Department of Pediatric Dentistry
Lutheran Medical Center
Resident’s Name: Craig Elice Date: 6/11/2010
Article title: Dental findings associated with the malformations of CHARGE
Author(s): Sheneifi TA, Cottrell DA, Hughes C..
Journal: Ped Dent: 24:1
Month, Year: 2002 43-6
Major topic: CHARGE
Type of Article: Case Report
What is affected in this syndrome? Major criteria for diagnosis include Colomba of the eye, Atresia of Choanae, multiple cranial nerve deficits including deafness, vision impairment, poor swallowing; and Ear abnormalities involving the inner, middle, and outer ear. The choanal atresia causes breathing difficulties and cyanosis within the first hour of life and requires surgical intervention early in life. Minor criteria include Heart defects like tetralogy of Fallot, septal defects or valvular stenosis, genital hypoplasia, orofacial clefting, trachoesophageal fistulas requiring a feeding tube, short stature and developmental delays.
Etiology: Unknown. Affects 1 in 10,000 live births
When is the diagnosis made? Diagnosis is usually made in infancy based on a clustering of congenital malformations. In cases of bilateral severe choanal atresia the patients turn blue in early infancy when resting.
What are the systemic or medical conditions associated with the syndrome that are critical concerns for dental work? Several conditions affect the methodology used to treat patients with CHARGE. Children usually have developmental delays affecting management of the patient. The patient in the case report exhibited behavioral difficulties requiring general anesthesia to manage the patient. Choanal atresia may affect airway management, Cardiac septal defects require antibiotic coverage because of the risk of endocarditis.
What factors influence dental care or cause the need for dental treatment? Because of behavior issues, the patients frequently have poor oral hygiene, and associated gingivitis. Rampant decay is a frequent finding. Delayed eruption of the permanent teeth and mandibular retrognatia have also been reported. In the case report, impacted teeth and congenitally missing teeth were noted as well as an odontogenic fibroma of the mandible.
New information? Genetic tests are available which have a limited degree of success in diagnosis because some children have a negative test, yet have CHARGE. Early intervention is a key element to success in encouraging development of the child. In many cases, intelligence is normal, but with vision and hearing loss this can be masked.
Showing posts with label 06/11/2010. Show all posts
Showing posts with label 06/11/2010. Show all posts
Thursday, June 10, 2010
06/11/2010 A Preventative Approach to Oral Self-mutilation in Lesch-Nyhan Syndrome: A Case Report
Resident: Hencler
Date: 06/11/2010
Article title: A Preventative Approach to Oral Self-mutilation in Lesch-Nyhan Syndrome- A Case Report
Author(s): Jeong et al.
Journal: Pediatric Dentistry-28:4 2006
Major topic: Oral Self-mutilation in Lesch-Nyhan Syndrome
Type of Article: Case Report
Main Purpose:
To present and discuss a Lesch-Nyhan syndrome patient who was treated successfully w/ noninvasive approaches, including a soft mouthguard and psychiatric pharmacologic therapy to prevent further damage of perioral soft tissues.
Background:
Lesch-Nyhan syndrome is a disorder of purine metabolism causing accumulation of sodium urate crystal in the joints, kidneys, CNS, and other tissues. Clinical symptoms include impaired kidney function, joint pain, and a progressive neurological disorder, resulting in retardation of mental and motor development. The most distressing aspect of the syndrome is the compulsive urge for self mutilation.
Case Description:
4 yo male w/ Lesch-Nyhan Syndrome and cerebral palsy receiving 2 mg diazepam daily to relieve biting action with limited success. EOE: Self-mutilated wounds noted on the lower lip and finger caused by primary central incisors. IOE: Revealed caries free intact primary dentition. A maxillary impression taken under sedation and a soft mouthguard was fabricated. Simultaneously, a psychiatric analysis was conducted. Child was biting lower lip since 12 months old whenever frustrated to control anxiety and express aggression. Sertraline and risperidone was prescribed to control the anxiety and self-mutilating behavior. After doubling dosages self-injurious behavior was markedly reduced at day 15. Continued pharmacological therapy at 1 month resulted in 1 attempts to self-mutilate per wk and at 4 months self-mutilating behavior ceased. At recall visits the lower lip was healed, mom was highly satisfied, and the patient appeared to be comfortable and less agitated.
