ORIGINAL ARTICLE. A New Staging System for Tympano-mastoid Cholesteatoma. Aziz Belal, Mahmoud Reda, Ahmed Mehana, Yousef Belal

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Int. Adv. Otol. 2012; 8:(1) 63-68 ORIGINAL ARTICLE A New Staging System for Tympano-mastoid Cholesteatoma Aziz Belal, Mahmoud Reda, Ahmed Mehana, Yousef Belal Alexandria Ear Hospital Alexandria Egypt (AB, MR, AM, YB) Objective: Objective: To present a new staging system of tympano-mastoid cholesteatoma that is based on the primary site of pathology in the middle ear (T), its spread to the mastoid (M), and to the surrounding structures either cranial, intra- or extracranial, i.e. presence of complications (C). The TMC staging system is based on correlation of preoperative otoscopy findings with CT examination of the temporal bone. Setting: Tertiary Care Center. Patients: We included 120 patients of tympano-mastoid cholesteatoma diagnosed preoperatively, staged and correlated with intraoperative findings. Results: Preoperative findings has also been correlated with intraoperative findings in 120 consecutive cases operated in the last five years. In 87 percent, pre-operative and intra-operative staging correlated well. Conclusion: The TMC staging system of cholesteatoma paves the way to a logical roadmap for functional surgery of the middle ear and mastoid, as well it makes the comparison of clinical studies about cholesteatoma meaningful. Submitted : 06 October 2011 Accepted : 01 January 2012 Introduction Surgical strategy of tympano-mastoid cholesteatoma may be based on factors related to the patient, surgeon, or disease. Patient`s factors include general factors like age, general medical status, occupation, and availability for follow ups. Local factors like status of hearing, only hearing ear, bilateral cholesteatoma, mastoid pneumatization, eustachian tube function, etc. Surgeon`s factors include preference and training of the surgeon at a particular time, ie. surgeon`s choice. Factors related to the disease include the anatomical site of origin, paths of spread inside and outside the temporal bone, histological characteristics and biological behavior of cholesteatoma. Pathology seems to be the most logical way to outline a surgical strategy for cholesteatoma. [1,2,3] Why Staging Cholesteatoma? Reviewing the techniques used to manage middle ear cholesteatoma showed that priority was often given to the mastoid, rather than the middle ear. [3,4,5,6] Furthermore, most of temporal bone dissection courses around the world emphasizes mastoidectomy as the key for chronic ear surgery. As a result, most of the ENT residents and trainees can do an excellent mastoidectomy and less than acceptable middle ear dissection. However, histopathologic and clinicoperative studies has shown that the involvement of middle ear is much higher than the mastoid in primary and revision cholesteatoma cases. [7,8,9,10,11] Thus, we need a new concept that is based on tailoring the surgical technique according to the site of the pathology and its extensions. Comparison of clinical and operative studies about tympano-mastoid cholesteatoma always had the problem of finding a standard to make a meaningful comparison. Terms of small, big, and huge cholesteatomas made comparison between the different studies like comparing apples to oranges. A standard staging system will definitely solve this problem. Corresponding address: Alexandria Ear Lomomba St. Al-Shalalat Alexandria - Egypt Phone: 0020123363987 E-mail: mahmoudreda99@yahoo.com Copyright 2005 The Mediterranean Society of Otology and Audiology 63

