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The Ekman-Westborg and Julin trait: report of a case

Judit A. Nemes, DDS,a and Marta Alberth, MD, LDS,b Debrecen, Hungary
FACULTY OF DENTISTRY, MEDICAL AND HEALTH SCIENCE CENTER, UNIVERSITY OF DEBRECEN

Macrodontia associated with multituberculism, central cusps, and pulpal invaginations is reported in a 7year-old Hungarian girl. Over 5 years’ follow-up the problems of the diagnosis, the similarity with and relationship to the Ekman-Westborg and Julin trait, and the difficulties of the treatment are discussed. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:659-62)

Macrodontia of a single tooth is a relatively uncommon condition, and the majority of cases are reported in mandibular molars or premolars.1-4 Macrodontia of incisors is extremely rare and may be confused with fusion or germination of adjacent teeth to form a single tooth.5,6 Multiple macrodontia is rare, but it may be associated with insulin-resistant diabetes, otodental syndrome, or facial hemihyperplasia.7-11 Large maxillary incisors have been reported in KBG syndrome. Generalized macrodontia may be caused by hormonal imbalance, as has been described in pituitary gigantism.7 In 47,XYY syndrome, generalized macrodontia and shovel-shaped incisors have been reported.12,13 It should be remembered that an illusion of generalized macrodontia will occur if the jaws are small relative to the size of the teeth. Combined dental morphologic anomalies have been rarely described in the literature. In 1974, EkmanWestborg and Julin14 reported a unique example of multiple dental malformations. In addition to multiple macrodontia and multituberculism of the posterior teeth, single conical molar roots, pulp invaginations and evaginations in premolars, and peak-shaped canines were demonstrated in the permanent dentition of a 14-year-old male.14 Similar cases were reported by Reichart et al.,15 Miikada et al.,16 Ritzau et al.,17 Nakagawa et al.,18 and Benjamin et al.19 Variants of this condition were reported by Mann et al.20 and by Yoda et al.21 Previously, the authors used the “Ekman-Westborg–Julin syndrome” name to determine this condia

tion. As this anomaly is not a syndrome, only a collection of unusual abnormalities isolated to teeth, Benjamin et al.19 proposed “multiple macrodontic multituberculism” as a better name for these typical dental alterations. Although the leading signs in these cases were generalized macrodontia and multituberculism, there were many other important abnormalities in all cases, so we propose “Ekman-Westborg and Julin trait” as a name to determine this condition. Our aim is to present a new case which appears to manifest all the clinical signs of the Ekman-Westborg and Julin trait and to discuss the problems of the treatment. CASE REPORT Clinical presentation
A 7-year-old Hungarian girl was referred to the Department of Pediatric Dentistry, Medical and Health Science Center, University of Debrecen, Hungary, for consultation concerning macrodontia combined with bimaxillary crowding. The patient’s medical history included the usual childhood infections without complications. The mother’s pregnancy had been normal, and no genetic disorders were found in the family history. The clinical and radiologic examination of the parents and sisters showed no dental anomalies. Consanguinity was denied. Results of physical examination and routine laboratory tests were within normal limits. A normal karyogram of the patient excluded any chromosomal anomalies. Mild mental retardation was noted. Extraoral inspection showed habitual open mouth posture with mouth breathing. Intraoral examination revealed anterior open-bite, Angle II–type occlusion with maxillary prominence. All the primary teeth and the upper permanent molars were carious. Otherwise the primary teeth were of normal shape and size. The upper left central permanent incisor exhibited extremely large shovel-shaped crown (mesiodistal width 11.7 mm, buccolingual width 10.5 mm) and a wide and deep invagination on the palatal side (Figs. 1 and 2). The permanent molars were enlarged and had several extra cusps. Roentgenographic examination showed generalized macrodontia of the permanent teeth, single conical roots of the permanent molars, the agenesia of the permanent lower central incisors, multituberculism of the molars and premolars,

Assistant Professor, Department of Pediatric Dentistry, Faculty of Dentistry, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. b Assistant Professor, Department of Pediatric Dentistry, Faculty of Dentistry, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. Received for publication Jan 24, 2005; returned for revision Sep 01, 2005; accepted for publication Sep 12, 2005. 1079-2104/$ - see front matter © 2006 Mosby, Inc. All rights reserved. doi:10.1016/j.tripleo.2005.09.007

