Dentin dysplasia

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Dentin dysplasia
Pantomog dntindysplasia.jpg
Preoperative panoramic radiographs showing features of dentin dysplasia type I
Specialty Dentistry

Dentin dysplasia (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting an autosomal dominant inheritance that causes malformation of the root. It affects both primary and permanent dentitions in approximately 1 in every 100,000 patients. [1] It is characterized by presence of normal enamel but atypical dentin with abnormal pulpal morphology. Witkop [1] in 1972 classified DD into two types which are Type I (DD-1) is the radicular type, and type II (DD-2) is the coronal type. DD-1 has been further divided into 4 different subtypes (DD-1a,1b,1c,1d) based on the radiographic features. [2]

Contents

Signs and symptoms

Clinically the teeth look normal in colour and morphologic appearance; however, they are commonly very mobile and exfoliated prematurely. [3]

Both primary and permanent dentitions can be affected by either type I or type II dentin dysplasia. However, deciduous teeth affected by type II dentin dysplasia have a characteristic blue-amber discolouration, whilst the other dentition appears normal. [3]

Causes

The mutation in collagen type 1 (COL1 A1, COL1 A2) causes DI-1. It is similar to the systemic condition dental features known as osteogenesis imperfect. [4] [5] [6] DI-2, DI-3 and DD-2 share the same genetic mutation of dentin sialophosphoprotein, that is located on chromosome 4. They are autosomal-dominant diseases with complete penetrance and variable expressivity. [1] [7] Due to the same genetic mutation, these diseases would often result in overlapping clinical and radiographic features. [8] [9] Therefore, prevailing theories suggests that DI-2, DI-3 and DD-2 are categorized as a single disease entity with variable severity of expression. [10] However, the causes of DD-1 have yet to be theorized.

Diagnosis

Diagnosis is mostly based on general examination and radiographs, and it should be taken when abnormality of the teeth is suspected as most of the affected teeth have normal clinical appearance. [1]

Differential diagnosis is very important to have a definitive diagnosis as some radiographic or histologic features of dentine dysplasia may bear a resemblance to different disorders: [11]

Type I: Radicular type

Type I has been known as radicular dentine dysplasia because the teeth have undeveloped root(s) with abnormal pulp tissue. Morphology and colour of the crown mostly appear normal, but occasionally teeth appear slightly amber coloured or bluish-brown shine in primary teeth with no or only immature root development. The teeth are mostly maligned and have higher risk of fracture. [12]

Radiographic feature

In other words, affected primary teeth usually have abnormal shaped or shorter than normal roots. “Crescent/half-moon shaped” pulp chamber remnant in permanent teeth can be seen on x-rays. The roots may appear to be darker or radiolucent/pointy and short with apical constriction. Dentine is laid down abnormally and causes excessive growth within the pulp chamber. This will reduce the pulp space and eventually cause incomplete and total pulp chamber obliteration in permanent teeth. [12] [13] Sometimes periapical pathology or cysts can be seen around the root apex. [11] Most cases of DD associated with peri-apical radiolucency/ pathology have been diagnosed as radicular cysts, but some of them have been as diagnosed peri-apical grauloma instead. [14]

Type II: Coronal type

Type II would mostly cause discolouration to the primary teeth. Affected teeth usually appear as brownish-blue, brown or yellow. Translucent “opalescence” is often one of the characteristics to describe teeth with DD-2. In some cases teeth might show slightly amber coloured but in most of the cases permanent teeth are unaffected and appear normal regardless of colour, shape and size. Dental X-rays are the key to diagnose dentine dysplasia, especially on permanent teeth. Abnormalities of the pulp chamber is the main characteristic to make a definitive diagnosis. [15] [16]

In the primary teeth, coronal dentin dysplasia may appear similar to Dentinogenesis Imperfecta type II (DG-II) but if abnormalities features appear to be more pronounced in the permanent teeth, then consider changing the diagnosis to DGI-II instead of DD-2. [5]

