What is the incidence of frontal sinus osteoma in patients with Gardner syndrome?

From the Guidelines

The incidence of frontal sinus osteoma in patients with Gardner syndrome is not explicitly stated in the provided evidence, but based on the information available, it can be inferred that osteomas, including those in the frontal sinus, are a common feature of Gardner syndrome. Gardner syndrome is a variant of familial adenomatous polyposis (FAP) characterized by extracolonic manifestations such as osteomas, skin cysts, congenital hypertrophy of the retinal pigmented epithelium, and desmoid tumors 1. While the exact incidence of frontal sinus osteoma in Gardner syndrome is not provided, the presence of osteomas, particularly in the skull and facial bones, is a well-documented aspect of the syndrome.

The genetic basis of Gardner syndrome, involving mutations in the APC gene on chromosome 5q21, leads to abnormal bone formation and the development of osteomas alongside other manifestations of the syndrome 1. Although the provided study does not specifically focus on the incidence of frontal sinus osteomas, it highlights the importance of considering Gardner syndrome when extracolonic manifestations such as osteomas are identified, especially in the context of familial adenomatous polyposis.

Key points to consider in the context of Gardner syndrome and frontal sinus osteomas include:

• The syndrome's genetic basis and its role in the development of osteomas
• The common locations of osteomas in Gardner syndrome, which include the skull and facial bones
• The potential for osteomas to be asymptomatic or to cause symptoms depending on their size and location
• The importance of early diagnosis of Gardner syndrome for the management of its potentially life-threatening intestinal manifestations.

From the Research

Incidence of Frontal Sinus Osteoma in Gardner Syndrome

• The incidence of frontal sinus osteoma in patients with Gardner syndrome is not explicitly stated in the provided studies, but it is known that osteomas are a common feature of Gardner syndrome 2, 3.
• Gardner syndrome is an autosomal dominant disease characterized by intestinal polyposis, osteomas, and cutaneous and soft tissue tumors, and the risk of developing colon cancer approaches 100% in affected individuals 3.
• Osteomas are common benign tumors of the paranasal sinuses, with the frontal sinus being a frequent location, and they may be locally destructive and aggressive with possible intracranial complications 4, 5.
• The study by Childrey in 1939 found an incidence of 0.43% of paranasal sinus osteomas in 3,510 consecutive sinus radiographs 5.
• However, there is no specific data available on the incidence of frontal sinus osteoma in patients with Gardner syndrome, and further research is needed to determine this incidence.

Clinical Manifestations and Complications

• Clinical manifestations of frontal sinus osteoma include headache, facial pain, rhinorrhea, and sinusitis 4, 3.
• Complications of frontal sinus osteoma can be life-threatening, including intracranial complications such as subdural empyema and pneumocephalus 4, 6.
• The management of frontal sinus osteoma depends on the size and location of the tumor, and surgical approaches such as craniofacial approach and osteoplastic frontal sinusectomy may be used 5, 6.

Association with Gardner Syndrome

• Osteomas are a key feature of Gardner syndrome, and patients with paranasal sinus osteomas should be evaluated for Gardner syndrome 2, 3.
• The presence of gastrointestinal symptoms and a positive family history of polyposis can indicate Gardner syndrome in patients with paranasal sinus osteomas 3.
• Otolaryngologists should be aware of the possibility of Gardner syndrome in patients with paranasal sinus osteomas and perform a complete workup, including lower gastrointestinal tract endoscopy, barium enema imaging, and DNA testing 3.