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Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence
AMER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Original contribution
Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence☆ Zulin Tan, MM a , Yang Zhang, BM a , Wei Chen, MD a , Weixi Gong, MM a , Jiapeng Zhao, MM b , Xianrong Xu, MM c,⁎ a b c
Department of Otorhinolaryngology, General Hospital of Air Force, Beijing, P.R.C Department of Neurosurgery Third Affiliated Hospital of Xinxiang Medical College, Xinxiang City, Henan Province P.R.C The Center of Clinical Aviation Medicine, General Hospital of Air Force, Beijing, P.R.C
ARTI CLE I NFO
A BS TRACT
Article history:
Objective: To study the role of total facial nerve decompression in preventing further
Received 13 September 2014
recurrence of facial palsy in Melkersson Rosenthal syndrome (MRS). Methods: Total facial nerve decompression was performed on nine patients with recurrent facial palsy in MRS, and prednisolone treatment was given to 6 cases who declined surgery. They were incorporated into surgery group and control group, respectively. Patients in surgery group and control group were followed up for 5.4 ± 1.4 years (range, 4 to 8 years) and 6.0 ± 1.4 years (range, 4 to 8 years), respectively. Results: Further episodes of facial palsy affected none of 9 cases (0.0%) in surgery group, while they affected 3 of 6 cases (50.0%) in control group, with significant difference (p < 0.05). Conclusions: Total facial nerve decompression was effective to prevent further episodes of facial palsy in MRS. © 2014 Published by Elsevier Inc.
1.
Introduction
Melkersson Rosenthal syndrome (MRS) was firstly described by Melkersson in 1928. Classically, it is a triad syndrome of recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue (lingua plicata, LP) [1]. When there are only two of the three symptoms present, it is called
oligosymptomatic MRS form [2]. It is rarely reported, with unknown etiology. The incidence of MRS with facial palsy is 0.36 in 100,000 per year [3]. Facial palsy is found in 33% of MRS patients [4], which is recurrent in 70% of the patients [5]. Recurrent facial palsy tends to reattack continuously [6], and facial nerve function seems to deteriorate after repetitive recurrence [7]. Total or subtotal facial nerve decompression was attempted in few
☆
Copyright transfer: In consideration of the American Journal of Otolaryngology's reviewing and editing my submission, "Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence", the authors undersigned transfers, assigns and otherwise conveys all copyright ownership to Elsevier Inc. in the event that such work is published in the American Journal of Otolaryngology.Signed by Zulin Tan, Yang Zhang, Wei Chen, Weixi Gong, Jiapeng Zhao, Xianrong Xu. ⁎ Corresponding author at: The Center of Clinical Aviation Medicine, General Hospital of Air Force, Beijing 100142, P.R.C. E-mail address: [email protected] (X. Xu). http://dx.doi.org/10.1016/j.amjoto.2014.12.001 0196-0709/© 2014 Published by Elsevier Inc.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
2
AMER ICA N JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D N E CK M EDI CI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
cases with recurrent facial palsy in MRS by a few authors, and it appeared that it was able to prevent further episodes of facial palsy [8,9]. We aim to present the role of total facial nerve decompression in preventing further episodes of facial palsy in MRS.
2.
Surgical techniques: We firstly performed standard mastoidectomy and decompressed mastoid segment and tympanic segment of facial nerve, and then decompressed facial nerve from geniculate ganglion to the internal auditory canal segment by middle cranial fossa approach. After total decompression, the sheath was cut along the nerve, and steroid soaked gel foams were placed over the nerve.
Materials and methods
We carried out a prospective study, which involved a consecutive series of 15 patients with recurrent facial palsy in MRS, 8 cases in triad and 7 in oligosymptomatic form. The diagnosis was all clinically confirmed without orofacial biopsy. There were 6 female and 9 male, and the aged ranged from 6 to 39 years, 24.6 ± 12.4 years on average. Among them, 9 cases underwent total facial nerve decompression through middle cranial fossa combined with transmastoid approach, and 6 cases who declined surgery were administered prednisolone (1 mg/kg/d for 10 days). They surgery was performed within 3 weeks after the last episode of facial palsy. They were classified into surgery group and control group, respectively. Facial nerve function was assessed by the House– Brackmann facial nerve grading system [10]. Patients in surgery group and control group were followed up for 5.4 ± 1.4 years (range, 4 to 8 years) and 6.0 ± 1.4 years (range, 4 to 8 years), respectively. Facial palsy recurrence rate of the two groups was compared by Fisher’s Exact Test, and mean age compared by t-test, with SPSS 16.0 software involved.
