当院における遅発性先天性横隔膜ヘルニア症例の検討  [in Japanese] Review of Late-Presenting Congenital Diaphragmatic Hernia in Children  [in Japanese]

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Abstract

【目的】先天性横隔膜ヘルニア(congenital diaphragmatic hernia: CDH)の中で生後30 日以降に診断される遅発性CDH は生後24 時間以内発症症例に比べ予後が良いとされているが発症後の診断が遅れることにより致死的な状態となることがある.今回,当科において経験した遅発性CDH について検討した.<br>【方法】1987 年1 月から2010 年12 月までに当院にて治療したCDH(bochdalek hernia)86 例のうち遅発性CDH であった10 例(11.6%)を対象とし,発症時期,初発症状,発症から診断に至るまでの時間,脱出臓器,ヘルニア囊の有無,予後について検討した.<br>【結果】遅発性CDH 10 例における診断時年齢の中央値は1 歳4 か月(生後76 日~15 歳)であった.初発症状は呼吸器症状が3 例(30%)であったのに対し消化器症状が6 例(60%)にみられた.発症から診断までの期間は平均4.2 日であった.脱出臓器は全例に消化管の脱出を認め,肝臓脱出は認められなかった.10 例中9 例は合併症なく救命しえたが,1 例は前医から当院へ搬送中に心肺停止をきたし死亡した.<br>【結論】新生児症例と異なり遅発性CDH では消化器症状で発症することが多く,診断までに時間がかかる場合がある.重篤な症状をきたす症例は少ないが,中には診断されるまでに脱出臓器の穿孔から急激に胸腔内圧が上昇し,心肺が圧迫されることによりショック状態となるような症例もあるため,小児の消化器症状においても本疾患が疑われた場合は早急に胸部画像検査を施行し,経鼻胃管挿入を早急に行い,胃の減圧を図るべきである.

<i>Purpose</i>: Among patients with congenital diaphragmatic hernia (CDH), those with late-presenting CDH (LP-CDH) have been reported to have a better prognosis. However, it has also been reported that the diagnosis is not always easy, and delayed diagnosis may sometimes be life-threatening. This study reports on LP-CDH cases experienced in an institution for 23 years.<br><i>Methods</i>: There were 10 LP-CDH cases (11.6%) between January 1987 and December 2010. They were retrospectively reviewed and investigated on the relationship with their ages at the LP-CDH onset, presenting symptoms, the period taken for diagnosis, contents of herniated organs, presence or absence of hernia sac and their outcome.<br><i>Results</i>: The median age at diagnosis of LP-CDH was 1 year and 4 months (76 days to 15 years). Presenting symptoms were respiratory failure in 3, digestive symptom such as abdominal pain with vomiting in 6 cases. One case had no symptoms, and was found on routine chest X-ray examination at healthy check-up. The mean period from onset to diagnosis was 4.2 days. Hernia occurred in the gastrointestinal tracts in all cases, and none had liver herniation. Nine of 10 cases were rescued without any complications, although 1 case developed cardiopulmonary arrest on the way to our hospital and died without surgical intervention.<br><i>Conclusions</i>: Unlike neonatal cases, LP-CDH cases often occur with digestive problems and diagnosis is often delayed. It should be noted that emergent chest X-ray and insertion of nasogastric tube to decompress stomach contents should be performed as soon as possible for their rescue when LP-CDH is suspected.

Journal

  • Journal of the Japanese Society of Pediatric Surgeons

    Journal of the Japanese Society of Pediatric Surgeons 49(5), 975-980, 2013-08-20

    The Japanese Society of Pediatric Surgeons

References:  18

Codes

  • NII Article ID (NAID)
    10031185843
  • NII NACSIS-CAT ID (NCID)
    AN00192281
  • Text Lang
    JPN
  • Article Type
    ART
  • ISSN
    0288609X
  • NDL Article ID
    024853349
  • NDL Call No.
    Z19-244
  • Data Source
    CJP  NDL  J-STAGE 
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