Airway and Feeding Outcomes in Pierre Robin Sequence: A Comparison of Three Management Strategies.

BACKGROUND: Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery. METHODS: All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate. RESULTS: 67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy. CONCLUSION: The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon.

Infant-Driven Feeding Systems: Do They "Normalize" the Feeding Experience of Infants With Cleft Palate?

OBJECTIVE: To examine feeding skills of infants with cleft palate with or without cleft lip (CP±L) using infant-driven feeding systems compared to healthy controls on standard bottles. DESIGN: Cross-sectional cohort. SETTING: Large pediatric academic medical center in the Midwestern United States. PARTICIPANTS: Infants with CP±L (n = 15) using the Dr. Brown's Specialty Feeding System and typically developing infants without CP±L (n = 15) using the Dr. Brown's Natural Flow Original or Options bottles. MAIN OUTCOME MEASURE(S): Bottle-feeding proficiency, duration, milk transfer, and signs of feeding difficulty. RESULTS: Five-minute feeding proficiency differed significantly between groups with the control group taking 44% of the feed compared to 15% for the CP±L group on level 1 (P < .001) and 21% on level 2 (P < .001) nipples. Proportion of milk transfer was 96% ± 7% for controls and 75% ± 24% for the CP±L group (P = .013). Feeding duration (minutes) differed between the control group (13 ± 3) and the CP±L groups on each nipple level (level 1: 29 ± 16; P = .003; level 2: 32 ± 11; P = .001). Milk transfer rate (mL/min) was 9 ± 3 for control infants compared to 3 ± 1 for infants with CP±L on level 1 (P < .001) and 5 ± 1 on level 2 (P = .007). Coughing occurred in 40% of infants with CP±L and 27% of controls. CONCLUSIONS: Even when using specialty bottles, infants with CP±L differ from noncleft infants in feeding proficiency, duration, and overall intake.

Feeding and Growth in Infants With Cleft Lip and/or Palate: Relationships With Maternal Distress.

OBJECTIVE: To examine relationships between early feeding and growth and maternal distress in infants with and without cleft lip and/or palate (CL/P). DESIGN: Cross-sectional. SETTING: Pediatric academic medical center in the Midwestern United States. PARTICIPANTS: Mothers of infants 1 to 12 weeks old with CL/P (n = 30) and without CL/P (control group, n = 30) were recruited at craniofacial clinic or pediatrician appointments. MAIN OUTCOME MEASURE(S): Maternal responses on the Feeding/Swallowing Impact Survey (FS-IS), Parenting Stress Index, Fourth Edition, Short Form (PSI-4-SF), and Edinburgh Postnatal Depression Scale. Infant feeding history and growth measurements were obtained. RESULTS: Having an infant with CL/P revealed greater impact on maternal health-related quality of life due to feeding problems (F = 4.83, P = .03). Mothers of infants with CL/P reported average range Total Stress scores on the PSI-4-SF, which were higher than controls (F = 4.12, P = .05). Edinburgh Postnatal Depression Scale scores did not differ between groups. Compared to controls, infants with cleft palate had lower percentiles for weight (t = 4.13, P = .04) and length (t = 2.93, P = .01). Higher FS-IS scores were associated with longer feeding duration (r = 0.32, P = .01) and lower weight (r = -0.31, P = .02) and length (r = -0.32, P = .02). CONCLUSIONS: Despite receiving early team care and feeding interventions, mothers of infants with CL/P reported higher stress and more challenges with feeding and growth. Future studies should examine targeted psychosocial interventions to improve feeding and growth outcomes in infants with CL/P.

Breast Milk Feeding Practices and Barriers and Supports Experienced by Mother-Infant Dyads With Cleft Lip and/or Palate.

OBJECTIVE: To examine trends in breast milk provision and to characterize the breast milk feeding practices experienced by mother-infant dyads with cleft lip and/or palate (CL/P) in a large US sample. DESIGN: Cross-sectional study. METHODS: An online survey was distributed through cleft-related social media sites in the United States and in a single cleft lip and palate clinic. Statistical analyses included Kruskal-Wallis H tests and post hoc Mann-Whitney tests to examine group differences based on cleft type and prenatal versus postnatal cleft diagnosis. Linear regression was used to estimate associations between obtained variables. PARTICIPANTS: One hundred fifty biological mothers of infants (8-14 months of age) with CL/P (15% cleft lip, 29% cleft palate, 56% cleft lip and palate). RESULTS: Forty-six percent of mothers of infants with CL/P provided breast milk to their infant for at least 6 months. Five percent of infants ever fed at breast, and 43% received pumped breast milk via bottle. The most commonly reported supports included lactation consultants, nurses, feeding therapists, and online support groups. Feeding therapy was received by 48% of infants. CONCLUSIONS: A lower percentage of mothers of infants with CL/P reported providing breast milk compared to national estimates of the general population of infants without clefting. Results suggested there are multiple barriers, as well as numerous medical and psychosocial supports that facilitated breast milk feeding success. Implications for care are discussed.

