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During the Eastern Section meeting of the Triologic Society, New York City, Jan 29, 1988, Drs Mark Levenson, Leslie Michaels, Simon Parisier, and Charles Juarbe presented their observations regarding congenital cholesteatomas in children and suggested that this entity results from an embryologic epidermoid rest present in the fetal mesotympanum.
The clinical findings in 37 children with congenital cholesteatoma of the middle ear were detailed in their presentation. The authors hypothesized that congenital cholesteatoma originates from an epidermoid formation that has been identified histologically in the anterior/superior lateral tympanic cavity adjacent to the anterior anulus during fetal development, which, if it fails to involute by 33 weeks' gestation, results in a congenital cholesteatoma and continues to grow. The authors have observed that the epidermoid formation may have a varied geometric shape but consists of stratified squamous epidermoid cells. This structure is observed to be present at a histologic differentiation point demarcating
PENSAK ML. Congenital Cholesteatomas in Children: An Embryologic Correlation. Arch Otolaryngol Head Neck Surg. 1988;114(8):835. doi:10.1001/archotol.1988.01860200017007
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