3.
Results
Table 1 lists summary of patients in the study. The median duration of facial palsy of the last episode was 4.1 ± 1.9 weeks (range, 2 to 8 weeks) and 4.0 ± 1.8 weeks (range, 2 to 7 weeks) in surgery group and control group, respectively. The sex ratio and facial palsy frequency before intervention of the two groups were similar (female/male ratio, 4/5 versus 2/4; facial palsy frequency, 31 times/109 years versus 15 times/62 years), and there was no significant difference in mean age between surgery group and control group (p > 0.05). None of 9 cases (0.0%) in surgery group suffered further episodes of facial palsy at the surgical side during the follow-up, although orofacial edema reattacked three cases, compared to 3 of 6 cases (50%) in control group (p < 0.05). 8 of 9 cases (88.9%) in surgery group recovered to normal or nearnormal level (Grade I or Grade II) in contrast to 4 of 6 cases (66.7%) in control group. There were no noticeable complications. During surgery, we didn’t find any soft tissue swelling around facial nerve but swelling of facial nerve itself. The swelling sites were mainly mastoid segment, tympanic segment, and geniculate ganglion.
Table 1 – Summary of 15 cases with Melkersson Rosenthal syndrome. No.
FPF before treatment (times/year)
Side
Total facial nerve decompression 1 2/8 L 2 3/13 R 3 2/9 L 6/20 R1/L5 4a 5 5/5 R 3/14 L 6b 5/13 R1/L4 7c 8 2/11 L 9 3/16 L Prednisolone treatment 10 2/9 L 11 2/6 R 12 2/13 L 3/6 R 13 d 14 4/16 L 15 2/12 R
FP after treatment
ROE before treatment
OE after treatment
Initial FNF
FP duration (weeks)
Final FNF
FT
FU (yr)
0 0 0 0 0 0 0 0 0
No No No Yes No Yes Yes Yes No
No No No Yes No Yes No Yes No
IV V IV VI IV III IV II IV
8 3 6 3 5 4 3 3 2
II II I III II I I I I
Yes Yes Yes Yes Yes Yes No Yes Yes
4 5 7 6 8 5 4 4 6
0 2 0 3/1 1 0
Yes No Yes Yes Yes No
No No No No Yes No
II III II IV V III
7 3 5 4 2 3
I II I III III I
Yes Yes Yes Yes Yes Yes
5 6 7 8 6 4
Cases 1–9 underwent total facial nerve decompression, and Cases 10–15 received prednisolone treatment. Duration of facial palsy refers to the last episode. FPF, facial palsy frequency; FP, facial palsy; ROE, recurrent orofacial edema; OE, orofacial edema; FNS, facial nerve function; FT, fissured tough; FU, follow-up. a Case 4 suffered facial palsy at the right side once and at the left side 5 times before surgery, and underwent surgery at the left side. b Case 6 underwent surgical decompression of the left facial nerve, and reported no further episodes of facial palsy at the left side, but suffered facial palsy at the right side once after surgery. c Case 7 suffered facial palsy at the right side once and at the left side 4 times before surgery, and underwent surgery at the left side. d Case 13 suffered further episodes of facial palsy at the right side three times and at the left side once after treatment.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
AMER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
4.