A Quality Improvement Initiative to Improve Feeding and Growth of Infants With Cleft Lip and/or Palate.

OBJECTIVE: The purpose of this quality improvement initiative was to improve feeding and growth outcomes in infants with cleft lip and/or palate (CL/P). DESIGN: Institute for Healthcare Improvement quality improvement model. SETTING: Large pediatric academic medical center in the Midwestern United States. PARTICIPANTS: One hundred forty-five infants with nonsyndromic CL/P ages 0 to 12 months. INTERVENTIONS: Key drivers included (1) caregiver education and resources, (2) care coordination and flow, and (3) provider education and training. Interventions were designed around these themes and included targeting improved team communication, increased social work consultations, patient tracking, staff education, improved access to feeding equipment, and the launch of a new cleft palate feeding team. MAIN OUTCOME MEASURE(S): The primary outcome measure was the percentage of new patients with CL/P who met criteria for failure to thrive (FTT) per month. The secondary outcome measure was the frequency of hospitalization for infants with CL/P with a primary reason for admission of feeding difficulties or FTT. RESULTS: The institutional FTT rate for infants with CL/P decreased from 17% to 7% ( P < .003). The frequency of hospitalization for FTT improved from once every 30 days to once every 118 days. CONCLUSIONS: Targeted interventions aimed at improving feeding efficiency and effectiveness, as well as changes in care delivery models, can reliably promote improvements in feeding and growth outcomes for infants with CL/P, even with psychosocial risk factors present.

Airway and Feeding Outcomes of Mandibular Distraction, Tongue-Lip Adhesion, and Conservative Management in Pierre Robin Sequence: A Prospective Study.

BACKGROUND: Pierre Robin sequence is characterized by mandibular retrognathia and glossoptosis resulting in airway obstruction and feeding difficulties. When conservative management fails, mandibular distraction osteogenesis or tongue-lip adhesion may be required to avoid tracheostomy. The authors' goal was to prospectively evaluate the airway and feeding outcomes of their comprehensive approach to Pierre Robin sequence, which includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion. METHODS: A longitudinal study of newborns with Pierre Robin sequence treated at a pediatric academic medical center between 2010 and 2015 was performed. Baseline feeding and respiratory data were collected. Patients underwent conservative management if they demonstrated sustainable weight gain without tube feeds, and if their airway was stable with positioning alone. Patients who required surgery underwent tongue-lip adhesion or mandibular distraction osteogenesis based on family and surgeon preference. Postoperative airway and feeding data were collected. RESULTS: Twenty-eight patients with Pierre Robin sequence were followed prospectively. Thirty-two percent had a syndrome. Ten underwent mandibular distraction osteogenesis, eight underwent tongue-lip adhesion, and 10 were treated conservatively. There were no differences in days to extubation or discharge, change in weight percentile, requirement for gastrostomy tube, or residual obstructive sleep apnea between the three groups. No patients required tracheostomy. The greatest reduction in apnea-hypopnea index occurred with mandibular distraction osteogenesis, followed by tongue-lip adhesion and conservative management. CONCLUSIONS: Careful selection of which patients with Pierre Robin sequence need surgery, and of the most appropriate surgical procedure for each patient, can minimize the need for postprocedure tracheostomy. A comprehensive approach to Pierre Robin sequence that includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion can result in excellent airway and feeding outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

Feed-Thickening Practices in NICUs in the Current Era: Variability in Prescription and Implementation Patterns.

BACKGROUND: Feed-thickening for infants in the Neonatal Intensive Care Unit (NICU) is performed due to concerns of dysphagia and gastroesophageal reflux disease (GERD). No standards currently exist regarding feed-thickening prescriptions and practices and this results in variable and potentially unsafe feeding approaches. METHODS: Electronic surveys were sent to neonatal feeding therapists and providers in order to determine the prescriptions and practices currently being used for feed-thickening in the NICU. RESULTS: A total of 313 responses were collected. Results revealed the majority of providers use thickened feeds for concerns of dysphagia or GERD with some reporting they thicken expressed breast milk. Variability of thickening prescriptions was noted regarding consistencies, thickening agents, and recipes used. Reported approaches for measuring, mixing, and warming thickened feeds varied. CONCLUSION: Variability was noted in the feed-thickening prescriptions and practices performed in the NICU. Further research and standardization are required to develop thickening guidelines.