鼻咽腔閉鎖機能不全による摂食・嚥下障害を伴った第1第2鰓弓症候群の1例  [in Japanese] A Case of First and Second Branchial Arch Syndrome with Eating and Swallowing Disorder Due to Velopharyngeal Insufficiency  [in Japanese]

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Author(s)

    • 玄 景華 GEN KEIKA
    • 朝日大学歯学部口腔病態医療学講座障害者歯科学分野 Department of Dentistry for the Disability and Oral Health, Division of Oral Pathogenesis and Disease Control, Asahi University School of Dentistry
    • 橋本 岳英 HASHIMOTO TAKEHIDE
    • 朝日大学歯学部口腔病態医療学講座障害者歯科学分野 Department of Dentistry for the Disability and Oral Health, Division of Oral Pathogenesis and Disease Control, Asahi University School of Dentistry
    • 片川 吉尚 KATAGAWA YOSHIHISA
    • 朝日大学歯学部口腔病態医療学講座障害者歯科学分野 Department of Dentistry for the Disability and Oral Health, Division of Oral Pathogenesis and Disease Control, Asahi University School of Dentistry
    • 安田 順一 YASUDA JUN-ICHI
    • 朝日大学歯学部口腔病態医療学講座障害者歯科学分野 Department of Dentistry for the Disability and Oral Health, Division of Oral Pathogenesis and Disease Control, Asahi University School of Dentistry

Abstract

【緒言】第1第2鰓弓症候群は主に顔面、口腔および耳などに変形を生じる先天性疾患であるが、摂食・嚥下障害は少ないとされている。今回は著明な哺乳障害を伴った本症候群患児に対して、摂食機能療法を行い良好な結果を得たので報告する。【症例】4ヵ月女児。主訴:某大学病院小児科より嚥下リハビリテーション依頼。38週2,296gで帝王切開にて出生し、左耳介欠損、左耳孔閉鎖等を認め、本症候群と診断された。既往歴:出生後は経管栄養で3週間入院したが、哺乳良好のため退院となった。現病歴:生後2ヵ月時にRSウイルス感染により誤嚥性肺炎を認め、某大学病院に入院。1ヵ月間気管内挿管するも喉頭軟化症のため呼吸困難が持続し、経鼻経管栄養の状態であった。【対応と治療経過】哺乳瓶でのミルク摂取で鼻腔や口唇からのもれやむせが強くみられたため、鼻咽腔閉鎖機能不全および口唇閉鎖不全による嚥下障害と診断した。P型乳首による哺乳、口唇や舌への間接訓練や味覚刺激を指導した。VE・VF検査では鼻咽腔閉鎖機能不全、中咽頭部麻痺および披裂軟骨部肥大、鼻腔逆流を認めたが、誤嚥所見はなかった。6ヵ月児よりプラスチック製吸い口でのミルク摂取と離乳初期食による摂食指導を開始した。10ヵ月児に離乳後期食でかじり取りの指導も行った。11ヵ月児にむせがほとんどなく、経口摂取量も十分なため経鼻経管栄養を中止した。現在は3歳4ヵ月で全身状態も安定しており、乳児食が摂取可能で水分摂取も自立した。【考察】本症例はウイルス感染により長期間にわたる気管内挿管が持続したことにより、軟口蓋の運動低下が生じた結果の鼻咽腔閉鎖機能不全と考える。母親の強い希望もあり経鼻経管栄養を持続しながら、本児の摂食・嚥下機能を評価して母親への摂食指導と支援が良好な成果を得たものと考える。

Introduction: First and second branchial arch syndrome is a congenital disease characterized by deformity of the oral cavity and ears, although it is less frequently associated with eating or swallowing disorders. We report the favorable outcome of eating function therapy for a child with first and second branchial arch syndrome accompanied by a pronounced suckling disorder. Case report: A 4-month-old girl was referred from the pediatric department of a university hospital for swallowing rehabilitation. She was born at 38 weeks gestation and weighed 2,296 grams. She exhibited a left auricular defect and left auditory atresia, and was diagnosed with first and second branchial arch syndrome. After birth, she was placed on tube feeding and hospitalized for three weeks, then discharged with favorable suckling function. At two months after birth, she developed aspiration pneumonia due to an RS virus infection and was admitted to a university hospital. She was maintained with endotracheal intubation for one month, but developed persistent dyspnea due to laryngomalacia and was hospitalized with nasal tube feeding. Treatment and clinical course: Severe milk leakage from the nasal and oral cavities and choking during bottle feeding led to a diagnosis of swallowing disorder caused by velopharyngeal insufficiency and insufficient lip closure. Bottle feeding with a P-shaped nipple, indirect training of the lips and tongue, and gustatory stimulation were prescribed. Videoendoscopy and videofluoroscopy revealed velopharyngeal insufficiency, oropharyngealparalysis, arytenoid cartilage swelling, and nasal regurgitation, without evidence of aspiration. At six months after birth, milk intake through a plastic suction mouth and eating instruction with early weaning food were started. At 10 months after birth, how to bite off food was taught using late weaning food. At 11 months after birth, she showed almost no choking and could eat a sufficient amount of food. Nasal tube feeding was discontinued. At the time of writing, she was three years and four months old with a stable overall condition and was able to eat infant food and drink water without assistance. Discussion: This case involved velopharyngeal insufficiency resulting from soft palate hypomotility due to prolonged endotracheal intubation that was required due to a viral infection. A favorable outcome was achieved by continuous nasal tube feeding, for which the mother was strongly in favor, combined with evaluation of thechild's eating and swallowing functions and the provision of appropriate eating instruction and support to the mother.

Journal

  • The journal of Gifu Dental Society

    The journal of Gifu Dental Society 37(3), 188-194, 2011-02-20

    Asahi University

References:  6

Codes

  • NII Article ID (NAID)
    110008138685
  • NII NACSIS-CAT ID (NCID)
    AN00053070
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    03850072
  • Data Source
    CJP  NII-ELS  IR 
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