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Clinical images. A) Frontal extraoral photograph; B) Frontal intraoral image of dental occlusion; C) Approximate photograph of skin lesion on the face; D) Approximate photograph showing hand injuries.
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Gorlin-Goltz Syndrome is a genetic disorder characterized by a series of clinical changes, including the presence of multiple odontogenic keratocysts and nevus basal cell carcinomas. As these lesions involve the maxillofacial region and can evolve to severe sequelae, it is essential that the dental surgeon recognize this pathology, in order to prom...
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Context 1
... 20-year-old female patient, melanoderma, was referred by her orthodontist to the Oral and Maxillofacial Surgery service, for presenting a cyst in anterior mandible region. The clinical examination (Fig. 1A) revealed a slight asymmetry of mandibular right body, asymptomatic, teeth 32 and 33 in infraocclusion (Fig. 1B) and nodular and brownish lesions in facial (Fig. 1C) and palmar region (Fig. 1D). Radiographically, well-defined radiolucent images were seen in region of teeth 41 to 34, in retromolar region involving tooth 48 and in right ...
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... 20-year-old female patient, melanoderma, was referred by her orthodontist to the Oral and Maxillofacial Surgery service, for presenting a cyst in anterior mandible region. The clinical examination (Fig. 1A) revealed a slight asymmetry of mandibular right body, asymptomatic, teeth 32 and 33 in infraocclusion (Fig. 1B) and nodular and brownish lesions in facial (Fig. 1C) and palmar region (Fig. 1D). Radiographically, well-defined radiolucent images were seen in region of teeth 41 to 34, in retromolar region involving tooth 48 and in right mandibular branch ( Fig. 2A). In addition, presence of tooth 13 included. A Cone Beam computed tomography was ...
Context 3
... patient, melanoderma, was referred by her orthodontist to the Oral and Maxillofacial Surgery service, for presenting a cyst in anterior mandible region. The clinical examination (Fig. 1A) revealed a slight asymmetry of mandibular right body, asymptomatic, teeth 32 and 33 in infraocclusion (Fig. 1B) and nodular and brownish lesions in facial (Fig. 1C) and palmar region (Fig. 1D). Radiographically, well-defined radiolucent images were seen in region of teeth 41 to 34, in retromolar region involving tooth 48 and in right mandibular branch ( Fig. 2A). In addition, presence of tooth 13 included. A Cone Beam computed tomography was performed for better evaluation, and a hypodense lesion ...
Context 4
... was referred by her orthodontist to the Oral and Maxillofacial Surgery service, for presenting a cyst in anterior mandible region. The clinical examination (Fig. 1A) revealed a slight asymmetry of mandibular right body, asymptomatic, teeth 32 and 33 in infraocclusion (Fig. 1B) and nodular and brownish lesions in facial (Fig. 1C) and palmar region (Fig. 1D). Radiographically, well-defined radiolucent images were seen in region of teeth 41 to 34, in retromolar region involving tooth 48 and in right mandibular branch ( Fig. 2A). In addition, presence of tooth 13 included. A Cone Beam computed tomography was performed for better evaluation, and a hypodense lesion in lingual region of tooth ...
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Aims:
The detection of odontogenic keratocysts (OKC) in the oral cavity is one of the main criteria for the clinical manifestation of Gorlin-Goltz syndrome (Nevoid Basal Cell Carcinoma Syndrome - NBCCS). From a clinical point of view, we distinguish between "syndromic" and "sporadic" OKC. Syndromic cysts, often multifocal, may be an accidental fin...
Citations
... En el presente caso se presenta un queratoquiste odontogénico en un paciente masculino joven, coincidiendo con los reportes de la literatura donde indican que la lesión es más frecuente en el sexo masculino, presentándose en un 60% de la población general. Sin embargo, se ha demostrado que cuando los queratoquistes están asociados al síndrome de Gorlin Goltz su relación es 1:1, es decir que hay igual afección tanto para hombres como para mujeres, tomando en cuenta que el paciente puede presentar otras alteraciones características del síndrome como carcinomas basocelulares múltiples, depresiones dígito-palmares, calcificación de la hoz del cerebro, costillas bífidas y mutación del gen supresor de tumores Patched 1 (Regezi, et al., 2012;Bachesk, et al., 2021). ...
