Home | About Us | Editorial Board | Current Issue | Archives | Search | Instructions | Subscription | Feedback | e-Alerts | Login 
Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
 Users Online: 2460  
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2021  |  Volume : 39  |  Issue : 2  |  Page : 221-224

Chairside endodontic management of a child with fibrodysplasia ossificans progressiva

1 KidsDentist® Exclusive Pediatric Dentistry Centers, Bengaluru, Karnataka, India
2 Department of Pedodontics and Preventive Dentistry, KAHER's KLE VK Institute of Dental Sciences, Belagavi, Karnataka, India

Date of Submission26-Apr-2020
Date of Decision09-Feb-2021
Date of Acceptance22-Feb-2021
Date of Web Publication29-Jul-2021

Correspondence Address:
Dr. Rajesh Hemant Bariker
KidsDentistR Exclusive Pediatric Dentistry Centers, HSR Layout, Sector-3, Bengaluru - 560 102
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JISPPD.JISPPD_192_20

Rights and Permissions



Fibrodysplasia ossificans progressiva (FOP) is an exceptionally atypical genetic disorder characterized by heterotopic bone formation within skeletal muscles, ligaments, tendons, and other connective tissues that affects one in 2 million people. A 4-year-old girl with FOP was referred to our practice with complaints of pain and discomfort in both sides of the lower jaw. Clinical examination revealed deep multiple caries and buccal gingival abscess in relation to multiple teeth. Endodontic care and conservative dental procedures were planned and performed chairside after a detailed discussion with FOP medical and dental experts. Very brief dental appointments were conducted with breaks to prevent muscle fatigue. No usage of regional anesthesia or dental dam clamps was done. The patient and her parents were counseled for oral hygiene maintenance, and periodic topical fluoride treatments were performed during successive follow-up appointments. The child is followed for 34 months post-treatment. The dental treatment modifications implemented for the present case were enough to institute good oral health and to prevent the creation of heterotopic ossifications in the maxillofacial region.

Keywords: Caries, fibrodysplasia ossificans progressiva, heterotopic ossification, pediatrics, pediatric dentistry

How to cite this article:
Bariker RH, Hugar SM. Chairside endodontic management of a child with fibrodysplasia ossificans progressiva. J Indian Soc Pedod Prev Dent 2021;39:221-4

How to cite this URL:
Bariker RH, Hugar SM. Chairside endodontic management of a child with fibrodysplasia ossificans progressiva. J Indian Soc Pedod Prev Dent [serial online] 2021 [cited 2022 Aug 11];39:221-4. Available from: http://www.jisppd.com/text.asp?2021/39/2/221/322496

   Introduction Top

Fibrodysplasia ossificans progressiva (FOP) is a particularly infrequent genetic disorder with autosomal dominant inheritance pattern branded by heterotopic bone formation within skeletal muscles, ligaments, tendons, and other connective tissues. Largely cases are caused by unprompted mutations within the ACVR1 gene, which is coupled with the activin A receptor, a bone morphogenetic protein receptor.[1],[2] ACVR1 was accredited as the gene responsible for FOP and the mutation c.617G>A, matching to Arg206His (R206H) of the ACVR1 gene, was found in all patients with classic FOP.[3] Misdiagnosis is common.[4] The solitary evident clinical sign of FOP at birth is malformation of the great toes. Soft tissue swellings leading to heterotopic ossification often appear in the first decade of life.[2],[5] The exacerbation of these ossifications can be caused by dental treatment, ensuing disease progression.[6] The temporomandibular joints (TMJ) possibly will be involved early on and are susceptible to trauma at any age. Proper oral health measures and prevention of caries are imperative in FOP.[7] Scores of patients with FOP are alas not treated for caries early in life owing to the trepidation that dental manipulation may deteriorate the condition. Other reported reasons for nontreatment are lack of familiarity on how to access decayed teeth for restoration and need of knowledge about proper local and general anesthetic care.[5]

   Case Report Top

Clinical history

A 4-year-old female patient of Asian race with a diagnosis of FOP was referred to our practice with the complaints of pain and discomfort in both sides of the lower jaw. The patient has two healthy siblings and the other members of the family had no similar findings of FOP. The patient had no previous history of dental treatment. The patient presented clinical characters of the FOP such as decreased mobility of peripheral joints (knees, elbows, and shoulders), vertebral column involvement, lateral deviation, and shortness of the great toes. Mouth opening was distinctly limited [Figure 1]. Intraoral examination revealed deep multiple caries and buccal gingival abscess in relation to 75 and 85 [Figure 2]a and [Figure 2]b, along with dental caries in few other teeth. Intraoral periapical radiographs [Figure 3]a and [Figure 3]b obtained and a diagnosis of chronic irreversible pulpitis with 74 and chronic furcal abscess with nondraining sinus with 75 and 85.
Figure 1: Photograph showing early diagnostic feature of fibrodysplasia ossificans progressiva. (note the hallux valgus deformity)

