Primary acquired cholesteatoma is an invagination of the tympanic membrane, of variable aggressiveness, with accumulation of desquamated keratin and a notable tendency to destroy bone. It is controversial whether cholesteatoma progression is driven by proliferative activity of the squamous layer or mucosal factors drawing the pouch from its medial aspect. Cholesteatoma has a strong association with mastoid hypopneumatization, is frequently associated with purulent discharge, and has a tendency to be bilateral.
While small cholesteatomas may be managed medically with periodic cleaning, especially in cooperative adults, most cholesteatomas ultimately come to surgery. There are two fundamental strategies in dealing with cholesteatoma: resection and exteriorization. Resection usually involves an intact canal wall tympanomastoidectomy with tympanic membrane repair with reinforcement using cartilage. Exteriorization in small and relatively indolent cases may be achieved via conservative atticotomy. Exteriorization of more advanced cholesteatoma involves canal wall down tympanomastoidectomy with creation of a cavity necessitating ongoing care facilitated by creation of a meatoplasty calibrated to the size of the mastoid bowl. Some advocate a retrograde approach removing only that portion of the mastoid overlying the cholesteatoma as an alternative to thorough removal of mastoid cellularity. Severe hypopneumatization tends to favor the exteriorization approach as the resulting cavity is small. By contrast, cholesteatoma invading a highly pneumatic mastoid is usually managed initially with an intact canal wall procedure. Radical mastoidectomy, removal of all middle ear contents and exteriorization of the tympanum, is not depicted in this atlas because it is largely a historical procedure. In contemporary practice, an effort is made to avoid exteriorization of tympanic mucosa, which can become a source of chronic discharge, by reconstruction of the tympanic membrane.
Unfortunately, cholesteatoma is frequently recidivistic. Recurrence refers to repeat invagination of the tympanic membrane, often via a narrow slit adjacent to the cartilage placed at the initial surgery. Recurrence is not rare following an intact canal wall procedure, especially when the disease is bilateral. The status of the opposite ear is an important determinant in the choice of exteriorization versus resection. The second mechanism is regrowth of residual cholesteatoma matrix left behind, most often in an inaccessible recess of the middle ear, at the initial stage. When cholesteatoma is situated in difficult-to-visualize area (e.g., sinus tympani, oval window, under the facial nerve, or in perilabyrinthine cells), staging is indicated. At second inspection, approximately 6 months later, inconspicuous microscopic fragments become identifiable as small white pearls which can often to be completely excised with confidence.
Congenital cholesteatoma most often originates from an ectopic squamous nest on the promontory. If detected in the first few years of life, it remains confined to the protympanum and can readily be removed in toto. If discovered in older childhood, it comes to involve the ossicular chain and anterior epitympanic space and requires more extensive surgery often including a staged procedure for excision validation and ossiculoplasty.