The Journal of International Advanced Otology Attempts to stage tympano-mastoid cholesteatoma: The classical etiological classification [7] into congenital, primary acquired, and secondary acquired cholesteatoma is an etio-pathological classification and describes well the site of origin of cholesteatoma, its paths of spread and histological behavior, but has little clinical significance. Tos [8] otoscopically classified cholesteatoma into attic type in the pars flaccida of the ear drum, sinus cholesteatoma starting as a postero-superior retraction or perforation of the pars tensa and extending to the tympanic sinus, posterior tympanum and beyond, and tensa cholesteatoma presenting as retraction and adhesion of the entire pars tensa involving the tympanic orifice of the Eustachian tube (may also extend further into the attic). Tos [12] latter modified his classification into: attic, pars tensa I (Marginal disease), and pars tensa II (Central disease) cholesteatomas. Meyerhoff and Truelson [13] classified cholesteatoma into primary acquired, secondary acquired, tertiary acquired, and congenital. Saleh and Mills [14] introduced the Site-Ossicles- Complications (SOC) classification System. It can be summarized in the following: S1: if the cholesteatoma is restricted to the site where it had started S2: when the disease extends to the other site S3: if it affects three sites S4: if it is installed in four sites S5 : cases in which the primary site is affected plus four or more are also involved Authors distinguished seven sites used to this classification: attic and antrum, middle ear, mastoid, auditory tube, labyrinth and middle fossa. O0: if the ossicle chain is intact O1: if incus is eroded and without chain discontinuity O2: if incus and stapes suprastructures are eroded O3: if the malleus head and incus are absent and stapes superstructure if eroded. C0: when there is no complications C1: if there is one complication C2: if there are two or more complications. As to complications, the authors considered lateral semicircular canal fistula, facial palsy, total sensorineural auditory loss, sinus thrombosis and intracranial invasion. We thought we still need an easy reliable clinical staging system that can be applied to every case of tympano-mastoid cholesteatoma, can be correlated with intraoperative findings, can work as a roadmap for functional surgery of the middle ear and mastoid, can objectively compare clinical studies about cholesteatoma. New Staging System for Tympano-mastoid Cholesteatoma: We designed a staging system of tympano-mastoid cholesteatoma from stage 1 to stage 5 depending on the site of disease in the tympanic cavity (T) the mastoid cavity (M), and the presence of any complication (C). Staging of any case is made according to the office clinical (otoscopic \ microscopic\ endoscopic) examination, the radiologic study (axial, coronal, and sagittal reconstruction views of high definition CT Petrous bone), and the clinicoradiological correlation. (Table 2, Fig. 1) Case Reports Case 1: 40 years old patient presented with recurrent discharging from the right ear. Otoscopy: attic cholesteatoma and small polyp on one side of the head of malleus, and normal mesotympanum (T1a) CT scan: showed attic cholesteatoma with involvement of the mastoid cavity only in the antrum (M1) (Fig.2) There was no clinical or radiological manifestation of complications (C0) Staging: T1M1C0 = Stage 3, intra-operative findings were corresponding to TMC staging. 64

A New Staging System for Tympano-mastoid Cholesteatoma Table 1. TMC Staging system of tympano-mastoid cholesteatoma. (T) Tympanic cavity involvement: T0: No tympanic cavity involvement Trp: Retraction pocket (precholesteatoma) T1: cholesteatoma involving one side of one region T1a: one side of epitympanum T1b: one side of mesotympanum T2: cholesteatoma involving both sides of one region T1a: both sides of epitympanum T1b: both sides of mesotympanum T3: Cholesteatoma extending from one region of the middle ear to another (vertical spread) T4: Holotympanic cholesteatoma filling the whole middle ear Regions (epity- or meso-, and hypotympanum) Sides (anterior or posterior in relation to a line along handle of malleus) (M) Mastoid cavity involvement: M0: No mastoid cavity involvement M1: Cholesteatoma extending to the mastoid antrum only M2: Cholesteatoma extending to mastoid cavity (C) Presence of complications: C0: uncomplicated Cholesteatoma C1: cranial or extracranial complication C2: intracranial complications Stages 1 2 3 4 5 Trp-1 M0 C0 T2 M0 C0 T3 M0 C0 T4 M0 C0 Any T Any M C2 Trp-3 MI C0 Any T M2 C0 Any T Any M C1 T= Tympanic M= mastoid C= complication rp = retraction pocket Figure 1. Staging system of tympano-mastoid cholesteatoma. 65

The Journal of International Advanced Otology Figure 2. Otoscopic and CT findings of case 1. Case 2: 24 years old male patient presented with right hearing loss and occasional ear discharge. Otoscopy: showed a white pearly mass behind an intact tympanic membrane (T3) CT scan: showed a soft tissue mass in the tympanic cavity, with well aerated mastoid (M0). The mass was eroding the basal turn of the cochlea (C1). (Fig.3) Staging: T3M0C1 = Stage 4, intra-operative findings were corresponding to TMC staging. Figure 3. Otoscopic and CT findings of case 2. Case 3: 35 years old male patient presented with persistent foul-smelling intermittent discharge from the right ear. Otoscopy: showed cholesteatoma involving the whole tympanic cavity (T4) CT scan: showed cholesteatoma filling the whole mastoid cavity (M2) There was no clinical or radiologic manifestation of complications (C0) (Fig.4) Staging: T4M2C0 = Stage 4, intra-operative findings were corresponding to TMC staging. Figure 4. Otoscopic and CT findings of case 3. 66

