Velocardiofacial Syndrome Treatment & Management
- Author: M Silvana Horenstein, MD; Chief Editor: Howard S Weber, MD, FSCAI more...
Evaluation of patients with velocardiofacial syndrome (VCFS) usually occurs in an outpatient setting. Perform workup in an outpatient setting for other associated problems as needed.
In newborns, however, the diagnosis may be made in the hospital. Admit a newborn with cyanosis for heart defect workup. Newborns with or without associated heart defects may require a prolonged hospitalization to work on feeding.
If needed, use medical therapy to treat heart failure, hypocalcemia, immune deficiency, feeding problems, and inadequate growth.
In terms of neurocognitive issues associated with velocardiofacial syndrome, during infancy and preschool years, feeding problems, cleft palate, and developmental disorders occupy most of the clinical management in these patients. During school years, management shifts to cognitive, behavioral, and learning disorders. The most common cognitive finding in these patients is functioning within the low-borderline range. Related features include significant visuospatial dysfunction, diminished math attainment, and executive dysfunction. In late adolescence and adult years, psychiatric illness such as schizophrenia or bipolar disorder become more of a concern because these may develop in 10% of adults with this condition.
Newborns who require heart surgery may need transfer to a tertiary center.
Diet and activity
No special diet is required, except as indicated for cardiac disease, feeding problems, growth problems, or other abnormalities.
Usually no restrictions are needed, except as indicated for cardiac disease or other abnormalities.
At the time of surgery, the complex cardiovascular anatomy in association with depressed immunological status, pulmonary vascular reactivity, neonatal hypocalcemia, bronchomalacia and bronchospasm, laryngeal web, and tendency for airway bleeding must be considered.
Irradiated cell blood products should be used in patients susceptible to developing graft versus host disease; if the immune status of the patient is not known, irradiated cell blood products must be used.
A cleft palate should be repaired.
Pharyngeal flap surgery may be required to treat velopharyngeal incompetence (VPI) and improvement in hypernasality; in planning the surgery, consider that the internal carotid is commonly medially displaced and tortuous.
Ventilation of ear tubes may be indicated.
Consultation with the following may be indicated:
Plastic surgeon (cleft palate)
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