Early diagnosis Gorlin-Goltz syndrome: A Rare Case Report in Surgery & Case Studies: Open Access Journal in Lupine Publishers
Gorlin-Goltz syndrome, is an uncommon, autosomal dominant inherited
disorder, which is characterized by numerous basal cell
carcinoma (known as nevoid basal cell carcinoma syndrome (NBCCS)). The
common manifestations include multiple odontogenic
keratocysts in the jaws are usually one of the first manifestations of
the syndrome, macrocephaly, frontal boosing, multiple basal cell
nevi on the skin with an early age onset, palmar pits, bifid or splayed
ribs, high arched palate, calcified diaphragm sellae, calcifications
of the falx cerebri. Although all the manifestations may not be present
in all the patients. We reported here a child patient, a 8-year-old
child boy visited maxillofacial department in Rizgary teaching hospital
for right premaxillary swelling emphasizing its clinical and
radiographic manifestations of Gorlin -Goltz syndrome with no family
history, the patient has multiple odontogenic keratocysts, rib
anomalies, calcifications of the falx cerebri, frontal and temporal
boosing, macrocephaly and thick eye brows. This study highlights
the importance of maxillofacial surgeon in the early diagnosis,
treatment and closes follow up is important to prevent long-term
complication including malignancy and oromaxillofacial deformation and
destruction.
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