A case of laryngeal atresia (congenital high airway obstruction syndrome) with chromosome 5p deletion syndrome rescued by ex utero intrapartum treatment |
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Authors: | Kanamori Yutaka Kitano Yoshihiro Hashizume Kohei Sugiyama Masahiko Tomonaga Tetsuya Takayasu Hajime Egami Satoshi Goishi Keiji Shibuya Kazuhiko Kawana Yukiko Marumo Genzo Kikuchi Akihiko Kozuma Shiro Taketani Yuji Sekiyama Yushi |
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Institution: | a Pediatric Surgery, The University of Tokyo Hospital, Tokyo, Japan b Department of Pediatrics, The University of Tokyo Hospital, Tokyo, Japan c Department of Gynecology and Obstetrics, The University of Tokyo Hospital, Tokyo, Japan d Department of Anesthesiology, The University of Tokyo Hospital, Tokyo, Japan |
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Abstract: | The authors report a case of laryngeal atresia (congenital high airway obstruction syndrome CHAOS]) that was diagnosed prenatally. The patient underwent successfully tracheostomy by ex utero intrapartum treatment (EXIT). The fetal ultrasonography and magnetic resonance imaging MRI showed a typical CHAOS pattern with expanded hyperechogenic lungs, inverted diaphragms, and a dilated trachea. Recently, 3 cases of prenatally diagnosed CHAOS were reported to be treated successfully by EXIT. The clinical manifestation and course of this case was not similar to these 3 cases. The 3 previous patients did not fare as well during gestation and were delivered earlier than that in our case. In our case, fetal hydrops was seen at 23 gestational weeks, but it gradually subsided and disappeared at 30 gestational weeks. The fetus was stable and well. After delivery at 39 weeks, the baby received respiratory assistance by ventilator assistance. After 3 days, she could breath well on her own. The patient also had chromosome 5p deletion syndrome and perineal groove. More experience in treating CHAOS cases with EXIT to fully estimate its clinical course and prognosis is needed. |
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Keywords: | Congenital high airway obstruction syndrome ex utero intrapartum treatment laryngeal atresia chromosome 5p deletion syndrome |
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