QUESTION

Is there any particular intervention improving pharyngeal clearance with the swallow. This infant has pharyngeal stasis post swallow on the swallow study. Diffuse residue, hard to quantify as it varied throughout the study, but definitely not a little…enough that poses a risk for aspiration post-swallow/as feeding continues. UES opened for a portion of the bolus to pass through, pharyngeal stripping was not great. No nasopharyngeal regurgitation that I recall. Reasons for the swallow study were bedside symptoms of desaturations during oral feeding at ~37 weeks PMA and a steady decline in his oral intake & feeding cues. Some background: He is an ex 33-weeker, now 39+5. Questionable dysmorphic features, we are awaiting genetic testing results. ENT did not find any structural issues, but his cry is described as “quiet” by nurses. Early on in my assessment (maybe ~35 weeks) I couldn’t elicit a tongue-lateralizing reflex, haven’t checked since. He also has a high palate and often is nasally congested. Recent ECHO showed a moderate ASD. Recent hearing test indicates possible sensorineural loss, but they want to re-do it. He also gets very constipated. Overall low energy and alertness for his age. All signs seem to be pointing to a neurological basis for his dysphagia and I’m just wondering if there are any last hail marys to try before we start to plan for home on NG, OP feeding therapy, etc. We put him on an oral rest for now. I’ve talked with team and parents both about aspiration risk and oral feeding aversion. Medical team is very supportive of therapy. Any ideas are greatly appreciated!

ANSWER

Thanks for such a detailed history to help to consider possibilities. Not an uncommon presentation that can go many directions as further data comes in.

I like to start with the whys to guide intervention options. Multiple co-morbidities at play it seems. The dysmorphic features stand out as worrisome, and often craniofacial and cardiac variants like an ASD can co-occur. None of the clinical or physiologic behaviors can be attributed strictly to being born preterm, though preterm birth would increase risk for co-morbidities. When I read your post with such  clear  clinical and radiologic presentation,  the possible etiologies/questions that popped in my mind as I read your post were: hypotonia (constipation, lethargy, oral-motor disintegrity understood thus far; wonder about postural control and movement patterns, sensory-moor function include trunk and head/neck), extra esophageal reflux (nasal congestion, lax pharyngeal constrictors, perhaps postural hypotonia), poor posterior driving force of tongue (often correlated with hypotonia, poor pressure generation  to help achieve UES relaxation and opening, posterior tongue tie and/or mandibular hypoplasia….seems at times ENTs miss that…). The quiet cry may suggest retracted tongue (? for stability and/or  r/t tethered oral tissues, r/t mild mandibular hypoplasia). Wondering about work of breathing, swallow-breathe interface with both pacifier dips and/or clinical observation of PO feeding. Has there been a neuro consult? MRI brain? Genetics consult? OT consult? Likely at risk for bolus mis-direction from below (refluxate), d/t what sounds like proximal hypotonia that could make  timely effective response to retrograde flow from the esophageal body unreliable.

All the etiologies that I mentioned, in isolation or in combination, could play a part in the diffuse pharyngeal stasis/residue observed, which is worrisome for bolus mis-direction during the course of a true feeding. I am surprised there was not retrograde flow into the nasopharynx (what some describe as nasopharyngeal regurgitation), given the diffuse residue. Pacifier dips in a secure swaddle elevated side lying position would allow for purposeful swallows and motor learning yet reduce risk for airway invasion, given that etiology is not fully clear. The motor learning from the pacifier dips would keep him learning but minimize risk.

What we know so far suggests a poor prognosis for being a full PO feeder at time of d/c from the NICU and the etiology(ies) is/are unlikely to resolve in the short term, given multiple complex co-morbidities, as yet not fully determined. As a result, pending further data, they might consider a G-Tube with a Nissen  (to optimize airway protection and more safely buy him time for response to therapy and evolution to guide the differential), instead of home NG, which would have its own attendant sequelae.

Search google scholar for this  article by my colleague and friend, Laura Brooks, which may or may not be pertinent, pending your further assessment and refection. Her paper is one I reference with neonatologist’s and intensivists when indicated.

Brooks, L., Landry, A., Deshpande, A., Marchica, C., Cooley, A., & Raol, N. (2020). Posterior tongue tie, base of tongue movement, and pharyngeal dysphagia: what is the connection?. Dysphagia, 35(1), 129-132.

Abstract: Ankyloglossia, or tongue tie, and its impact on the oral phase of feeding has been studied and debated for decades. However, the impact of posterior tongue ties on the pharyngeal phase of swallowing is not well documented in the literature. A videofluoroscopic swallow study (VFSS) allows for visualization of the oral, pharyngeal, and esophageal phases of the swallow. When decreased base of tongue movement, impaired pharyngeal pressure generation, and presence of pharyngeal residue are noted during a VFSS, a neurologic etiology can be suspected. However, in the setting of a normal MRI with normal motor development, other etiologies need to be explored. If it is not neurologic, could it be anatomic? We present a 21-month-old patient with significant pharyngeal phase dysphagia which was most saliently characterized by impaired base of tongue movement, poor pressure generation, and diffuse residue resulting in aspiration. He was eventually diagnosed with a posterior tongue tie and underwent a frenulectomy. Results via subsequent VFSS revealed significant improvement in base of tongue movement, pharyngeal pressure generation, and pharyngeal constriction, resulting in efficient movement of the bolus through the pharynx into the esophagus, no nasopharyngeal regurgitation, no aspiration, and near resolution of his pharyngeal dysphagia. Patients with impaired base of tongue movement and impaired pressure generation resulting in pharyngeal residue in the setting of a normal neurologic workup could possibly present with a posterior tongue tie which should be examined and included in the differential diagnosis.

I hope this is helpful.