Background
Materials and methods
Results
Literature review results are presented in the Table 1
Author | No. | Age | Sex | Symptoms | Form | Treatment |
---|---|---|---|---|---|---|
Present study | 1 | 7 | M | Vertigo, nausea, vomiting, tinittus, headache, local swelling | Monostotic | Nonsurgical |
2 | 16 | F | Headaches, skull asymmetry, HL, mass, EAC stenosis | Monostotic | Modified lateral petrosectomy with complete removal of the lesion | |
3 | 13 | F | HL, vertigo, tinittus, vomiting, visual disorder | Polyostotic | Nonsurgical | |
4 | 16 | M | Mastoiditis, HL, EAC stenosis, otalgia, otorrhea, mass, left ear cholesteatoma | Monostotic | Mastoidectomy, perisinus abscess drainage, revision | |
Previously published reports of temporal bone FD in children | ||||||
Articles added after screening the references | ||||||
Celenk et al. [34] | 1 | 17 | F | Painless, unilateral mandibular swelling | Polyostotic | Nonsurgical-periodic follow up |
Fattah et al. [7] | 2 | 6 | – | Facial asymmetry, malocclusion | Monostotic | No data |
3 | 6 | – | Mass | Monostotic | No data | |
4 | 17 | – | Mass, EAC stenosis | Monostotic | No data | |
5 | 14 | – | Mass | Polyostotic | No data | |
6 | 8 | – | Snoring, epistaxis, deteriorating acuity; | Polyostotic | No data | |
Couturier et al. [6] | 7 | 10 | M | EAC stenosis (normal hearing) | Monostotic | Canaloplasty, osseointegrated implant |
Reports not listed in PubMed reviewed by Megerian | ||||||
Megerian et al. [12] | 8 | 5 | F | Vertigo, SNHL, FN paralysis | Monostotic | Nonsurgical |
9 | 7 | F | HL, EAC stenosis | Monostotic | Nonsurgical | |
10 | 16 | M | Mass | Monostotic | Mastoid surgery | |
11 | 3 | M | Otitis, EAC stenosis, SNHL, EAC cholesteatoma | Polyostotic | Mastoidectomy, canaloplasty | |
12 | 17 | F | HL, EAC stenosis | Monostotic | Canaloplasty, mastoidectomy | |
13 | 11 | M | HL | Monostotic | Canaloplasty, mastoidectomy | |
14 | 11 | M | HL, mass, EAC stenosis, EAC cholesteatoma, FN palsy | Monostotic | Mastoid surgery | |
15 | 9 | M | HL, EAC stenosis | Polyostotic | Canaloplasty | |
16 | 16 | F | HL, otalgia, EAC stenosis | Monostotic | Nonsurgical | |
17 | 14 | M | HL, EAC stenosis | Monostotic | Mastoid surgery | |
Articles listed in PubMed | ||||||
Pardo-Maza et al. [11] | 18 | 16 | F | Recurrent suppurative otitis media, HL | Monostotic | Mastoidectomy, A surgical revision of the ear cavity, resecting the lesion and regularizing the cavity |
Du et al. [35] | 19 | 16 | M | Painless vision loss in both eyes | Polyostotic | Nonsurgical |
Shakeel et al. [36] | 20 | 16 | M | Painless swelling—temporal area, no other symptoms | Polyostotic | Affected temporal bone was resected, and gross total removal was achieved |
Yang et al. [37] | 21 | 15 | F | HL, EAC stenosis, EAC cholesteatoma | Monostotic | Canaloplasty |
Cai et al. [38] | 22 | 15 | M | Local lump, facial malformation | Monostotic | Surgical resection |
23 | 6 | M | Atypical headaches | Polyostotic | Surgical resection | |
24 | 15 | F | Atypical headaches, proptosis | Polyostotic | Complete removal and reconstruction with a titanium allegation implant | |
25 | 11 | M | Local lump | Monostotic | Complete removal and reconstruction with a titanium implant | |
26 | 14 | F | Local lump, HL | Polyostotic | Complete removal and reconstruction with a titanium implant | |
Jethanamest et al. [39] | 27 | 17 | M | HL, EAC atresia, cholesteatoma | Monostotic | Mastoidectomy, canaloplasty |
Kim et al. [13] | 28 | 17 | F | HL, EAC stenosis | Monostotic | Mastoidectomy, canaloplasty |
29 | 13 | M | HL, EAC stenosis, cholesteatoma | Monostotic | Mastoidectomy, canaloplasty | |
30 | 16 | M | HL, EAC stenosis | Monostotic | Mastoidectomy, canaloplasty | |