Further Reading
Britze A, Møller ML, Ovesen T. Incidence, 10-year recidivism rate and prognostic factors for cholesteatoma. J Laryngol Otol 2017;131(4):319–328 PubMed
Calli C, Pinar E, Oncel S, Tatar B, Tuncbilek MA. Measurements of the facial recess anatomy: implications for sparing the facial nerve and chorda tympani during posterior tympanotomy. Ear Nose Throat J 2010;89(10):490–494 PubMed
Castle JT. Cholesteatoma pearls: practical points and update. Head Neck Pathol 2018;12(3):419–429 PubMed
Dornhoffer JL. Retrograde mastoidectomy. Otolaryngol Clin North Am 2006;39(6):1115–1127 PubMed
Jackler RK, Santa Maria PL, Varsak YK, Nguyen A, Blevins NH. A new theory on the pathogenesis of acquired cholesteatoma: mucosal traction. Laryngoscope 2015;125(Suppl 4):S1–S14 PubMed
Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin North Am 1989;22(5):883–896 PubMed
Kerckhoffs KG, Kommer MB, van Strien TH, et al. The disease recurrence rate after the canal wall up or canal wall down technique in adults. Laryngoscope 2016;126(4):980–987 PubMed
Kuo CL, Liao WH, Shiao AS. A review of current progress in acquired cholesteatoma management. Eur Arch Otorhinolaryngol 2015;272(12):3601–3609 PubMed
Lim J, Gangal A, Gluth MB. Surgery for cholesteatomatous labyrinthine fistula. Ann Otol Rhinol Laryngol 2017;126(3):205–215 PubMed
Magliulo G, Iannella G. Endoscopic versus microscopic approach in attic cholesteatoma surgery. Am J Otolaryngol 2018;39(1):25–30 PubMed
Mostafa BE, El Fiky L. Congenital cholesteatoma: the silent pathology. ORL J Otorhinolaryngol Relat Spec 2018;80(2):108–116 PubMed
Soldati D, Mudry A. Knowledge about cholesteatoma, from the first description to the modern histopathology. Otol Neurotol 2001;22(6):723–730 PubMed
Westerberg J, Mäki-Torkko E, Harder H. Cholesteatoma surgery with the canal wall up technique combined with mastoid obliteration: results from primary surgery in 230 consecutive cases. Acta Otolaryngol 2018;138(5):452–457 PubMed
8.2 Growth Patterns of Cholesteatoma
Fig. 8.1 Acquired cholesteatoma begins as a retraction pocket in the tympanic membrane, most often in the pars flaccida.
Fig. 8.2 Cholesteatoma draws into the epitympanum along the incus body and malleus head and begins to accumulate keratin.
Fig. 8.3 With further growth, the cholesteatoma continues to accumulate keratin as it expands toward the aditus-ad-antrum.
Fig. 8.4 Infected cholesteatoma penetrating the mastoid via the aditus-ad-antrum. The excluded air cells contain purulence and granulation tissue.
Fig. 8.5 Chronic otitis media without cholesteatoma.
Fig. 8.6 Existing theories of cholesteatoma formation. (Used with permission from Jackler RK, Santa Maria PL, Varav KY, Gguyen A, Blevins NB. A new theory on the pathogenesis of acquired cholesteatoma: mucosal traction. Laryngoscope 2015;125(Suppl 4):S1–S14.)
Fig. 8.7 Mucosal traction theory of cholesteatoma formation. (Used with permission from Jackler RK, Santa Maria PL, Varav KY, Gguyen A, Blevins NB. A new theory on the pathogenesis of acquired cholesteatoma: mucosal traction. Laryngoscope 2015;125(Suppl 4):S1–S14.)
Fig. 8.8 Primary acquired cholesteatoma arises from the epitympanum, posterior mesotympanum, or both, but almost never from the anterior or inferior aspects of the tympanic membrane. This means that primary acquired cholesteatomas almost universally are intimately involved with the ossicles.
Fig. 8.9 The origin of cholesteatoma is from the posterior epitympanum (1) most commonly, followed by posterior mesotympanum (2), and then anterior epitympanum (3). Sometimes pockets occur in combinations such as posterior epitympanic with posterior mesotympanic or both posterior and anterior epitympanic together.
Fig. 8.10 Cholesteatomas invade the middle ear and mastoid along fairly predictable pathways. Their growth is constrained by ligaments and mesenteries which are embryological remnants of the first branchial arch pouches which form the tympanic cavity. (Used with permission from Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin NA 1989;22:883–896.)
Fig. 8.11 Cholesteatoma growth tends to follow spaces defined by ligaments and mesenteries which compartmentalize the middle ear. (Used with permission from Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin NA 1989;22:883–896.)
Fig. 8.12 Posterior epitympanic cholesteatoma penetrates the epitympanum and posterior mesotympanum lateral to the ossicles. (Used with permission from Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin NA 1989;22:883–896)
Fig. 8.13 Posterior epitympanic cholesteatoma penetrating the aditus-ad-antrum.
