Resident: J. Hencler
Date: 05/07/2010

Article title: Oral health considerations in muscular dystrophies (MD)
Author(s): Balasubramaniam, Sollecito, Stoopler
Journal: Spec Care Dentist 28(6) 2008

Major topic: MD and oral/dental considerations
Type of Article: Review

Main Purpose: Present oral health considerations in MD patients.

Background:
MD’s are a heterogenous grp of inherited neuromuscular disorders characterized by muscle necrosis and progressive muscle weakness. Severity of the disease range from mild to severe, and is caused by mutations in genes that encode for proteins that are critical for maintaining muscle fiber integrity for functional muscle contraction and relaxation. There is no specific tx for any of the MDs and it is considered an incurable disease. In pts suspected of having MD, percussion of the tongue w/ a tongue depressor and the occurrence of lingual myotonia may confirm the presence of MD in some cases.

Orofacial Manifestations:
The muscles of the head and neck are affected by MD and manifest as altered craniofacial morphology and dental malocclusion. Weakness of perioral muscles may produce deformities of the face and difficulty in chewing and phonation. MD patients usually have long, thin faces, high-arched palates mandibular prognathism, and dental malocclusion. The most consistent clinical finding is malocclusion related to vertical aberrations in craniofacial growth due to reduced function of the masticatory muscles and the less affected suprahyoid muscles. The resultant lowering of the mandible and tongue does not counterbalance the forces from the stretched facial musculature. This change in force balance affects the teeth transversely, decreases the width of the palate, and causes posterior crossbite. The combo of lowering of the mandible and relative decrease in bite force permits the overeruption of posterior teeth and development of anterior open bite and deep palatal vault. Delayed eruption of the permanent dentition seen in MD patients may be related to the calcification stages of dental devel. There have been few reports on TMJ abnormalities in patients with MD. OH declines with loss of muscle function of the arms and hands.

Dental Management Considerations:
Consultation w/ patient’s physician to determine stability of MD and presence of complications such as cardiomyopathy, arrhythmias, pulmonary hypoventilation, and neuropsychiatric traits. Patients with stable MD may receive tx in outpt setting, however persons w/ severe muscle contracture and/or med complications may require tx under GA. GA may cause complications b/c the risk of malignant hypertension (MH) associated w/ certain neuromuscular blocking agents. In general, neuromuscular blocking agents should be avoided. Induction and maintenance with N20, inhalation agents, barbiturates, and benzodiazepines may be used usually w/out complication. Proper pt positioning may be difficult due to kyphoscoliosis or flexion contractures. Post op complications are typically pulmonary related. Pt should be inclined at 45 degress post op to prevent dyspnea and obstruction. In the outpt setting, MD pts ofter require assistance w/ ambulation. MD patients should not be put in supine position and b/c of facial and/or perioral muscle wealness require vigilant suctioning of fluids to prevent aspiration. OH is usually poor due to limb weakness. Potential for drug interaction and oral manifestations w/ commonly used meds for MD pts such as prednisone must be considered.

Recommendations:
Routine dental visits and good OH habits established early. Education for pt/caregiver regarding proper diet, OH, fluoride, sealants, and recall visits. Children w/ MD w/ low caries risk can begin tooth brushing at the age of 1 w/out toothpaste, and at age of 3, toothpaste, topical F prophylaxis, and F varnishes should be introduces. Children w/ MD that are mod-high risk for caries should receive F varnish at 6 month and 3-6month intervals, respectively. The indication for ortho tx to improve masticatory funct is difficult to determine as progressive devel of the dentofacial abnormalities in MD renders prognosis unpredictable. MD patients require special considerations when providing their OH care. Oral healthcare providers need to familiarize themselves with the orofacial characteristics and dental tx considerations for pts w/ MD so that they can deliver safe and appropriate tx.

Assessment of article:
Good article. Good to be aware of the OH characteristic of patients w/ MD and the tx challenges one would face while tx a MD child.