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© Borgis - New Medicine 4/2019, s. 127-134 | DOI: 10.25121/NewMed.2019.23.4.127
Jolanta Jadczyszyn, Karolina Raczkowska-Łabuda, *Lidia Zawadzka-Głos
Congenital cholesteatoma of the middle ear in children: a hidden pathology
Perlak wrodzony ucha środkowego u dzieci ? utajona zmiana chorobowa
Department of Paediatric Otolaryngology, Medical University of Warsaw, Poland
Head of Department: Lidia Zawadzka-Głos, MD, PhD
Streszczenie
Wstęp. Perlak wrodzony jest rzadko występującą zmianą ucha środkowego u dzieci. Aby zakwalifikować zmianę chorobową jako perlaka wrodzonego muszą być spełnione odpowiednie kryteria kliniczne.
Cel pracy. Przedstawienie pacjentów z perlakiem wrodzonym hospitalizowanych w Klinice Otolaryngologii Dziecięcej WUM w latach 2018-2019, u których zmiana została wykryta przypadkowo w badaniu pediatrycznym i laryngologicznym, przebiegała skrycie za niezmienioną błoną bębenkową, nie dając żadnych objawów klinicznych.
Materiał i metody. U pacjentów przeprowadzono dokładny wywiad laryngologiczny, badanie mikrootoskopowe i badanie słuchu. W wykonanym przedoperacyjnym badaniu tomografii komputerowej kości skroniowej dokonano analizy rozległości zmian w obrębie ucha środkowego. U wszystkich pacjentów wykonano jednoetapowy zabieg operacyjny z usunięciem zmian z ucha środkowego, a następnie oceniono wynik słuchowy i rezultaty po przeprowadzonej operacji.
Wyniki. U dwóch pacjentów śródoperacyjnie stwierdzono masy perlaka w przednio-górnej części jamy bębenkowej i okolicy trąbki Eustachiusza, u jednego pacjenta perlak wypełniał całe mezotympanum. U pacjentów nie stwierdzono zmian zapalnych w obrębie komórek i jamy wyrostka sutkowego. W dotychczasowej obserwacji nie stwierdzono wznowy perlaka.
Wnioski. 1. Perlaki wrodzone u dzieci są najczęściej wykrywane przypadkowo i zlokalizowane w przednio-górnym kwadrancie jamy bębenkowej. 2. Perlak wrodzony jest zmianą wolno rosnącą i rozwija się za niezmienioną błoną bębenkową. 3. Rozpoznanie opiera się o badanie mikrootoskopii. 4. Jedyną metodą leczenia jest operacyjne usunięcie zmiany.
Summary
Introduction. Congenital cholesteatoma is a rare middle ear pathology in children. An abnormality can be classified as congenital cholesteatoma if it meets certain clinical criteria.
Aim. To present patients with congenital cholesteatoma hospitalised at the Department of Paediatric Otolaryngology of the Medical University of Warsaw in 2018-2019 in whom the abnormality was detected incidentally during paediatric and ENT examination. The disease was hidden behind an intact tympanic membrane with no clinical symptoms.
Material and methods. A thorough ENT history was taken and microotoscopy and a hearing test were conducted. Preoperative computed tomography of the temporal bone was performed to assess the extent of the pathology in the middle ear. A single-stage surgical procedure was performed in all the patients to remove the pathological growths from the middle ear. Subsequently the patients’ hearing and surgical outcomes were evaluated.
Results. During surgery, in two patients cholesteatoma masses were found in the anterosuperior part of the tympanic cavity and in the Eustachian tube area; in one patient, the growth filled the whole mesotympanum. No inflammatory lesions were found in the mastoid cells and antrum. No cholesteatoma recurrence has been found found in follow-up to date.
Conclusions. 1. Congenital cholesteatoma in children is most often detected accidentally and localized in the antero-upper quadrant of the tympanic cavity. 2. Congenital cholesteatoma is a slow-growing lesion and develops behind the unchanged eardrum.3. Diagnosis is based on microotoscopy. 4. The only method of treatment is surgical removal of the lesion.