Fig. 8.14 Posterior epitympanic cholesteatoma involving the mastoid which penetrated the epitympanum and posterior mesotympanum lateral to the ossicles.
Fig. 8.15 Posterior mesotympanic cholesteatoma illustrating the pathways to the mastoid and middle ear. Note that the penetration to the mastoid is medial to the ossicles. Posterior mesotympanic cholesteatoma tends to involve the posterior tympanic spaces: the sinus tympani and facial recess (see section 8.8 Sinus Tympani and Facial Recess in Cholesteatoma). (Used with permission from Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin NA 1989;22:883–896.)
Fig. 8.16 Anterior epitympanic cholesteatoma penetrates the supratubal recess and can involve the geniculate ganglion of the facial nerve (see section 8.9 Anterior Epitympanic Cholesteatoma). (Used with permission from Jackler RK. The surgical anatomy of cholesteatoma. Otolaryngol Clin NA 1989;22:883–896.)
8.3 Canal Wall Down Mastoidectomy
Fig. 8.17 Canal wall down tympanomastoidectomy cavity with fascia tympanoplasty, cartilage graft, and partial ossicular replacement prosthesis.
Fig. 8.18 Completed canal wall down mastoidectomy before middle ear reconstruction.
Fig. 8.19 Epitympanic cholesteatoma with involvement of the mastoid.
Fig. 8.20 Raising tympanomeatal flap.
Fig. 8.21 Incision is placed superiorly and carried into the cholesteatoma pocket. No inferior incision is made.
Fig. 8.22 Incising the margin of the cholesteatoma sac.
Fig. 8.23 Raising the inferiorly based tympanomeatal flap.
Fig. 8.24 Scutum removal to expose the oval window region. This is done early to determine whether or not the ossicular chain is intact.
Fig. 8.25 Removal of the scutum (posterior superior ear canal).
Fig. 8.26 Completed exposure of the incus and stapes which are intact in this drawing, but are often eroded.
Fig. 8.27 Beginning removal of the posterior ear canal wall.
Fig. 8.28 Removing the ear canal wall with a rongeur.
Fig. 8.29 The bridge is the arch of bone over the epitympanum.
Fig. 8.30 Curetting away the last remnant of the bridge brings the cholesteatoma into view. Switching from a drill to curette is especially important in cases of an intact ossicular chain.
Fig. 8.31 The anterior and posterior buttresses need to be removed.
Fig. 8.32 Removal of the anterior and posterior buttresses.
Fig. 8.33 Lowering of the canal wall, also known as the facial ridge because the facial nerve lies in its base.
Fig. 8.34 Lowering facial ridge does not require exposure of the facial nerve. It is best to leave 2 to 3 mm of bone over the nerve to maintain a middle ear space.
Fig. 8.35 Lowering the floor of the middle ear to the level of the facial ridge (base of the ear canal).
Fig. 8.36 Removal of the mastoid tip creates a smaller cavity. This is indicated in canal wall down surgery in an extensively pneumatized temporal bone.
Fig. 8.37 Removal of the mastoid tip. The facial nerve is not related to the tip itself: it exits the mastoid bone anteriorly beneath the digastric ridge.
Fig. 8.38 Completing removal of the mastoid tip.
Fig. 8.39 After removal of the mastoid tip.
Fig. 8.40 Osseous anatomy of a completed canal wall down mastoidectomy. In modern times, middle ear reconstruction is almost always performed creating a modified radial mastoidectomy. A true radial mastoidectomy, leaving the middle ear open, is seldom used today but may be necessary when the cholesteatoma adheres tenaciously to the tympanic section of the facial nerve and/or stapes footplate, especially when matrix prolapses into the mouth of the Eustachian tube.
Fig. 8.41 Typical cartilage and fascia reconstruction in a canal wall down mastoidectomy. The skin flaps are not shown.
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