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Oral Rehabilitation in Neurofibromatosis Type 1- A Six Year Follow-Up Case Report


Affiliations
1 Department of Periodontics and Implantology, Aditya Dental College, Beed – 431122, Maharashtra, India
2 Department of Periodontology, Government Dental College and Hospital, Nagpur - 440003, Maharashtra, India
 

Neurofibromatosis is an autosomal dominant disorder that affects the bone, the nervous system, soft-tissue, and the skin. The expressivity of the disease is extremely variable, with manifestations ranging from mild lesions to several complications and functional impairment. The penetrance, otherwise, is 100%. Approximately 72% of Neurofibromatosis Type1 patients present with oral manifestations. These patients have an estimated 3-15% additional risk of malignant disease in their life-time. The following report presents a 32 year old lady diagnosed with Neurofibromatosis Type 1 in childhood who presented to department with complain of bleeding gums. Radiograph showed shallow sigmoid notch on left side, obtuse and thinned out left angle of mandible which are some additional features. A multidisciplinary approach of dental and medical specialties was taken here for oral rehabilitation and to manage and treat periodontal manifestations and other oral complaints. Case was followed for more than 6 years. Neurofibromatosis has no cure hence when such patients report to dental professionals it is up to them to act as primary care giver and instill positive frame of mind to help reduce stressful life events.


Keywords

Multidisciplinary Approach, Neurofibromatosis Type 1, Oral Manifestations, Oral Rehabilitation, Periodontal Manifestations, Shallow Sigmoid Notch.
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  • Friedman JM. Neurofibromatosis 1. Clinical Genetics. In: Friedman JM, Gutmann DH, MacCollin M, Riccardi VM, editors. Neurofibromatosis: Phenotype, Natural History and Pathogenesis. 3rd ed. Baltimore: Johns Hopkins University Press; 1999. p. 110–8.

  • Gorlin RJ, Cohen MM, Levin LF. Syndromes of the head and neck. p. 353–416. Oxford: Oxford University Press, 1990.

  • Gutmann DH, Aylsworth A, Carey JC et al. The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 1997; 278:51–57. https://doi.org/10.1001/jama.1997.03550010065042

  • Eiti Singh, Khushboo Singh, Sunita Gupta, Mayank Shailat. Use of advanced imaging in diagnosis of neurofibromatosis 1. J Oral Maxillofac Radiol 2018; 6: 36–41. https://doi.org/10.4103/jomr.jomr_15_18

  • D’Ambrosio JA, Langlais RP, Young RS. Jaw and skull changes in neurofibromatosis. Oral Surg Oral Med Oral Pathol 1988; 66: 391–396. https://doi.org/10.1016/00304220(88)90252-6

  • Marx RE, Stern D. Oral and Maxillofacial Pathology: A Rationale for diagnosis and treatment. 2nd ed. Illinois: Quintessence Publishing Co Inc; 2003. p. 422–7.

  • Shapiro SD, Abramovitch K, Van Dis ML et al Neurofibromatosis: oral and radiographic manifestations.

  • Oral Surg Oral Med Oral Pathol 1984; 58: 493–498. https://doi.org/10.1016/0030-4220(84)90350-5

  • Neville BW, Damm DD, Allen CM et al Oral and Maxillofacial Pathology. Philadelphia: WB Saunders Company, 1995, pp. 381–383.

  • Visnapuu V, Peltonen S, Tammisalo T, et al. Radiographic findings in the jaws of patients with Neurofibromatosis 1. J Oral Maxillofac Surg., 2012; 70: 1351–1357. https://doi.org/10.1016/j.joms.2011.06.204

  • Krishnamoorthy B, Singh P, Gundareddy S.N, et al. Notching in the posterior border of the ramus of mandible in a patient with Neurofibromatosis Type I- A case report. J Clin Diagn Res., 2013; 7: 2390–2391. https://doi.org/10.7860/JCDR/2013/5952.3534

  • Friedrich RE, Giese M, Schmelzle R, Mautner VF, Scheuer HA. Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1: a descriptive analysis of 48 patients based on panoramic radiographs and oral findings. J Cranio Maxillofac Surg. 2003; 31: 1–9.

  • https://doi.org/10.1016/S1010-5182(02)00160-9

  • Mahajan A,Dixit J and Bharadwaj A. Gingival enlargement in Neurofibromatosis Type I: A Case report and literature review. J. Contemp. Dent. Pract., 2010; 11: 57–63. https://doi.org/10.5005/jcdp-11-2-57

  • Doufexi A, Mina M and Ionnidou E. Gingival overgrowth in children: Epidemiology, Pathogenesis and Complications. A literarture review. J Periodontol, 2005; 76: 3–10. https://doi.org/10.1902/jop.2005.76.1.3

  • Reinhard E. Freidrich and Anika Reul. Decayed, missing and restored teeth in patients with Nuerofibromatosis Type I, J Clin Exp Dent, Feb 2018; 10(2): e107–115.

  • Riccardi VM, Eichner JE. Neurofi bromatosis: Phenotype, Natural History, and Pathogenesis. 1sted. Baltimore: John Hopkins University Press; 1986, p. 37–55.

  • Daphne L. Wang, Kelly B. Smith, Sonia Esparza. et al. Emotional functioning of patients with neurofibromatosis tumour suppressing syndrome. Genet Med. Dec 2012; 14 (12): 977–982. https://doi.org/10.1038/gim.2012.85


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  • Oral Rehabilitation in Neurofibromatosis Type 1- A Six Year Follow-Up Case Report

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Authors

Somnath Pralhad Pachrupe
Department of Periodontics and Implantology, Aditya Dental College, Beed – 431122, Maharashtra, India
Mangesh Phadnaik
Department of Periodontology, Government Dental College and Hospital, Nagpur - 440003, Maharashtra, India
Pooja Gupta
Department of Periodontics and Implantology, Aditya Dental College, Beed – 431122, Maharashtra, India

Abstract


Neurofibromatosis is an autosomal dominant disorder that affects the bone, the nervous system, soft-tissue, and the skin. The expressivity of the disease is extremely variable, with manifestations ranging from mild lesions to several complications and functional impairment. The penetrance, otherwise, is 100%. Approximately 72% of Neurofibromatosis Type1 patients present with oral manifestations. These patients have an estimated 3-15% additional risk of malignant disease in their life-time. The following report presents a 32 year old lady diagnosed with Neurofibromatosis Type 1 in childhood who presented to department with complain of bleeding gums. Radiograph showed shallow sigmoid notch on left side, obtuse and thinned out left angle of mandible which are some additional features. A multidisciplinary approach of dental and medical specialties was taken here for oral rehabilitation and to manage and treat periodontal manifestations and other oral complaints. Case was followed for more than 6 years. Neurofibromatosis has no cure hence when such patients report to dental professionals it is up to them to act as primary care giver and instill positive frame of mind to help reduce stressful life events.


Keywords


Multidisciplinary Approach, Neurofibromatosis Type 1, Oral Manifestations, Oral Rehabilitation, Periodontal Manifestations, Shallow Sigmoid Notch.

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DOI: https://doi.org/10.18311/ijmds%2F2021%2F26284