Multiple odontogenic keratocysts associated with Gorlin-Goltz syndrome

Dixit, Siddharth; Acharya, Sonu; Dixit, Punam Basnet

doi:10.3126/kumj.v7i4.2765

Cited by 7 publications

(11 citation statements)

References 11 publications

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“…Radical treatment methods involve partial resection of the tumour-invaded bone together with a 5-millimetre margin of healthy bone tissue and are, undoubtedly, associated with the lowest recurrence rate. Nevertheless, in children whose tooth eruption or bone forming processes have not finished, the radical procedures should be replaced by conservative ones [7,52,53]. Whenever dental practitioners encounter cases of multiple or recurrent cysts (Figure 3), they are obliged to provide such patients with comprehensive dental care and carry out diagnostic tests or refer them for such tests, because the cysts might be the first noticeable symptoms of the nevoid basal cell carcinoma syndrome (GGS) [1,7,24].…”

Section: Treatment Of a Kcotmentioning

confidence: 99%

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: Treatment Of a Kcotmentioning

confidence: 99%

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

Kiwilsza

Sporniak‐Tutak

2012

Med Sci Monit

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“…Stoelinga advocated that the 3-minute application time to be safer. 12 Cryosurgery involves the use of liquid nitrogen that has a unique ability to devitalize the bone in situ while leaving the inorganic framework intact. Both these techniques are advocated in killing any epithelial remnants and dental lamina within osseous keratocysts, thus, helping in reducing the rate of recurrences.…”

Section: Discussionmentioning

confidence: 99%

Multiple Odontogenic Keratocysts in Nonsyndromic Patients—A Case Report and Review of Literature

Jamwal¹,

K²,

Ramakanth³

et al. 2019

Journal of Contemporary Dentistry

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Aim:The aim of this study is to highlight a behavioral pattern of odontogenic keratocysts (OKCs) in syndromic and nonsyndromic patients in terms of clinical course, genetic makeup, local destruction, management modalities, and recurrences such that detection in initial stages and intervention when done prevents extensive morbidity and a better quality of life. Background: OKC is a common developmental odontogenic cyst affecting the oral and the maxillofacial region, which arises from the dental lamina or its remnants. Multiple OKCs are usually seen in association with the nevoid basal cell carcinoma syndrome (NBCCS) but approximately 5% of the cases have multiple cysts without concomitant syndromic presentation. As it spreads by way of the bone marrow, it results in more destruction before any clinical manifestation appears. Although lesions are benign but are aggressive, locally destructive, and highly recurrent. Case description: A 15-year-old male patient, a rare case of nonsyndromic multiple OKCs, is presented here along with a review of literature with emphasis on the importance of thorough evaluation, diagnosis, treatment strategy, and strict follow-up. Conclusion: Although our case does not fulfill the major and minor criteria of syndrome, but considering the reported literature and clinical presentation, early age, multiple OKCs, involvement of both the jaws, cleft lip and cleft palate, and histological correlation, we suggest our case to be a partial expression of NBCCS. Clinical significance: Any case presenting with single or multiple OKCs should be thoroughly investigated locally and systemically for any other signs of NBCCS or other syndrome. A definite treatment protocol should be formulated depending on patient's age, number and size of lesion, remaining subsequent growth, and proximity to adjacent vital structures, to provide a better quality of life with a minimal amount of morbidity and kept on strict regular follow-up.

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“…The most important criteria to make a diagnosis for this syndrome are the presence of pigmented basal cell carcinomas, OKC, palmar and/or plantar pits, and ectopic calcifications of the falx cerebri [5][6][7][8]. Together with these major features, more than 100 minor features have been described.…”

Section: Discussionmentioning

confidence: 99%

Non-Syndromic Multiple Odontogenic Keratocysts: A Case Report and Comparison with Syndromic Multiple Odontogenic Keratocysts

Golgire¹,

Varekar²

2016

J Clin Case Rep

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The Odontogenic Keratocyst (OKC) is a relatively common developmental odontogenic cyst arising from the dental lamina remnants. Multiple OKCs are rare and occur either in syndromic and non-syndromic groups. The Keratocyst demands special consideration because of its aggressive clinical nature and propensity for recurrence. Epithelial proliferation plays a significant role in behaviour of OKC and suggests that more aggressive behaviour is present in case of syndromic OKC than non-syndromic ones. Here we report a case of multiple OKCs in a 20 year old female patient having simultaneous occurrence of cystic lesions in maxilla and mandible without any syndromic manifestations. Comparison between syndromic and non syndromic multiple OKCs is also discussed.

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Multiple odontogenic keratocysts associated with Gorlin-Goltz syndrome

Cited by 7 publications

References 11 publications

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

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

Multiple Odontogenic Keratocysts in Nonsyndromic Patients—A Case Report and Review of Literature

Non-Syndromic Multiple Odontogenic Keratocysts: A Case Report and Comparison with Syndromic Multiple Odontogenic Keratocysts

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