Acta Oto-Laryngologica. 2014; 134: 425–428
ORIGINAL ARTICLE
Subtotal facial nerve decompression for recurrent facial palsy in Melkersson Rosenthal syndrome
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
CHUANFU DAI1,2, JIANDONG LI3, SHIMING YANG2, LIANG ZHAO4, SHUI FENG4, YANG LI4, ZHIYAO SONG4, JIEYU LU4 & KUN ZHAO4 1
Department of Otolaryngology Head and Neck Surgery, Medical Center Tsinghua University, 2Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, 3Department of Otolaryngology, State Grid Electric Power Hospital and 4Department of Otolaryngology Head and Neck Surgery, Beijing Shijitan Hospital of Capital Medical University, Beijing, PR China
Abstract Conclusions: Subtotal facial nerve decompression seems effective to prevent further episodes of facial palsy and promote facial nerve recovery for recurrent facial palsy in Melkersson Rosenthal syndrome (MRS). The main inflammatory sites of recurrent facial palsy in MRS may be the mastoid segment, tympanic segment, geniculate ganglion, and labyrinthine segment. Objective: To present our surgical experience in preventing further episodes of facial palsy and improving facial nerve recovery of patients with recurrent facial palsy in MRS. Methods: We performed transmastoid subtotal facial nerve decompression on eight patients with recurrent facial palsy in MRS. They were followed up for 3.3 years on average (range 2–5 years). Results: There were no further attacks of facial palsy in any of the cases. Seven cases (87.5%) recovered to grade I or grade II, and three of eight cases (37.5%) recovered completely. We found obvious edema of the facial nerve at the mastoid segment in all cases, at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in only one case (12.5%).
Keywords: Facial nerve function, inflammatory site, mastoid segment, tympanic segment, geniculate ganglion, labyrinthine segment
Introduction Melkersson Rosenthal syndrome (MRS) is a rare disease, with unknown etiology and undetermined incidence, which was first described by Melkersson in 1928. MRS is classically defined as a triad of recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue (lingua plicata, LP) [1]. When two of the three symptoms appear, it is called the oligosymptomatic form [2]. Facial palsy is common in MRS patients, it is found in about one-third of cases [3], and recurrent facial palsy acounts for 70% of facial palsy in MRS [4]. Crego et al. found that 50%
of recurrent facial palsy had a second recurrence, and the interval between the first and second recurrence was almost half of that between the first episode of facial palsy and the first recurrence [5]. Recurrent facial palsy showed a worse prognosis when compared with non-recurrent palsy, and the facial nerve tended to deteriorate after repetitive recurrence [6]. Total or subtotal facial nerve decompression has been attempted in one or two cases with recurrent facial palsy in MRS, respectively, and no further episodes of facial palsy were reported during the follow-up [7,8]. We present our surgical experience of the largest case series of recurrent facial palsy in MRS.
Correspondence: Jiandong Li MD, Department of Otorhinolaryngology, State Grid Electric Power Hospital, Beijing 100073, PR China. E-mail: [email protected] and Shiming Yang MD, Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing 100039, PR China. E-mail: [email protected]
(Received 14 October 2013; accepted 28 October 2013) ISSN 0001-6489 print/ISSN 1651-2251 online Ó 2014 Informa Healthcare DOI: 10.3109/00016489.2013.863431
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
426
C. Dai et al.
Material and methods
Discussion
We performed subtotal facial nerve decompression in eight patients with recurrent facial palsy in MRS, which were clinically diagnosed as four with the triad form and four with the oligosymptomatic form. There were three females and five males, and the mean age was 23.5 ± 10.2 years (range 7–37 years). Preoperative facial nerve function was either grade V or grade VI based on the House-Brackmann grading system [9]. The patients had received prednisolone treatment in other hospitals for at least 3 weeks. They became doubtful about their treatment, since there were no signs of facial nerve recovery, and came to our Facial Lab for help. We performed subtotal facial nerve decompression, because electroneurography showed more than 95% degeneration of the facial nerve. They were followed up after surgery.
