TABLE II.
Clinical Findings of CdLS by System and Recommended Interventions in Adulthood
Childhood involvement | Adult involvement | Adult management |
---|---|---|
Congenital heart disease | Typically stable | Echocardiogram if never had |
Primary teeth tend to be retained | Panorex X-ray can be taken | |
Secondary teeth eruption delayed Sinusitis and/or nasal polyps may produce symptoms |
Lingual caries may develop with GERD Sinusitis and/or nasal polyps may produce symptoms |
(Pediatric) dental visits every 4–6 months. ENT visits as needed; nasal polypectomy may be helpful |
Cleft palate—should be repaired Pyloric stenosis requiring surgery Gastroesophageal reflux disease Complications of GERD Malrotation may be present |
Cleft palate stable—may need repair Constipation may persist GERD may worsen Barrett esophagus may occur Risk for volvulus, intestinal perforation |
Craniofacial team visits as needed Diet, medications as needed Regular gastrointestinal follow-up Biopsies every 1–3 years after diagnosis All patients need upper GI series and warning about presenting signs of volvulus (e.g. bilious vomiting) |
Renal malformation, vesiculo-ureteral reflux (VUR) |
Renal malformation, VUR | Renal ultrasound on all patients, as indicated clinically |
Cryptorchidism in males | Cryptorchidism in males | Orchiopexy and/or orchiectomy in childhood, monitor hormones later |
Slightly delayed puberty females | Irregular or no menses | Hormonal treatment as needed; routine gynecology care with Pap smears every 3 years |
Lacrimal duct malformations | Retinal detachment can occur with severe myopia |
Regular ophthalmology visits, surgery as needed |
Blepharitis Hip dislocations |
Blepharitis tends to improve Leg length discrepancy, scoliosis, bunions may occur |
Baby shampoo rinses Orthopaedic visits as needed |
Seizures may occur or worsen Peripheral neuropathy may produce symptoms |
Seizures may occur or worsen Peripheral neuropathy may produce symptoms |
Pediatric neurology, medications Medications may be helpful |
Behavioral issues (SIB, anxiety, aggression) may worsen |
Behavioral issues (SIB, anxiety, aggression) may worsen |
Psychiatrist or psychologist intervention may be helpful |