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Tuesday, November 20, 2012

Ectodermal dysplasia

INTRODUCTION :

Ectodermal dysplasia syndrome is a large,heterogeneous group of inherited disorders , the manifestations of which could be seen in more than one ectodermal derivatives.


Common syndromes :
A. Hypohidrotic ED
B. Hidrotic ED

Etiology : 

 Ectodermal dysplasia syndrome results from aberant development of ectodermal derivatives in early embryonic life.


Symptoms

Teeth

In the development of tooth buds frequently result in congenitally absent teeth (in many cases a lack of a permanent set) and/or in the growth of teeth that are peg-shaped or pointed. The enamel may also be defective. Cosmetic dental treatment is almost always necessary and children may need dentures as early as two years of age. Multiple denture replacements are often needed as the child grows, and dental implants may be an option in adolescence, once the jaw is fully grown. Nowadays this option of extracting the teeth and substituting them with dental implants is quite common. In other cases, teeth can be crowned. Orthodontic treatment also may be necessary. Because dental treatment is complex, a multi-disciplinary approach is best.


People with ectodermal dysplasia may not sweat or may have decreased sweating because of a lack of sweat glands.

Children with the disease may have difficulty controlling fevers. Mild illness can produce extremely high fevers, because the skin cannot sweat and control temperature properly.
Affected adults are unable to tolerate a warm environment and need special measures to keep a normal body temperature.
Other symptoms include:
  • Abnormal nails
  • Abnormal or missing teeth
  • Absent or decreased tears
  • Decreased skin color (pigment)
  • Heat intolerance
  • Inability to sweat
  • Large forehead
  • Lower than normal number of teeth
  • Low nasal bridge
  • Poor hearing
  • Poor temperature regulation
  • Poor vision
  • Thin, sparse hair
  • Thin skin

    Treatment

    There is no specific treatment for this disorder.
    Some things you can do include:
    • Wear a wig and dentures to improve appearance.
    • Use artificial tears to replace normal tearing and prevent drying of the eyes.
    • Spray the nostrils with saline nose spray often to remove debris and prevent infection.
    • Live in a cooler climate and take cooling water baths or use water sprays to keep a normal body temperature (water evaporating from the skin replaces the cooling function of sweat evaporating from the skin).

      Reference :
      Wikipedia , Shafers text book of oral pathology


     


Thursday, November 8, 2012

Chronic Hyperplastic Pulpitis ( Pulp polyp )

A pulp polyp, also called as Chronic Hyperplastic Pulpitis, is found in an open carious lesion, fractured tooth or when a dental restoration is missing. Due to lack of intrapulpal pressure in an open lesion pulp necrosis does not take place as would have occurred in a closed caries case. Also a good vascular and immune supply is necessary, therefore found in adults. It is a productive pulpal inflammation where the development of granulation tissue is seen.



Causes

Causes of a pulp polyp include the following:
  • Carious tooth with significant loss of tooth structure
  • Loss of a dental restoration that results in pulpal exposure
  • Fractured tooth due to trauma with a pulpal exposure
  • Pulpal tissue with access to a good blood supply
  • Possible hormonal (estrogen and progesterone) influence


Clinical features :

1. Occurs mostly in children and young adults , who possess a high degree of resistance and reactivity.
2.It involves teeth with large,open carious lesions.
3.Pulp appears as a pinkish red globule of tissue protruding from the pulp chamber and not only fills the caries defect but also extends beyond.
4.The teeth most commonly involved by this phenomenon are the deciduous molars and the first permanent molars.


Note : On occasion the gingival tussue adjacent to a broken carious tooth may proliferate into the carious lesion and superficially resemble an example of hyperplastic pulpitis.In such cases , the distinction can be made to determine whether the connection is with pulp or gingiva.  

 Histological features :

1.It is basically a granulation tissue made of delicate connective tissue fibers interspersed with variable numbers of small capillaries.
2. It becomes epithelialized and the origin of these epithelial cells is a matter of controversy.
3.The epithelium is stratified squamous in type and closely resemles the oral muscosa,even to the extent of developing well formed rete ridges.


Epidemiology

Frequency

United States

Pulp polyps are reportedly uncommon in the United States, and no epidemiologic studies specifically document the frequency of this entity. Although this lesion is reported to be uncommon with only isolated references in the literature, the true prevalence of this reactive pulpal disease is likely to be underestimated because it is a well-recognized sequela of extensive dental caries in children.

International

Pulp polyps are uncommon in countries with routine access to dental care, but they are encountered more frequently in developing countries. In a study of Vietnamese refugees who sought dental care, the prevalence of pulp polyps was 6%. This high number of cases is an indication of the severity of dental disease in this impoverished population.

