Abstract
Noonan syndrome is an autosomal dominant disorder which includes cardinal symptoms such as specific facial features, congenital heart disorder, and skeletal anomaly. Although suckling disorder during the neonatal period and feeding difficulties during infancy have been observed, there have been no reports regarding the development of feeding and swallowing function in Noonan syndrome. Here, we report on a patient with Noonan syndrome who underwent long-term functional therapy for eating.
Case Presentation：At the initial examination（3 years and 2 months old）, the patient could suck the thumb, but refused a spoon and paste food. Therefore, we diagnosed the case as oral intake preparatory dysfunction accompanied by refusal to orally ingest. We performed taste stimulation training by having a researcher wet a finger with dashi stock and then wetting the patient’s lower lip with it to make the patient be stimulated by the taste and swallow the secreted saliva. We used toys and similar items to stimulate the oral cavity tactilely.
Course：As the patient accepted liquids well, liquid paste-based foods were gradually introduced. At 4 years and 5 months old, the patient acquired the ability to capture food and at 5 years and 6 months the patient could chew and grind food. From 6 years old, the patient started to refuse assistance and ate independently, thus losing the ability to eat paste foods. From 8 years and 2 months old, the patient was able to accept assistance and soft solid-shaped foods. At 8 years and 6 months, the patient regained the ability to eat paste foods and at 9 years and 5 months the patient could bite through food using the anterior teeth. Our current aim is to enable the patient to acquire chewing function through masticatory training.
Discussion：In the case of this sensory biased and finicky patient, we had to consider pediatric psychological and social development achieved by rehabilitation and education, so we did not force the patient to accept assistance or engage in direct training when they refused to do so. We also found it necessary to offer continuous feeding support so that we could give functional therapy for eating at the appropriate time.
