A variant of the Ekman-Westborg and Julin trait

Abstract

Ekman-Westborg and Julin is a trait that shows multiple macrodontia and multituberculism affecting only the teeth with no other anomalies (E-WJ). The aim of this report is to present a case which appears to manifest all the clinical signs of the E-WJ trait including odontoma formation. A 18-year-old girl with gingival inflammation particularly in the maxillary insicor area was referred to the authors’ department. Panoramic, periapical and cephalometric radiographs were examined and complex odontoma associated with unerupted maxillary permanent lateral incisors was revealed. Intraoral examination revealed anterior crossbite, Angle Class III type malocclusion with mandibulary prominence and macrodontia of teeth 37, 12, 11 and 21. The patient was accepted as a new sporadic case of E-WJ. More case reports are needed to elucidate the causes and pathogenesis of this condition.

Ekman-Westborg and Julin (E-WJ) described multiple macrodontia and multituberculism affecting only the teeth with no other systemic anomalies. B enjamin et al. proposed ‘multiple macrodontic multituberculism’ as a more suitable term to identify these typical dental alterations, because this anomaly is not a syndrome but only a collection of unusual abnormalities isolated to teeth . In 1974, E-WJ reported the first case of multiple dental malformations which include multiple macrodontia and multituberculism of the posterior teeth, single conical molar roots, pulp invaginations and evaginations in premolars and peak shaped canines . Despite the fact that macrodontia has been reported to affect mostly the mandibular premolars and molars, incisors are rarely affected . Macrodontia, one of the characteristric findings of the E-WJ trait, is an uncommon condition in which the teeth appear larger than normal. It rarely affects the entire dentition, but commonly involves a group of teeth or a single tooth. In the dental literature, only a few cases of E-WJ trait and its variants have been reported . The pathogenesis and the role of genetic factors in this trait is unclear .

Odontoma is a benign odontogenic tumour or hamartoma, composed of a mixture of odontogenic tissues and classified into two types: compound and complex odontomas. Compound odontomas show considerable anatomic resemblance to normal teeth whereas complex odontomas are irregular mixtures of odontogenic hard and soft tissues that bear no morphologic similarity to normal teeth. The reasons for odontoma formation are unknown. The aim of this report is to present a case which appears to manifest all the clinical signs of the E-WJ trait with the addition of a formation odontoma and to discuss treatment options.

Case report

A 18-year-old girl with gingival inflammation particularly in the maxillary insicor area was referred to the authors’ department in December 2009. She underwent pedodontic and orthodontic consultations for macrodontia, agenesia and anterior crossbite. Her medical history was unremarkable, she was the second child of healthy parents, her mother’s pregnancy had been normal and consanguinty was denied. The clinical examination of the parents and the sibling showed no dental anomalies. She was mentally healthy and had no history of trauma. In order to eliminate the possibility of systemic illnesses, biochemical tests to determine sex hormone levels, growth hormone levels, thyroid hormone levels, complete blood count, alkaline phosphatase, and diabetes were carried out and to be found normal.

Intraoral examination revealed anterior crossbite, Angle Class III type malocclusion with mandibulary prominence and macrodontia of teeth 37, 12, 11 and 21 ( Fig. 1 ). The mesiodistal widths of the upper left central and upper right central incisors were 9 mm and 10 mm, respectively. The buccolingual widths of the upper left central and upper right central incisors were 10 mm and 11 mm, respectively. Intraoral examination showed late mixed dentition. The persistant primary teeth 52 and 53 were extracted and the some suspected pulpal obliteration was resolved by histopathologic evaluation. Histopathologic findings revealed that these teeth showed osteodentin formation in the pulp chamber ( Fig. 2 ). The upper permanent incisors exhibited large shovel-shaped crown and peak shaped canines. The maxillary incisors had remarkable lingual tubercules. The permanent molars had several extra cusps. Dens in dente was seen on teeth 12, 11, 21. Based on radiographic, clinical and laboratory evaluations this case was considered to be consistent with the previously reported findings of the E-WJ trait.