Recurrent facial palsy is usual in MRS with unknown etiology, and it tends to reattack after conservative treatment or self-resolution. It has been reported that outcomes of facial nerve function progressively deteriorate after repetitive attacks of facial palsy [6,10,11]. An electrophysiological study revealed that there is loss of action potential amplitude in cases of recurrent facial palsy in contrast to those with a single attack [12]. Furthermore, the risk of a further episode of facial palsy increases with every recurrence, from 15% on the second episode to 50% on the fourth attack [13]. From those perspectives, it appears that even prophylactic decompression of the facial nerve is reasonable to protect the facial nerve from potential damage. Some authors have attempted total or subtotal facial nerve decompression in a small number of patients with recurrent facial palsy in MRS, and it seemed that either total or subtotal decompression was effective to prevent further episodes of facial palsy [7,8,10]. Graham et al. [8] decompressed the whole course of the facial nerve in a young woman who had experienced eight episodes of facial palsy always accompanied by facial swelling within 8 years, and the follow-up showed that she was completely free of facial paralysis in spite of six episodes of facial edema during 30 months. Another example was a 27-yearold woman who had suffered a left-sided facial palsy at the age of 4, which recurred on an annual basis until the age of 14, and who underwent a decompression of the mastoid segment of the left facial nerve. The follow-up showed that the left facial palsy recurred but decreased in frequency, from an annual basis to a 3-year basis. Then she selected total facial nerve decompression, and two episodes of partial left facial
Results A summary of the cases with recurrent facial palsy in MRS is shown in Table I. The follow-up period was 3.3 ± 1.1 years (range 2–5 years).The median duration of the last facial palsy was 4 weeks (range 3– 13 weeks). Seven cases (87.5%) recovered to normal or near-normal level (grade I or grade II), among which three of eight cases (37.5%) recovered completely. There were no episodes of facial palsy on the surgical side during the follow-up, although orofacial edema reattacked one case. During the surgery, we found noticeable edema of the facial nerve at the mastoid segment in all cases (100%), at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in one case (12.5%). No cases developed serious hearing loss due to the surgical procedures.
Table I. Summary of cases with recurrent facial palsy in Melkersson-Rosenthal syndrome (MRS). Frequency of preoperative Postoperative Preoperative Postoperative Initial Duration of Final facial facial palsy episode facial palsy recurrent orofacial facial nerve facial palsy nerve Fissured Follow-up Case no. (times per year) episode orofacial edema edema function (weeks) function tongue (years) 1
2/10
0
Yes
No
V
11
I
Yes
2
2
2/10
0
Yes
No
VI
3
4/27
0
No
No
VI
3
II
Yes
2.5
7
III
Yes
4
4
3/9
0
No
No
5
2/7
0
Yes
No
V
4
II
Yes
4
VI
4
I
Yes
5
6
6/18
0
No
No
7
4/4
0
Yes
Yes
VI
13
II
Yes
3.5
V
3
II
Yes
3.5
8
2/6
0
No
No
V
3
I
Yes
2
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
Subtotal facial nerve decompression in MRS palsy occurred during the first year after surgery, each resolving within 24 h. She reported no further episodes during the next 10 years of follow-up [10]. It seemed that decompression of the mastoid segment only was not essential to prevent further recurrence of facial palsy, and total facial nerve decompression was more effective. In the present study, the mean interval required for one facial palsy recurrence for case nos 4, 5, 6, and 7 was 3 years, 3.5 years, 3 years, and 1 year, respectively, and there were no further episodes of facial palsy during the follow-up of 4 years, 5 years, 3.5 years, and 3.5 years, respectively, indicating that subtotal facial nerve decompression seemed effective to prevent further episodes of facial palsy in MRS. Typically, case no. 7 suffered episodes of facial palsy accompanied by orofacial edema on an annual basis for 4 years, and no further episodes of facial palsy occurred after surgery, although orofacial edema affected the patient three times within more than 3 years. However, case nos 1, 2, 3, and 8 were not followed up for a relatively long period to evaluate the further episodes sufficiently, and further follow-up was expected. Surgical intervention is controversial for Bell’s palsy, but most authors advocate the benefits of surgical decompression when electroneurography shows over 90–95% degeneration of the facial nerve. According to Fisch [14], patients with Bell’s palsy of more than 95% non-excitable fibers on the 14th day had a less than 50% chance of obtaining satisfactory facial nerve recovery, and 78.8% of those undergoing surgical decompression obtained good recovery. Gantz et al. [15] performed facial nerve decompression on patients with over 90% degeneration on electroneurography and no voluntary motor unit potentials on electromyography within 14 days of total paralysis, and found that 91% of 19 cases undergoing surgical decompression obtained grade I or II, versus only 42% of non-surgically treated patients. In the present study, all cases had more than 95% degeneration of the facial nerve, and they had a history of prednisolone treatment. After surgery, seven cases (87.5%) recovered to grade I or II, among which three cases recovered completely. In contrast, only 3 of 12 (25%) cases with recurrent facial palsy who had 90% or more denervation of the facial nerve and received prednisolone treatment only recovered to grade I or II based on Fisch’s experience [16]. It appeared that subtotal facial nerve decompression was valuable to promote facial nerve recovery from recurrent facial palsy. Surgical timing is also contentious. Yanagihara and his colleagues [17] proposed surgical treatment before day 30 in patients with more than 95% degeneration.
