References

El-Naggar A, Chan J, Grandis J, Takata T, Slootweg P. WHO Classification of Head and Neck Tumours.Lyon, France: IARC; 2017
Barnes L. Pathology and Genetics of Head and Neck Tumours.Lyon, France: IARC; 2005
Song Y-L, Zhang W-F, Peng B, Wang C-N, Wang Q, Bian Z. Germline mutations of the PTCH gene in families with odontogenic keratocysts and nevoid basal cell carcinoma syndrome. Tumor Biol. 2006; 27:175-180
MacDonald-Jankowski D. Keratocystic odontogenic tumour: systematic review. Dentomaxillofac Radiol. 2011; 40:1-23
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Surgical/orthodontic management of a large mandibular odontogenic keratocyst (OKC)

From Volume 46, Issue 5, May 2019 | Pages 473-479

Authors

Amarpreet Atwal

BDS (Hons), MJDF, MOrth RCS

Post CCST Orthodontics, University of Sheffield and Royal Derby Hospital

Articles by Amarpreet Atwal

Email Amarpreet Atwal

Hannah Cottom

BDS(Hons), MJDF, FRCPath

Specialist Registrar in Oral and Maxillofacial Pathology, Royal London Hospital

Articles by Hannah Cottom

Peter Doyle

FDS RCS, FRCS(OMFS)

Consultant Oral and Maxillofacial Surgeon, Chesterfield Royal Hospital

Articles by Peter Doyle

Jonathan Sandler

BDS (Hons), MSc, PhD, MOrth RCS, FDS RCPS

Consultant Orthodontist, Chesterfield Royal Hospital, Chesterfield, UK

Articles by Jonathan Sandler

Abstract

The odontogenic keratocyst (OKC) is the third most common cyst of the jaw. OKCs are benign developmental cysts that are often locally destructive and large at presentation. They are usually identified during radiographic examination and require appropriate treatment planning to ensure optimal care. We present a very challenging case of a 12-year-old boy with a large mandibular OKC. The roots of several teeth were displaced, with both LR3 and LR5 unerupted and severely malpositioned. A multidisciplinary approach was therefore essential in view of the complexity, with joint planning and close liaison of treatment between the surgeon, orthodontist and general dental practitioner (GDP). This was fundamental to ensure the best possible outcome was achieved for the patient.

CPD/Clinical Relevance: This case report aims to increase awareness of OKCs to GDPs and outlines the surgical, orthodontic and restorative challenges faced in the management of such cases.

Article

Odontogenic keratocysts (OKCs) are benign developmental odontogenic cysts, which comprise approximately 10%–20% of all odontogenic cysts of the jaw.1 There is a peak in incidence in the second to third decade, with a greater prevalence reported in men compared to women (2:1).1 They occur most commonly in the mandible, especially the posterior body and angle regions, and almost always occur within bone, although a small number of peripheral OKCs have been reported.1 OKCs may initially appear small and unilocular, but early detection can be difficult, as they are often asymptomatic and growth is preferentially within the marrow spaces of the bone, with little cortical expansion. Therefore, OKCs are often detected later, when they are of a sufficiently large enough size to cause cortical expansion, perforation and affect adjacent structures.

In approximately 5%, OKCs may occur as part of Naevoid Basal Cell Carcinoma syndrome or Gorlin-Goltz syndrome.1 These syndromic cases tend to develop in younger patients, with greater tendency for multifocality and localization to the posterior maxilla.1 The OKC was renamed the Keratocystic Odontogenic Tumour (KCOT) in 2005 by the World Health Organization (WHO).2 At that time it was considered that this nomenclature more appropriately reflected its behaviour and proposed neoplastic nature. This was based on the knowledge that syndromic OKCs show mutation and inactivation of the PTCH1 gene, with a suggestion that the PTCH1 gene could also be important in the development of sporadic OKCs.2, 3 Similar genetic alterations have now been detected in other developmental cysts. Therefore, there is currently insufficient evidence to support a neoplastic nature of OKCs, which have now been reinstated as developmental in the current edition of the WHO Classification of Head and Neck tumours.1

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