Mortality/Morbidity

Pulp polyps tend to be asymptomatic and are not associated with any significant morbidity or mortality except for gross caries destruction with premature tooth loss in many cases.

Race

No racial predilection is recognized for this sequela of dental caries; however, it is more common in individuals of lower socioeconomic background who have limited access to dental care than in other people.

Sex

No sexual predilection has been documented for this oral lesion.

Age

This pulpal disease occurs almost exclusively in children and young adults, and it can occur in both the primary dentition and the permanent dentition.


Treatment :

Extraction of the tooth or by Pulp extirpation.



Sunday, November 4, 2012

Macroglosia

Macroglossia, meaning large tongue, has been a documented anatomical anomaly for several centuries but remains an entity defined more by presentation than by strict cephalometric analysis. The earliest known written description of tongue lesions comes from the Egyptian Papyrus Ebers, originally thought to be from around 1550 BC.
Obviously, tongue lesions have since been categorized by their etiologies. Macroglossia has an extensive list of possible causes. Its treatment has been largely surgical in the modern era.



Problem

Multiple studies have attempted to define macroglossia by objective measurements based on a variety of clinical and radiographic tests. However, because of the difficulty in performing these tests and because intervention is not based on measurements but on clinical presentation, macroglossia is most often diagnosed subjectively.
Ueyama and others defined macroglossia as occurring when  of the following  criteria is met:extravasation of the lingual apex or lingual border onto or outside the dentition;  the impression of one or more teeth on the lingual border visualized when the mouth is open; or  following surgery for correction, a relapse of increased interdental space, open bite deformity, and/or jaw deformation with malocclusion occurs.
As with many lesions, medicine has identified a triad for those with macroglossia. It includes open bite deformity, mandibular prognathism, and malalignment.



Epidemiology

Frequency

Although the exact incidence of macroglossia is unknown (because the etiologies are too numerous to quantify), some congenital syndromes often express macroglossia in their phenotypes, most commonly Down syndrome (1 per 700 live births) and Beckwith-Wiedemann syndrome (0.07 pre 1000 live births). In Beckwith-Wiedemann syndrome, 97.5% of patients have macroglossia.The literature documents 2 families with autosomal dominant inheritance of isolated macrogloss


Pathophysiology

Because the pathophysiology of the enlarged tongue is related to the specific etiology, defining the pathophysiology of each is beyond the scope of this discussion. However, in all cases, the locoregional complications of macroglossia are generally the same relative to the magnitude of the enlargement. Mechanical obstruction in the oral cavity can directly occlude the airway. This occlusion is usually worsened by lying supine when an enlarged tongue base is more directly acted upon by gravity to block the oropharynx and hypopharynx. Depending on muscle mass and tone, speech and swallowing may be affected as well.
Several studies document the role of the tongue in shaping the oral cavity. Just as reduced pressure of the tongue on the palate and mandible may lead to an adenoid facies, increased pressure on the surrounding anatomy can have opposite effects. Upper incisors can be pushed horizontally, inducing forward maxillary growth. Other morphologic changes include open bite deformities, prognathism, class III malocclusion, anterior and/or posterior crossbites, buccal tipping of posterior teeth, accentuated curve of Spee in the maxillary arch, reverse curve of Spee in the mandibular arch, increased transverse width of mandibular and/or maxillary arches. Furthermore, difficulty with mastication may lead to temporomandibular joint pain.
If the tongue protrudes beyond the lips and is exposed to the air, drying of the tongue with resultant glossitis and bleeding is common. Prior to the 1900s, this was not an uncommon occurrence for patients.


Indications

Indications for surgical intervention are varied. The most important is airway compromise. A tracheostomy may be required as a first step in surgical care (in some cases an elective tracheostomy is performed prior to surgical correction). Other indications include dysarthria, dysphagia, and cosmesis.
The goal of nearly all surgery is to return the patient to an anatomically and physiologically normal condition; the same is also true in surgery for macroglossia. The goal is to reduce tongue size and thereby improve function. Those main functions include articulation, mastication, deglutition, protection of the airway, and gustation. Of these, only gustation is not often improved with surgical intervention.


Contraindications

As with all intervention, whether medical or surgical, the benefits of the operation must outweigh the risks. Relative contraindications are those associated with most surgeries and include coagulopathies and other comorbidities that make general anesthesia more dangerous. In the pediatric population, many cases of macroglossia are associated with syndromes that may have lesions that increase the risk of general anesthesia.


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