Medical ConditionsBehavior & neurodevelopment: Cri du chat syndrome
Medical ConditionsBehavior & neurodevelopment: Cri du chat syndrome
Cri du chat syndrome
Overview
Board takeaway: Assess communication, dysphagia, cardiac disease, and laryngeal anatomy before selecting dental behavior guidance or anesthesia.
What causes Cri du chat syndrome?
A deletion of the short arm of chromosome 5 causes this syndrome. Most deletions arise sporadically; some result from a parental chromosomal rearrangement. The characteristic kitten-like cry reflects laryngeal and neurologic differences during infancy.
Which systemic and craniofacial findings occur in Cri du chat syndrome?
- Growth and development: low birth weight, microcephaly, developmental disability, and marked speech impairment.
- Feeding and movement: hypotonia, dysphagia, reflux, and later changes in muscle tone.
- Other systems: congenital heart defects, recurrent respiratory or ear infections, hearing impairment, and scoliosis may occur.
- Face and jaws: a broad nasal bridge, widely spaced eyes, low-set ears, micrognathia, and a face that becomes longer with age; clefting is possible.
What oral findings are reported in Cri du chat syndrome?
A high or otherwise abnormal palate, bifid uvula or cleft palate, mandibular retrusion, open bite, and Class II malocclusion may occur. The guide reports delayed eruption, enamel defects, and reflux-related erosion. Caries and periodontal risk depend on hygiene access and exposures rather than the chromosome deletion alone.
How should dental care be adapted for Cri du chat syndrome?
Use the child's established communication methods and caregiver knowledge of distress. Adapt visits for sensory and behavioral needs, and provide caregiver-supported prevention. Assess dysphagia and reflux before positioning and isolation. If sedation or general anesthesia is needed, review laryngeal abnormalities, micrognathia, respiratory status, and cardiac disease with anesthesia. Assess antibiotic prophylaxis from the actual cardiac lesion and repair status.
What treatment and prognosis should be understood for Cri du chat syndrome?
There is no treatment that replaces the missing chromosome material. Early communication, educational, physical, and occupational support helps function; medical care addresses feeding and associated organ disease. Survival into adulthood is common after early medical problems are addressed, although lifelong assistance may be required.