Introduction
A cholesteatoma, also known as pearly tumour, is a tissue mass composed of matrix, i.e. deposits of keratinised epithelium, keratin, cholesterol crystals and a capsule built of keratinising stratified squamous epithelium. The mass develops at an abnormal anatomical location in the middle ear. Otitis media with cholesteatoma in children can involve acquired cholesteatoma or, much less frequently, congenital cholesteatoma. Congenital cholesteatoma accounts for approximately 2-28% of all cholesteatomas in children (1-3). Congenital cholesteatoma was first described by House in 1953 (4). In 1965, Derlacki and Clemis proposed clinical criteria for this pathology (5). These initial diagnostic criteria were modified by Levenson et al. (6) who regarded a one-time history of otitis media as not excluding congenital cholesteatoma (tab. 1).
Tab. 1. Clinical criteria for the diagnosis of congenital cholesteatoma
 Clinical criteria for congenital cholesteatoma
1A whitish abnormality in the tympanic cavity visible through the tympanic membrane
2Normal appearance of the pars flaccida and pars tensa of the tympanic membrane
3No ear discharge, no tympanic perforation, no ear trauma
4Negative history of ear surgery
5A history of uncomplicated acute otitis media does not warrant the exclusion of congenital cholesteatoma
Potsic distinguished between 4 stages of congenital middle ear cholesteatoma (7):
I: cholesteatoma confined to the anterosuperior quadrant of the tympanic membrane,
II: cholesteatoma of the mesotympanum and a few quadrants of the tympanic membrane without ossicular involvement,
III: cholesteatoma of the mesotympanum and a few quadrants of the tympanic membrane with ossicular involvement but with no mastoid involvement,
IV: mastoid involvement.
Aim
The aim of the study is to characterize and present a rare disease in children that runs secretly behind a properly preserved eardrum, initially gives no clinical symptoms and is most often detected accidentally in a paediatric and ENT examination.
Material and methods
In 2018-2019, three boys with congenital cholesteatoma of the middle ear were hospitalised at the Department of Otolaryngology of the Medical University of Warsaw. Two patients (1 and 2) were referred to the hospital by a paediatrician with suspected foreign body in the ear, while another patient (3) was referred by an ENT physician with suspected congenital cholesteatoma. On admission a thorough ENT history was taken: the patients had not had any episodes of otitis media or middle ear trauma and had not had any ear surgery. The patients did not report any ear pain, tinnitus, a sense of fullness in the ear, dizziness or headaches. The patients’ history was negative for chronic diseases. No family history of cholesteatoma was found either. Otoscopy revealed a normal, wide and inflammation-free external auditory canal in all patients; no foreign body was found there. However, a whitish, spherical mass was found in the tympanic cavity behind an intact tympanic membrane (both pars flaccida and pars tensa were normal). The first, 4-year-old boy, developed a cholesteatoma in the anterosuperior part of the tympanic cavity of the right ear near the tympanic end of the Eustachian tube (fig. 1a). The second, 5-year-old boy, developed a cholesteatoma in the tympanic cavity of the left ear also near the tympanic end of the Eustachian tube (fig. 1b). In the third, 7-year-old patient, the abnormality filled the whole mesotympanum and part of the epitympanic recess (fig. 1c, tab. 2).
Fig. 1a-c. Microotoscopy upon admission to hospital. Congenital cholesteatoma visible behind a normal tympanic membrane, a) right ear (patient 1), b) left ear (patient 2), c) left ear (patient 3)
Tab. 2. Characteristics of patients with congenital cholesteatoma of the middle ear
No.SexAge (years)Congenital cholesteatoma of the middle ear
1Boy4Right
2Boy5Left
3Boy7Left
Laboratory test results were normal and inflammatory markers were negative. Whisper and tuning fork hearing tests were performed. Impedance audiometry and pure tone audiometry were also conducted. Before the planned surgery, computed tomography of the temporal bone was also performed in order to assess the pathology in the middle ear. In the first patient, a round soft tissue mass with a diameter of 3.5 mm was present in the medial part of the right tympanic cavity anterior from the manubrium of malleus; the tympanic cavity was inflammation-free and mastoid cells were normally pneumatised (fig. 2a, b).
