September 2019 HSC Section 1 Congenital and Pediatric Problems

Yuhan et al

Odontogenic benign masses In comparison to cysts, odontogenic tumors represent approximately 20% of all odon- togenic lesions. 38 Although most of these tumors are incidentally found on radio- graphic imaging, rapid expansion may lead to displacement of teeth and facial asymmetry. These more aggressive benign tumors may require radical ablative sur- gery with reconstruction. 46 Ameloblastomas are tumors of epithelial origin and represent the second most aggressive benign odontogenic tumors in the children at 20.8%. 38 Unicystic masses are more common in the pediatric age group. 47 Painless jaw swelling and slow expansion of the jaw are characteristic symptoms. Pathologic diagnosis occurs postenucleation because most unicystic ameloblastomas are often confused initially with dentigerous cysts. 48 Radiographic evidence of irregular border and expansion may indicate the presence of an underlying malignant etiology, specifically amelo- blastic sarcoma. Clinicians should be wary of under-treatment that may lead to multiple recurrences of the tumor—the most appropriate treatment is determined by a multidisciplinary team working with a patient’s pediatric ear, nose, and throat physician. Keratocystic odontogenic tumors (KCOTs) were historically called odontogenic ker- atocysts in an attempt to describe a benign but locally destructive and recurrent odon- togenic cyst. Recurrence is attributed to the existence of daughter cysts found between the cystic lining and oral mucosa. 46 They classically present as a syndromic manifestation due to mutations in the patched gene. 49 Therefore, a child with multiple or recurrent KCOTs should be evaluated for associated genetic syndromes; specif- ically, nevoid basal cell carcinoma syndrome, also known as Gorlin-Goltz syndrome, involves multiple basal cell carcinomas, keratocysts of the jaw, and bifid ribs. 50 Definitive diagnosis of KCOT is made after histologic confirmation obtained after enucleation or curettage. No general consensus has been made regarding the man- agement of KCOT. The goal of treatment, however, should be to initially remove the body of the tumor with subsequent serial surgeries for the removal of residual daughter cells. 51 Simple enucleation alone is not acceptable due to the high rate of recurrence associated with KCOT. 46 Odontomas are benign tumors of mixed epithelial and mesenchymal origin. They are hamartomatous malformations as opposed to true neoplasms. 52 Composed of dentin and enamel, they are slow growing and are often incidentally diagnosed in the first 2 decades of life by panoramic radiographs. 47 Early detection significantly in- creases successful preservation of the impacted tooth with simple enucleation and curettage. 46 Odontogenic abscesses should be considered in the differential diagnosis of pedi- atric oral lesions. A majority of dental abscesses are restricted to the gingiva around the infected tooth, presenting as an acute-onset localized swelling that is exquisitely tender to palpation. In general, these limited abscesses can be treated with an in- office incision and drainage, although management may require general anesthesia and/or sedation in younger or less cooperative patients. Although anaerobic bacteria are a common culprit, many cases are polymicrobial in origin, 53–55 and familiarity with local resistance patterns is critical in determining whether aminopenicillins, metroni- dazole, or other antibiotics are sufficient while awaiting culture sensitivities. Far more worrying are the significant potential consequences of an untreated infection spreading into various oral cavity compartments and neck spaces, including sub- mandibular and sublingual abscesses causing floor of mouth swelling (Ludwig angina). Infections in this area can rapidly result in airway obstruction, necessitating emergent intubation and even the need for tracheostomy. 56 Hence, close

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