Radiographic features

In coronal type, teeth show normal roots containing enlarged pulp with abnormal extensions towards the roots, which is often described as “thistle tube” shaped on dental radiographs. As well as this, numerous pulp stones can be often found in the pulp chambers due to abnormal calcifications. [15] In primary teeth, the pulp chamber is usually completely obliterated but in permanent teeth, the pulp may become partially obliterated after eruption. [11]

Histological features

There are a few studies and proposals that were designed to explain the pathogenesis of DD but the main reason of the cause still remains unclear in the dental literature. Logan et al., [17] suggested that dental papilla is the cause of abnormal root growth or development. They also proposed that calcification of multiple degenerative foci within the papillae reduce the growth and eventually leads to pulpal obliteration. Wesley et al., [18] suggested that odontoblasts with abnormal function and/ or differentiation are mainly due to atypical interaction between odontoblasts and ameloblasts. Histopathologically, deeper layer of the teeth shows abnormal dentine tubular pattern with unstructured, unorganised, atubular areas with normal enamel appearance. Globular or small mass of rounded or irregular shape of atypical dentine is often seen in the pulp. [19]

Treatment

With various options available to dentists, the treatment of this condition can still be difficult. Endodontic treatment is not advised for teeth with complete obliteration of root canals and pulp chambers. [3] An alternative treatment for teeth with periapical abscesses and pulpal necrosis is dental extraction. Retrograde fillings and periapical surgery is a treatment option for teeth with longer roots, as well as orthodontic treatment. However, orthodontic treatment can lead to even more resorption of the roots, which could lead to further tooth mobility and premature exfoliation. [3] Another proposed treatment, for successful oral rehabilitation, is to extract all teeth, curette any cysts and provide the patient with a complete denture. A combination of bone grafting and a sinus lift technique can also be successful to accomplish implant placement. [3]

Management

The best method of maintaining the health of teeth is to practice exemplary oral hygiene. More tooth loss is likely to occur if intervention takes place. However, factors such as present complaint, patient age, severity of the problem, can affect the treatment plan or options. [11]

Stainless steel crowns

Stainless steel crowns which also known as "hall crowns" can prevent tooth wear and maintain occlusal dimension in affected primary teeth. However, if demanded, composite facings or composite strip crowns can be added for aesthetic reasons. [11]

Endodontics Treatment

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. [14]

Removable dentures

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. [11]

Dental implants

Dental implant is one of the treatment options that can be considered when growth is fully attained. For patients who experience maxillo-mandibular alveolar atrophy due to early loss of teeth, alveolar ridge augmentation procedure is recommended prior to the implant placement. Both onlay bone grafting and sinus lift techniques can be carried out together to accomplish implant placement. [20]

Related Research Articles

<span class="mw-page-title-main">Human tooth</span> Calcified whitish structure in humans mouths used to break down food

The human teeth function to mechanically break down items of food by cutting and crushing them in preparation for swallowing and digesting. As such, they are considered part of the human digestive system. Humans have four types of teeth: incisors, canines, premolars, and molars, which each have a specific function. The incisors cut the food, the canines tear the food and the molars and premolars crush the food. The roots of teeth are embedded in the maxilla or the mandible and are covered by gums. Teeth are made of multiple tissues of varying density and hardness.

<span class="mw-page-title-main">Dentin</span> Calcified tissue of the body; one of the four major components of teeth

Dentin or dentine is a calcified tissue of the body and, along with enamel, cementum, and pulp, is one of the four major components of teeth. It is usually covered by enamel on the crown and cementum on the root and surrounds the entire pulp. By volume, 45% of dentin consists of the mineral hydroxyapatite, 33% is organic material, and 22% is water. Yellow in appearance, it greatly affects the color of a tooth due to the translucency of enamel. Dentin, which is less mineralized and less brittle than enamel, is necessary for the support of enamel. Dentin rates approximately 3 on the Mohs scale of mineral hardness. There are two main characteristics which distinguish dentin from enamel: firstly, dentin forms throughout life; secondly, dentin is sensitive and can become hypersensitive to changes in temperature due to the sensory function of odontoblasts, especially when enamel recedes and dentin channels become exposed.

