, y las patologías ováricas se distribuyeron de manera desigual (fibromas en dos) y la mayoría exhibió signos de estar sana y posmenopáusica. Las patologías hepáticas tenían patrones opuestos entre los sexos, con más patologías hepáticas entre los hombres sin HFI (particularmente congestión pasiva y estasis biliar). Las excepciones fueron que el único caso de neoplasia hepática se encontró en un varón con HFI y se presentó esteatosis en dos casos con HFI y un caso sin HFI. En las mujeres, todos los casos de patologías hepáticas (esteatosis, hepatitis, congestión pasiva, fibrosis y estasis biliar) se asociaron con HFI. Al parecer la patología gonadal está más estrechamente asociada con la HFI en los hombres que en las mujeres, lo que sugiere un rol del estradiol en este crecimiento inusual de hueso en hombres de avanzada edad. Sería importante realizar investigaciones más detalladas precisas respecto a la hiperostosis frontal interna.

Uremic lion face syndrome / Face Leonina na Síndrome Urêmica

Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.

Resumo O distúrbio mineral e ósseo é uma característica comum da doença renal crônica. A síndrome da face leonina é uma complicação rara do hiperparatireoidismo grave em pacientes com doença renal terminal, que tem sido menos relatada devido aos avanços na diálise e tratamento médico na última década. O reconhecimento precoce da deformidade facial característica é crucial para estimular o tratamento precoce e prevenir a desfiguração severa. Os autores apresentam um caso raro de hiperparatireoidismo grave, apresentando síndrome da face leonina e fraturas ósseas.

Presentación de una paciente con síndrome de Morgagni-Stewart-Morel / Presentation of a Patient with Morgagni-Stewart-Morel Syndrome

El síndrome de Morgagni-Stewart-Morel es una rara enfermedad que se caracteriza por hiperostosis frontal interna bilateral asociada a alteraciones metabólicas, psiquiátricas, hipertensión arterial y disfunción de pares craneales de etiología no definida. Se presentó una paciente femenina de 73 años, hipertensa, diabética, obesa, con trastornos psiquiátricos; padeciendo de cefalea, hiposmia e hipoacusia y se constató tomográficamente el engrosamiento frontal interno en relación con el estadio A de la clasificación de Hershkovitz de dicha enfermedad.

Morgagni-Stewart-Morel Syndrome is a rare disease characterized by bilateral hyperostosis frontalis interna associated to metabolic and psychiatric disorders, with hypertension and cranial nerve dysfunction of undefined etiology. A female patient of 73 years, hypertensive, diabetic, obese, was presented with psychiatric disorders; suffering from headache, hyposmia and hearing loss. In the tomographic study a stage A of Hershkovitz classification of the disease was found.

Hyperostosis of the Frontal, Temporal, and Sphenoid Bones: Case Report and Review of Literature.

Hyperostosis of the internal table of the frontal bone is not an uncommon phenomenon. However, such hyperostosis of the temporal and/or sphenoid bone is rarely discussed in the available literature, especially in the absence of meningioma. We report a case of hyperostosis of the frontal, temporal, and sphenoid bones found during routine cadaveric dissection of an 81 year-old female. Histology revealed thickening and increased density of the lamellar bone. The periosteum was unaffected. Possible etiology is discussed.