Overview of Treatment Efficacy

Dysphagia in VCFS can be caused by a number of factors such as airway compromise, vascular anomalies, palatal and pharyngeal anomalies such as cleft palate, slow gastric emptying, hypotonia, chronic constipation, and irritable temperament (Fullman & Boyer, 2012).  Because cleft palate is one of the most commonly seen characteristics of VCFS and one of the most significant contributors to dysphagia in the syndrome, treatment of cleft palate will be highlighted here.

Commonly recommended/studied treatments for cleft palate include the following:

      · Special feeding equipment – e.g. squeezable bottle and/or special nipple to compensate for the 

       difficulty in creating negative pressure due to the cleft (Bessell et al., 2011; Glass & Wolf, 1999; Miller, 

      2011; Reid, 2004; Shprintzen, 2008)

     · Maternal advice/support  (Bessell et al., 2011; Masarei, Wade, Mars, Sommerlad, & Sell, 2007; 

      Reid, 2004)

     · Feeding position – e.g. upright to avoid fluids leaking into eustachian tube and excess air from going to 

      the stomach. (Bessell et al., 2011; Boyer, Fullman, & Bruns, 2012; Forsyth & Morrison, 2009; Glass & 

      Wolf, 1999; “Velo-Cardio-Facial Syndrome Knowledge is Hope,” 2007)

      · Obturators/maxillary plates- a custom molded appliance used to seal off the cleft. (Glass & Wolf, 

      1999; Karayazgan, Gunay, Gurbuzer, Erkan, & Atay, 2009; Masarei et al., 2007; Miller, 2011; Reid, 

      2004)

     · Interdisciplinary care - (American Cleft Palate-Craniofacial Association, 2009; “Velo-Cardio-Facial 

      Syndrome Knowledge is Hope,” 2007)

     · Surgery/surgeries - to close the cleft (Marsh, 2009; Sell, Mars, & Worrell, 2006)

The above list is not all-inclusive and there is great variation amongst providers in cleft palate treatment.  In fact, a survey by Shaw et al., (2001) found out of 201 treatment teams working on one subtype of cleft palate, 194 treatment protocols were used.  There is consensus that there is a dearth of high level evidence for cleft palate care (Bessell et al., 2011; Miller, 2011; Reid, 2004), which results in this significant variation in treatment across providers.

To address the lack of consensus regarding evidence base practices, several recent reviews of cleft palate intervention literature have been done that have systematically assessed levels of evidence for some commonly recommended interventions (Bessell et al., 2011; Reid, 2004).  Below is a summary of their findings.



2 bottles used by Glass et al. (1999)

Special feeding equipment: Reid (2004) found moderate to strong evidence in one randomized controlled trial (RCT) (Shaw, Bannister, & Roberts, 1999) for the combination of a squeezable bottle and a NUK orthodontic nipple combined with parental counseling vs. a rigid bottle with a NUK orthodontic nipple and parental counseling, evidenced by increased weight at 12 months and increased head circumference in the squeezable bottle group.  In another RCT (Brine et al., 1994), Reid (2004) found that both a squeezable bottle with a long narrow crosscut nipple and a rigid bottle with a standard (crosscut) nipple provided adequate weight gain.  Bessell et al. (2011) reviewed the same two RCTs (Brine et al., 1994; Shaw et al., 1999) and pooled the data from both.  Analysis of the pooled data found no difference between the two bottle types for weight or head circumference, however because it was more common for babies to be switched from a rigid to a squeezable bottle (due to feeding difficulties) they theorized that the squeezable bottle may be easier to use.  Importantly, it must be noted that the subjects in both of these trials were nonsyndromic and results cannot be assumed applicable to the VCFS population.

Maternal advice/support:  Of the five RCTs included for review by Bessell et al., (2011), none examined the effect of maternal advice/support on the success of breastfeeding or bottle feeding.  Reid (2004) reports on one study (Pandya & Boorman, 2001), a prospective audit that was done following an intervention consisting of home visits, breast-feeding support, feeding education, and growth monitoring.  The study found significant decrease in failure-to-thrive rates (49% to 26% for cleft palate, 32% to 9% for unilateral cleft palate, and 38% to 20% for bilateral cleft palate) for all infants, including syndromic cases.




"Feeding a Child with a Cleft Lip or
Cleft Palate." (2014)

.

Feeding position:  Reid (2004) discussed two reports (Danner, 1992; Willis, 2000) based on personal experience advocating a variety of breast-feeding positions, noting that the recommendations were not backed up by evidence.  Bessell et al. (2011) were also unable to find any high level-of-evidence studies regarding feeding position in their study of the literature, dating back to 1950.





Example of an obturator

Obturators/maxillary plates: Reid (2004) reported on one small (n=8) study (Turner et al., 2001) with multiple baselines (with and without the obturator) and multiple interventions (obturator and breast/bottle feeding advice) that provided limited evidence that the obturator combined with advice can improve time taken to feed, volume of intake, and growth at 4 weeks of age when begun with newborn infants who have cleft palate or combined cleft lip and palate.  However, this study was open to bias because mothers self-selected to participate.  Bessell et al. (2011) found two RCTs (Masarei et al., 2007; Prahl, Kuijpers-Jagtman, Van  ’t Hof, & Prahl-Andersen, 2005) comparing outcomes with and without a maxillary plate.  One found a statistically significant difference in weight with the plate at 6 months in a portion of the babies, however, this difference was not observed at any other time (babies were followed through 18 months).  No statistically significant differences were observed between groups with regard to length or head circumference.

 

Interdisciplinary care: Neither Reid (2004) nor Bessell et al., (2011) evaluated interdisciplinary care, however the results of a small descriptive study on interdisciplinary care are reported on here.

Surgery: Efficacy of specific surgery types are beyond the scope of this website, however it is important to note that none of the above interventions are intended to be substitutions for surgery, but rather treatment for deficits prior to the surgery and/or remaining deficits after a surgery.

Summary:  The evidence for commonly recommended interventions is weak.  91% of 55 studies examined by Reid (2004) being level IV (opinion of respected authorities, based on clinical experience, descriptive studies, or reports of expert committee) and only five studies since 1950 qualifying for Bessell et al's (2011) more stringent inclusion criteria (all studies had to be RCTs).  More high quality research is needed in order to inform best practices.

Note that the specific articles reviewed in detail elsewhere on this website:

     · Feeding Obturator Appliance for an Infant With Cleft Lip and Palate

     · A Preoperative Appliance for a Newborn with Cleft Palate

     · Presurgical nasoalveolar moulding treatment in cleft lip and palate patients

    · A randomized control trial investigating the effect of presurgical orthopedics on feeding in infants with cleft lip and/or palate.

   ·  Interdisciplinary craniofacial teams compared with individual providers: is orofacial cleft care more comprehensive and do parents perceive better outcomes?

These do not represent all of the most commonly recommended/studied interventions for cleft palate, but rather were chosen as representative of some of the most recent research (published in the last five years).

References

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Bessell, A., Hooper, L., Shaw, W.C.., Reilly, S., Reid, J., & Glenny, A.M. (2011). Feeding interventions for 
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