Discussion:
Self-mutilation can result in destruction of the lower lip. EXT of primary and permanent incisors are frequently advocated as a solution, however, canines and perhaps premolars may also be involved. Oral devices to prevent self-mutilation include soft mouthguard, bite block, various types of shields that guard the tongue and lips, and lip bumpers. These methods have an advantage of preserving the teeth, but patient compliance is poor. Also, devices covering the skin may result in dermatitis. In this case, after 6 months the soft mouthguard was ill fitting due to wear and needed replacing. It is important to maintain POEs to reassess integrity of any oral appliance.
Summary of conclusions:
There are no standard methods for the prevention of self-mutilation. Appropriate preventative methods need to be developed on an individual patient basis. As an alternative treatment to EXT, a therapy combining psychiatric, pharmacologic, and dental soft mouth guard treatment may be a ideal option in treating transient and acute episodes of self-injurious behavior involving the lower lip.
Assessment of article:
Interesting case. If the medication can reduce oral self-mutilation, then combination with a soft mouthguard would be a great alternative to EXTs, just as long as the compliance is there. It would be important to preserve the models for appliance fabrication for as long as possible because to get an accurate impression on this patient population would indicate impressing under sedation of some kind. Captain obvious signing off.
Date: 06/11/2010
Article title: A Preventative Approach to Oral Self-mutilation in Lesch-Nyhan Syndrome- A Case Report
Author(s): Jeong et al.
Journal: Pediatric Dentistry-28:4 2006
Major topic: Oral Self-mutilation in Lesch-Nyhan Syndrome
Type of Article: Case Report
Main Purpose:
To present and discuss a Lesch-Nyhan syndrome patient who was treated successfully w/ noninvasive approaches, including a soft mouthguard and psychiatric pharmacologic therapy to prevent further damage of perioral soft tissues.
Background:
Lesch-Nyhan syndrome is a disorder of purine metabolism causing accumulation of sodium urate crystal in the joints, kidneys, CNS, and other tissues. Clinical symptoms include impaired kidney function, joint pain, and a progressive neurological disorder, resulting in retardation of mental and motor development. The most distressing aspect of the syndrome is the compulsive urge for self mutilation.
Case Description:
4 yo male w/ Lesch-Nyhan Syndrome and cerebral palsy receiving 2 mg diazepam daily to relieve biting action with limited success. EOE: Self-mutilated wounds noted on the lower lip and finger caused by primary central incisors. IOE: Revealed caries free intact primary dentition. A maxillary impression taken under sedation and a soft mouthguard was fabricated. Simultaneously, a psychiatric analysis was conducted. Child was biting lower lip since 12 months old whenever frustrated to control anxiety and express aggression. Sertraline and risperidone was prescribed to control the anxiety and self-mutilating behavior. After doubling dosages self-injurious behavior was markedly reduced at day 15. Continued pharmacological therapy at 1 month resulted in 1 attempts to self-mutilate per wk and at 4 months self-mutilating behavior ceased. At recall visits the lower lip was healed, mom was highly satisfied, and the patient appeared to be comfortable and less agitated.
Discussion:
Self-mutilation can result in destruction of the lower lip. EXT of primary and permanent incisors are frequently advocated as a solution, however, canines and perhaps premolars may also be involved. Oral devices to prevent self-mutilation include soft mouthguard, bite block, various types of shields that guard the tongue and lips, and lip bumpers. These methods have an advantage of preserving the teeth, but patient compliance is poor. Also, devices covering the skin may result in dermatitis. In this case, after 6 months the soft mouthguard was ill fitting due to wear and needed replacing. It is important to maintain POEs to reassess integrity of any oral appliance.
Summary of conclusions:
There are no standard methods for the prevention of self-mutilation. Appropriate preventative methods need to be developed on an individual patient basis. As an alternative treatment to EXT, a therapy combining psychiatric, pharmacologic, and dental soft mouth guard treatment may be a ideal option in treating transient and acute episodes of self-injurious behavior involving the lower lip.
Assessment of article:
Interesting case. If the medication can reduce oral self-mutilation, then combination with a soft mouthguard would be a great alternative to EXTs, just as long as the compliance is there. It would be important to preserve the models for appliance fabrication for as long as possible because to get an accurate impression on this patient population would indicate impressing under sedation of some kind. Captain obvious signing off.
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