Pediatric Dentistry · patient case question
Which additional finding would you expect in this patient?
Patient case: A father who says his son has no teeth
demo: 6-year-old male, accompanied by his father
chief: The father is worried that his son still has almost no teeth.
history: Born at term. The father reports the boy overheats badly in summer and has to be cooled with wet towels; he rarely seems to sweat. He has never been seen by a specialist.
meds: None.
findings: Sparse fine scalp hair, prominent periorbital wrinkling with darkening around the eyes, frontal bossing, a depressed nasal bridge and everted lips. The skin is dry and scaly. Only two conical teeth are present in each arch.
Show answer and explanation
Correct answer: B. Dry eyes from deficient lacrimal secretion
- A. Bifid ribs are a feature of Gorlin-Goltz syndrome, alongside multiple odontogenic keratocysts and basal cell carcinomas.
- B. Correct. Lacrimal glands are ectodermal derivatives, so they are hypoplastic in this condition. Reduced tear production causes dry eyes and photophobia. Nail dystrophy, absent or hypoplastic salivary and mucous glands, and a hoarse voice from dry respiratory mucosa follow the same logic.
- C. Palmoplantar hyperkeratosis belongs to Papillon-Lefevre syndrome. This distractor has appeared as a miskeyed answer in earlier files in the series, so it is worth fixing in memory.
- D. The adrenal cortex arises from intermediate mesoderm, not ectoderm, so adrenal function is unaffected. This is the reasoning behind the 'all except' version of this question.
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