September 2019 HSC Section 1 Congenital and Pediatric Problems

Yuhan et al

Papillomatous lesions of the oral mucosa may be the first signs of Cowden syn- drome, a rare autosomal dominant disease. These lesions often appear by the second or third decade. 70 In the case of functional impairment or pain, local excision of the oral lesions may be done with an electric loop. A thorough history and physical examina- tion for early detection are indicated to rule out malignancy every 6 months. Mucosal neuromas are associated with multiple endocrine neoplasia type 2B, which also presents with medullary thyroid carcinoma, pheochromocytoma, and marfanoid habitus. 70 Affected patients usually present early in childhood with a thyroid mass or vague gastrointestinal symptoms. These masses may be confused with plexiform neurofibromas and underlying neurofibromatosis. To avoid misdiagnosis, immunocy- tochemistry highlights differing pattern of growth and may be useful in delineating the etiology of these nodules. 71 Children with vitamin D–resistant rickets may develop spontaneous gingival ab- scesses, or parulis, in the gingiva. Defects in tooth formation allow invasion by bac- teria into the gingiva and subsequent necrosis. 30 These gum boils are fluctuant swellings containing pus. 30 Treatment involves root canal therapy or tooth extraction. Antibiotics are reserved for immunocompromised patients and include penicillins or clindamycin. 25 Oral candidiasis, or thrush, is a yeast infection seen in milk-fed infants or in chil- dren treated with antibiotics or corticosteroids. 8 It is also the second most common AIDS-defining infection and seen in HIV-positive infants and in children treated with chemotherapy or post–bone marrow transplant. 72 Visually, the pseudomembranous form appears as erythematous macules on oral mucosa with a white cottage cheese exudate that may be scraped away. 30 For young children, swabs of oral nystatin or ketoconazole are both acceptable options for primary treatment. For children with a compromised immune system, systemic antifungal therapy is indicated. Primary herpetic gingivostomatitis is an oral manifestation of acute infection by her- pes simplex virus type 1 that presents before 5 years of age. 25 Transmission occurs via saliva or direct contact with a primary or recurrent lesion. Infants and young chil- dren often present with vesicles on the distal phalanx, also known as herpetic whitlow, due to their oral habits. 30 These painful vesicles heal spontaneously within 2 weeks. Rinsing and swallowing an acyclovir suspension (15 mg/kg) 5 times per day during the first 3 symptomatic days, however, expedite resolution and decrease pain. 25 Kawasaki disease, or mucocutaneous lymph node syndrome, is a systemic vascu- litis that affects children younger than 5 years of age. 73,74 The American Heart Asso- ciation published diagnostic criteria in 2004, 75 requiring fever for at least 5 days and at least 4 of 5 of the following symptoms: (1) changes of the oral cavity, including straw- berry tongue; (2) polymorphous rash; (3) bilateral nonpurulent conjunctivitis; (4) ery- thema and desquamation of the hands and feet; and (5) cervical lymphadenopathy. The oral mucosa and lips may be chapped, cracked, crusted, and erythematous. 73 Intravenous immunoglobulin and high-dose aspirin have been the mainstay of treatment. 75 Malignant lesions Although exceedingly uncommon, missing a malignant oral cavity lesion can have devastating consequences on functional morbidities, outcomes, and survival. Hence, the authors cannot emphasize enough the importance of pursuing appropriate work- up and obtaining a tissue diagnosis for any lesion for which there is not an obvious diagnosis. Persistent masses must be biopsied if conservative management does

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