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This disease has not been shown to be life-threatening or the cause of death in patients. However, treatment is necessary to maintain a healthy lifestyle.
Most recent methods of treatment take the form of surgeries such as oral prophylaxis, followed by post-surgical therapies to monitor, provide proper oral hygiene, and correct the deformity. Although, the nature of recurrence post-treatment is virtually unknown, let alone what type of treatment is most effective for HGF. (SOURCE 2) In some cases, there is re-growth after surgical removal of the excess gingival tissues, in others there is minimal. No cases yet have shown any particular treatment or form of medicine to permanently remove HGF.
One type of procedure that can be executed is as follows: Removal of excess tissue under anesthesia through an internal bevel gingivectomy or undisplaced flap followed by gingivoplasty and continuous sling suture placements and periodontal dressing; after about a week of recovery after the surgery, remove sutures and periodically do observational evaluations to look for any signs of re-occurrence.
No intervention is usually recommended unless they are causing difficulty to the infant or mother.
However some recommend that they be removed as the tooth can cut or amputate the tip of the tongue.
They should be left in the mouth as long as possible to decrease the likelihood of removing permanent tooth buds with the natal tooth. They should also not be removed if the infant has hypoprothrombinemia. In case of complications when the natal teeth need to be removed, dental radiographs should be obtained whenever possible, and evaluated and followed up with pediatric dentists.
In 2006, retinoids and antibiotics have been used with a successful dental maintenance for one year. In the past, only Extraction of all teeth and construction of a complete denture were made.
An alternative to rehabilitation with conventional dental prothesis after total loss of the natural teeth was proposed by Drs Ahmad Alzahaili and his teacher Jean-François Tulasne (developer of the partial bone graft technique used). This approach entails transplanting bone extracted from the cortical external surface of the parietal bone to the patient’s mouth, affording the patient the opportunity to lead a normal life.
Notwithstanding this treatment do not scope the disease itself. Actually it is the repositioning of bone from calvaria to the maxillary bones, and placement of dental implants in a completely edentulous maxilae, when the patient has already lost all teeth. An already developed method to reconstruct maxillae in edentulous elderly people by other dental professionals.
There's still no real treatment to help those who suffer from this disease to keep all their natural teeth, though their exfoliation and loss can be delayed.
The maintenance of teeth is done by dental professionals with a procedure called scaling and root planing with the use of systemic antibiotics. The syndrome should be diagnosed as earlier as possible, so the teeth can be kept longer in the mouth, helping the development of the maxillary bones.
Unerupted microdonts may require surgical removal to prevent the formation of cysts. Erupted microdonts, peg laterals especially, may cause cosmetic concern. Such teeth may be restored to resemble normal sized teeth, typically with composite build ups or crowns. Orthodontics may be required in severe cases to close gaps between the teeth.
Endodontic intervention can help conserve the existing health of affected permanent teeth. It is difficult to perform an endodontic therapy on teeth that develop abscesses as a resultant of obliteration of the pulp chambers and root canals. An alternative to conventional therapy would be retrograde filling and periapical curettage. However, these therapies are not recommended for teeth with roots that are too short.
The treatment aims are to eliminate the bacteria from the exposed surface of the root(s) and to establish the anatomy of the tooth, so that better plaque control can be achieved. Treatment plans for patients differ depending on the local and anatomical factors.
For Grade I furcation, scaling and polishing, root surface debridement or furcationplasty could be done if suitable.
For Grade II furcation, furcationplasty, open debridement, tunnel preparation, root resection, extraction, guided tissue regeneration (GTR) or enamel matrix derivative could be considered.
As for Grade III furcation, open debridement, tunnel preparation, root resection, GTR or tooth extraction could be performed if appropriate.
Tooth extraction is usually considered if there is extensive loss of attachment or if other treatments will not obtain good result (i.e. achieving a nice gingival contour to allow good plaque control).