Discussions
Recurrent facial palsy tended to recur after conservative treatment or self-resolution, and a few reports showed that outcomes of facial nerve became progressively poor after repetitive episodes [7,11,12], although Pitts et al. [13] stated that the recovery profile and recovery index of patients with RFP were not affected by recurrence. An electrophysiological study also indicated that there was a greater loss of action potential amplitude in cases who had multiple episodes of facial palsy compared to those with a single attack [14]. Thereby, it was reasonable to prevent further episodes of facial palsy in order to avoid potential damage. It appeared that either total or subtotal facial nerve decompression was able to successfully prevent further episodes of facial palsy [8,9,11]. Malcolm D et al. [9] performed total facial nerve decompression on a young woman, who had 8 episodes of facial palsy always accompanied by facial swelling within eight years, and she was then completely free of facial palsy despite 6 episodes of facial edema within 30 months. Yetiser S et al. [8] reported three cases of recurrent facial palsy, who underwent subtotal facial nerve decompression and revealed no further episodes of facial palsy within 4 to 9 years. However, decompression of mastoid segment sole appeared insufficient to prevent further attacks of facial palsy [11]. Since the reports above didn’t have control group, which couldn’t provide a relatively solid evidence, we designed the clinical trial and aimed to document the effect of total facial nerve decompression in preventing further episodes of facial palsy in MRS. In our study, facial palsy recurrence rate in surgery group was 0.0% in contrast to 50% in control group during the followup (p < 0.05). Given that there was no marked difference in follow-up period, sex ratio, age and facial palsy frequency before intervention between surgery group and control group and the related bias could be ignored, our results demonstrated that total facial nerve decompression was effective to prevent further episodes of facial palsy in MRS. Typically, episodes of facial palsy were always accompanied by orofacial edema in Case 4, 6 and 8 in surgery group before operation, while they were completely free of facial palsy after operation in spite of further attacks of orofacial edema. Regarding outcomes of facial nerve, 88.9% of the patients in surgery group recovered to normal or near-normal level, while 66.7% of the patients in control group returned to Grade I or Grade II. There was no significant difference (p > 0.05), indicating that total facial nerve decompression could not directly promote recovery of facial nerve in MRS and the main value was to prevent potential damage of facial nerve caused by repetitive episodes of facial palsy. Subtotal facial nerve decompression was also a surgical option to be considered. Yetiser S [8] decompressed facial nerve between stylomastoid foramen and geniculate ganglion in 4 cases with recurrent facial palsy in MRS through transmastoid approach, and it appeared that subtotal facial nerve decompression was also effective to prevent recurrence of facial palsy at the surgical side. However, there was no control group in the study and subtotal facial nerve decompression via transmastoid approach may result in mild conductive hearing loss in minor cases [15–17].
3
The middle fossa approach was another possible choice. Nyberg and Fish [18] decompressed the labyrinthine segment, geniculate ganglion and meatal portion of facial nerve in patients with recurrent facial palsy via middle cranial fossa approach, and successfully prevented recurrence of facial palsy in 80% of the patients. Similar to subtotal facial nerve decompression, it also lacked control group and the conclusion was not convincing enough. In the past, there was no robust evidence to indicate whether surgery could really prevent recurrence of facial palsy, and the literature offered only a small number of case series which reported their follow-up results without control group. Our study was the first one, to our knowledge, which provided a relatively robust evidence to demonstrate the value of total facial nerve decompression in preventing further episodes of facial palsy in MRS.
Conflict of interests None
REFERENCES
[1] Greene RM, Rogers III RS. Melkersson–Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21: 1263–70. [2] Rogers III RS. Melkersson–Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371–9. [3] Peitersen E. Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies. Acta Otolaryngol Suppl 2002;549:4–30. [4] Zimmer WM, Rogers III RS, Reeve CM, et al. 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;74:610–9. [5] Kanerva M, Moilanen K, Virolainen S, et al. Melkersson– Rosenthal syndrome. Otolaryngol Head Neck Surg 2008;138: 246–51. [6] Crego F, Galindo J, Quesada P, et al. Recurrent peripheral facial paralysis. Our case load from 1995. Acta Otorrinolaringol Esp 1998;49:280–2. [7] Ralli G, Magliulo G. Bell’s palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387–90. [8] Yetiser S, Satar B, Kazkayasi M. Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis. Otol Neurotol 2002;23:772–8. [9] 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. [10] Hous JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146–7. [11] Doshi J, Irving R. Recurrent facial nerve palsy: the role of surgery. J Laryngol Otol 2010;124:1202–4. [12] Boddie HG. Recurrent Bell’s palsy. J Laryngol Otol 1972;86: 117–20. [13] Pitts DB, Adour KK, Hilsinger Jr RL. Recurrent Bell’s palsy: analysis of 140 patients. Laryngoscope 1988;98: 535–40. [14] Mamoli B, Neumann H, Ehrmann L. Recurrent Bell's palsy. Etiology, frequency, prognosis. J Neurol 1977;216: 119–25.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
4
AMER ICA N JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D N E CK M EDI CI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
[15] Yanagihara N, Hato N, Murakami S, et al. Transmastoid decompression as a treatment of Bell palsy. Otolaryngol Head Neck Surg 2001;124:282–6. [16] Dai C, Li J, Guo L, et al. Surgical experience of intratemporal facial nerve neurofibromas. Acta Otolaryngol 2013;33:893–6.