<span class="mw-page-title-main">Toothache</span> Medical condition of the teeth

Toothache, also known as dental pain, is pain in the teeth or their supporting structures, caused by dental diseases or pain referred to the teeth by non-dental diseases. When severe it may impact sleep, eating, and other daily activities.

<span class="mw-page-title-main">Pulp (tooth)</span> Part in the center of a tooth made up of living connective tissue and cells called odontoblasts

The pulp is the connective tissue, nerves, blood vessels, and odontoblasts that comprise the innermost layer of a tooth. The pulp's activity and signalling processes regulate its behaviour.

<span class="mw-page-title-main">Enamel organ</span>

The enamel organ, also known as the dental organ, is a cellular aggregation seen in a developing tooth and it lies above the dental papilla. The enamel organ which is differentiated from the primitive oral epithelium lining the stomodeum.The enamel organ is responsible for the formation of enamel, initiation of dentine formation, establishment of the shape of a tooth's crown, and establishment of the dentoenamel junction.

<span class="mw-page-title-main">Dentinogenesis imperfecta</span> Medical condition

Dentinogenesis imperfecta (DI) is a genetic disorder of tooth development. It is inherited in an autosomal dominant pattern, as a result of mutations on chromosome 4q21, in the dentine sialophosphoprotein gene (DSPP). It is one of the most frequently occurring autosomal dominant features in humans. Dentinogenesis imperfecta affects an estimated 1 in 6,000-8,000 people.

Dens invaginatus (DI), also known as tooth within a tooth, is a rare dental malformation where there is an infolding of enamel into dentine. The prevalence of condition is 0.3 - 10%, affecting more males than females. The condition is presented in two forms, coronal and radicular, with the coronal form being more common.

Dens evaginatus is a rare odontogenic developmental anomaly that is found in teeth where the outer surface appears to form an extra bump or cusp.

Regional odontodysplasia is an uncommon developmental abnormality of teeth, usually localized to a certain area of the mouth. The condition is nonhereditary. There is no predilection for race, but females are more likely to get regional odontodysplasia. The enamel, dentin, and pulp of teeth are affected, to the extent that the affected teeth do not develop properly. These teeth are very brittle. On radiographs the teeth appear more radiolucent than normal, so they are often described as "ghost teeth". Most cases are considered idiopathic, but some cases are associated with syndromes, growth abnormalities, neural disorders, and vascular malformations. Permanent teeth usually show effects of regional odontodysplasia if the deciduous tooth was affected. Many of these teeth do not erupt, and those that do have an increased risk of caries and periapical inflammation.

<span class="mw-page-title-main">Root canal treatment</span> Dental treatment

Root canal treatment is a treatment sequence for the infected pulp of a tooth which is intended to result in the elimination of infection and the protection of the decontaminated tooth from future microbial invasion. Root canals, and their associated pulp chamber, are the physical hollows within a tooth that are naturally inhabited by nerve tissue, blood vessels and other cellular entities. Together, these items constitute the dental pulp.

Dental pertains to the teeth, including dentistry. Topics related to the dentistry, the human mouth and teeth include:

<span class="mw-page-title-main">Pulpotomy</span>

Pulpotomy is a minimally invasive procedure performed in children on a primary tooth with extensive caries but without evidence of root pathology. The minimally invasive endodontic techniques of vital pulp therapy (VPT) are based on improved understanding of the capacity of pulp (nerve) tissues to heal and regenerate plus the availability of advanced endodontic materials. During the caries removal, this results in a carious or mechanical pulp exposure (bleeding) from the cavity. During pulpotomy, the inflamed/diseased pulp tissue is removed from the coronal pulp chamber of the tooth leaving healthy pulp tissue which is dressed with a long-term clinically-successful medicament that maintains the survival of the pulp and promotes repair. There are various types of medicament placed above the vital pulp such as Buckley’s Solution of formocresol, ferric sulfate, calcium hydroxide or mineral trioxide aggregate (MTA). MTA is a more recent material used for pulpotomies with a high rate of success, better than formocresol or ferric sulfate. It is also recommended to be the preferred pulpotomy agent in the future. After the coronal pulp chamber is filled, the tooth is restored with a filling material that seals the tooth from microleakage, such as a stainless steel crown which is the most effective long-term restoration. However, if there is sufficient remaining supporting tooth structure, other filling materials such as amalgam or composite resin can provide a functional alternative when the primary tooth has a life span of two years or less. The medium- to long-term treatment outcomes of pulpotomy in symptomatic permanent teeth with caries, especially in young people, indicate that pulpotomy can be a potential alternative to root canal therapy (RCT).

<span class="mw-page-title-main">Tooth resorption</span> Medical condition

Resorption of the root of the tooth, or root resorption, is the progressive loss of dentin and cementum by the action of odontoclasts. Root resorption is a normal physiological process that occurs in the exfoliation of the primary dentition. However, pathological root resorption occurs in the permanent or secondary dentition and sometimes in the primary dentition.

Dentin sialophosphoprotein is a precursor protein for other proteins found in the teeth. It is produced by cells (odontoblasts) inside the teeth, and in smaller quantities by bone tissues. It is required for normal hardening (mineralisation) of teeth. During teeth development, it is broken down into three proteins such as dentin sialoprotein (DSP), dentin glycoprotein (DGP), and dentin phosphoprotein (DPP). These proteins become the major non-collagenous components of teeth. Their distribution in the collagen matrix of the forming dentin suggests these proteins play an important role in the regulation of mineral deposition. Additional evidence for this correlation is phenotypically manifested in patients with mutant forms of dentin sialophosphoprotein. Such patients suffer dental anomalies including type III dentinogenesis imperfecta.

<span class="mw-page-title-main">Amelogenesis imperfecta</span> Genetic disorder resulting in abnormal enamel

Amelogenesis imperfecta (AI) is a congenital disorder which presents with a rare abnormal formation of the enamel or external layer of the crown of teeth, unrelated to any systemic or generalized conditions. Enamel is composed mostly of mineral, that is formed and regulated by the proteins in it. Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel as a result of abnormal enamel formation via amelogenesis.

<span class="mw-page-title-main">Pulp stone</span>

Pulp stones are nodular, calcified masses appearing in either or both the coronal and root portion of the pulp organ in teeth. Pulp stones are not painful unless they impinge on nerves.

<span class="mw-page-title-main">Regenerative endodontics</span> Dental specialty

Regenerative endodontic procedures is defined as biologically based procedures designed to replace damaged structures such as dentin, root structures, and cells of the pulp-dentin complex. This new treatment modality aims to promote normal function of the pulp. It has become an alternative to heal apical periodontitis. Regenerative endodontics is the extension of root canal therapy. Conventional root canal therapy cleans and fills the pulp chamber with biologically inert material after destruction of the pulp due to dental caries, congenital deformity or trauma. Regenerative endodontics instead seeks to replace live tissue in the pulp chamber. The ultimate goal of regenerative endodontic procedures is to regenerate the tissues and the normal function of the dentin-pulp complex.

<span class="mw-page-title-main">Pulp capping</span>

Pulp capping is a technique used in dental restorations to prevent the dental pulp from necrosis, after being exposed, or nearly exposed during a cavity preparation, from a traumatic injury, or by a deep cavity that reaches the center of the tooth causing the pulp to die. When dental caries is removed from a tooth, all or most of the infected and softened enamel and dentin are removed. This can lead to the pulp of the tooth either being exposed or nearly exposed which causes pulpitis (inflammation). Pulpitis, in turn, can become irreversible, leading to pain and pulp necrosis, and necessitating either root canal treatment or extraction. The ultimate goal of pulp capping or stepwise caries removal is to protect a healthy dental pulp and avoid the need for root canal therapy.