Teeth with short thin roots and marked cervical constrictions are less favourable for indirect restorations such as crown placements. If endodontics treatment fails, and abscess develops around the root apex, extraction of the affected teeth would be the best treatment option. Dentures or over dentures can be considered, as rehabilitation until growth is completed. Cast partial dentures could also be an alternative treatment option and it only works if there are a few teeth that has enough root length to serve as retentive purpose.
Bisphosphonates have recently been introduced to treat several bone disorders, which include osteogenesis imperfecta.
A recognized risk of this drug relevant to dental treatments is bisphosphonate-associated osteonecrosis of the jaw (BRONJ). Occurrences of this risk is associated with dental surgical procedures such as extractions.
Dental professionals should therefore proceed with caution when carrying out any dental procedures in patients who have Type 2 DI who may be on bisphosphonate drug therapy.
Preventive and restorative care are important as well as esthetics as a consideration. This ensures preservation of the patient's vertical face height between their upper and lower teeth when they bite together. The basis of treatment is standard throughout the different types of DI where prevention, preservation of occlusal face height, maintenance of function, and aesthetic needs are priority. Preventive efforts can limit pathology occurring within the pulp, which may render future endodontic procedures less challenging, with better outcomes.
- Challenges are associated with root canal treatment of teeth affected by DI due to pulp chamber and root canal obliteration, or narrowing of such spaces.
- If root canal treatment is indicated, it should be done in a similar way like with any other tooth. Further consideration is given for restoring the root-treated tooth as it has weaker dentine which may not withstand the restoration.
Preservation of occlusal face height may be tackled by use of stainless steel crowns which are advocated for primary teeth where occlusal face height may be hugely compromised due to loss of tooth tissue as a result of attrition, erosion of enamel.
In most cases, full-coverage crowns or veneers (composite/porcelain) are needed for aesthetic appearance, as well as to prevent further attrition. Another treatment option is bonding, putting lighter enamel on the weakened enamel of the teeth and with lots of treatments of this bonding, the teeth appear whiter to the eye, but the teeth on the inside and under that cover are still the same. Due to the weakened condition of the teeth, many common cosmetic procedures such as braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they were intended to correct.
Dental whitening (bleaching) is contraindicated although it has been reported to lighten the color of DI teeth with some success; however, because the discoloration is caused primarily by the underlying yellow-brown dentin, this alone is unlikely to produce normal appearance in cases of significant discoloration.
If there is considerable attrition, overdentures may be prescribed to prevent further attrition of remaining teeth and for preserving the occlusal face height.
The oral rehabilitation of hypodontia, especially where a significant number of teeth have not developed, is often a multidisciplinary process, involving a specialist orthodontist, a consultant in restorative dentistry, and a paediatric dentist in the earlier years. The process of treating and managing hypodontia begins in the early years of the patient's dentition where absent teeth are identified and the process of maintaining the remaining teeth begins. This is largely conducted by the paediatric dentist with orthodontic input. Once all the adult teeth have erupted the orthodontist is likely to liaise with the restorative dentist regarding optimal positioning of teeth for subsequent replacement with prosthodontic methods. This may include the utilisation of a resin-retained bridge and implants for spaces or composite resin, veneers or crowns where teeth are diminutive or misshaped.
Prosthetic replacement of missing teeth is possible using dental implant technology or dentures. This treatment can be successful in giving patients with anodontia a more aesthetically pleasing appearance. The use of an implant prosthesis in the lower jaw could be recommended for younger patients as it is shown to significantly improve the craniofacial growth, social development and self-image. The study associated with this evidence worked with individuals who had ectodermal dysplasia of varying age groups of up to 11, 11 to 18 and more than 18 years. It was noted that the risk of implant failure was significantly higher in patients younger than 18 years, but there is significant reason to use this methodology of treatment in those older. Overall the use of an implant-prosthesis has a considerable functional, aesthetic and psychological advantage when compared to a conventional denture, in the patients.
