Few syndromes in which Multiple OKCs can occur as a component are Orofacial

digital syndrome, Ehler-Danlos syndrome, Noonan syndrome,

Simpson-Golabi-Behmel syndrome.

Thank you Dr Adhikari.

can you nominate a reference for me?

Probably we are talking about a Gorlin-Goltz syndrome. How old is this patient? Did you search for other skeletal chances? I did not hear or read something else describing multiple keratocysts in another population. Maybe these multiple lesions at the jawbones could be the first sign for this syndrome. 

The patient is 8 years old male, with a KOT in one side of the mandible, another in the maxilla and an odontogenic myxoma in the other side of the mandible.  No other skeletal findings.

Interestingly, his mother and brother presented also with multiple keratocysts. His mother is 37 years old. She didn't show features of Gorlin-Goltz syndrome neither.

May be it is Gorlin-Goltz syndrome with partial expression, as there are several published case reports suggesting the same.

Dear Omyama;

To the best of my knowledge multiple odontohenic keratocysts is reported in association with the Gorlin-Goltz syndrome. I am not aware of any syndromic conditions involve multiple odontogenic keratocysts. So your patient will either have no syndrome or partial or mild expression of clinical signs and symptoms of the condition. It is also possible that the patient develops syndrome signs and symptoms later as he/she grows older. Therefore, it is recommended to continue follow up the patient and monitor any additional clinical expressions. Best regards!

Dear Omayma, 

Since family history is positive; the case is likely to be Gorlin Goltz Syndrome with partial expression due to incomplete penetrance. Follow up is required, and you might also look for microdeletion at 9q22.3 (PCTH1 gene) to be sure about Gorlin Goltz

 Bhoj Raj and Agnieszka mentioned syndromes apart from that orofacial digital syndrome also associated with odotogenic keratocyst.

Dear all

I thank you for your contributions. I found reports in the literature where multiple keratocysts occurred in Noonan and Ehler-Danlos syndromes. As soon as I access the full articles, I will share them with you.

Dear Nirnala

Can you send us the article of Bhoj Raj and Agnieszka?

Thank you all.

Dear Omayma,

I have published an article regarding Conservative Management of Multiple Odontogentic Keratocyst in a Young Patient with 2 Years Follow Up - A case report

follow the citation: Nirmala SVSG,Sandeep C, Sindhuri V,Vimala Devi P. Conservative Management of Multiple Odontogentic Keratocyst in a Young Patient with 2 Years Follow Up. J Dent App. 2015;2(2): 149-152.