Click here to view
Figure 2: (a) Dental caries with 84, 85, and buccal gingival abscess with 85 (b) Dental caries with 74, 75, and buccal gingival abscess with 75

Click here to view
Figure 3: (a) Preoperative radiograph with 84 85 (b) Preoperative radiograph with 74,75

Click here to view


Corticosteroid prophylaxis was given after a detailed discussion with medical and dental experts, and consent for dental treatment was taken from the child's parents. It included tablet prednisolone 1–2 mgs/kg, (per oral) one day before treatment and three days post visit to prevent flare-ups and then was slowly tapered.[8]

Endodontic and conservative care

The execution of endodontic care and conservative dental procedures were planned and performed chairside. The dental chair was positioned at 45° to endow with more soothe and to shun exacerbating the disease. Positioning concern is fundamental as patients' bodies are often fused in a rigid position. However, all pressure points must be padded and the neck supported. Pulpectomy with 74, 75, and 85 was carried out under intrapulpal anesthesia and in multiple appointment sessions over an 8-week period and obturated with calcium hydroxide with iodoform (Metapex, Meta Biomed/Korea), followed by a restorative glass ionomer cement (GIC) (Ketac Molar, 3M ESPE, St. Paul, MN, USA) and stainless steel crown (3M ESPE, St. Paul, MN, USA) for final restoration. Excavation and review were done in relation to 84, followed by restoration with stainless steel crown Hall's technique (3M ESPE, St. Paul, MN, USA). Other carious teeth were restored with GIC (Ketac Molar, 3M ESPE, St. Paul, MN, USA) [Figure 4].
Figure 4: Post pulp therapy and final restoration with stainless steel crown with 74,75 and 85. Glass ionomer cement restoration with 84

Click here to view


The timeline of treatment is detailed in [Table 1].
Table 1: Timeline of the patient

Click here to view


Post-treatment, the patient and her parents were counseled for oral hygiene maintenance, and recurrent fluoride application was performed during successive follow-up appointments. The patient was evaluated both clinically and radiographically for consecutive periods of 3, 12, 24, and 34 months, respectively [Figure 5]. A orthopantomograph was taken [Figure 6]. No adverse effects including heterotopic ossifications were noticed during the course of follow-up period. A dislodged restoration was during the followup (tooth #84) and was restored with GIC, followed by a stainless steel crown (Hall's technique).
Figure 5: Thirty-four-month follow-up mandibular arch

Click here to view
Figure 6: Thirty-four-month follow-up orthopantomograph

Click here to view

   Discussion Top

Clinical diagnosis of FOP is apparent in the late stages; genetic analysis be confirmatory. In the pediatric age group, misdiagnosis is frequent as the active lesions are mistaken for infection and the chronic lesions are mistaken for benign bone tumors or fibromatosis.[9] Hence, genotyping of the ACVR1gene is of assistance for confirming or excluding a diagnosis of FOP in clinically affected or ambiguous patients. The most universal recurrent allele c.617 G>A, p.R206H, is also the most common in Indian patients with FOP.[10],[11]

Patients with FOP have plentiful oral health-care issues that entail enduring and diligent attention to prevent and minimization of risk when restorative care is necessary.[5] To the best of our knowledge, it is the first report of chairside endodontic treatment in a child with FOP with follow-up for 34 months. This report shows that chairside dental management is possible, with assiduous precautions and under extenuating circumstances for patients with FOP. It is recommended that dental anesthesia be limited to intrapulpal only. The extent of mouth opening plays a crucial role and requires multiple short appointments. It is recommended not exceed more than 15 s, after which an interval of 30 s can be given before resuming the procedure. Limit the overall appointment to time to <10 min for each appointment. Corticosteroids are recommended as first-line treatment at the start of flare-ups or for flare-up prophylaxis in conjunction with extensive dental procedures or operative intervention.[8],[12]

Attentive measures must be taken every time to minimize ossifications, including avoiding regional anesthesia, and surgical procedures must be implemented whenever possible.[11] Mandibular blocks in patients with FOP are illicit because they will escort to the ossification of the pterygoid muscles and hurried ankylosis of the TMJ. General anesthesia can be safely administered to patients with FOP for dental procedures with attention to perioperative and airway management via a multidisciplinary approach. An awake nasal fiberoptic intubation should be considered the first preference for airway management. Majority of patients can be discharged home on the same day as their dental procedure.[6],[12]

   Conclusion Top

This case report outlines few necessary guidelines for chairside treatment of children with FOP. The dental treatment modifications implemented with chair and mouth opening period, position considerations, and strict intrapulpal anesthesia are important to establish good oral health and to thwart the configuration of heterotopic ossifications in the maxillofacial region.