[17] Dai C, Li J, Zhao L, et al. Surgical experience of nine cases with intratemporal facial hemangiomas and a brief literature review. Acta Otolaryngol 2013;133:1117–20. [18] Nyberg P, Fisch U. Surgical treatment and results of idiopathic recurrent facial palsy. In: Portmann M, editor. Facial nerve. New York: Masson; 1985. p. 259–68.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Original contribution
Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence☆ Zulin Tan, MM a , Yang Zhang, BM a , Wei Chen, MD a , Weixi Gong, MM a , Jiapeng Zhao, MM b , Xianrong Xu, MM c,⁎ a b c
Department of Otorhinolaryngology, General Hospital of Air Force, Beijing, P.R.C Department of Neurosurgery Third Affiliated Hospital of Xinxiang Medical College, Xinxiang City, Henan Province P.R.C The Center of Clinical Aviation Medicine, General Hospital of Air Force, Beijing, P.R.C
ARTI CLE I NFO
A BS TRACT
Article history:
Objective: To study the role of total facial nerve decompression in preventing further
Received 13 September 2014
recurrence of facial palsy in Melkersson Rosenthal syndrome (MRS). Methods: Total facial nerve decompression was performed on nine patients with recurrent facial palsy in MRS, and prednisolone treatment was given to 6 cases who declined surgery. They were incorporated into surgery group and control group, respectively. Patients in surgery group and control group were followed up for 5.4 ± 1.4 years (range, 4 to 8 years) and 6.0 ± 1.4 years (range, 4 to 8 years), respectively. Results: Further episodes of facial palsy affected none of 9 cases (0.0%) in surgery group, while they affected 3 of 6 cases (50.0%) in control group, with significant difference (p < 0.05). Conclusions: Total facial nerve decompression was effective to prevent further episodes of facial palsy in MRS. © 2014 Published by Elsevier Inc.
1.
Introduction
Melkersson Rosenthal syndrome (MRS) was firstly described by Melkersson in 1928. Classically, it is a triad syndrome of recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue (lingua plicata, LP) [1]. When there are only two of the three symptoms present, it is called
oligosymptomatic MRS form [2]. It is rarely reported, with unknown etiology. The incidence of MRS with facial palsy is 0.36 in 100,000 per year [3]. Facial palsy is found in 33% of MRS patients [4], which is recurrent in 70% of the patients [5]. Recurrent facial palsy tends to reattack continuously [6], and facial nerve function seems to deteriorate after repetitive recurrence [7]. Total or subtotal facial nerve decompression was attempted in few
☆
Copyright transfer: In consideration of the American Journal of Otolaryngology's reviewing and editing my submission, "Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence", the authors undersigned transfers, assigns and otherwise conveys all copyright ownership to Elsevier Inc. in the event that such work is published in the American Journal of Otolaryngology.Signed by Zulin Tan, Yang Zhang, Wei Chen, Weixi Gong, Jiapeng Zhao, Xianrong Xu. ⁎ Corresponding author at: The Center of Clinical Aviation Medicine, General Hospital of Air Force, Beijing 100142, P.R.C. E-mail address: [email protected] (X. Xu). http://dx.doi.org/10.1016/j.amjoto.2014.12.001 0196-0709/© 2014 Published by Elsevier Inc.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
2
AMER ICA N JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D N E CK M EDI CI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
cases with recurrent facial palsy in MRS by a few authors, and it appeared that it was able to prevent further episodes of facial palsy [8,9]. We aim to present the role of total facial nerve decompression in preventing further episodes of facial palsy in MRS.
2.
Surgical techniques: We firstly performed standard mastoidectomy and decompressed mastoid segment and tympanic segment of facial nerve, and then decompressed facial nerve from geniculate ganglion to the internal auditory canal segment by middle cranial fossa approach. After total decompression, the sheath was cut along the nerve, and steroid soaked gel foams were placed over the nerve.
Materials and methods
We carried out a prospective study, which involved a consecutive series of 15 patients with recurrent facial palsy in MRS, 8 cases in triad and 7 in oligosymptomatic form. The diagnosis was all clinically confirmed without orofacial biopsy. There were 6 female and 9 male, and the aged ranged from 6 to 39 years, 24.6 ± 12.4 years on average. Among them, 9 cases underwent total facial nerve decompression through middle cranial fossa combined with transmastoid approach, and 6 cases who declined surgery were administered prednisolone (1 mg/kg/d for 10 days). They surgery was performed within 3 weeks after the last episode of facial palsy. They were classified into surgery group and control group, respectively. Facial nerve function was assessed by the House– Brackmann facial nerve grading system [10]. Patients in surgery group and control group were followed up for 5.4 ± 1.4 years (range, 4 to 8 years) and 6.0 ± 1.4 years (range, 4 to 8 years), respectively. Facial palsy recurrence rate of the two groups was compared by Fisher’s Exact Test, and mean age compared by t-test, with SPSS 16.0 software involved.