Fig. 1
Frontal and lateral view of the patient.

Fig. 2
(A–E) Clinical appearance. (F) Histopathologic evaluation and osteodentin formation.

Panoramic, periapical and cephalometric radiographs were examined and complex odontoma associated with unerupted maxillary permanent lateral incisors and impacted or half impacted third molars was revealed ( Fig. 3 ). The operation, consisting of the enucleation of the odontoma and the extraction of impacted 22, was performed. The patient’s postoperative course was uneventful and histopathologic evaluation of the enucleated mass was consistent with odontoma ( Fig. 4 ).

Fig. 3
Panoramic and cephalometric radiographs indicating dental anomalies and odontoma.

Fig. 4
Complex odontoma associated with unerupted maxillary permanent lateral incisors.

In order to achieve an acceptable level of oral hygiene, periodontal treatment consisting of scaling and polishing was applied. The patient was shown how to manage mechanical plaque by brushing her teeth thoroughly and using dental floss. Gingival inflammation in the maxillary incisor area was thought to be due to the aberrant frenulum attachment and shallow vestibular sulcus. In order to remove the excessively high frenulum attachment and simultaneously increase the depth of vestibular sulcus, free gingival graft procedures were performed. Free gingival grafts were used to increase the zone of attached gingiva and to extend the depth of the vestibule at the same time.

The girl had class III malocclusion caused by mandibular prognathism and dental class III malocclusion with a narrow maxillary arch. In order to eliminate the maxillary crossbite, surgical asistant rapid palatal extension was applied. Extraction of the first mandibular premolars to treate the severe mandibular crowding has been planned. Two jaw surgical correction will be applied to resolve the skeletal problems.

Discussion

Macrodontic incisors and multiple macrodontia are rare conditions ( Table 1 ), but may be associated with insulin-resistant diabetes, otodental syndrome or facial hemihyperplasia. Large maxillary incisors have been reported in KBG syndrome (KBG stands for the initials of the affected patients in the original report). Generalized macrodontia may be caused by hormonal imbalance, as has been described in pituitary gigantism. Generalized macrodontia and shovel shaped incisors have been reported in 47, XXY syndrome. Combined dental morphologic anomalies have been rarely described in the literature. In 1974, E kman-Westborg and Julin reported a unique example of multiple dental malformations . In this article, a patient with clinical and radiological findings consistent with the previously reported cases of E-WJ trait was presented. This case was considered to be a variant of the E-WJ trait because of the presence of the dental morphological anomalies including macrodontia, shovel shaped incisors, peak shaped canines, dental agenesis and orthodontic disorders. Radiographs indicated the presence of complex odontoma associated with unerupted maxillary permanent lateral incisors as shown in one of the cases previously presented . Mental retardation was reported in three of the previously reported cases but no such disorder was found in the present case. With orthodontic problems, including anterior and posterior cross bite and mandibular prognatism, the presented case also has similarities to the cases presented in some previous reports . E-WJ trait macrodontia of incisors is extremly rare and few cases of E-WJ trait and this trait’s variants have been presented in the literature . A hereditary character was found in one of the cases; both mother and son diagnosed with the E-WJ trait had multiple macrodontic teeth and odontoma in the jaws . Owing to the limited number of cases reported, only one familial case, suggesting autosomal dominant or X linked dominant inheritence has been reported. In the present case, the authors examined the mother of the patient, but similar findings were not found. The present patient was accepted as a new sporadic case. More case reports are need to elucidate the causes and pathogenesis of this condition.

Table 1
Common clinical features of E-WJ trait.
Clinical features 1974 1978 1990 1997 1997 1998 2003 2006 2009 2011
This report
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 + +
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Jan 26, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on A variant of the Ekman-Westborg and Julin trait
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