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Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome.

Manjima S, Naik Z, Keluskar V, Bagewadi A - Contemp Clin Dent (2015)

Bottom Line: Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome.Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement.Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, KLE VKIDS, Belgaum, Karnataka Belgaum, India.

ABSTRACT
Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

No MeSH data available.


Related in: MedlinePlus

Extraoral examination revealing facial asymmetry and eyes showing hypertelorism and strabismus
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Figure 1: Extraoral examination revealing facial asymmetry and eyes showing hypertelorism and strabismus

Mentions: A 16-year-old male patient reported to the Department of Oral Medicine and Radiology, KLE VK Institute of Dental Sciences, Belgaum with a chief complaint of a swelling in the Left back region of the jaw since 15 days. The history revealed that the swelling was of sudden onset and was not associated with pain or any kind of discharge. Patient's medical, family, dental, and personal histories were noncontributory. On general physical examination, the patient was moderately built and nourished, presented with normal gait and kyphosis. Extraoral examination revealed facial asymmetry, mandibular prognathism, hypertelorism and strabismus with left eye [Figure 1]. On intraoral examination, a swelling measuring 3 cm ×1 cm in size was present obliterating the buccal vestibule from 34 to 36 region with missing 33. [Figure 2] and 33 was missing. On palpation, swelling was firm in consistency; nontender with expansion of buccal cortical plate extending from 34 to 36 region. Based on the history and clinical findings, we arrived at a provisional diagnosis of dentigerous cyst and odontogenic keratocyst was considered as a differential diagnosis. Panoramic radiograph showed four multilocular radiolucencies and one unilocular radiolucency with scalloped margins. Multilocular radiolucencies involved the right and left mandibular body region extending to the ramus and also left symphysis region. Unilocular radiolucency was seen in the left maxillary tuberosity area [Figure 3]. Fine-needle aspiration cytology of the swelling was performed using 24 gauge needle and the aspirate was creamy white in color, and the protein estimate was 3.3 g/100 ml. Considering the possibility of the Gorlin-Goltz syndrome, further evaluation was done with chest radiographs, computed tomography (CT) scan and lateral cephalogram. Chest radiograph revealed bifid ribs in relation to right 2nd, 3rd and 4th, and left 3rd and 4th ribs [Figure 4]. CT scan showed bilateral expansile lytic lesion with mandible [Figure 5], and lateral cephalogram showed bridging of sella turcica [Figure 6].


Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome.

Manjima S, Naik Z, Keluskar V, Bagewadi A - Contemp Clin Dent (2015)

Extraoral examination revealing facial asymmetry and eyes showing hypertelorism and strabismus
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4374303&req=5

Figure 1: Extraoral examination revealing facial asymmetry and eyes showing hypertelorism and strabismus
Mentions: A 16-year-old male patient reported to the Department of Oral Medicine and Radiology, KLE VK Institute of Dental Sciences, Belgaum with a chief complaint of a swelling in the Left back region of the jaw since 15 days. The history revealed that the swelling was of sudden onset and was not associated with pain or any kind of discharge. Patient's medical, family, dental, and personal histories were noncontributory. On general physical examination, the patient was moderately built and nourished, presented with normal gait and kyphosis. Extraoral examination revealed facial asymmetry, mandibular prognathism, hypertelorism and strabismus with left eye [Figure 1]. On intraoral examination, a swelling measuring 3 cm ×1 cm in size was present obliterating the buccal vestibule from 34 to 36 region with missing 33. [Figure 2] and 33 was missing. On palpation, swelling was firm in consistency; nontender with expansion of buccal cortical plate extending from 34 to 36 region. Based on the history and clinical findings, we arrived at a provisional diagnosis of dentigerous cyst and odontogenic keratocyst was considered as a differential diagnosis. Panoramic radiograph showed four multilocular radiolucencies and one unilocular radiolucency with scalloped margins. Multilocular radiolucencies involved the right and left mandibular body region extending to the ramus and also left symphysis region. Unilocular radiolucency was seen in the left maxillary tuberosity area [Figure 3]. Fine-needle aspiration cytology of the swelling was performed using 24 gauge needle and the aspirate was creamy white in color, and the protein estimate was 3.3 g/100 ml. Considering the possibility of the Gorlin-Goltz syndrome, further evaluation was done with chest radiographs, computed tomography (CT) scan and lateral cephalogram. Chest radiograph revealed bifid ribs in relation to right 2nd, 3rd and 4th, and left 3rd and 4th ribs [Figure 4]. CT scan showed bilateral expansile lytic lesion with mandible [Figure 5], and lateral cephalogram showed bridging of sella turcica [Figure 6].

Bottom Line: Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome.Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement.Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, KLE VKIDS, Belgaum, Karnataka Belgaum, India.

ABSTRACT
Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

No MeSH data available.


Related in: MedlinePlus