3.
Results
Table 1 lists summary of patients in the study. The median duration of facial palsy of the last episode was 4.1 ± 1.9 weeks (range, 2 to 8 weeks) and 4.0 ± 1.8 weeks (range, 2 to 7 weeks) in surgery group and control group, respectively. The sex ratio and facial palsy frequency before intervention of the two groups were similar (female/male ratio, 4/5 versus 2/4; facial palsy frequency, 31 times/109 years versus 15 times/62 years), and there was no significant difference in mean age between surgery group and control group (p > 0.05). None of 9 cases (0.0%) in surgery group suffered further episodes of facial palsy at the surgical side during the follow-up, although orofacial edema reattacked three cases, compared to 3 of 6 cases (50%) in control group (p < 0.05). 8 of 9 cases (88.9%) in surgery group recovered to normal or nearnormal level (Grade I or Grade II) in contrast to 4 of 6 cases (66.7%) in control group. There were no noticeable complications. During surgery, we didn’t find any soft tissue swelling around facial nerve but swelling of facial nerve itself. The swelling sites were mainly mastoid segment, tympanic segment, and geniculate ganglion.
Table 1 – Summary of 15 cases with Melkersson Rosenthal syndrome. No.
FPF before treatment (times/year)
Side
Total facial nerve decompression 1 2/8 L 2 3/13 R 3 2/9 L 6/20 R1/L5 4a 5 5/5 R 3/14 L 6b 5/13 R1/L4 7c 8 2/11 L 9 3/16 L Prednisolone treatment 10 2/9 L 11 2/6 R 12 2/13 L 3/6 R 13 d 14 4/16 L 15 2/12 R
FP after treatment
ROE before treatment
OE after treatment
Initial FNF
FP duration (weeks)
Final FNF
FT
FU (yr)
0 0 0 0 0 0 0 0 0
No No No Yes No Yes Yes Yes No
No No No Yes No Yes No Yes No
IV V IV VI IV III IV II IV
8 3 6 3 5 4 3 3 2
II II I III II I I I I
Yes Yes Yes Yes Yes Yes No Yes Yes
4 5 7 6 8 5 4 4 6
0 2 0 3/1 1 0
Yes No Yes Yes Yes No
No No No No Yes No
II III II IV V III
7 3 5 4 2 3
I II I III III I
Yes Yes Yes Yes Yes Yes
5 6 7 8 6 4
Cases 1–9 underwent total facial nerve decompression, and Cases 10–15 received prednisolone treatment. Duration of facial palsy refers to the last episode. FPF, facial palsy frequency; FP, facial palsy; ROE, recurrent orofacial edema; OE, orofacial edema; FNS, facial nerve function; FT, fissured tough; FU, follow-up. a Case 4 suffered facial palsy at the right side once and at the left side 5 times before surgery, and underwent surgery at the left side. b Case 6 underwent surgical decompression of the left facial nerve, and reported no further episodes of facial palsy at the left side, but suffered facial palsy at the right side once after surgery. c Case 7 suffered facial palsy at the right side once and at the left side 4 times before surgery, and underwent surgery at the left side. d Case 13 suffered further episodes of facial palsy at the right side three times and at the left side once after treatment.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
AMER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY X X (2 0 1 4) XXX – XXX
4.