Why is this paper important?

  • Each child with FOP differs in stage of progression. It is extremely important to understand the present condition of the child with FOPA before planning the treatment under typical chairside/hospital setting.
  • This case report provides the first data about chairside management of FOP patients and recommends position consideration, use of intrapulpal anesthesia for chairside dental procedures, and mouth opening time periods and intervals.
  • It recommends mouth opening periods of 15 s with a break interval of 30 s before resuming the procedure to prevent muscle fatigue. Recommends limiting the total appointment time to <10 min for each appointment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for the patient's images and other clinical information to be reported in the journal. The patient's parents understand that the patient's name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors thank Dr. Frederick S.Kaplan for his continued guidance and support since the initial treatment planning and review this report. The authors thank the Center for Research in FOP and Related Disorders at the University of Pennsylvania for their support and Friends and families of FOP patients across the world.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Hüning I, Gillessen-Kaesbach G. Fibrodysplasia ossificans progressiva: Clinical course, genetic mutations and genotype-phenotype correlation. Mol Syndromol 2014;5:201-11.  Back to cited text no. 1
Liu JX, Hu R, Sun Y, Jiang H. General anesthesia in fibrodysplasia ossificans progressive: A case report and clinical review. Int J Clin Exp Med 2014;7:1474-9.  Back to cited text no. 2
Shore EM, Xu M, Feldman GJ, Fenstermacher DA, Cho TJ, Choi IH, et al. A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressiva. Nat Genet 2006;38:525-7.  Back to cited text no. 3
Kitterman JA, Kantanie S, Rocke DM, Kaplan FS. Iatrogenic harm caused by diagnostic errors in fibrodysplasia ossificans progressiva. Pediatrics 2005;116:e654-61.  Back to cited text no. 4
Oliveira FA, Fernandes CP, Araujo KS, Alves AP, Sousa FB, Mota MR. Clinical aspects and conservative dental management of a patient with fibrodysplasia ossificans progressiva. J Contemp Dent Pract 2014;15:122-6.  Back to cited text no. 5
Kilmartin E, Grunwald Z, Kaplan FS, Nussbaum BL. General anesthesia for dental procedures in patients with fibrodysplasia ossificans progressiva: A review of 42 cases in 30 patients. Anesth Analg 2014;118:298-301.  Back to cited text no. 6
Apfelbaum JL, Hagberg CA, Caplan RA, Blitt CD, Connis RT, Nickinovich DG, et al. Practice guidelines for management of the difficult airway: An updated report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway. Anesthesiology 2013;118:251-70.  Back to cited text no. 7
Roberts T, Stephen L, Scott C, Urban M, Sudi S, Beighton P. Fibrodysplasia ossificans progressiva (FOP) in South Africa: Dental implications in 5 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:11-8.  Back to cited text no. 8
Nussbaum BL, O'Hara I, Kaplan FS. Fibrodysplasia ossificans progressiva: Report of a case with guidelines for pediatric dental and anesthetic management. ASDC J Dent Child 1996;63:448-50.  Back to cited text no. 9
Kaplan FS, Le Merrer M, Glaser DL, Pignolo RJ, Goldsby RE, Kitterman JA, et al. Fibrodysplasia ossificans progressiva. Best Pract Res Clin Rheumatol 2008;22:191-205.  Back to cited text no. 10
Madhuri V, Santhanam M, Sugumar LK, Rajagopal K, Chilbule SK. Classical and atypical Fibrodysplasia Ossificans Progressiva in India. Ann Hum Genet 2015;79:245-52.  Back to cited text no. 11
Kaplan FS, Al Mukaddam M, Baujat G, Brown M, Cali A, Cho TJ, et al. The medical management of fibrodysplasia ossificans progressiva: Current treatment considerations. Proc Intl Clin Council FOP 2019;1:1-111.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

  [Table 1]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,177 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded271    
    Comments [Add]    

Recommend this journal

Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer | Privacy Notice
  2005 - Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow 
Online since 1st May '05