I have a 34 week premature infant with Atrial flutter and Atrial septal defect that I just started seeing who has Beckwith Wiedeman Syndrome. The pediatrician recommended solid food feedings due to not taking his formula consistently. Mom only gives him baby food at night and he eats about half a container but seems to enjoy it. He had a MBS since he was receiving thickened formula with oatmeal on 6/5/19. He was clear for thin liquids via Avent level 1. He got frustrated with nipple so SLP in outpatient ok’d him to use Avent level 2. According to his MBS, he had oral transport/lingual motion impairment and oral residue. His swallow response is impaired at level of pyriforms, laryngeal elevation and anterior hyoid excursion are also impaired. At rest, child’s tongue protrudes and when he sucks on the bottle and when spoon feedings are tried, he has a tongue thrust. Coughing is noted during bottle and spoon feedings and when the bottle is pulled out of his mouth every 2-3 sips to help him breathe, he seems to have difficulty latching onto the bottle. I’ve tried palpating gently under his chin and noticed his tongue recessed into his mouth for a brief time, and have tried a ‘j scoop’ with the spoon when fed him. I just wonder what other treatments you would recommend I try since his doctor wants to try therapy before surgical treatment of macroglossia.
Sounds like he is a former 34 weeker who you are following in the community. What is his adjusted age now?
Being a former preemie, as opposed to a former full term infant, adds some other challenges to his BWS that might be playing a role, such a respiratory co-morbidities. Sounds as though he was sent home from the NICU as a full oral feeder, but quality may not have been part of the assessment for readiness to be a full PO feeder, rather volume was.
What looks like tongue thrust may actually be the tongue moving forward with onset of motion, due to the lack of space in the oral cavity for his enlarged tongue as it moves posteriorly and an adaptive response on the part of the infant to maintain his upper airway.
Sounds like he was sent home without any respiratory support to assist with airway maintenance. Many infants with BWS require trachs to assure a consistent patent upper airway. Even so, some who manage to be discharged without airway support can readily destabilize a fragile upper airway with the act of swallowing during feeding. The need for the base of tongue to retract, and the large thickened blade to move posteriorly as part of the swallow, can actually “create” a sense of further airway obstruction with the act of swallowing. In addition, the thickened blade with intrinsic tongue muscles that lack thinning and cupping can create challenges with bolus control orally.
The macroglossia also can alter mandibular alignment, leading to subluxation. It also inhibits a closed mouth posture, which can work against establishing the necessary anterior seal on the bolus to help drive the swallow.
Often behaviors during feeding may be related to the struggle during feeding to manage the bolus and maintain the upper airway, and can lead to adaptive behaviors, which then create maladaptive feeding behaviors and long-term struggles with eating/drinking.
Sounds like with Avent 1 flow in radiology there was a delay in swallow initiation with premature entry to the pyriforms. It is likely the bolus is poorly managed due to the adverse effects of BWS noted above, so it would be important to objectify the effect of the change/increase in flow rate with Avent 2 on his swallowing physiology, as his physiology sounds precarious. The coughing with spoon and bottle suggest fluid has approached or entered his airway.
Even if he there was no witnessed aspiration or laryngeal penetration during the VFSS (which is a moment in time), he is at high risk for airway invasion and onset of aversions due to the struggle to feed. Most events of aspiration are silent in the infant population, and he is also giving us some signs, so he is likely more worrisome.
I think the doctor’s well-intentioned goal of wanting him to PO feed before surgery may have been made without his understanding of the impact of BWS on swallowing physiology and the swallow-breathe interface, and may be counter productive and perhaps not safe for this former preemie. Muscular and structural restrictions such as those with BWS often require us to take a step back and look at the co-morbidities and their overriding influence on swallowing physiology not just on intake, which is at times challenging for our medical colleagues.