Discussions
Recurrent facial palsy tended to recur after conservative treatment or self-resolution, and a few reports showed that outcomes of facial nerve became progressively poor after repetitive episodes [7,11,12], although Pitts et al. [13] stated that the recovery profile and recovery index of patients with RFP were not affected by recurrence. An electrophysiological study also indicated that there was a greater loss of action potential amplitude in cases who had multiple episodes of facial palsy compared to those with a single attack [14]. Thereby, it was reasonable to prevent further episodes of facial palsy in order to avoid potential damage. It appeared that either total or subtotal facial nerve decompression was able to successfully prevent further episodes of facial palsy [8,9,11]. Malcolm D et al. [9] performed total facial nerve decompression on a young woman, who had 8 episodes of facial palsy always accompanied by facial swelling within eight years, and she was then completely free of facial palsy despite 6 episodes of facial edema within 30 months. Yetiser S et al. [8] reported three cases of recurrent facial palsy, who underwent subtotal facial nerve decompression and revealed no further episodes of facial palsy within 4 to 9 years. However, decompression of mastoid segment sole appeared insufficient to prevent further attacks of facial palsy [11]. Since the reports above didn’t have control group, which couldn’t provide a relatively solid evidence, we designed the clinical trial and aimed to document the effect of total facial nerve decompression in preventing further episodes of facial palsy in MRS. In our study, facial palsy recurrence rate in surgery group was 0.0% in contrast to 50% in control group during the followup (p < 0.05). Given that there was no marked difference in follow-up period, sex ratio, age and facial palsy frequency before intervention between surgery group and control group and the related bias could be ignored, our results demonstrated that total facial nerve decompression was effective to prevent further episodes of facial palsy in MRS. Typically, episodes of facial palsy were always accompanied by orofacial edema in Case 4, 6 and 8 in surgery group before operation, while they were completely free of facial palsy after operation in spite of further attacks of orofacial edema. Regarding outcomes of facial nerve, 88.9% of the patients in surgery group recovered to normal or near-normal level, while 66.7% of the patients in control group returned to Grade I or Grade II. There was no significant difference (p > 0.05), indicating that total facial nerve decompression could not directly promote recovery of facial nerve in MRS and the main value was to prevent potential damage of facial nerve caused by repetitive episodes of facial palsy. Subtotal facial nerve decompression was also a surgical option to be considered. Yetiser S [8] decompressed facial nerve between stylomastoid foramen and geniculate ganglion in 4 cases with recurrent facial palsy in MRS through transmastoid approach, and it appeared that subtotal facial nerve decompression was also effective to prevent recurrence of facial palsy at the surgical side. However, there was no control group in the study and subtotal facial nerve decompression via transmastoid approach may result in mild conductive hearing loss in minor cases [15–17].
3
The middle fossa approach was another possible choice. Nyberg and Fish [18] decompressed the labyrinthine segment, geniculate ganglion and meatal portion of facial nerve in patients with recurrent facial palsy via middle cranial fossa approach, and successfully prevented recurrence of facial palsy in 80% of the patients. Similar to subtotal facial nerve decompression, it also lacked control group and the conclusion was not convincing enough. In the past, there was no robust evidence to indicate whether surgery could really prevent recurrence of facial palsy, and the literature offered only a small number of case series which reported their follow-up results without control group. Our study was the first one, to our knowledge, which provided a relatively robust evidence to demonstrate the value of total facial nerve decompression in preventing further episodes of facial palsy in MRS.
Conflict of interests None
REFERENCES
[1] Greene RM, Rogers III RS. Melkersson–Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21: 1263–70. [2] Rogers III RS. Melkersson–Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371–9. [3] Peitersen E. Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies. Acta Otolaryngol Suppl 2002;549:4–30. [4] Zimmer WM, Rogers III RS, Reeve CM, et al. 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;74:610–9. [5] Kanerva M, Moilanen K, Virolainen S, et al. Melkersson– Rosenthal syndrome. Otolaryngol Head Neck Surg 2008;138: 246–51. [6] Crego F, Galindo J, Quesada P, et al. Recurrent peripheral facial paralysis. Our case load from 1995. Acta Otorrinolaringol Esp 1998;49:280–2. [7] Ralli G, Magliulo G. Bell’s palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387–90. [8] Yetiser S, Satar B, Kazkayasi M. Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis. Otol Neurotol 2002;23:772–8. [9] 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. [10] Hous JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146–7. [11] Doshi J, Irving R. Recurrent facial nerve palsy: the role of surgery. J Laryngol Otol 2010;124:1202–4. [12] Boddie HG. Recurrent Bell’s palsy. J Laryngol Otol 1972;86: 117–20. [13] Pitts DB, Adour KK, Hilsinger Jr RL. Recurrent Bell’s palsy: analysis of 140 patients. Laryngoscope 1988;98: 535–40. [14] Mamoli B, Neumann H, Ehrmann L. Recurrent Bell's palsy. Etiology, frequency, prognosis. J Neurol 1977;216: 119–25.
Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001
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Please cite this article as: Tan Z, et al, Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression.., Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.12.001