A Rare Case of Gorlin-Goltz Syndrome in Children

Abstract

The Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome is an autosomal dominant condition disorder with high variability expression. It presents a series of relevant clinical manifestations that suggest its diagnosis in cutaneous, bone, dental, soft tissue, nervous, and ocular system disorders. This condition requires a great interaction of several specialists to improve the patient’s life. In this case, we presented a 9-year-old male patient referred to the Department of Oral and Maxillofacial Surgery reporting failure in the normal chronology of dental eruption. After evaluation, it was observed that the patient had 13 typical characteristics of the syndrome, including keratocysts, bifid ribs, palmoplantar pits, and 10 other minor characteristics. In conclusion, the expression of so many features of Gorlin-Goltz syndrome is rare in infants, and early diagnosis is important to decrease morbidity and mortality associated with basal cell carcinomas.

Oral and maxillofacial surgeon's perspective on Gorlin–Goltz syndrome - A report of two cases

“…None of our patients had BCCS which was in concordance with Ahn et al ., suggesting the incidence of BCC low in Asia. [ 9 ]…”

Common Odontogenic Cysts in Pediatric Patients: Traditional Review

“…Diğer bulguları arasında; frontal çıkıntı, palmar/plantar çukurlaşma, sindaktili, polidaktili, desmoplastik medulloblastoma, yumurtalık/kardiyak fibromlar, lenfomezenterik kistler, oküler anormallikler (hipertelorizm, şaşılık, konjenital katarakt), radyolojik bulgular (bifid kaburga, falks serebri kalsifikasyonu, kifoskolyoz, vertebral anormallikler, kısalmış 4. metakarpal), makrosefali, dudak damak yarığı, maloklüzyon, bifid uvula ve ektopik diş pozisyonları olabilmektedir. 4,33,42 İntramural epitel artıkları ve uydu kistleri ilişkili olarak daha sık görülebilmektedir. 1…”

Radiological evaluation of odontogenic keratocysts in patients with nevoid basal cell carcinoma syndrome: A review

“…In some cases, carcinomas will occur following the occurrence of OKs. According to the literature, the frequency of NBCCS has been reported to be 1 in 50,000 to 150,000 in general population ( Khodaverdi et al, 2018 , Kumar et al, 2018 , Santander et al, 2018 , Al-Jarboua et al, 2019 , Bartos et al, 2019 , Boos Lima et al, 2019 , Moramarco et al, 2019 , Nilius et al, 2019 , Sahu et al, 2019 , Cesinaro et al, 2020 , Lata and Kaur, 2020 , Silva et al, 2020 , Gao et al, 2021 , Rafiq et al, 2021 , Singh and Mishra, 2021 , de Lima et al, 2022 , Katayama et al, 2022 , Pazdera et al, 2022 , Pitak-Arnnop et al, 2022 , Rao and Taksande, 2022 , Reaz et al, 2022 , Spadari et al, 2022 ).…”