Dental, dermatological and radiographic findings in a case of Gorlin-Goltz Syndrome: report and review

Kumar, Nilesh; Tewary, Shivsagar; Zope, Sameer; Patel, J.G.; Vande, Aaditee

doi:10.11604/pamj.2017.27.96.12025

Cited by 11 publications

(19 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…There were 38 studies included in this review. This comprises 19 case series, 9,17,18,21–35 12 case reports 36–47 and seven case‐control studies 6,16,48–51 . Sample sizes ranged from 1 to 78 cases.…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Sella turcica morphology in patients with genetic syndromes: A systematic review

Roomaney

2020

Orthod Craniofacial Res

View full text Add to dashboard Cite

The sella turcica is an important anatomical reference used in orthodontics for the evaluation of craniofacial growth. Studies have found variations in the sella turcica morphology in patients with syndromes affecting the craniofacial complex. This review aims to determine whether genetic syndromes involving the craniofacial complex are associated with abnormal radiographic sella turcica morphology and whether there is a pattern of malformation which is consistent within each syndrome. An electronic database search was conducted to identify relevant studies. We included primary studies describing the morphology of the sella turcica on lateral radiographs in human subjects with genetic syndromes involving the craniofacial complex. No restrictions were placed on language or timeframe. PROSPERO registration CRD42019148060. Thirty‐eight studies were included in this review. A ‘J’‐shaped sella was found in patients with Hutchinson‐Gilford‐Progeria syndrome and other syndromes. A bulbous dorsum sellae was highly prevalent Cleidocranial dysplasia, and a bulbous dorsum sellae and uneven contours of the clivus was found in Cri du chat syndrome. A steep clivus was described in patients with Axenfeld‐Rieger syndrome. An oblique anterior wall was the most frequent malformation found in Down’s syndrome. Genetic syndromes affecting the craniofacial complex are associated with abnormal morphology of the radiographic sella turcica. Clinicians should be observant of abnormal sella turcica morphology which can be a sign of undiagnosed or subclinical syndromes. More high‐quality studies are needed which use standardized and objective methods of determining the morphology of the sella turcica.

show abstract

Section: Resultsmentioning

confidence: 99%

“…All included studies were available in English. The dates of publication ranged from 1967 to 2020, with many (14) studies being published in the last decade 6,11,17,18,22,25,34,35,37,40,42–45,47 …”

Section: Resultsmentioning

confidence: 99%

Sella turcica morphology in patients with genetic syndromes: A systematic review

Roomaney

2020

Orthod Craniofacial Res

View full text Add to dashboard Cite

show abstract

“…BCCs may also appear on the trunk and limbs [ 9 ]. Another feature of GGS is the occurrence of multiple KCOTs of the jaw [ 11 ]. The occurrence of GGS-associated KCOTs is approximately a decade earlier than that of KCOTs not associated with the syndrome [ 12 ]; moreover, KCOTs are considered to be among the most frequent and consistent features of GGS.…”

Section: Discussionmentioning

confidence: 99%

Gorlin-Goltz Syndrome: A Case Report and Literature Review

Al-Jarboua¹,

Al-Husayni²,

Al-Mgran³

et al. 2019

Cureus

View full text Add to dashboard Cite

Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant inherited disorder characterized by the presence of multiple keratocystic odontogenic tumors (KCOT) in the jaws, multiple basal cell nevi carcinomas, and skeletal abnormalities. Early diagnosis of Gorlin-Goltz syndrome is essential as it may progress to aggressive basal cell carcinomas and neoplasias. Gorlin-Goltz syndrome has rarely been reported in Saudi Arabia. This article reports a case of a 13-year-old Saudi female patient with Gorlin-Goltz syndrome and includes an extensive literature review of the syndrome. To the extent of our knowledge, this is the first case reported by dentists in the Kingdom of Saudi Arabia.

show abstract

“…Multiple basal cell carcinomas are considered one of the most common characteristics of GGS, especially in the head and neck region. 10 Another feature of GGS is the occurrence of multiple KCOTs of the jaw. 11 The occurrence of GGS-associated KCOTs is approximately a decade earlier than that of KCOTs not associated with the syndrome.…”

Section: The Minor Criteria Arementioning

confidence: 99%

Gorlin Goltz syndrome: a rare case report

et al. 2020

View full text Add to dashboard Cite

Gorlin-Goltz syndrome is uncommon multisystemic disease with an autosomal dominant trait, with complete penetrance and variable expressivity, though sporadic cases have been described. We report a case of 18 years old male patient having features of Gorlin Goltz syndrome. Gorlin-Goltz syndrome is characterized by multiple basal cell nevi or carcinomas, odontogenic keratocysts, palmar and/or plantar pits, calcification of the falx cerebri, and is associated with internal malignancies. It is important to know the major and minor criteria for the diagnosis and early preventive treatment of this syndrome.

show abstract

Dental, dermatological and radiographic findings in a case of Gorlin-Goltz Syndrome: report and review

Cited by 11 publications

References 6 publications

Sella turcica morphology in patients with genetic syndromes: A systematic review

Sella turcica morphology in patients with genetic syndromes: A systematic review

Gorlin-Goltz Syndrome: A Case Report and Literature Review

Gorlin Goltz syndrome: a rare case report

Contact Info

Product

Resources

About