... A pesar de tratarse de una lesión benigna, es caracterizada por su comportamiento biológico agresivo, generalmente se localiza en el cuerpo y rama mandíbula, esta ubicación es la más frecuente reportada dentro de la literatura, sin embargo, puede presentarse también en el maxilar superior y generalmente es asociada con la presencia de órganos dentales retenidos. Cabe mencionar que los queratoquistes odontogénicos hacen parte de las características principales que conforman el Síndrome de Gorlin Goltz, que, a pesar de presentar la misma histología, en este trastorno suelen presentarse de forma múltiples y son más agresivos que los queratoquistes odontogénicos no sindrómicos (Regezi, et al., 2012;Moctezuma, et al., 2020;Bachesk, et al., 2021). ...
... Aunque técnicamente el tratamiento de marsupialización y descompresión son distintos al ser autoinducidos y realizados mediante un dispositivo respectivamente, ambos tienen como objetivo comunicar la luz de quiste con el exterior para reducir su presión, inducir un cambio en el epitelio del quiste y disminuir de esta manera el tamaño de la lesión. En el presente caso se utilizó el fragmento de una sonda vesical, pero autores como Moctezuma sugieren el uso de tubo de Penrose suturado a la mucosa y Bachesk recomienda un dispositivo estabilizado con un alambre de acero, también se ha reportado el uso de catéteres intravenosos o de alimentación pediátrica; todos ellos han mostrado resultados favorables para la descompresión (Pogrel, 2013;Moctezuma, et al., 2020;Bachesk, et al., 2021). ...
Keratocyst is a benign odontogenic lesion with aggressive behavior, probably derived from the dental lamina. It is frequently located in the posterior part of the mandibular bone in the area of the third molar, mandibular angle and can progress towards the ramus and the body, presenting a direct association with retained dental organs. There is a wide variety of techniques for the treatment of this lesion, such as decompression, marsupialization, enucleation, and en bloc resection, as well as the combination of these with adjuvant methods. The interest in this lesion stems from its high recurrence rate, which is estimated to be 20-30% in the general population, however, at present the use of conservative treatments such as marsupialization and decompression has been chosen. demonstrated greater effectiveness and less recurrence. This is why after treating the lesions it is important to give a long-term follow-up. The objective of the publication is to present the report of a clinical case of a 21-year-old male patient with a diagnosis of odontogenic keratocyst treated with a decompression technique for five months for subsequent surgical enucleation. It has been proven that decompression treatment followed by enucleation and accompanied by adjuvant methods is an adequate therapeutic management for keratocysts as it demonstrates its lower rate of recurrence and its noble behavior with neighboring vital structures. However, in all cases, regular monitoring should be carried out to prevent recurrence of the lesion.
... Such decompression treatment results in thickening of the cyst lining, allowing easier removal with a lower recurrence rate. (12,21,(48)(49)(50)(51) When OKC is small and easy to access, enucleation is chosen with two adjuvant management protocols. (37,52,53) Recurrence rates with different treatment modalities are mentioned below: (11,54) Decompression is "a modified marsupialization technique that causes the cyst to decrease significantly in size, and the cystic lining becomes thicker, resembling oral mucosa that allows for easier enucleation. ...
Article history:The odontogenic keratocysts are developmental cysts of the jaws that require proper
diagnosis due to their potential for local aggressive growth, recurrences, and association with
an inherited syndrome. The renaming of odontogenic keratocysts as keratocystic odontogenic
tumors in 2005 then, after 12 years, denomination as an odontogenic cystic lesion, by the
WHO in 2017, has been one of the most debatable changes in the terminology of odontogenic
lesions in recent years. This article reviews the etiopathogenesis, clinical, radiographic,
histopathological, immunohistochemical and treatment; with the aim of describing the
importance of this lesion.
