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 Table of Contents  
REVIEW ARTICLE
Year : 2015  |  Volume : 6  |  Issue : 3  |  Page : 88-92

Generalized severe short root anomaly: A diagnostic dilemma


1 Department of Periodontics, Sudha Rustagi College of Dental Sciences and Research, Faridabad, Haryana, India
2 Department of Oral and Maxillofacial Surgery, Sudha Rustagi College of Dental Sciences and Research, Faridabad, Haryana, India
3 Department of Pediatrics, Fortis Hospital, Faridabad, Haryana, India

Date of Submission11-Oct-2014
Date of Acceptance09-Jun-2015
Date of Web Publication4-Dec-2015

Correspondence Address:
Sumidha Bansal
Department of Periodontics, Sudha Rustagi College of Dental Sciences and Research, Faridabad, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-6944.171071

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  Abstract 

A short root anomaly is a very rare condition, more so, if found in a generalized state. The reasons for the presence of short roots can vary from idiopathic to childhood environmental insults such as radiotherapy, chemotherapy, or dental and systemic hereditary disorders. This anomaly may sometimes, not always, be associated with short stature or short stature syndromes. Short roots, whether generalized or localized, may or may not pose any clinical problem. Abnormalities in the size or color or the number of teeth always demand some extra care and more so if associated with facial abnormalities and short stature also. A good clinician is one who keeps an eagle's eye on the overall appearance of the patient so as to advise some nonroutine investigations that may unearth some undiagnosed dental or systemic hereditary disorder. A case of generalized short roots affecting only permanent dentition in two brothers is presented here. This report reviews short root anomaly and also gives us an insight to the series of investigations required and the possible diagnosis in such cases.

Keywords: Rhizomicry, root dwarfism, short roots, short stature syndromes


How to cite this article:
Bansal S, Bansal P, Gupta A. Generalized severe short root anomaly: A diagnostic dilemma. Indian J Oral Sci 2015;6:88-92

How to cite this URL:
Bansal S, Bansal P, Gupta A. Generalized severe short root anomaly: A diagnostic dilemma. Indian J Oral Sci [serial online] 2015 [cited 2019 Sep 20];6:88-92. Available from: http://www.indjos.com/text.asp?2015/6/3/88/171071


  Introduction Top


The literature describes the condition of short root anomaly, whether generalized or localized, as a constitutional anomaly whose prevalence varies with sex, race, and hereditary predisposition. [1] A perusal of the related literature reveals that while localized short roots are still found, the presence of generalized short roots is absolutely rare with very few cases reported all over the world. The patient might not even be aware of the condition as presented in the case below. The serendipitous discovery of the presence of generalized short roots was made when a 16 years old male reported for prosthetic rehabilitation of congenitally missing mandibular anterior teeth.


  Case Report Top


A 16-year-old male, disproportionately short statured [Figure 1], reported for prosthetic rehabilitation in relation to the mandibular anterior region.
Figure 1: Standing photograph of patient

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Clinical features

Oral examination [Figure 2] and [Figure 3] revealed retained 64, missing 31, 32, 41, 41, 43 without any history of extraction, generalized microdontia, short clinical crown length with upper anterior crowding, spade-shaped centrals and shovel-shaped laterals, normal physiologic mobility, and crossbite with an end of relationship of molars. Enamel showed normal opalescence except for the presence of slight hypoplastic enamel with brown staining but no pitting. Caries, systemic illness, any relevant medical history were not found. Facial features, which were otherwise proportionate, presented a depressed nasal bridge and slightly hypoplastic maxilla [Figure 4] and [Figure 5]. Height was 4 ft 3 inches (127 cm) with relatively short legs, and hence, a seemingly disturbed leg body ratio. [2] Accompanying younger brother, 9 years of age, also appeared to be short-statured according to his age. Orthopantomograph (OPG) was advised by both the brothers.
Figure 2: Clinical photograph of oral cavity showing crossbite

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Figure 3: Clinical photograph of oral cavity showing crowding

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Figure 4: Patient's front face showing saddle nose, frontal bossing, and deficient premaxilla

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Figure 5: Patient's profile showing saddle nose, frontal bossing, and deficient premaxilla

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Radiographic features

OPG [Figure 6] revealed the presence of generalized, extremely short, and conical but completely formed roots without any periapical radiolucencies. Crown root ratio was approximately 1:0.7 in posteriors and 1:0.8 in anteriors. There was a presence of tooth buds of all third molars but a congenital absence of aforementioned clinically missing teeth. There seemed to be an absence of proper lamina dura and periodontal ligament space. Jaw bones were free of any pathology with normal height and trabecular pattern. The bifurcation and trifurcation of posterior teeth were not clearly visible. Pulp chambers seemed to be obliterated except in posterior teeth while a rudimentary pulp canal was present in all other teeth.
Figure 6: Patient's orthopantomograph

