The role of the orthodontist in the diagnosis of Gorlin’s syndrome

Maroto, Martín Romero; Porras, Jose L.; Saez, Rafael Salvan; Rios, Marta Hoyos de los; Gonzalez, Luis Bravo

doi:10.1016/s0889-5406(99)70321-5

Cited by 18 publications

(16 citation statements)

References 17 publications

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“…In spite of a considerable size of the tumours, pathological bone fractures hardly ever occur. Other rare anomalies in the oral cavity include occlusal problems related to the adjustment, shape and number of teeth, as well as mild mandibular prognathism manifested in soft tissues by protrusion of the lower lip [29]. In many cases, a high palatal arch or a close relationship between the canal and the lower border of the mandible [30] were observed.…”

Section: Symptoms and Complicationsmentioning

confidence: 99%

“…In many cases, a high palatal arch or a close relationship between the canal and the lower border of the mandible [30] were observed. Less frequent symptoms included cleft lip, cleft palate [31] and alveolar process [27,29], as well as other deformities of alveolar processes caused by tumours. Tumours developing within the nasal sinuses may lead to a deteriorated patency of nasal passages [32].…”

Section: Symptoms and Complicationsmentioning

confidence: 99%

“…Apart from the earlier mentioned symptoms of NBCCS, patients may have numerous disorders affecting various systems and organs (Table 2) [2,4,5,10,17,22,25,27,29,30,34–39]. …”

Section: Symptoms and Complicationsmentioning

confidence: 99%

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Gorlin-Goltz syndrome – a medical condition requiring a multidisciplinary approach

Kiwilsza

Sporniak‐Tutak

2012

Med Sci Monit

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SummaryGorlin-Goltz syndrome is a rare genetic condition showing a variable expressiveness. It is inherited in a dominant autosomal way. The strongest characteristic of the disease includes multiple basal cell carcinomas, jaw cysts, palmar and plantar pits, skeletal abnormalities and other developmental defects. Owing to the fact that the condition tends to be a multisystemic disorder, familiarity of various medical specialists with its manifestations may reduce the time necessary for providing a diagnosis. It will also enable them to apply adequate methods of treatment and secondary prevention. In this study, we present symptoms of the disease, its diagnostic methods and currently used treatments.We searched 2 scientific databases: Medline (EBSCO) and Science Direct, for the years 1996 to 2011. In our search of abstracts, key words included nevoid basal cell carcinoma syndrome and Gorlin-Goltz syndrome.We examined 287 studies from Medline and 80 from Science Direct, all published in English. Finally, we decided to use 60 papers, including clinical cases and literature reviews.Patients with Gorlin-Goltz syndrome need particular multidisciplinary medical care. Knowledge of multiple and difficult to diagnose symptoms of the syndrome among professionals of various medical specialties is crucial. The consequences of the disease pose a threat to the health and life of patients. Therefore, an early diagnosis creates an opportunity for effective prevention and treatment of the disorder. Prevention is better than cure.

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Section: Symptoms and Complicationsmentioning

confidence: 99%

Section: Symptoms and Complicationsmentioning

confidence: 99%

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Gorlin-Goltz syndrome – a medical condition requiring a multidisciplinary approach

Kiwilsza

Sporniak‐Tutak

2012

Med Sci Monit

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“…According to Marotto et al [13], some of the most common clinical findings of the syndrome are discovered through radiography commonly used in orthodontic treatment. In the case described in this study, a panoramic radiograph for orthodontic purposes showed radiolucent areas in the maxilla and mandible, suggesting the presence of KCOTs.…”

Section: Discussionmentioning

confidence: 99%

Early diagnosis of Gorlin-Goltz syndrome: case report

et al. 2011

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The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the KCOTs are usually one of the first manifestations of the syndrome. This article paper reports the case of a patient, a 10-year-old boy with NBCCS, emphasizing its clinical and radiographic manifestations. This study highlights the importance of health professionals in the early diagnosis of NBCCS and in a preventive multidisciplinary approach to provide a better prognosis for the patient.

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“…Male to female ratio is 1:0.62 for odontogenic keratocyst not associated with NBCCS, and 1:1 for odontogenic keratocyst in NBCCS, that is, simple keratocysts are more common in males, but more females with NBCCS develop odontogenic keratocysts. 11,12 Odontogenic keratocyst associated with NBCCS have greater predilections for the mandible than the maxilla, with 69% occurring in the mandible and 31% in the maxilla. 10 The odontogenic keratocyst is now termed as 'keratocystic odontogenic tumor.…”

Section: Introductionmentioning

confidence: 99%