427
However, Gantz et al. [15] recommended surgical decompression before day 14. In the present study, the duration of facial palsy ranged from 3 to 13 weeks, with a median of 4 weeks. Three cases with facial palsy duration of 7 weeks or more also recovered well. We recommend early surgical decompression for cases with more than 95% degeneration of the facial nerve on electroneurography if possible, but surgical intervention could also be considered for those who had facial palsy duration of 1–3 months. Many surgeons opted for total facial nerve decompression for Bell’s palsy, since Fisch and Esslen proposed that the most likely site for neural compression and conduction block in Bell’s palsy was at the entrance to the meatal foramen [18]. However, there is no evidence demonstrating that total facial nerve decompression is better than subtotal facial nerve decompression either in preventing further episodes of facial palsy or in promoting facial nerve recovery. It is important to note that total facial nerve decompression via middle cranial fossa increases the risk of complications, such as seizure, sensorineural hearing loss, and cerebrospinal fluid leak. Of note, we found dramatic edema of the facial nerve at the mastoid segment in all cases, at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in one case (12.5%). The findings above indicate that the main inflammatory sites of recurrent facial palsy in MRS may be the mastoid segment, tympanic segment, and geniculate ganglion, with the labyrinthine segment accidentally involved. In the surgery, we decompressed the facial nerve from the stylomastoid foramen to the labyrinthine segment by the transmastoid approach. The surgical approach has been used by Yanagihara et al. [17], and we have also described the surgical procedures and possible complications in our previous papers [19,20]. To expose the geniculate ganglion and labyrinthine segment adequately, the incus is removed temporarily and then repositioned exactly. This procedure is significant to avoid surgical damage to the stapes and the inner ear, although there is a risk of the patient developing mild conductive hearing loss. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References [1] Greene RM, Rogers RS 3rd. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21:1263–70.
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
428
C. Dai et al.
[2] Rogers RS 3rd. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371–9. [3] Zimmer WM, Rogers RS 3rd, Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Pathol 1992;74:610–19. [4] Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A. Melkersson-Rosenthal syndrome. Otolaryngol Head Neck Surg 2008;138:246–51. [5] Crego F, Galindo J, Quesada P, Naches S, Piñas J, Vila J, et al. Recurrent peripheral facial paralysis. Our case load from 1995. Acta Otorrinolaringol Esp 1998;49:280–2. [6] Ralli G, Magliulo G. Bell’s palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387–90. [7] Yetiser S, Satar B, Kazkayasi M. Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis. Otol Neurotol 2002;23:772–8. [8] Graham MD, Kemink JL. Total facial nerve decompression in recurrent facial paralysis and the Melkersson-Rosenthal syndrome: a preliminary report. Am J Otol 1986;7:34–7. [9] House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Heck Surg 1985;93:146–7. [10] Doshi J, Irving R. Recurrent facial nerve palsy: the role of surgery. J Laryngol Otol 2010;124:1202–4.
[11] Boddie HG. Recurrent Bell’s palsy. J Laryngol Otol 1972;86: 117–20. [12] Mamoli B, Neumann H, Ehrmann L. Recurrent Bell’s palsy. Etiology, frequency, prognosis. J Neurol 1977;216:119–25. [13] Pitts DB, Adour KK, Hilsinger RL Jr. Recurrent Bell’s palsy: analysis of 140 patients. Laryngoscope 1988;98:535–40. [14] Fisch U. Surgery for Bell’s palsy. Arch Otolaryngol 1981; 107:1–11. [15] Gantz BJ, Rubinstein JT, Gidley P, Woodworth GG. Surgical management of Bell’s palsy. Laryngoscope 1999;109:1177–88. [16] No authors listed The facial nerve. Am J Otol 1986;7:235–7. [17] Yanagihara N, Hato N, Murakami S, Honda N. Transmastoid decompression of the facial nerve in Bell palsy. Arch Otolaryngol 1979;105:530–4. [18] Fisch U, Esslen E. Total intratemporal exposure of the facial nerve. Pathologic findings in Bell’s palsy. Arch Otolaryngol 1972;95:335–41. [19] Dai C, Li J, Guo L, Song Z. Surgical experience of intratemporal facial nerve neurofibromas. Acta Otolaryngol 2013; 33:893–6. [20] Dai C, Li J, Zhao L, Liu Y, Song Z, Li Y, et al. Surgical experience of nine cases with intratemporal facial hemangiomas and a brief literature review. Acta Otolaryngol 2013;133: 1117–20.
