Role of dental clinical and imaging exam during McCune Albright Syndrome diagnosis process: Case Report/ Importância do exame clínico e imaginológico odontológico durante o processo diagnóstico da Síndrome de McCune Albright: Relato de Caso
DOI:
https://doi.org/10.34119/bjhrv2n5-066Keywords:
Mccune Albright Syndrome, craniofacial complex, diagnosis process.Abstract
McCune Albright Syndrome (MAS) is a rare disease characterized by the classic triad of early puberty that begins in the embryonic period and mainly affects women. The present study aims to report the case of a 27-year-old patient, melanodermic, referred to the oral and maxillofacial surgery service of the Walter Cantídio University Hospital, complaining of pain in the right mandibular body region. During the anamnesis, the patient reported having had her menarche at the age of 6. Clinically, it was observed body asymmetry, prominence of the right zygomatic, frontal and parietal bones, and expansion of the right hemimandible. Panoramic radiography showed images with ground-glass appearance in the region of the direct hemi-mandible with mixed radiopacity and root resorption, suggestive of MAS. The patient was referred to the gynecologist and endocrinologist, and it was observed that the patient had the presence of cyst in the right ovary. Incisional biopsy was performed on the right maxilla, which showed histopathological characteristics compatible with fibrous dysplasia, with final diagnosis of MAS. In summary, the present study reinforces the importance of a meticulous and interdisciplinary clinical examination allied to the patient's complaint in order to establish an adequate treatment planReferences
Albright F, Butler AM, Hampton AO, Smith P. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation, and endocrine dysfunction, with precocious puberty in females. N Engl J Med 1937;216:727-41.
Vasilev V, Daly AF, Thiry A, Petrossians P, Fina F, Rostomyan L, Silvy M, Enjalbert A, Barlier A, Beckers A (2014) McCune- Albright syndrome: a detailed pathological and genetic analysis of disease effects in an adult patient. J Clin Endocrinol Metab 99: E2029–E2038
Collins MT, Singer FR, Eugster E. McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia. Orphanet J Rare Dis. 2012;7(Suppl 1):S4.
Robinson C, Collins MT, Boyce AM. Fibrous Dysplasia/McCune-Albright syndrome: clinical and translational perspectives. Curr Osteoporos Rep 2016; 14:178–86. 10.1007/s11914-016-0317-0
Collins MT, Riminucci M, Bianco P. Fibrous dysplasia. In: Rosen C, editor. Primer on the metabolic bone diseases and disorders of mineral metabolism. 8th American Society of Bone and Mineral Research; Washington, D.C: 2013. pp. 786–793.
Lietman SA, Levine MA. Fibrous dysplasia. Pediatr Endocrinol Rev. 2 01;;2:389-96.
ROKA, Yam Bahadur et al. Turkish Neurosurgery, Vol: 20, No: 4, 508-511508Clinical, radiological and Endocrinological Findings in a Case of McCune-Albright Syndrome. Turkish Neurosurgery. Nepal, p. 508-511. out. 2010;
Leopardi, M., Parziale, V., Ciavarella, D., & Chimenti, C. Report Maxillary bone lesions in McCune-Albright syndrome: a case. Progress in Orthodontics, 2011; 12(1), 84–89;
Dumitrescu CE Collins MT McCune-Albright syndrome. Orphanet J Rare Dis. 2008;3:1-12. 10:1186/1750-1172-3-12.
Ziadi S, Trimeche M, Mokni M, Sriha B, Khochtali H, Korbi S. Eighteen cases of craniofacial fibrous dysplasia. Rev Stomatol Chir Maxillofac. 2009; 110:318–22
Collins MT, Singer FR, Eugster E. McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia. Orphanet J Rare Dis. 2012;7 Suppl 1:S4.
Deshpande A, Naidu GS, Dara BG, Gupta M. Craniofacial fibrous dysplasia: A summary of findings with radiological emphasis. J Indian Acad Oral Med Radiol. 2016; 28:403–8.
Bowers CA, et al. Pregnancy-induced cystic degeneration of fibrous dysplasia. Can J Neurol Sci. 2012;39(6):828–9.
Boyce AM, et al. Association of hearing loss and otologic outcomes with fibrous dysplasia. JAMA Otolaryngol Head Neck Surg. 2018. 144(2):102–107
Kabali, T.M., Moshy, J.R., Owibingire, S.S. et al. Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports. BMC Oral Health. 2019
Wong SC, Zacharin M. Long-term health outcomes of adults with McCune- Albright syndrome. Clin Endocrinol. 2017;87(5):627–34.
Laven JS, et al. Management of infertility in a patient presenting with ovarian dysfunction and McCune-Albright syndrome. J Clin Endocrinol Metab. 2004;89(3):1076–8.
Chanson P, Salenave S, Young J. Ovarian dysfunction by activating mutation of GS alpha: McCune-Albright syndrome as a model. Ann Endocrinol (Paris). 2010;71(3):210–3.
Lavoue V, et al. Restoration of ovulation after unilateral ovariectomy in a woman with McCune-Albright syndrome: a case report. Eur J Endocrinol. 2008;158(1):131–4.
Collins MT, et al.Renal phosphate wasting in fibrous dys- plasia of bone is part of a generalized renal tubular dysfunction similar to that seen in tumor-induced osteomalacia. J Bone Miner Res. 2001. 16(5):806–813.
Riminucci M, et al.FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. J Clin Invest. 2003. 112(5):683–692.
Javaid MK, Boyce A, Appelman-Dijkstra N, et al. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 2019;14(1):139. Published 2019 Jun 13. doi:10.1186/s13023-019-1102-9
Lee JS, et al.Normal vision despite narrowing of the optic canal in fibrous dysplasia. N Engl J Med 347(21):1670–1676. 2002
Burke AB, Collins MT, Boyce AM. Fibrous dyspla- sia of bone: craniofacial and dental implications. Oral Dis. 2017. 23(6):697–708.
Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 2001;127:1239–1247.
Yongjing G, Huawei L, Zilai P, Bei D, Hao J, Kemin C. McCune-Albright syndrome: radiological and MR findings. JBR-BTR. 2001;84:250–252.
Sunday O. Akintoye, Alison M. Boyce, Michael T. Collins. Dental Perspectives in Fibrous Dysplasia and Mccune-Albright Syndrome. Oral Surgery, Oral Medicine, Oral Pathology, and Oral Radiology. 2013.
Ben hadj Hamida F, Jlaiel R, Ben Rayana N, Mahjoub H, Mellouli T, Ghorbel M, et al. Craniofacial fibrous dysplasia: A case report. J Fr Ophtalmol. 2005.
Bulakba?i N, Bozlar U, Karademir I, Kocao?lu M, Somuncu I. CT and MRI in the evaluation of craniospinal involvement with polyostotic fibrous dysplasia in McCune-Albright syndrome. Diagn Interv Radiol. 2008;14:177–81
Chevalier N, et al. Postpubertal persistent Hyperestrogenemia in McCune- Albright syndrome: unilateral oophorectomy improved fertility but detected an unexpected borderline epithelial ovarian tumor. J Pediatr Adolesc Gynecol. 2015;28(6):e169–72.