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OPG of younger brother [Figure 7] shows generalized microdontia, missing tooth bud of 18, rotated permanent maxillary lateral incisors. All second molars show single conical root while mandibular first molars seem to be taurodontic because of shortness of root. The primary teeth show normal shape, size, pulp chamber, resorption pattern, enamel, and dentin translucency. Erupting premolars are quite radiopaque with pulp chamber not being clearly visible although same is not true for erupted and erupting molars.
Figure 7: Patient's younger brother's orthopantomograph

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  Review and Discussion Top


Rhizomicry or short root anomaly or root dwarfism, of the upper incisors or premolars, has been reported [3] in approximately 1 person out of 100 but the same in generalized form is considered extremely rare. Both dental and systemic hereditary disorders can be associated with generalized shortness of the roots; the most probable examples of former being dentinal dysplasia and dentinogenesis imperfecta, [4],[5] whereas the latter includes some short stature syndromes. [3],[6] Arrested root formation may be the result of environmental insults during tooth development, such as radiation therapy or chemotherapy also, for childhood malignancies. More rarely, short root anomaly may be idiopathic, probably the result of fresh mutations or variable penetration of an autosomal dominant trait. [7]

The only study regarding this subject carried out by Lind [1] reported normal crown root ratio as 1:1.6. The occurrence of short roots was found to be more common in a Mongolian and Japanese population and thrice more frequently in females, implying that prevalence differs with race and sex. Familial occurrence and high frequency of frontal crossbite interpreted as a deficiency in premaxillary growth were reported. It was stressed that differentiation should always be made between developmental short roots and roots short due to resorption.

Edwards and Roberts [8] reported a case of generalized short root anomaly unassociated with any systemic illness, radiotherapy, short stature, or dental syndrome, but the history of familial occurrence was there. Crown root ratio of more than 1:1 in all except permanent molars, complete roots with closed apices, normal lamina dura were present. Diagnosis or differential diagnosis of the condition was, however, not mentioned.

The presence of short roots may or may not be associated with short stature which when present can be either proportionate or disproportionate. Forty percent of short statured people fall in normal variant short stature category, which is precisely defined as being characterized by a current height and adult height prediction < third percentile, a birth weight > 2.5 kg, no organic cause from growth retardation, an average peak serum growth hormone >12 ng/ml. The average height for a 16-year-old Indian male is 154-194 cm, which in the presented case was much lesser (approximately 127 cm). The legs compose approximately half of adult human height, with women having a tendency to have a higher leg to body ratio than men. [2] The subdivision of proportional and disproportional is quite subjective. Disproportionate short stature is further subdivided into short limb short stature (includes achondroplasia and other chondrodysplasias), and short trunk dwarfism (includes spondyloepiphyseal dysplasia, Morquio's, and metatropic dwarfism). Routine laboratory values would include complete blood count, urinalysis, glucose, creatinine, and alkaline phosphatase. Radiographs should include a wrist for bone age in the proportionate group, and a lateral of the spine, neck and skull, and an additional anteroposterior view of the pelvis in the disproportionate group. Consultation with an endocrinologist and/or dysmorphologist is suggested. [9],[10]


  Short Roots Associated with Short Stature and Syndrome Top


When associated with short stature [6],[11],[12],[13],[14],[15] syndromes such as Down, Aarskog, Seckel (bird headed dwarfism), Rothmund-Thomson can be thought of although a presence of syndrome is not necessary. [16] Kantaputra [17],[18],[19] reported a new syndrome involving skeletal dysplasia and dental anomalies with autosomal recessive inheritance. Anomalies consist of disproportionately short stature, broad and depressed nasal bridge, anteroposteriorly broad chest, kyphosis, protruded abdomen while skeletal radiographs indicate a large sella turcica, platyspondyly, hypoplastic acetabulum, and a small body of mandible. Both deciduous and permanent dentition are hypodontic, microdontic, opalescent, rootless with obliterated pulp chambers, and severely hypoplastic alveolar process. The combination of microdontia, shortness of roots, delayed dental development, and hypoplastic alveolar processes has also been described in microcephalic osteodysplastic primordial dwarfism. The  Rothmund-Thomson syndrome More Details [7] is a rare genodermatosis characterized by poikilodermatous rash starting in infancy, with variable associated clinical findings such as juvenile cataracts, small stature, skeletal abnormalities, sparse hair, dystrophic or thin nails, dental malformations, and predisposition to skin and bone cancers with increased risk of osteosarcoma often arising in previously dysplastic long bones. This particular case [7] showed abnormally short roots in a patient of normal height, growth, and intelligence during the radiographic examination, affecting a complete permanent dentition including partially erupted third molars. Affected teeth had normal crowns, physiologic mobility, and normal lamina dura. Other syndromes associated with short roots are an otodental syndrome in which it occurs along with abnormal globe-shaped crown morphology of molars. Another related syndrome "familial otodentodysplasia" is characterized by the posterior teeth with abnormal morphology, large pulp chambers, short root length compared to crown height, and the absent or small premolars in association with high frequency hearing loss. [20]