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Fig. metacarpal. Fig. The crowns of these maxillary premolars were mesiodistally 68% and buccolingually 57% larger than average22 (Fig. however. . 3. Palatal aspect of the maxillary left central incisor. the diagnosis of hemifacial hypertrophy was excluded. The invagination is divided into compartments by enamel crests. Mesiodistal width 12. Because the lack of the enlargement of the jaws and the soft tissues was obvious and the face was symmetrical. Note the signs of resorption on the mesial root. Enlarged multituberculated molars are a commonly reported feature of otodental syndrome. before surgical removal of the mandibular right first molar.5. Periapical radiograph showing abnormally large invaginated maxillary incisors. and skeletal abnormalities such as vertebral.XYY syndrome generalized macrodontia and shovelshaped incisors have been reported. When the patient was 9. Differential diagnosis The presence of macrodontic maxillary central incisors and the mild mental retardation was suggestive of KBG syndrome. we removed teeth #4 and #13 under general anesthesia. 4). buccolingual width 14. and femoral anomalies excluded this diagnosis. Surgically removed maxillary second premolar. The oral features reported in this case were consistent with a diagnosis of Ekman-Westborg and Julin trait.2 mm. Orthopantomogram at the age of 9. Based on the laboratory tests we excluded insulin-resistant diabetes and hyperpituitarism. Although in 47. 4. namely to prevent further crowding.5 years old. we had to remove tooth #19 because of a serious pulpal inflammation. 1. brachy- dactyly. The extraction of tooth #30 was performed under general anesthesia. so for orthodontic considerations. the normal karyogram ruled out this diagnosis. the root of the lower right first molar showed the signs of resorption secondary to the pressure of the erupting second premolar (Fig. When the patient was 8 years old.6 mm. the lack of the characteristic face. central cusps on the lower premolars. the disparity between the size of the upper teeth and the upper jaw was evident. but there was no sensorineural hearing loss to support this diagnosis. When the patient was 11 years old. in which multiple or generalized macrodontia have been reported. Fig. and invaginations on all the upper incisors. 3). 2.660 Nemes and Alberth OOOOE November 2006 Fig.

from a pit or fissure. central evaginations. and invaginations. evagina- Table I. In most cases a fine pulpal extension was demonstrable in the dentinal core of the cusp. can be categorized as follows: true generalized. (1997) Nakagawa et al. Number 5 Nemes and Alberth 661 Present authors (2005) DISCUSSION Similarities between our case and the 6 formerly reported cases are evident (Table I). there has been no direct evidence or any clues to clarify the cause of this trait. (2003) . (1995) Ritzau et al. Macrodontia of single teeth or groups of teeth has occasionally been described. which was assumed to be the leading clinical sign in our case. (1978) Miikada et al.23 True generalized macrodontia associated with hyperpituitarism or other (unknown) etiology is rare. Although 7 patients have been reported and studied. Central evaginations (cusps) were seen on the lower premolars. The generalized macrodontia results in serious orthodontic anomalies. In this group the crown has a normal or “near-normal” appearance but the occlusal/palatal surface shows a complicated fissure pattern. The enamel lining is often deficient at the base of invagination. an invagination may arise. In our case we found enlarged teeth in normal-size jaws. Multituberculism. Mild mental retardation was present in 3 of the 7 reported cases. With our report the male:female ratio is 5:2. Pathogenetically the underlying mechanism of macrodontia is unknown. All the 4 leading signs mentioned above raise special treatment problems. so it can be classified as true generalized macrodontia (Table II). of the 4 leading signs we have to mention the invaginations. but all the premolars and upper incisors had enlarged multitubercular crowns. relative generalized. (1997) Bejamin et al. We think that this new case demonstrates a variant of the Ekman-Westborg and Julin phenotype. According to Oehlers’ classification25 the investigated invaginations belong to group I. not only the molars were multituberculated. such as anterior open bite and crowding. Multituberculism and the presence of central cusps are important characteristics of the Ekman-Westborg and Julin trait. The invagination in the present case seems to be the result of an inclusion of a portion of the enamel organ. Invagination could be the result either of active proliferation of an area of the enamel organ. Genetic cause could not be demonstrated in any of the cases of Ekman-Westborg and Julin trait.OOOOE Volume 102.24 Finally. multituberculism. In our case. whereas relative generalized macrodontia is more common but difficult to define. Clinical features of Ekman-Westborg and Julin trait Ekman-Westborg and Julin (1974) Macrodontia—permanent teeth Macrodontia—primary teeth Multituberculism Central cusp Dental invagination Shovel-shaped incisors Peak-shaped canines Single conical roots Dental agenesis Dental crowding Dental impaction Open bite Mental retardation Reichart et al. Macrodontia. and isolated macrodontia of single teeth. All are sporadic cases and there is no parental consanguinity. or of displacement of part of the enamel organ into the papilla as a result of abnormal pressure from the surrounding tissue. with infolding of the proliferating cells into the dental papilla. The 4 leading signs in our case are macrodontia. The coronal invaginations observed in the upper incisors are difficult to explain.

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