Audit on pediatric Cholesteatoma

S. Lakshmipriya

ENT PG, Kauvery Hospital, Trichy, Cantonment

Editor’s Note: This paper is an adaptation of a presentation made at a clinical meeting

Introduction

Anatomy of Ear

Ear consists of 3 parts

External ear
Middle ear
Internal ear

Middle ear contains 3 tiny bones – the ossicles

Malleus/Hammer
Incus/Anvil
Stapes/Stirrup

Middle ear is divided into

Epitympanum
Mesotympanum
Hypotympanum

Facial nerve leaves the brainstem at pontomedullary junction; it travels through the posterior cranial fossa and enters the IAM. At the fundus of the IAM, it takes a turn posteriorly forming a genu, from the genu the nerve passes backward above the oval window and below the SCC till the level of pyramidal eminence(2nd genu). The nerve continues vertically downward along the posterior wall of tympanic cavity and leaves the temporal bone through the stylomastoid foramen

What is cholesteatoma?

The presence of keratinized squamous epithelium in middle ear or mastoid is characteristic of cholesteatoma – “Skin in the wrong place”

The term ‘cholesteatoma’ is a misnomer; it contains neither cholesterol crystals nor a tumor.

Definition of Cholesteatoma

Cholesteatoma is a mass formed by keratinizing squamous epithelium in the middle ear and/or mastoid, subepithelial connective tissue and by progressive accumulation of keratin debris with/without surrounding inflammatory reaction.

It is aggressive in children, if left untreated, can cause bone erosion which in turn can lead on to serious complications.

Bone resorption in Cholesteatoma

Enzymatic-induced necrosis and resorption – Collagenase, Acid phosphatase, proteolytic enzymes
Cytokines induced bone remodelling – IL-1a and 1b, TNF-alpha, TNF-beta
Prostaglandin induced bone remodelling – PG E2
Pyogenic osteitis theory – Pseudomonas aeruginosa, Streptococcus sp, proteus, E.coli
Pressure necrosis theory – unlikely

Objectives

To describe our methodology and outcome in treating pediatric patients with Cholesteatoma.

Materials and Methods

A retrospective study was carried out from July 2023–June 2024.
The study includes 10 patients, of whom
- 4 were boys
- 6 were girls
Between the age of 7 and 16 years.

The evaluated parameters

Clinical symptoms
Site of infection
Extension of the disease
Ossicular chain involvement
Facial nerve integrity
Imaging
Treatment modality
Outcome
Complications

*congenital chloesteatoma excluded from this study

Symptoms and Signs

All patients invariably presented with Foul smelling ear discharge and Hard of hearing.
50% of patients had blood stained ear discharge.
20% of patients had facial asymmetry.

Preoperative Assessment of Facial Nerve

House-Brackmann grade 4
He also had Giddiness, Vomiting, Headache

Preoperative Assessment of Facial Nerve

(1) – Complete left eye closure only with maximum effort

(2) – Asymmetry of mouth with maximum effort

(3) – Inability to blow the cheek

House-Brackmann grade 3

Otoendoscopy

CT Temporal Bone – Axial Plane

CT Temporal Bone – Coronal Plane

Diagnosis

Diagnosis	No. of. Cases
Chronic otitis media with cholesteatoma, active, squamosal disease with facial nerve compression	2
Chronic otitis media with cholesteatoma, active, squamosal disease with intact facial nerve	8

Management

Surgery	No. of. Cases
Canal wall down mastoidectomy, tympanoplasty with facial nerve decompression	2
Canal wall down mastoidectomy with tympanoplasty	8

Intraoperative Findings

Cholesteatoma Site of Involvement

Ossicular Status

Fallopian Canal and Semicircular Canal Status

Left ear CWD Mastoidectomy cavity of a 7-year-old girl

Ossicular Chain Reconstruction

Graft materials	No.of. Cases	Placed over	Tympanoplasty type
Harvested conchal cartilage	2	Head of stapes	III
Septal cartilage	1	Head of stapes	III
Sculptured Malleus	1	Head of stapes	III
Sculptured Incus	1	Head of stapes	III
Sculptured Incus	2	Footplate of stapes	IV
PORP	1	Head of stapes	III
TORP	1	Footplate of stapes	IV

Left ear CWD Mastoidectomy of a 9-year-old boy

Pre OP – HB Grade 4

Post OP – Normal

Pre OP – HB Grade 3

Post OP – Normal

Otoendoscopy of Postoperative Mastoid Cavity

Comparison

Variables	Our study	Acta Ital
Number of patients	10 in 1 year	36 in 6 years
Age group	7–16 years	6–14 years
Surgery
Canal wall down mastoidectomy	100%	39.39%
Canal wall intact mastoidectomy	0	60.60%
Complications
Residual disease	Nil	18.18%
Recurrence	Nil (On Avg 5 months follow-up)	6.06%
Hearing
Subjective test	Hearing improvement present	Hearing improvement present
Objective test (Audiometry)	Not done	ABG between 0 and 20 db in 54%

B. sergi et al. Ital. 2014 34(2): 138-143

Surgery

Primary role of surgery	Secondary role of surgery
Remove all original cholesteatoma squamous epithelial matrix	Improve hearing (aim is to restore the best possible hearing in an ear that will never be normal).
Prevent further erosion and complications
Give a dry, watertight ear
Give a ear that will be self-cleaning
Prevent the occurrence of recurrent cholesteatoma.

What to be cautious in pediatric Cholesteatoma?	Why/how?
More extensive and destructive in children than adults	Hence early intervention is important to prevent further damage
Increases risk of complications	More prone to facial paralysis, Meningitis, Brain abscess
Impact on development	if left untreated, it affects speech, language and cognitive development
Difficulty in diagnosis	Due to nonspecific symptoms
Surgical challenges	Surgery can be more complex in children due to extensive spread in developing temporal bone
High recurrence rates	More likely to recur due to poor Eustachian tube function. Hence long term follow-up will be necessary
Impact on hearing and balance	As it cause erosion of ossicles and labyrinth, patient develops hard of hearing and giddiness respectively
Potential for congenital associations	Down’s syndrome, Craniofacial syndrome, di-George syndrome
Emotional and psychological impact	Children and families may experience emotional distress and concerns about hearing

Conclusion

Management of pediatric cholesteatoma requires a highly individualized approach that takes into account anatomic, clinical and social factors to determine the most successful treatment paradigm.

Prompt evaluation, management, and early intervention are very important to avoid life- threatening events.

Dr. S. Lakshmipriya
ENT PG

Audit on pediatric Cholesteatoma

Audit on pediatric Cholesteatoma

S. Lakshmipriya

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