  Short Roots Associated with Short Stature but No Syndrome Top


Generalized short roots associated with microdontia, taurodontism of posterior teeth, multiple dens invaginatus along with short stature but no syndrome and history of spontaneous exfoliation of several teeth [17] was reported. Microdontic teeth normal in shape and color, short tapered roots with crown root ratio more than 1:1 and partially obliterated pulp chambers in all except molars were found. Endocrinologic evaluation for growth hormone, thyroxine, and cortisol were normal. The final diagnosis of dentin dysplasia Type I (DDI), made on the basis of clinical and radiographic features, was ruled out on histologic evaluation, and idiopathic generalized short root anomaly was rather suggested. Similarly, a radiological skeletal survey of an extremely short (99 cm) 17-year-old male, with missing mandibular incisors, mobile canine teeth, short trunk and limbs, showed abnormal and unusual findings. [15] Teeth were found to have short, conical roots and obliterated pulp chambers. In both cases, extensive biochemical and the endocrinological investigation concluded that the findings were inconsistent with any recognized skeletal dysplasia or recognized syndrome affecting the oral and dental tissues.


  Short Roots with or without Syndrome Top


Rootless teeth have been reported in DDI, which is a rare disturbance in dentin formation. It is postulated that abnormal dentin formation in DDI can cause different teeth morphology as taurodontism, short roots and rootless teeth all in the same patient [21] because of regionalized abnormalities in cellular function and proliferation as occurs in regional odontodysplasia. A subclassification of DDI explains the occurrence of variation in root length from rootless to almost normal root length in different teeth afflicted by the same disease. [22] Features include periapical radiolucencies, mobility, and spontaneous exfoliation. DDI can occur as isolated condition or as component of certain syndromes [17] such as  Singleton-Merten syndrome More Details; tricho-onycho-dental syndrome; with skeletal dysplasia and sclerotic bone; calcinosis universalis; rheumatoid arthritis and vitaminosis D; tumoral calcinosis. Regional odontodysplasia or ghost teeth show short roots with wide open apices and very wide pulp canals that often become infected. Irradiation to jaws or chemotherapy during the period of root development leads to arrested development and can give a radiographic appearance of DDI. Vitamin D-dependent and resistant rickets both have clinical and radiographic features of dentinogenesis imperfecta and DDI, respectively. Although, former is characterized by yellowish to brown enamel, chronic periodontal disease, large quadrangular pulp chambers, and short roots [23] features of latter include attrition and the exposure of abnormally formed dentine of primary teeth and abscessed noncarious primary or permanent teeth.


  Short Stature Associated with Dental Aberrations other than Short Roots Top


 Ellis-van Creveld syndrome More Details [24] which affects primary dentition and there is a dental aberration in tooth form, single conical roots in primary molars, hypodontia, and hypoplasia. Systemically disproportionate dwarfism with short limbs and exceptionally long trunk, bilateral postaxial polydactyly of the hands, dystrophic nails, congenital cardiac malformations are present. Kosowicz and Rzymski [25] studied abnormalities of tooth development in pituitary dwarfism where along with other abnormalities like delayed shedding and overcrowding of teeth, short roots were not found. Dental development in patients having somatotropin deficiency [26],[27] indicate crowding, need for orthodontic treatment, anomalies of eruption, and dental morphogenesis owing to decrease in the level of circulating growth factors especially epidermal growth factor but no short roots.


  Conclusion Top


Generalized short root anomaly, which is only a finding and not the diagnosis per se, has an autosomal dominant pattern of inheritance like DDI. These anomalous teeth function remarkably well throughout life unless exposed to severe stress. Mobility is not always a feature and chance discovery of short roots may be there when radiographs are taken for some other purpose like prosthetic rehabilitation or orthodontic treatment. [4] The pretreatment radiographic evaluation of root length is mandatory when orthodontic, prosthetic, periodontal, or endodontic therapy is contemplated. Prophylactically, teeth should be protected from strong masticatory and orthodontic stress, pressure from retained/crowded teeth and traumatic occlusion, which can cause root resorption further shortening of roots. Emphasis should be placed on dental care aiming at prevention of tooth loss from caries or periodontal disease. During the extraction, elevators should be used with caution so as to protect the adjacent tooth from inadvertent mobilization. When associated with short stature especially, investigations should be carried out to find out the presence of any dental or systemic abnormality, syndrome or skeletal dysplasia so as to head the treatment, whether curative or preventive, incorrect direction accordingly.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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