What Is 22q11.2 Deletion Syndrome?

22q11.2 deletion syndrome is a common genetic condition often associated with cleft palate. Here is what to know.

Cleft Lip Schematic

22q11.2 deletion syndrome (also called DiGeorge syndrome or velocardiofacial syndrome) is caused by a small missing piece of chromosome 22. It is the most common genetic syndrome associated with cleft palate.

  • Key features:
  • Cleft palate (especially submucous cleft)
  • Heart defects (in about 75% of cases)
  • Immune system problems (thymus issues)
  • Low calcium levels (hypocalcemia)
  • Characteristic facial features
  • Learning difficulties (variable)
  • Speech and feeding problems
  • Hearing loss
  • Palate issues:
  • Cleft palate is present in about 70% of cases
  • Submucous cleft palate is common
  • Velopharyngeal insufficiency is very common (even without a visible cleft)
  • Hypernasal speech is a frequent symptom
  • Diagnosis:
  • Genetic test (FISH or microarray)
  • Can be detected before or after birth
  • Often diagnosed when a child has a cleft palate and other features
  • Treatment:
  • Cleft palate repair (if needed)
  • Heart surgery (if needed)
  • Speech therapy
  • Immune and calcium management
  • Educational support
  • Feeding therapy

Long-Term Outlook

Most children with 22q11.2 deletion syndrome lead full lives with appropriate support. Early intervention for speech, learning, and medical issues is essential.

Clinical Guidance

22q11.2 deletion syndrome is a common genetic condition that features cleft palate, heart defects, immune issues, and learning challenges. Children need multidisciplinary care addressing medical and developmental needs.