A New Staging System for Tympano-mastoid Cholesteatoma Correlation of pre- and intra-operative findings: 120 cases of tympano-mastoid cholesteatoma examined and operated at Alexandria Ear Hospital, Egypt in the period 2005 2010 were included in this study. Cases were examined pre-operatively (otoscopy, microscopy, and endoscopy), CT scanned (coronal and sagittal views), then pre-operatively staged according to the TMC staging system. Cases were operated upon then staged again according to the intra-operative findings. Staging correlated well in 87 percent of cases. Pre-operative staging under-estimated the size of cholesteatoma in 11 percent of cases, and overestimated it in one percent of cases. Table 2 summarizes the pre- and intra-operative findings in these cases. Our study has been performed in comply with the ethical principles of Alexandria Faculty of Medicine board. Table 2. Comparison of pre- and intra-operative findings. Stage No. of Good Correlation Under-estimated Over-estimated % Correlation Average Correlation Cases Cases Cases T1 5 5 - - 100% T2 42 40 2-95% T3 58 40 16 2 68% 87.3% T4 15 13 1 1 86.5% MO 37 18 9-66.6% M1 39 20 9 1 74% 77.7% M2 54 50-4 92.5% C0 115 104 11-90% C1 3 3 - - 100% 96.6% C2 2 2 - - 100% Total No. of Cases 120 87% 11% 1% Conclusion There are many advantages to the TMC staging system. It is a simple clinico-radiological system that can be applied by any clinician or resident. Because there is high correlation between pre- and intra-operative findings, TMC staging can be easily used to plan a roadmap of functional surgery in cases of tympanomastoid cholesteatoma. Furthermore, it standardizes and simplifies the terminology used to describe a case of tympano-mastoid cholesteatoma. Thus, it facilitates a reliable type-specific comparison of published data reporting the results of tympano-mastoid surgery. References 1.Graham M, Delap T. Closed tympanomastoidectomy. Otolaryngol Clin North Am. 1999, 32:547-55. 2. Syms M, Luxford W. Management of cholesteatoma: status of the canal wall. Laryngoscope. 2003,113:443-8. 3. Kapur R. Causes of failure of combined approach tympanoplasty in the treatment of acquired cholesteatomas of the middle ear and the mastoid. J Laryngol Otol. 1995, 109:710-2. 4. Reddy T, Dutt S, Sangeeta M. Transcanal atticoaditotomy and transcortical mastoidectomy for cholesteatoma: the farrior-olaizola technique revisited. Ann Otol Rhinol Laryngol. 2001,110:739-45. 5. Uzun C, Yagiz R, Tas A, Adali M, Koten M, Karasalihoglu A. Combined Heermann and Tos (CHAT) technique in cholesteatoma surgery: surgical technique and preliminary results. J Laryngol Otol. 2005, 119:429-35. 6. Dornhoffer J. Retrograde mastoidectomy with canal wall reconstruction: a follow-up report. Otol Neurotol 2004, 25:653-60. 7. Belcadhi M. Predictive factors of recurrence in pediatric cholesteatoma surgery. J Int. Adv. Otol. 2008, 4:118-24. 67

The Journal of International Advanced Otology 8. Lau T, Tos M. Long-term Results and Recurrence Rate. Arch Otolaryngol Head Neck Surg. 1988; 114:1428-34. 9. Sethom A, Akkari K, Dridi I. Preoperative CT Scan in middle ear cholesteatoma. Tunis Med. 2011; 89(3):248-53. 10. Alzoubi F, Odat HA, Al-Balas H. The role of preoperative CT scan in patients with chronic otitis media. Eur Arch Otorhinolaryngol. 2009; 266:807-9. 11. Williams M, Ayache D. Imaging in adult chronic otitis. J Radiol. 2006; 87:1743-55. 12. Tos M, Lau T. Late results of surgery in different cholesteatoma types. ORL J Otorhinolaryngol Relat Spec. 1989; 511:33-49. 13. Meyerhoff WL, Truelson J. Cholesteatoma staging. Laryngoscope. 1986; 96:935-9. 14. Saleh H, Mills R. Classification and staging of cholesteatoma. Clin Otolaryngol Allied Sci. 1999; 24:355-9. 68