<span class="mw-page-title-main">Tooth discoloration</span> Medical condition

Tooth discoloration is abnormal tooth color, hue or translucency. External discoloration is accumulation of stains on the tooth surface. Internal discoloration is due to absorption of pigment particles into tooth structure. Sometimes there are several different co-existent factors responsible for discoloration.

Periapical granuloma, also sometimes referred to as a radicular granuloma or apical granuloma, is an inflammation at the tip of a dead (nonvital) tooth. It is a lesion or mass that typically starts out as an epithelial lined cyst, and undergoes an inward curvature that results in inflammation of granulation tissue at the root tips of a dead tooth. This is usually due to dental caries or a bacterial infection of the dental pulp. Periapical granuloma is an infrequent disorder that has an occurrence rate between 9.3 to 87.1 percent. Periapical granuloma is not a true granuloma due to the fact that it does not contain granulomatous inflammation; however, periapical granuloma is a common term used.

References

  1. 1 2 3 4 Kim, J.-W.; Simmer, J.P. (2007-05-01). "Hereditary Dentin Defects". Journal of Dental Research. 86 (5): 392–399. doi:10.1177/154405910708600502. ISSN   0022-0345. PMID   17452557. S2CID   1279513.
  2. ↑ Shields, E.D.; Bixler, D.; El-Kafrawy, A.M. (1973). "A proposed classification for heritable human dentine defects with a description of a new entity". Archives of Oral Biology. 18 (4): 543–IN7. doi:10.1016/0003-9969(73)90075-7. PMID   4516067.
  3. 1 2 3 4 5 Malik, Sangeeta; Gupta, Swati; Wadhwan, Vijay; Suhasini, GP (2015). "Dentin dysplasia type I – A rare entity". Journal of Oral and Maxillofacial Pathology. 19 (1): 110. doi:10.4103/0973-029X.157220. PMC   4451656 . PMID   26097326.
  4. ↑ Wang, Shih-Kai; Chan, Hui-Chen; Makovey, Igor; Simmer, James P.; Hu, Jan C.-C. (2012-12-05). "Novel PAX9 and COL1A2 Missense Mutations Causing Tooth Agenesis and OI/DGI without Skeletal Abnormalities". PLOS ONE. 7 (12): e51533. Bibcode:2012PLoSO...751533W. doi: 10.1371/journal.pone.0051533 . ISSN   1932-6203. PMC   3515487 . PMID   23227268.
  5. 1 2 Barron, Martin J.; McDonnell, Sinead T.; MacKie, Iain; Dixon, Michael J. (2008-11-20). "Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia". Orphanet Journal of Rare Diseases. 3: 31. doi:10.1186/1750-1172-3-31. ISSN   1750-1172. PMC   2600777 . PMID   19021896.
  6. ↑ Zhang, Xianqin; Chen, Lanying; Liu, Jingyu; Zhao, Zhen; Qu, Erjun; Wang, Xiaotao; Chang, Wei; Xu, Chengqi; Wang, Qing K (2007-08-08). "A novel DSPP mutation is associated with type II dentinogenesis Imperfecta in a chinese family". BMC Medical Genetics. 8: 52. doi:10.1186/1471-2350-8-52. ISSN   1471-2350. PMC   1995191 . PMID   17686168.
  7. ↑ McKnight, Dianalee A.; Suzanne Hart, P.; Hart, Thomas C.; Hartsfield, James K.; Wilson, Anne; Wright, J. Timothy; Fisher, Larry W. (2008-12-01). "A comprehensive analysis of normal variation and disease-causing mutations in the human DSPP gene". Human Mutation. 29 (12): 1392–1404. doi:10.1002/humu.20783. ISSN   1098-1004. PMC   5534847 . PMID   18521831.