Fig. 2a. A soft tissue mass in the tympanic cavity of the right middle ear located medially from the ossicles (thick arrow). Normally pneumatised mastoid cells (fine arrow), patient 1
Fig. 2b. Congenital cholesteatoma of the right middle ear (thick arrow): coronal view, patient 1
In the second patient, a well-defined, round mass 4 mm in diameter was found directly at the manubrium of malleus in the left tympanic cavity, the epitympanic recess was clear and the ossicular chain was normal (fig. 3a, b).
Fig. 3a. Cholesteatoma “pearl” in the left middle ear (thick arrow). Epitympanic recess with no inflammatory lesions, patient 2
Fig. 3b. Cholesteatoma “pearl” (marked with a thick arrow) in the left tympanic cavity. Bilaterally intact ossicular chain, patient 2
In the third patient, a soft tissue mass was revealed in the left tympanic cavity, which was 7.5 x 8 mm in diameter; the abnormality was present in the mesotympanum and was directed towards the hypotympanum and epitympanic recess; the mass was adjacent to the ossicles but did not cause them to become disconnected (fig. 4a, b). All diagnostic imaging procedures revealed normally pneumatised middle ear cells and mastoid antrum without lesions typical for chronic otitis media. The patients were assessed and scheduled for surgery.
Fig. 4a. Congenital cholesteatoma covering the medial part of the tympanic cavity, the tympanic end of the Eustachian tube and the epitympanic recess, patient 3
Fig. 4b. Congenital cholesteatoma covering the mesotympanum and developing towards the hypotympanum, patient 3
Results
In the hospitalised patients congenital cholesteatoma limited to the tympanic cavity of the middle ear was diagnosed. The mean age of the patients was 5 years and the mean hospitalisation time was 6 days. Microotoscopy revealed a normal, intact, translucent tympanic membrane which had no perforation, signs of thickening or inflammatory lesions of the myringosclerosis type. All congenital cholesteatomas adhered tightly to the tympanic membrane. Normal sound reception was observed on whisper and tuning fork hearing tests in a quiet room with no external noise. Impedance audiometry produced type A tympanometric curves (fig. 5a, b).
Fig. 5a. Impedance audiometry, preoperative assessment, patient 2
Fig. 5b. Impedance audiometry, preoperative assessment, patient 3
The stapedius reflex was present symmetrically in both ears. Pure tone audiometry showed a normal hearing threshold for air and bone conduction in both ears (fig. 6).
Fig. 6. Pure tone audiometry: preoperative hearing test
Computed tomography revealed the presence of abnormalities in tympanic cavities with an intact tympanic membrane. The cholesteatoma was a well-defined tissue mass located medially from the ossicles; it did not cause them to erode. An additional computed tomography scan was performed to make a thorough anatomical analysis of the temporal bone, evaluate the extent of pathology and select the right surgical technique. A single-stage surgical procedure was performed in all the patients during which the masses were removed from the middle ear. In two patients the cholesteatoma “pearls” were removed through an intra-aural approach. These cholesteatomas were well-separated from the surrounding area, had a capsule and adhered to the manubrium of the malleus without causing bone erosion (fig. 7).
Fig. 7. Cholesteatoma “pearl” removed intraoperatively, patient 2

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otrzymano: 2019-11-27
zaakceptowano do druku: 2019-12-18

Adres do korespondencji:
*Lidia Zawadzka-Głos
Klinika Otolaryngologii Dziecięcej Warszawski Uniwersytet Medyczny
ul. Żwirki i Wigury 63A, 02-091 Warszawa
tel.: +48 (22) 317-97-21
laryngologia@litewska.edu.pl

New Medicine 4/2019
Strona internetowa czasopisma New Medicine