Melkersson-Rosenthal Syndrome in Pediatric Age Group

Abstract

Melkersson-Rosenthal Syndrome (MRS) is a disease characterized by peripheral facial paralysis, facial edema and fissured tongue. Appearance of this triad is rare, generally monosymptomatic or oligosymptomatic involvement is observed and difficulties or delays in diagnosis can occur due to scarcity of the disease. One or two of the findings and presence of cheilitis granulomatosa in biopsy are sufficient for the diagnosis. Melkersson Rosenthal Syndrome should be considered in differential diagnosis of recurrent facial paralysis. Although findings of the syndrome can regress spontaneously or with medical treatment, it can take a progressive course in some patients, requiring surgical treatment (facial nerve decompression). In this article a pediatric case with diagnosis of Melkersson Rosenthal Syndrome is presented.

Keywords

• Melkersson-Rosenthal syndrome, orofacial edema, recurrent facial paralysis.

Melkersson-Rosenthal Syndrome in Pediatric Age Group

Abstract

Melkersson-Rosenthal sendromu (MRS); periferik fasiyal paralizi, fasiyal ödem ve cografik dil triadı ile karakterize bir hastalıktır. Hastalık yüz, dudaklar, oral kavite ve fasiyal sinirde ödeme yol açan granülomatöz patoloji ile seyreder. Sendromun bulgularının kendiliğinden veya medikal tedaviyle gerileyebilmesine karşın, bazı hastalarda progresif seyredebilmekte ve cerrahi tedavi (fasiyal sinir dekompresyonu) gerektirmektedir. Klasik belirtiler triadının görülmesi nadirdir ve genellikle monosemptomatik veya oligo semtomatik tutulum izlenir ayrıca hastalığın nadir görülmesinden dolayı tanı koymada güçlükler veya gecikmeler söz konusu olmaktadır. Bulgulardan bir veya ikisi ile biyopside granülamotöz keilitin varlığı kesin tanı için yeterlidir. Melkerson Rosenthal sendromu tekrarlayan fasiyal paralizilerin ayırıcı tanısında düşünülmesi gereken bir hastalıklardandır ve bu yazıda Melkersson Rosenthal sendromu tanısı konulan bir pediatrik hasta sunulmuştur

Keywords

• Melkersson Rosenthal Sendromu, orofasiyal ödem, tekrarlayan fasiyal paralizi

References

1. Ang KL, Jones NS. Melkersson-Rosenthal syndrome. J Laryngol Otol 2002; 116: 386-8.
2. Kesler A, Vainstein G, Gadot N. Melkersson-Rosenthal syndrome treated by methylprednisolone. Neurology 1998;51:1440-1.
3. Micheal S, Sara P, Henry S. Melkersson-Rosenthal syn- drome in the perioculer area: A review of the literature and case report. Ann Plastic Surg 2003;150:664-8.
4. Ziem PE, Pfrommer C, Goerdt S, Orfanos CE, Blume- Peytavi U. Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 2000; 143: 860-3.
5. Pèrez-Calderòn R, Gonzalo-Garijo MA, Chaves A, De Argila D. Cheilits granulomatosa of Melkersson Rosenthal syndrome: Treatment with intralesional corticosteroid in- jections. Allergol Immunopathol 2004; 32(1): 36-8.
6. Camacho-Alonso F, Bermejo-Fenoli A, Lòpez-Jornet P. Miescher’s cheilitis granulomatosa. A presentation of five cases. Med Oral Patol 2004; 9: 425-9.
7. Shapiro M, Peters S, Spinelli HM. Melkersson-Rosenthal syndrome in the periocular area: a review of the litera- ture and case report. Ann Plast Surg 2003; 50: 644-8.
8. Van der Waal R, Shulten E, Van de Scheur MR,Wauters I, Starink TM, Van der Waal I. Cheilitis granulomatosa. JEADV 2001; 15: 519-23

9. Zimmer WM, Rogers RS, 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;74:610-9.
10. Cockerham KP, Hidayat AA, Cockerham GC, et al. Melkersson- Rosenthal syndrome: new clinicopathologic findings in 4 cases. Arch Ophthalmol 2000;118:227-32.
11. Greco F, Barbagallo M L, Guglielmino R, Sorge G. Recurrent facial nevre palsy associated with anti-GQ1b IgG antibod- ies. Brain & Development 2008; 30: 606-8.
12. Melek H, Köken R, Bükülmez A, et al. Melkersson- Rosenthal Sendromu: Bir Olgu Sunumu. Güncel Pediatri 2007; 5: 82-4.
13. Devriese PP, Schumacher T, Scheide A, de Jongh RH, Houtkooper JM. Incidence, prognosis and recovery of Bell's palsy. A survey of about 1000 patients (1974-1983). Clin Otolaryngol Allied Sci 1990;15:15-27
14. Dodi I, Verri R, Brevi B, et al. Monosymptomatic Melkersson-Rosenthal syndrome in an 8-year old boy. Acta Biomed 2006; 77: 20-3.
15. Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A. Melkersson-Rosenthal syndrome. Otolaryngol Head Neck Surg 2008;138(2):246-51.
16. Pearce JM. Melkersson’s syndrome. J Neurol Neurosurg Psychiatry 1995;58(3):340.
17. Kemal Ö, Özgürsoy OB, Dursun G, Tulunay Ö. Turkiye Klinikleri J Med Sci 2007, 27:128-31.
18. Pino Rivero P, Gonzalez Palomino A, Pantoja Hernandez CG, et al. Melkersson-Rosenthal syndrome. Report of a case with bilateral facial palsy. An Otorrinolaringol Ibero Am 2005;32:437-43.
19. Gerressen M, Alitreza G, Stockbrinck G, Riediger D, Zadeh MZ.Melkersson-Rosenthal syndrome: case report of a 30- year misdiagnosis. J Oral Maxillofac Surg 2005;63:1035-9.
20. Khandpur S, Malhotra AK, Khanna N. Melkersson- Rosenthal syndrome with diffuse facial swelling and mul- tiple cranial nevre palsies. J Dermatol 2006;33:411-4.
21. Smeets E, Fryns JP, Van den Berghe H. Melkersson- Rosenthal syndrome and de novo autosomal t(9;21) (p11;p11) translocation. Clin Genet 1994;45:323-4.
22. Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis: presentation,pathology and management of 13 cases. J Oral Pathol Med 2003;32:576-85.
23. Khouri JM, Bohane TD, Day AS. Is orofacial granulomato- sis in children a feature of Crohn's disease? Acta Paediatr 2005;94:501-4.
24. Sunil N, Showat M, Richard M. Total decompression offa- cial nerve for Melkersson-Rosenthal syndrome. J Laryngol Otol 2000;114:8703.