Child with complete anodontia and dry skin

Correct answer: A. Ectodermal dysplasia

• A. Correct. Hypohidrotic ectodermal dysplasia is defined by defects in ectodermally derived structures: anodontia/hypodontia with conical teeth, HYPOHIDROSIS (reduced sweating causing HEAT INTOLERANCE), fine sparse hair, and dry skin with periorbital/perioral wrinkling and hyperpigmentation. The absence of a family history does not exclude it — the X-linked form can arise from a new mutation, and carrier mothers may be only mildly affected.
• B. Cleidocranial dysplasia features SUPERNUMERARY teeth, retained primary teeth, delayed eruption, absent or hypoplastic clavicles and open fontanelles — not anodontia with heat intolerance.
• C. Gorlin-Goltz (nevoid basal cell carcinoma syndrome) features multiple odontogenic keratocysts, basal cell carcinomas, bifid ribs and calcified falx cerebri.
• D. Papillon-Lefèvre features palmoplantar hyperkeratosis with severe early-onset periodontitis and premature loss of both dentitions — the teeth erupt normally first.

Correct answer: C. Dry eyes

• A. Bifid ribs belong to Gorlin-Goltz syndrome.
• B. Microcephaly is not a feature; the frontal bossing and depressed nasal bridge give a characteristic facies but head size is normal.
• C. Correct. Because lacrimal and other exocrine glands are also ectodermal derivatives, they are hypoplastic — producing DRY EYES (and dry mouth, dry nasal mucosa) alongside the reduced sweating.
• D. Palmoplantar keratosis belongs to Papillon-Lefèvre syndrome.

Correct answer: D. Refer to a pediatric dentist and a multidisciplinary team

• A. Implants are generally deferred until skeletal growth is complete because an osseointegrated implant behaves like an ankylosed tooth and will become infrapositioned as the jaws grow. The anterior mandible is the one region sometimes considered earlier in carefully selected cases.
• B. Deferring all treatment neglects function, nutrition, speech development and the psychosocial impact of anodontia in childhood.
• C. Definitive fixed prostheses are inappropriate in a growing child for the same reason.
• D. Correct. Management is MULTIDISCIPLINARY and staged — pediatric dentistry, prosthodontics, orthodontics, oral surgery, and often a geneticist and dermatologist. Children are typically restored with removable overdentures beginning around age 3-5 to support function, speech, appearance and psychosocial development, and these are remade as the child grows.