Selective disruption of trigeminal sensory neurogenesis and differentiation in a mouse model of 22q11.2 deletion syndrome

22q11.2 Deletion Syndrome (22q11DS) is a neurodevelopmental disorder associated with cranial nerve anomalies and disordered oropharyngeal function including pediatric dysphagia. Using the LgDel 22q11DS mouse model, we asked whether sensory neuron differentiation in the trigeminal ganglion (CNgV) , which is essential for normal orofacial function, is disrupted. We did not detect changes in cranial placode cell translocation or neural crest migration at early stages of LgDel CNgV development. As the ganglion coalesces, however, proportions of placode-derived LgDel CNgV cells increase relative to neural crest cells. In addition, local aggregation of placode-derived cells increases and aggregation of neural crest-derived cells decreases in LgDel CNgV. This change in cell-cell relationships was accompanied by altered proliferation of placode-derived cells at E9.5, and premature neurogenesis from neural crest-derived precursors, reflected by increased frequency of asymmetric neurogenic divisions for neural crest-derived precursors by E10.5. These early differences in LgDel CNgV genesis prefigure changes in sensory neuron differentiation and gene expression by P8, when early signs of cranial nerve dysfunction associated with pediatric dysphagia are observed in LgDel mice. Apparently, 22q11 deletion destabilizes CNgV sensory neuron genesis and differentiation by increasing variability in cell-cell interaction, proliferation, and sensory neuron differentiation. This early developmental divergence and its consequences may contribute to oropharyngeal dysfunction including suckling, feeding and swallowing disruptions at birth and additional orofacial sensory/motor deficits throughout life.

Pediatric Dysphagia: Survey Results Describing Speech-Language Pathologists' Education and Experience

Purpose Evaluation and treatment of pediatric dysphagia, swallowing difficulties occurring during childhood, is in the speech-language pathologists' (SLPs) scope of practice; however, little is known regarding the education SLPs receive during their graduate work and afterwards to effectively assess, treat, and manage this diagnosis. Method An 11-question survey was developed to examine the experience and opinions of SLPs related to the type and extent of pediatric dysphagia education and training they have and/or should have received during their graduate education. The survey was disseminated to SLPs across the United States via social media and e-mail Listservs. Results Survey results from 134 participants across the country, with varying clinical backgrounds, indicated a lack of pediatric dysphagia education during graduate school. SLPs reported overcoming these insufficiencies through gaining work experience and pursuing continuing education units on pediatric dysphagia. SLPs overwhelmingly (> 95% of respondents) recommended a full course or unit focusing on pediatric dysphagia for master's students in speech-language pathology. Survey results found a statistically significant increase in the likelihood of completion of a pediatric dysphagia unit or course for students graduating in the last 10 years; however, this positive trajectory shows a modest increase in the availability of coursework to prepare pediatric SLPs to evaluate and treat the growing pediatric dysphagia population. Conclusions Some positive changes in SLP education can be inferred from these results; however, there is still additional work to be done to equip pediatric SLPs to provide adequate care for a growing population with pediatric dysphagia.

Pharyngeal Manometry in Pediatric Dysphagia Assessment

Purpose Reliable measures to capture swallowing pathophysiology and outcomes from therapeutic interventions are needed in the pediatric setting. Therefore, this review presents the current best evidence for manometry investigation and its application in pediatric dysphagia. For oropharyngeal dysphagia assessment, videofluoroscopic swallow study is considered the gold standard, and fiberoptic endoscopic examination of swallowing is also employed in some settings. In clinical practice, both tests mostly rely on qualitative interpretation of image data and have variable reliability to detect pathophysiology. Conclusion Acknowledging that swallowing is a pressure-driven event, the role of manometry is now being considered as an objective, nonradiological technique that can be performed as an adjunct to other clinical swallow evaluations. The latest high-resolution manometry catheter technologies are miniaturized and simple to use, enhancing their potential application in pediatric clinical and research contexts.