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Case Study. Patient: Pam Halpert Age: 24 years of age Gender: Female Height: 5’7” Weight: 150 lbs. Vital Signs: HR: 70 bpm Respiratory rate: 15 rpm Blood.

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Presentation on theme: "Case Study. Patient: Pam Halpert Age: 24 years of age Gender: Female Height: 5’7” Weight: 150 lbs. Vital Signs: HR: 70 bpm Respiratory rate: 15 rpm Blood."— Presentation transcript:

1 Case Study

2 Patient: Pam Halpert Age: 24 years of age Gender: Female Height: 5’7” Weight: 150 lbs. Vital Signs: HR: 70 bpm Respiratory rate: 15 rpm Blood Pressure:130/70 mmHg Temperature: 98.9 degrees F Chief complaint Pain in R. Mn. Molar region Medical Alert Name of Syndrome: Gardner Syndrome Cause of Syndrome - Medical History (if applicable) Gardner syndrome, a variant of familial adenomatous polyposis (FAP), is an autosomal dominant disease characterized by GI polyps, multiple osteomas, and skin and soft tissue tumors. Cutaneous findings of Gardner syndrome include epidermoid cysts, desmoid tumors, and other benign tumors. Polyps have a 100% risk of undergoing malignant transformation; consequently, early identification of Gardner syndrome is critical.familial adenomatous polyposis (FAP)

3 Orofacial Clinical Features Gardner’s syndrome has prominent orofacial features characterized by hyperdontia, multiple impacted supernumerary teeth, compound odontomas, prominent tori, and jaw osteomas. Because the disease has such variable expressivity, affected patients may have one or more of the foregoing findings. Age/race/sex predilections and Systemic Clinical Features: Epidermoid cysts of Gardner syndrome occur at an earlier age (around puberty) than ordinary cysts and in less common locations, such as the face, the scalp, and the extremities. Gardner syndrome cysts tend to be multiple and are present in the multiple form in 50-65% of patients. Similar to ordinary epidermal inclusion cysts, cysts in Gardner syndrome are usually asymptomatic; however, they may be pruritic and/or inflamed. Other skin signs in Gardner syndrome include: Fibromas, Lipomas, Leiomyomas, Neurofibromas, Pigmented skin lesions Desmoid tumors occur as swelling in the anterior abdominal wall and are often preceded by surgical trauma. Osteomas are required to make the diagnosis of Gardner syndrome. The mandible is the most common location. Dental abnormalities (i.e. unerupted teeth, supernumerary teeth) may occur. Radiographic Features of this Syndrome Maxillofacial radiographs often reveal several round osteomas of varying size. In some patients, the osteomas may be large and interfere with jaw function, whereas other patients develop multiple small diffuse enostoses that impart a cotton-wool appearance to the jaws.

4 Special considerations in Treatment of this patient? How is it Diagnosed? How common is it? Is it a horse or zebra? Treatment: Depends on the symptomatic or cosmetic nature and the location of the cysts. Treatment is similar to that used for ordinary cysts and involves excision or use of intralesional steroids if the cysts are inflamed. Colectomy is recommended if 30 or more polyps are detected on colonoscopy or if biopsy reveal dysplasia or malignant degeneration. Cutaneous findings do not require treatment. Diagnosis: Diagnosis of Gardner’s syndrome is made by demonstrating the classic triad of adenomatous intestinal polyposis, hard tissue tumors, and soft tissue tumors Gastrointestinal findings are confirmed by double-contrast barium enema (air and barium introduced), fiberoptic colonoscopic examination and biopsy. **The reported incidence of familial adenomatous polyposis (FAP) varies from 1 in 7,000 to 1 in 22,000 individuals. **Estimated 1 person per million population is diagnosed with Gardner’s**

5 Pictures of Gardner’s Syndrome

6 Intraoral Pictures


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