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The Natural History of Congenital Cholesteatoma
Peter J. Koltai, MD;
Marc Nelson, MD;
Roberto J. Castellon, MD;
Erea-Noel Garabedian, MD;
Jean-Michel Triglia, MD;
Stephane Roman, MD;
Gilles Roger, MD
Arch Otolaryngol Head Neck Surg. 2002;128:804-809.
Objectives To describe the natural history of congenital cholesteatoma (CC) and
to determine whether such a description provides clues about the origins and
end points of these lesions.
Design A retrospective qualitative analysis of intraoperative illustrations
of 34 consecutive patients with 35 CCs (1 bilateral).
Setting Two tertiary care children's hospitals.
Patients Thirty-four children with CC, mean age, 5.6 years (range, 2-13 years).
Results Congenital cholesteatoma originates generally, but not universally,
in the anterior superior quadrant. The progression of growth is toward the
posterior superior quadrant and attic and then into the mastoid. Contact with
the ossicular chain generally results in loss of ossicular continuity and
in conductive hearing loss.
Conclusions Congenital cholesteatoma appears to have a predictable trajectory of
growth, starting as a small pearl in the middle ear, eventually growing to
involve the ossicles and mastoid, and causing varying degrees of destruction
and functional impairment. The clinical picture of a young child with otorrhea,
conductive hearing loss, tympanic membrane perforation in a nontraditional
location, and a mastoid filled with cholesteatoma may represent the end point
in the natural history of CC, despite the fact that this type of lesion is
outside the accepted definition of CC.
From the Section of Pediatric Otolaryngology, The Cleveland Clinic
Foundation, Cleveland, Ohio (Drs Koltai and Nelson); Division of Otolaryngology,
Albany Medical College, Albany, NY (Dr Castellon); and Children's Hospital
Armand Trousseau, Paris (Drs Garabedian and Roger), and La Timone Children's
Hospital, Marseille (Drs Triglia and Roman), France.
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Congenital Cholesteatoma: Classification, Management, and Outcome
Marc Nelson, Gilles Roger, Peter J. Koltai, Erea-Noel Garabedian, Jean-Michel Triglia, Stephane Roman, Roberto J. Castellon, and Jeffrey P. Hammel
Arch Otolaryngol Head Neck Surg. 2002;128(7):810-814.
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