Regarding a girl who was diagnosed with Beckwith-Wiedemann syndrome and in whom suckling disorder due to macroglossia was observed, we herein report on the handling of pediatric ingesting/swallowing outpatient services in our department and the patient’ s course until she achieved sufficient ingesting/swallowing functions.
The patient was a 3-month-old girl with macroglossia observed from birth, on whom tongue reduction surgery was conducted for macroglossia approximately 3 months after birth in the Plastic Surgery Department of this hospital. However, because suckling disorder was observed with no improvement in her protruding tongue due to macroglossia, she was referred to our department for the purpose of dysphagia training. At the initial diagnosis, an imperfect oral lip seal due to macroglossia was observed without any sucking reflex. A nasogastric tube was placed and strongly attached around the upper and lower lips with medical tape.
Clinical diagnosis: Dysfunction at the preparatory stage. Desensitization was initiated to remove irritation, after which swallowing-facilitation training was conducted via gustatory stimuli using yogurt, encouraging her to swallow saliva. At this time, we explained the purpose of the training to her mother and attending nurse as well and asked her family to actively participate in the training. Specifically, we encouraged them to actively assist her lips. Once she became capable of moving her tongue backwards and closing her mouth with the assistance of her lips, she shifted from swallowing-facilitation training to direct training, wherein she started taking stage 2 baby food from a spoon using her lips.
Second tongue reduction surgery for modification purposes was conducted, after which rejection of oral intake was temporarily observed; however, adjustment of the form of food enabled the intake of infant foods.
In this case, tongue reduction surgery and oropharyngeal intubation for airway maintenance were conducted after birth and the patient was forced to endure various uncomfortable situations around the oral cavity including frequent intraoral suction and long-term nasogastric tube feeding, making oral intake difficult. However, early ingesting/swallowing instructions and intervention by the family were believed to lead to the successful acquisition of ingesting/swallowing functions.
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