31 | 14 | M | EAC stenosis, ear fullness | Monostotic | Canaloplasty | |
32 | 14 | M | Sudden HL-fluctuating SNHL, vertigo | Monostotic | Transmastoid labyrinthectomy | |
Keskin et al. [40] | 33 | 16 | F | Painful facial swelling-mandible | Polyostotic | Segmental mandibulectomy, mass resection |
Martinez et al. [20] | 34 | 7 | M | HL, EAC stenosis, mass, proptosis, trismus, bleeding, head pain | Polyostotic | Nonsurgical-medical therapy, EAC debris removed |
Sreetharan et al. [41] | 35 | 16 | M | Postauricular swelling, EAC stenosis | Monostotic | Nonsurgical-managed conservatively |
Tweddle et al. [42] | 36 | 16 | M | Recurrent otitis externa, tinnitus, EAC stenosis, normal hearing | Monostotic | Mastoidectomy, EAC reconstruction |
Ozbek et al. [43] | 37 | 10 | F | HL, postauricular swelling, recurrent otitis externa, EAC stenosis | Monostotic | Nonsurgical-periodic CT scanning |
Magu et al. [44] | 38 | 15 | F | Headache, facial asymmetry–FN palsy | Polyostotic | No data |
Lustig et al. [45] | 39 | 15 | – | HL, EAC stenosis | McCune–Albright syndrome | Canalplasty |
40 | 11 | – | Proptosis | Polyostotic | Surgical drainage, cystoethmoidistomy, pericranial closure | |
41 | 17 | – | Headache | Monostotic | Middle fossa mass excision | |
Chinski et al. [25] | 42 | 16 | M | HL, EAC stenosis | Monostotic | Nonsurgical-periodically examined |
Ohta et al. [46] | 43 | 16 | M | Tumor | Monostotic | Surgical |
Megerian et al. [12] | 44 | 4 | M | Tumor, HL, otalgia, otorrhea, EAC stenosis, recurrent otitis externa | Monostotic | Nonsurgical |
45 | 9 | F | HL, EAC stenosis, otalgia, otitis externa | Monostotic | Surgical, infratemporal approach | |
46 | 12 | F | HL, EAC stenosis, canal cholesteatoma, aural pressure, facial asymmetry | Polyostotic | Mastoidectomy, canaloplasty | |
47 | 12 | F | HL, EAC stenosis, trismus, otalgia, otorrhea | Monostotic | Canaloplasty TMJ arthroplasty | |
48 | 5 | F | HL, EAC stenosis, mass | Polyostotic | Conservative | |
49 | 10 | F | HL, EAC stenosis, frontal region protrusion, aural fullness, facial asymmetry, otitis externa | Polyostotic | Surgical-maxilla | |
50 | 2 | M | EAC stenosis, HL in audio, FN paralysis | Monostotic | Mastoidectomy, canaloplasty, tympanoplasty, FN decompression | |
Mizuno et al. [47] | 51 | 16 | F | Temporal pain, temporal area deformation, malocclusion | McCune–Albright syndrome | Surgical-mandible |
Kessler et al. [48] | 52 | 15 | M | HL, EAC stenosis | Monostotic | Nonsurgical |
Talmi et al. [49] | 53 | 15 | F | Recurrent otitis externa, HL, EAC stenosis, ear fullness | Monostotic | Surgical, canaloplasty |
Younus et al. [50] | 54 | 9/12 | M | Mass, EAC stenosis | Monostotic | Mastoidectomy, canaloplasty |
Smoucha et al. [51] | 55 | 14 | M | HL, EAC stenosis, mass, otitis externa | Monostotic | Mastoidectomy, canaloplasty |
Sataloff et al. [52] | 56 | 9 | F | HL, EAC stenosis | Polyostotic | Mastoidectomy, canalplasty |
Nishioka et al. [53] | 57 | 11 | M | Epileptic attacks, mass, EAC stenosis | Monostotic | No data |
Nager et al. [54] | 58 | 10 | M | HL, EAC stenosis, EAC cholesteatoma | McCune–Albright syndrome | Canaloplasty |
59 | 10 | M | HL, EAC stenosis | Monostotic | PE tube | |
Barrionuevo et al. [17] | 60 | 11 | F | Mass, otalgia | Monostotic | Nonsurgical |
61 | 3 | F | Premature puberty | McCune–Albright syndrome | Nonsurgical | |
Williams et al. [55] | 62 | 10 | F | HL, EAC stenosis | Monostotic | Mastoidectomy, canalooplasty |
Talbot et al. [56] | 63 | 12 | M | HL, EAC stenosis | Monostotic | Canaloplasty |
Cohen et al. [57] | 64 | 8 | F | Mass, EAC stenosis, FN paralysis, SNHL | Polyostotic | Mastoid surgery |
65 | 9 | M | Mass, EAC stenosis | Monostotic | Mastoidectomy, canaloplasty | |