If the condition is not affecting patient, no treatment is needed. Concrescence teeth could be reshaped and replaced with full crowns. However, if the teeth are having recurrent problems, non-restorable or pain, extraction should be considered.
The major treatment is surgery for most babies. The type of surgery which they would undergo differs from age and strength they have. The main reason of doing the surgery is to alleviate pressure on the brain, and create a space for brain developing and growing. It would improve infant’s appearance.
The first one is Traditional surgery. During surgery, they make an incision in the baby's scalp and cranial bones, and reshape the portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.
After surgery, your baby remains in the hospital for at least three days. Some children may require a second surgery later because, the craniosynostosis recurs. Also, children with facial deformities often require future surgeries to reshape their faces.
Another one is Endoscopic surgery. This less invasive form of surgery isn't an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby's brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss, and shortens the hospital stay, often to one day after surgery.
The frequency of first permanent molar treatment for children with MIH is nearly 10 times greater compared to children without MIH. The available treatment modalities for MIH is extensive but the decision on which treatment should be used is complex and multi-factorial. Factors may include: condition severity, the patient’s dental age, the child/parent’s social background and expectations. There are treatment modalities available to manage children affected by MIH; however, the evidence supporting these modalities are still weak.
There is currently no specified treatment for individuals suffering from otodental syndrome. Considering that there are many possible genetic and phenotypic associations with the condition, treatment is provided based on each individual circumstance. It is recommended that those affected seek ear, nose & throat specialists, dental health specialists, and facial oral health specialists immediately; in order to determine potential treatment options.
Common treatment methods given are:
- Dental treatment/management – which can be complex, interdisciplinary and requires a regular follow up. Tooth extraction(s)and if needed, medications may be administered for pain, anxiety, and anti-inflammation. The affected individual is usually placed on a strict and preventative dental regiment in order to maintain appropriate oral hygiene and health.
- Endodontic treatment – individuals consult with an endodontist to analyze the individuals dental pulp. Typically endodontic treatment proves to be difficult due to duplicated pulp canals within the affected teeth. There may be a need for multiple extractions as well. Dental prosthesis and/or dental implants may be necessary for individuals that lack proper oral function, appearance, and comfort.
- Orthodontic treatment – given the predicament of the size and location of the affected oral area, molars and canines, orthodontic treatment is generally required in order treat any problems associated with the individuals bite pattern and tooth appearance.
- Hearing aids – in some cases affected individuals will suffer from hearing imparities and it may be necessary for hearing aid use.
The functional prognosis is mostly good with those that suffer from otodental syndrome. Appropriate dental treatment, hearing aids, and visitation to necessary specialists are recommended. Quality of life may be affected by psychological and functional aspects. It is also recommended that genetic counseling be given to families that have or may have this condition.
Involves repeated cycles of etching with 37% phosphoric acid followed by applying 5% sodium hypochlorite until improvement of discolouration is achieved. Clear resin composite or resin infiltrate can be used to seal the lesion after the technique.
Each child is different and it entirely depends on which sutures are fused and how it is affecting the child as to how it is treated. Some children have severe breathing issues due to shallow mid face and may require a tracheostomy. All should be treated at a specialist centre. Cranio bands are not used in the UK.
Surgery is typically used to prevent the closure of sutures of the skull from damaging the brain's development. Without surgery, blindness and mental retardation are typical outcomes. Craniofacial surgery is a discipline of both plastic surgery and oral and maxillofacial surgery (OMFS) . To move the orbits forward, craniofacial surgeons expose the skull and orbits and reshape the bone. To treat the midface deficiency, craniofacial surgeons can move the lower orbit and midface bones forward. For jaw surgery, either plastic surgeons or OMFS surgeons can perform these operations.
Crouzon patients tend to have multiple sutures involved, most specifically bilateral coronal craniosynostoses, and either open vault surgery or strip craniectomy (if child is under 6 months) can be performed. In the later scenario, a helmet is worn for several months following surgery.
