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) . When there are only two of the three symptoms present, it is called oligosymptomatic MRS form . It is rarely reported, with unknown etiology.

The incidence of MRS with facial palsy is 0.36 in 100,000 per year . Facial palsy is found in 33% of MRS patients , which is recurrent in 70% of the patients . Recurrent facial palsy tends to reattack continuously , and facial nerve function seems to deteriorate after repetitive recurrence . Total or subtotal facial nerve decompression was attempted in few 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 . We aim to present the role of total facial nerve decompression in preventing further episodes of facial palsy in MRS.

2

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 . 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.

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.

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 near-normal level (Grade I or Grade II) in contrast to 4 of 6 cases (66.7%) in control group. There were no noticeable complications.

Table 1

Summary of 15 cases with Melkersson Rosenthal syndrome.

No.	FPF before treatment (times/year)	Side	FP after treatment	ROE before treatment	OE after treatment	Initial FNF	FP duration (weeks)	Final FNF	FT	FU (yr)
Total facial nerve decompression
1	2/8	L	0	No	No	IV	8	II	Yes	4
2	3/13	R	0	No	No	V	3	II	Yes	5
3	2/9	L	0	No	No	IV	6	I	Yes	7
4 a	6/20	R1/L5	0	Yes	Yes	VI	3	III	Yes	6
5	5/5	R	0	No	No	IV	5	II	Yes	8
6 b	3/14	L	0	Yes	Yes	III	4	I	Yes	5
7 c	5/13	R1/L4	0	Yes	No	IV	3	I	No	4
8	2/11	L	0	Yes	Yes	II	3	I	Yes	4
9	3/16	L	0	No	No	IV	2	I	Yes	6
Prednisolone treatment
10	2/9	L	0	Yes	No	II	7	I	Yes	5
11	2/6	R	2	No	No	III	3	II	Yes	6
12	2/13	L	0	Yes	No	II	5	I	Yes	7
13 d	3/6	R	3/1	Yes	No	IV	4	III	Yes	8
14	4/16	L	1	Yes	Yes	V	2	III	Yes	6
15	2/12	R	0	No	No	III	3	I	Yes	4

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