  8. ↑ Kim, Jung-Wook; Hu, Jan C.-C.; Lee, Jae-Il; Moon, Sung-Kwon; Kim, Young-Jae; Jang, Ki-Taeg; Lee, Sang-Hoon; Kim, Chong-Chul; Hahn, Se-Hyun (February 2005). "Mutational hot spot in the DSPP gene causing dentinogenesis imperfecta type II" (PDF). Human Genetics. 116 (3): 186–191. doi:10.1007/s00439-004-1223-6. hdl: 2027.42/47595 . ISSN   0340-6717. PMID   15592686. S2CID   10035732.
  9. ↑ Li, Li; Shu, Yi; Lou, Beiyan; Wu, Hongkun (2012). "Candidate-gene exclusion in a family with inherited non-syndromic dental disorders". Gene. 511 (2): 420–426. doi:10.1016/j.gene.2012.09.042. PMID   23018043.
  10. ↑ Neville, Brad W.; Damm, Douglas D.; Chi, Angela C.; Allen, Carl M. (2015). Oral and maxillofacial pathology. Neville, Brad W.,, Damm, Douglas D.,, Allen, Carl M.,, Chi, Angela C. (Fourth ed.). St. Louis, MO. ISBN   9781455770526. OCLC   908336985.
  11. 1 2 3 4 5 6 Fulari, SangameshG; Tambake, DeeptiP (2013-10-01). "Rootless teeth: Dentin dysplasia type I". Contemporary Clinical Dentistry. 4 (4): 520–2. doi:10.4103/0976-237x.123063. PMC   3883336 . PMID   24403801.
  12. 1 2 "Dentin Dysplasia Type I - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). Retrieved 2017-12-07.
  13. ↑ O Carroll, M. K.; Duncan, W. K. (September 1994). "Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family". Oral Surgery, Oral Medicine, and Oral Pathology. 78 (3): 375–381. doi:10.1016/0030-4220(94)90071-x. ISSN   0030-4220. PMID   7970601.
  14. 1 2 Ravanshad, Shohreh; Khayat, Akbar (2006-04-01). "Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia Type 1". Australian Endodontic Journal. 32 (1): 40–42. doi:10.1111/j.1747-4477.2006.00008.x. ISSN   1747-4477. PMID   16603045.
  15. 1 2 "Dentin Dysplasia Type II - NORD (National Organization for Rare Disorders)". NORD (National Organization for Rare Disorders). Retrieved 2017-12-07.
  16. ↑ Burkes, E. Jeff; Aquilino, Steven A.; Bost, Michael E. (1979). "Dentin dysplasia II". Journal of Endodontics. 5 (9): 277–281. doi:10.1016/s0099-2399(79)80175-2. PMID   297771.
  17. ↑ Logan, James; Becks, Hermann; Silverman, Sol; Pindborg, Jens J. (1962). "Dentinal dysplasia". Oral Surgery, Oral Medicine, Oral Pathology. 15 (3): 317–333. doi:10.1016/0030-4220(62)90113-5. PMID   14466295.
  18. ↑ Wesley, Richard K.; Wysocki, George P.; Mintz, Sheldon M.; Jackson, Jack (1976). "Dentin dysplasia Type I". Oral Surgery, Oral Medicine, Oral Pathology. 41 (4): 516–524. doi:10.1016/0030-4220(76)90279-6. PMID   1063351.
  19. ↑ Malik, Sangeeta; Gupta, Swati; Wadhwan, Vijay; Suhasini, GP (2015-01-01). "Dentin dysplasia type I - A rare entity". Journal of Oral and Maxillofacial Pathology. 19 (1): 110. doi:10.4103/0973-029x.157220. PMC   4451656 . PMID   26097326.
  20. ↑ Muñoz-Guerra, Mario F.; Naval-Gías, Luis; Escorial, Verónica; Sastre-Pérez, Jesús (September 2006). "Dentin dysplasia type I treated with onlay bone grafting, sinus augmentation, and osseointegrated implants". Implant Dentistry. 15 (3): 248–253. doi: 10.1097/01.id.0000234638.60877.1b . ISSN   1056-6163. PMID   16966898. S2CID   23474265.
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