Frequent middle ear infections, nasal speech, and early feeding difficulties may be the first indicators that a child has a submucosal cleft palate.
A submucosal cleft palate (SMCP) results from a lack of normal fusion of the muscles within the soft palate as the baby is developing in utero. It occurs in about 1 in 1,200 children. There is no single cause of SMCP; however, current research suggests a combination of genetic and environmental factors. The most common syndrome (genetic disorder) associated with SMCP is 22q11.2 deletion syndrome.
A SMCP can include any, or all, of the following signs:
- A very wide or split (bifid) uvula
- Translucency of the tissue along the middle of the soft palate
- A notch in the back of the hard palate.
All newborns undergo an examination of the mouth at birth, but it is not uncommon for the diagnosis of SMCP to be missed. In some cases, an infant may struggle with feeding or display leakage of breastmilk or formula through the nose.
In other cases, feeding may progress without difficulty, but the child may develop chronic ear infections and effusions (fluid in the middle ear space), often requiring placement of ventilation tubes. Finally, a SMCP may be identified when a child’s speech is noted to have a nasal quality (hypernasality), either occurring on its own or following adenoidectomy.
Regardless of when or how the diagnosis is made, it is important for all patients with a confirmed or suspected SMCP to be referred to a Cleft Lip and Palate Center or Velopharyngeal Dysfunction (VPD) Program for interdisciplinary team evaluation. The team typically includes a speech-language pathologist with specialized training in cleft palate and VPD and a surgeon with expertise in cleft palate and VPD surgery. Additional team members may also assess the child, such as a dentist, otolaryngologist, psychologist, geneticist or audiologist, based on the child’s specific needs.
If the speech evaluation confirms the presence of hypernasality or nasal air emission, specialized imaging, such as nasopharyngoscopy, may be recommended to evaluate velopharyngeal (VP) closure. VP closure is the process by which the soft palate attempts to close off the oral cavity from the nasal cavity during speech.
If imaging reveals a lack of VP closure, then VPD is confirmed and this information can be helpful for surgical planning. Once the diagnosis of SMCP is made, surgery is typically recommended to repair the SMCP if speech symptoms are significant. However, if a SMCP is present but speech is not affected, surgical management is typically not immediately needed, and instead, the condition should be monitored by the team for several years to ensure that speech does not change (i.e., become hypernasal) with growth and development.
In addition, adenoidectomy may need to be avoided or limited, in children with SMCP, as this may increase their risk for VPD. Lastly, speech therapy may be needed for children with SMCP who also have articulation or language disorders.
For more information about submucous cleft palate and velopharyngeal dysfunction, or referrals for evaluation and treatment, click here.
For professionals interested in learning more about SMCP, VPD and surgical management, check out our International Symposium on Velopharyngeal Dysfunction, which will be held in Columbus, OH, September 14-15, 2018, at www.nationwidechildrens.org/ivpd