ORIGINAL ARTICLE
Subtotal facial nerve decompression for recurrent facial palsy in Melkersson Rosenthal syndrome
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
CHUANFU DAI1,2, JIANDONG LI3, SHIMING YANG2, LIANG ZHAO4, SHUI FENG4, YANG LI4, ZHIYAO SONG4, JIEYU LU4 & KUN ZHAO4 1
Department of Otolaryngology Head and Neck Surgery, Medical Center Tsinghua University, 2Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, 3Department of Otolaryngology, State Grid Electric Power Hospital and 4Department of Otolaryngology Head and Neck Surgery, Beijing Shijitan Hospital of Capital Medical University, Beijing, PR China
Abstract Conclusions: Subtotal facial nerve decompression seems effective to prevent further episodes of facial palsy and promote facial nerve recovery for recurrent facial palsy in Melkersson Rosenthal syndrome (MRS). The main inflammatory sites of recurrent facial palsy in MRS may be the mastoid segment, tympanic segment, geniculate ganglion, and labyrinthine segment. Objective: To present our surgical experience in preventing further episodes of facial palsy and improving facial nerve recovery of patients with recurrent facial palsy in MRS. Methods: We performed transmastoid subtotal facial nerve decompression on eight patients with recurrent facial palsy in MRS. They were followed up for 3.3 years on average (range 2–5 years). Results: There were no further attacks of facial palsy in any of the cases. Seven cases (87.5%) recovered to grade I or grade II, and three of eight cases (37.5%) recovered completely. We found obvious edema of the facial nerve at the mastoid segment in all cases, at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in only one case (12.5%).
Keywords: Facial nerve function, inflammatory site, mastoid segment, tympanic segment, geniculate ganglion, labyrinthine segment
Introduction Melkersson Rosenthal syndrome (MRS) is a rare disease, with unknown etiology and undetermined incidence, which was first described by Melkersson in 1928. MRS is classically defined as a triad of recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue (lingua plicata, LP) [1]. When two of the three symptoms appear, it is called the oligosymptomatic form [2]. Facial palsy is common in MRS patients, it is found in about one-third of cases [3], and recurrent facial palsy acounts for 70% of facial palsy in MRS [4]. Crego et al. found that 50%
of recurrent facial palsy had a second recurrence, and the interval between the first and second recurrence was almost half of that between the first episode of facial palsy and the first recurrence [5]. Recurrent facial palsy showed a worse prognosis when compared with non-recurrent palsy, and the facial nerve tended to deteriorate after repetitive recurrence [6]. Total or subtotal facial nerve decompression has been attempted in one or two cases with recurrent facial palsy in MRS, respectively, and no further episodes of facial palsy were reported during the follow-up [7,8]. We present our surgical experience of the largest case series of recurrent facial palsy in MRS.
Correspondence: Jiandong Li MD, Department of Otorhinolaryngology, State Grid Electric Power Hospital, Beijing 100073, PR China. E-mail: [email protected] and Shiming Yang MD, Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing 100039, PR China. E-mail: [email protected]
(Received 14 October 2013; accepted 28 October 2013) ISSN 0001-6489 print/ISSN 1651-2251 online Ó 2014 Informa Healthcare DOI: 10.3109/00016489.2013.863431
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
426
C. Dai et al.
Material and methods
Discussion
We performed subtotal facial nerve decompression in eight patients with recurrent facial palsy in MRS, which were clinically diagnosed as four with the triad form and four with the oligosymptomatic form. There were three females and five males, and the mean age was 23.5 ± 10.2 years (range 7–37 years). Preoperative facial nerve function was either grade V or grade VI based on the House-Brackmann grading system [9]. The patients had received prednisolone treatment in other hospitals for at least 3 weeks. They became doubtful about their treatment, since there were no signs of facial nerve recovery, and came to our Facial Lab for help. We performed subtotal facial nerve decompression, because electroneurography showed more than 95% degeneration of the facial nerve. They were followed up after surgery.