Wong et al. [58] | 66 | 13 | F | HL, mass, EAC stenosis, EAC cholesteatoma | Monostotic | Canaloplasty |
Case 1—7-year-old boy with tinnitus and vertigo
Anamnesis
Physical examination
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Treatment: We decided not to perform surgery because of considerable expansion of the FD in the skull base and risk of vestibular damage.
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Clinical course of the disease: The vertigo recurred throughout the 3-year follow-up period. Gradual deterioration in vestibular function was observed (35–65%). 3 years after the first hospitalization, the patient was again admitted to our hospital because of an episode of sudden deafness. His hearing gradually returned to normal after intravenous steroid treatment.
Case 2—16-year-old girl with conductive hearing loss and skull asymmetry
Anamnesis
Physical examination
Imaging findings
Treatment
Clinical course of the disease
Case 3—13-year-old girl with sensorineural hearing loss, tinnitus, and vertigo
Anamnesis
Clinical examination
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Treatment: Considering the extent and localization of the lesion, the decision was made not to operate on the patient.
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Clinical course of the disease: Long-term follow-up of the patient showed no evidence of lesion expansion but periodic worsening of her vertigo. At the time of this writing, the patient’s hearing was stable.
Case 4—16-year-old boy with acute mastoiditis, a left ear cholesteatoma, external auditory canal stenosis, and hearing loss
Anamnesis
Clinical examination
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Treatment: The patient was immediately qualified for surgical treatment. During the surgery, we observed secondary complete occlusion of the external auditory canal and a large cholesteatoma involving the auditory canal and middle ear. Extensive destruction of the left temporal bone was noted. The patient underwent canal wall down mastoidectomy. The walls of the sigmoid sinus were thickened due to an inflammatory process and perisinus abscess. We performed surgical drainage and ossicular reconstruction (incus interposition). The diagnosis of FD initially based on the imaging findings was confirmed pathologically. The initial surgical outcome and hearing improvement were good, but within a few months, severe conductive hearing loss recurred. Repeat imaging studies demonstrated disease progression with involvement of the tympanic cavity and destruction of the sound-transferring system. Postoperative MRI showed the possibility of residual cholesteatoma. The patient underwent revision surgery 12 months after the first surgery, and the tissue that had been observed by MRI appeared to be scar tissue and silastic sheeting.
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Clinical course of the disease: Over the next 4 years of follow-up, neither recurrent cholesteatoma nor FD progression was encountered. The patient was qualified for and successfully implanted with a transcutaneous bone conduction hearing aid and was under the care of an audiologist at the time of this writing.
Discussion
Symptoms
Diagnosis
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Pagetoid: This is the most common type, affecting about 56% of patients. It is characterized by areas of intermixed tissues of different density, with dense areas of sclerosis and radiolucent areas of fibrosis, resulting in a ground-glass appearance.
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Sclerotic: This is the second most common form, affecting about 23% of patients. It is characterized by homogeneous dense areas.