Guidebook
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Guidebook
Home
Table
of Contents
Preface
Introductory Comments
General
Questions About FOP
Genetics of FOP
How FOP Affects the Body
Care and Treatment
Activities
Feelings About FOP
Helpful Addresses
Family Resources
Ideas for Independence
Medical Articles
Acknowledgments and Contributions
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A
Dental Guide for People with FOP
Brief
Definition of FOP: Fibrodysplasia Ossificans Progressiva (FOP)
is a rare disease in which bone forms in abnormal locations, leading
to stiffness, locking, and permanent immobility. Extra bone growth
can occur without any warning or as a result of trauma, which can
be as minimal as a bump or a fall. Malformations of the great toe
are commonly noted at birth. Ectopic bone formation usually begins
in the first decade of life and progresses episodically in characteristic
anatomic patterns.
Special
Concerns for Dental Care:
The temporomandibular joints in the jaw are characteristically
among the last joints to be affected by FOP. However, involvement
of the TMJ area has been reported following dental procedures or
oral trauma at any age. This, combined with the age-increasing risk
that the jaw will become affected by FOP (71% of patients have jaw
restriction by age 18), points to the need to take certain precautions
to minimize the need for invasive procedures which could result
in jaw restriction, or the need for procedures which may be more
difficult to deal with as a result of existing jaw restriction.
Even in routine circumstances, stretching of the jaws and neck to
open or turn can have dire consequences in people with FOP.
People
with FOP can have preventative oral health care including prophys,
fluoride, and sealants. Restorative care must be accomplished but
using great caution. Similarly, dental extractions should be avoided
unless absolutely necessary. Orthodontic care can be accomplished
using short visits and caution; All bonded appliances are recommended
to avoid extra pressure on the jaws. The condition prevents use
of intramuscular injections of any type including block anesthesia.
Local anethesia can be applied as infiltrations, periodontal (PDL),
and intraligamentary. Electronic anesthesia is also recommended.
But remember: no intramuscular injections! If general anethesia
is needed, the person must be intubated using a fiberoptic laryngoscope
through the nose to avoid TMJ fusion.
To
help us evaluate dental care for people with FOP and to participate
in an on-going study, please send copies of your dental records
on a yearly basis to Dr. Nussbaum.
For
additional questions about dental care and people with FOP:
Burton
L. Nussbaum, D.D.S.
Dentistry for Special People, P.A
1910 Marlton Pike E Ste 9
Cherry Hill, NJ 08003-2123
Phone: (856) 667-2123
Fax: (856) 482-7825
Email: bikr2th@aol.com
www.dentistryforspecialpeople.com
Frederick
S. Kaplan, M.D.
University of Pennsylvania Medical Center
Department of Orthopaedics
Silverstein Pavilion-2nd Floor
34th & Spruce Streets
Philadelphia, PA 19104
Phone: (215) 349-8727
Fax: (215) 349-5928
Luchetti
W, Cohen RB, Hahn GV, Rocke DM, Helpin M, Zasloff MA, Kaplan, FS.
"Temporomandibular joint ankylosis following routine injection of
local anesthetic in patients who have fibrodysplasia ossificans
progressiva. Oral Surgery. 81: 1996. 21-25.
Nussbaum,
B, O'Hara I, Kaplan FS. "Fibrodysplasia Ossificans Progressiva,
Report of a Case with Guidelines for Pediatric Dental and Anesthetic
Management." J Dent Child, Nov-Dec 1996. 338-450.
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