Recurrent facial palsy is usual in MRS with unknown etiology, and it tends to reattack after conservative treatment or self-resolution. It has been reported that outcomes of facial nerve function progressively deteriorate after repetitive attacks of facial palsy [6,10,11]. An electrophysiological study revealed that there is loss of action potential amplitude in cases of recurrent facial palsy in contrast to those with a single attack [12]. Furthermore, the risk of a further episode of facial palsy increases with every recurrence, from 15% on the second episode to 50% on the fourth attack [13]. From those perspectives, it appears that even prophylactic decompression of the facial nerve is reasonable to protect the facial nerve from potential damage. Some authors have attempted total or subtotal facial nerve decompression in a small number of patients with recurrent facial palsy in MRS, and it seemed that either total or subtotal decompression was effective to prevent further episodes of facial palsy [7,8,10]. Graham et al. [8] decompressed the whole course of the facial nerve in a young woman who had experienced eight episodes of facial palsy always accompanied by facial swelling within 8 years, and the follow-up showed that she was completely free of facial paralysis in spite of six episodes of facial edema during 30 months. Another example was a 27-yearold woman who had suffered a left-sided facial palsy at the age of 4, which recurred on an annual basis until the age of 14, and who underwent a decompression of the mastoid segment of the left facial nerve. The follow-up showed that the left facial palsy recurred but decreased in frequency, from an annual basis to a 3-year basis. Then she selected total facial nerve decompression, and two episodes of partial left facial
Results A summary of the cases with recurrent facial palsy in MRS is shown in Table I. The follow-up period was 3.3 ± 1.1 years (range 2–5 years).The median duration of the last facial palsy was 4 weeks (range 3– 13 weeks). Seven cases (87.5%) recovered to normal or near-normal level (grade I or grade II), among which three of eight cases (37.5%) recovered completely. There were no episodes of facial palsy on the surgical side during the follow-up, although orofacial edema reattacked one case. During the surgery, we found noticeable edema of the facial nerve at the mastoid segment in all cases (100%), at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in one case (12.5%). No cases developed serious hearing loss due to the surgical procedures.
Table I. Summary of cases with recurrent facial palsy in Melkersson-Rosenthal syndrome (MRS). Frequency of preoperative Postoperative Preoperative Postoperative Initial Duration of Final facial facial palsy episode facial palsy recurrent orofacial facial nerve facial palsy nerve Fissured Follow-up Case no. (times per year) episode orofacial edema edema function (weeks) function tongue (years) 1
2/10
0
Yes
No
V
11
I
Yes
2
2
2/10
0
Yes
No
VI
3
4/27
0
No
No
VI
3
II
Yes
2.5
7
III
Yes
4
4
3/9
0
No
No
5
2/7
0
Yes
No
V
4
II
Yes
4
VI
4
I
Yes
5
6
6/18
0
No
No
7
4/4
0
Yes
Yes
VI
13
II
Yes
3.5
V
3
II
Yes
3.5
8
2/6
0
No
No
V
3
I
Yes
2
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
Subtotal facial nerve decompression in MRS palsy occurred during the first year after surgery, each resolving within 24 h. She reported no further episodes during the next 10 years of follow-up [10]. It seemed that decompression of the mastoid segment only was not essential to prevent further recurrence of facial palsy, and total facial nerve decompression was more effective. In the present study, the mean interval required for one facial palsy recurrence for case nos 4, 5, 6, and 7 was 3 years, 3.5 years, 3 years, and 1 year, respectively, and there were no further episodes of facial palsy during the follow-up of 4 years, 5 years, 3.5 years, and 3.5 years, respectively, indicating that subtotal facial nerve decompression seemed effective to prevent further episodes of facial palsy in MRS. Typically, case no. 7 suffered episodes of facial palsy accompanied by orofacial edema on an annual basis for 4 years, and no further episodes of facial palsy occurred after surgery, although orofacial edema affected the patient three times within more than 3 years. However, case nos 1, 2, 3, and 8 were not followed up for a relatively long period to evaluate the further episodes sufficiently, and further follow-up was expected. Surgical intervention is controversial for Bell’s palsy, but most authors advocate the benefits of surgical decompression when electroneurography shows over 90–95% degeneration of the facial nerve. According to Fisch [14], patients with Bell’s palsy of more than 95% non-excitable fibers on the 14th day had a less than 50% chance of obtaining