Once treated for the cranial vault symptoms, Crouzon patients generally go on to live a normal lifespan.
There is no standard treatment for the hand malformations in Apert due to the differences and severity in clinical manifestations in different patients. Every patient should therefore be individually approached and treated, aiming at an adequate balance between hand functionality and aesthetics.
However, some guidelines can be given depending on the severity of the deformities.
In general it is initially recommended to release the first and fourth interdigital spaces, thus releasing the border rays.
This makes it possible for the child to grasp things by hand, a very important function for the child's development. Later the second and third interdigital spaces have to be released.
Because there are three handtypes in Apert, all with their own deformities, they all need a different approach regarding their treatment:
- Type I hand usually needs only the interdigital web space release. First web release is rarely needed but often its deepening is necessary. Thumb clynodactyly correction will be needed.
- In type II hands it is recommended to release the first and fifth rays in the beginning, then the second and the third interdigital web spaces have to be freed. The clynodactyly of the thumb has to be corrected as well. The lengthening of the thumb phalanx may be needed, thus increasing the first web space. In both type I and type II, the recurrent syndactyly of the second web space will occur because of a pseudoepiphysis at the base of the index metacarpal. This should be corrected by later revisions.
- Type III hands are the most challenging to treat because of their complexity. First of all, it is advised to release the first and fourth webspace, thus converting it to type I hand. The treatment of macerations and nail-bed infections should also be done in the beginning. For increasing of the first web space, lengthening of the thumb can be done. It is suggested that in severe cases an amputation of the index finger should be considered. However, before making this decision, it is important to weigh the potential improvement to be achieved against the possible psychological problems of the child later due to the aesthetics of the hand. Later, the second and/or third interdigital web space should be released.
With growing of a child and respectively the hands, secondary revisions are needed to treat the contractures and to improve the aesthetics.
All impacted teeth, unless otherwise contraindicated, are considered for surgical removal. Thus, dental extractions will often take place. The type of extraction (simple or surgical) often depends on the location of the teeth.
In some cases, for aesthetic purposes, a surgeon may wish to expose the canine. This may be achieved through open or closed exposure. Studies show no advantage of one method over another.
Although these teeth are usually asymptomatic and pose no threat to the individual, they are often extracted for aesthetic reasons. This is done particularly if the mesiodens is positioned in the maxillary central incisor region. The traditional method of removal is done by using bone chisels, although a more advanced technique has been found to be more beneficial, especially if surgery is required . Through the use of Piezoelectricity, Piezoelectric ultrasonic bone surgery may be more time consuming than the traditional method but it seems to reduce the post-operative bleeding and associated complications quite significantly.
Management of teeth with PFE can include extractions of affected teeth, followed by orthodontic space closure or placement of a prosthetic implant with a bone graft. This option can only be applied to a single tooth that is affected. If multiple teeth are affected then, a segmental osteotomy may be performed to bring the entire segment into occlusion. However, minimal success has been shown following this procedure. These teeth usually are "non-responsive" to the orthodontic force and studies have shown that ankylosis of these teeth can occur if force applied.
Treatment and prognosis are usually based upon keeping these teeth and preserving the alveolus. For erupted teeth, endodontics is an option if the tooth is devitalized and restorable. For unerupted teeth, function can be restored with a removable partial denture until all major growth has been completed and a final restoration can be placed.
Before root canal treatment or extraction are carried out, the clinician should have thorough knowledge about the root canal morphology to avoid complications.
Teeth affected by macrodontia are either contoured, aligned or extracted. Contouring involves shaving the tooth down to change shape and size. However, the result is minimal change as this could be dangerous for the dentin and dental pulp. Aligning involves the use of braces to straighten, align, and make space for larger teeth to grow. When extracted, they are replaced with an implant or bridge. This is done in cases in which the patient suffers from pain that cannot be treated by other methods.