日本未熟児新生児学会雑誌　第24巻第2号　92～98頁（2012年）

日本未熟児新生児学会雑誌　24(2)：304-310；2012　

受付日：平成24．02．16

受理日：平成24．03．07

先天性横隔膜ヘルニア修復術後の長期にわたる重度肺高血圧に対し経鼻的に一酸化窒素吸入療法を施行した1 例

Inhaled Nitric Oxide Via Nasal Cannula in an Infant with Congenital Diaphragmatic Hernia Complicated with Prolonged Pulmonary Hypertension：An Alternative to Endotracheal Intubation

＊1東京女子医科大学　母子総合医療センター　新生児部門，＊2島根大学　医学部　小児科　新生児集中治療部

＊1Maternal and Perinatal Center, Tokyo Women’s Medical University，＊2Department of Pediatrics, Shimane University School of Medicine

中西秀彦＊1・石田宗司＊1・首里京子＊1・今井　憲＊1・柴田直昭＊1＊2・鷲尾洋介＊1・増本健一＊1・戸津五月＊1・内山　温＊1・楠田　聡＊1

Hidehiko NAKANISHI＊1，Syuji ISHIDA＊1，Kyoko SYURI＊1，Ken IMAI＊1，Naoaki SHIBATA＊1＊2， Yosuke WASHIO＊1，Kenichi MASUMOTO＊1，Satsuki TOTSU＊1，Atsushi UCHIYAMA＊1，Satoshi KUSUDA＊1

Key Words：congenital diaphragmatic hernia，pulmonary hypertension，inhaled nitric oxide，nasal cannula

　重度の先天性横隔膜ヘルニア（CDH）修復術後，長期に遷延する肺高血圧（PH）に対し，経鼻カニューラから一酸化窒素吸入療法（Nasal-iNO）を施行した早産児の1例を報告する。児は在胎期間31週6日，出生体重1,832gの男児。左横隔膜全欠損型のCDHに対し，再発も含め計4回の修復術を施行後，PHに対し経鼻酸素と血管拡張薬で管理していたが，修正7カ月時にPH増悪を来し，再度人工呼吸管理とNO投与を行った。PHは改善したが，児は重度の肺低形成であり，今後再燃が考えられたため，抜管後，一時的にNasal-iNO（初回投与量5ppm）で管理した。PHのコントロールは良好で，修正1歳時にNO離脱可能となり，以後，経鼻酸素，血管拡張薬，利尿薬で管理している。Nasal-iNO は，長期のNO 投与が必要なPHに対する新たな治療戦略の一つとして有効であると考えられる。

　Inhaled nitric oxide（iNO）is a selective pulmonary vasodilator, mainly administered to infants with hypoxic respiratory failure via an endotracheal tube under mechanical ventilation. Here we report an infant with a severe type of congenital diaphragmatic hernia（CDH）who was treated by inhaled nitric oxide through nasal cannula（Nasal-iNO）in a chronic phase. The male infant was born at 31 and 6/7 weeks’gestation by emergency cesarean section due to an uncontrollable labor onset. Soon after birth, he was successfully intubated under deep sedation, and had been under mechanical ventilation with iNO. The type of CDH was a total deficiency of the left diaphragm with liver herniation into the left chest cavities. He received a total of four surgical repairs due to the relapses of CDH eventually. Since the final operation, no relapse of CDH had been observed. However, he had gradually developed pulmonary hypertension, and finally needed mechanical ventilation and iNO seven months after birth. During the weaning period from this treatment, we chose Nasal-iNO for a long duration, and Nasal-iNO was started at a 5ppm of dose. Under this concentration, NO and NO2 in environment was undetectable. Since starting nasal-iNO, his PH was well controlled, and finally nasal-iNO treatment was discontinued at the age of one year. Nasal-iNO seems to be safe and effective for the infants with pulmonary hypertension in a chronic phase.