satisfactory facial nerve recovery, and 78.8% of those undergoing surgical decompression obtained good recovery. Gantz et al. [15] performed facial nerve decompression on patients with over 90% degeneration on electroneurography and no voluntary motor unit potentials on electromyography within 14 days of total paralysis, and found that 91% of 19 cases undergoing surgical decompression obtained grade I or II, versus only 42% of non-surgically treated patients. In the present study, all cases had more than 95% degeneration of the facial nerve, and they had a history of prednisolone treatment. After surgery, seven cases (87.5%) recovered to grade I or II, among which three cases recovered completely. In contrast, only 3 of 12 (25%) cases with recurrent facial palsy who had 90% or more denervation of the facial nerve and received prednisolone treatment only recovered to grade I or II based on Fisch’s experience [16]. It appeared that subtotal facial nerve decompression was valuable to promote facial nerve recovery from recurrent facial palsy. Surgical timing is also contentious. Yanagihara and his colleagues [17] proposed surgical treatment before day 30 in patients with more than 95% degeneration.
427
However, Gantz et al. [15] recommended surgical decompression before day 14. In the present study, the duration of facial palsy ranged from 3 to 13 weeks, with a median of 4 weeks. Three cases with facial palsy duration of 7 weeks or more also recovered well. We recommend early surgical decompression for cases with more than 95% degeneration of the facial nerve on electroneurography if possible, but surgical intervention could also be considered for those who had facial palsy duration of 1–3 months. Many surgeons opted for total facial nerve decompression for Bell’s palsy, since Fisch and Esslen proposed that the most likely site for neural compression and conduction block in Bell’s palsy was at the entrance to the meatal foramen [18]. However, there is no evidence demonstrating that total facial nerve decompression is better than subtotal facial nerve decompression either in preventing further episodes of facial palsy or in promoting facial nerve recovery. It is important to note that total facial nerve decompression via middle cranial fossa increases the risk of complications, such as seizure, sensorineural hearing loss, and cerebrospinal fluid leak. Of note, we found dramatic edema of the facial nerve at the mastoid segment in all cases, at the tympanic segment and geniculate ganglion in five cases (62.5%), and at the labyrinthine segment in one case (12.5%). The findings above indicate that the main inflammatory sites of recurrent facial palsy in MRS may be the mastoid segment, tympanic segment, and geniculate ganglion, with the labyrinthine segment accidentally involved. In the surgery, we decompressed the facial nerve from the stylomastoid foramen to the labyrinthine segment by the transmastoid approach. The surgical approach has been used by Yanagihara et al. [17], and we have also described the surgical procedures and possible complications in our previous papers [19,20]. To expose the geniculate ganglion and labyrinthine segment adequately, the incus is removed temporarily and then repositioned exactly. This procedure is significant to avoid surgical damage to the stapes and the inner ear, although there is a risk of the patient developing mild conductive hearing loss. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References [1] Greene RM, Rogers RS 3rd. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21:1263–70.
Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15 For personal use only.
428
C. Dai et al.
[2] Rogers RS 3rd. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371–9. [3] Zimmer WM, Rogers RS 3rd, Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Pathol 1992;74:610–19. [4] Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A. Melkersson-Rosenthal syndrome. Otolaryngol Head Neck Surg 2008;138:246–51. [5] Crego F, Galindo J, Quesada P, Naches S, Piñas J, Vila J, et al. Recurrent peripheral facial paralysis. Our case load from 1995. Acta Otorrinolaringol Esp 1998;49:280–2. [6] Ralli G, Magliulo G. Bell’s palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387–90. [7] Yetiser S, Satar B, Kazkayasi M. Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis. Otol Neurotol 2002;23:772–8. [8] Graham MD, Kemink JL. Total facial nerve decompression in recurrent facial paralysis and the Melkersson-Rosenthal syndrome: a preliminary report. Am J Otol 1986;7:34–7. [9] House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Heck Surg 1985;93:146–7. [10] Doshi J, Irving R. Recurrent facial nerve palsy: the role of surgery. J Laryngol Otol 2010;124:1202–4.
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