胎児食道閉鎖症におけるポーチサインの描出

Naomi Takamura; 高村奈緒美; Motoyoshi Kawataki; 川滝 元良; Rei Sunami; 須波 玲; Hiroshi Ishikawa; 石川 浩史

doi:10.11272/jss.42.631

胎児食道閉鎖症におけるポーチサインの描出Visualization of Pouch Sign in Congenital Esophageal Atresia Cases

高村奈緒美^1,3，川滝元良^2,3，須波玲⁴，石川浩史⁵Naomi Takamura^1,3, Motoyoshi Kawataki^2,3, Rei Sunami⁴, Hiroshi Ishikawa⁵

¹ 川崎協同病院検査科Clinical Laboratory, Kawasaki Kyodo Hospital

² 東北大学産婦人科Obstetrics and gynecology, Tohoku University

³ 神奈川県立こども医療センター新生児科Neonatology, Kanagawa Children’s Medical Center

⁴ 山梨県立中央病院総合周産期母子医療センター産科Obstetrics, Yamanashi Prefectural Central Hospital

⁵ 神奈川県立こども医療センター産婦人科Obstetrics and gynecology, Kanagawa Children’s Medical Center

受付日：2017年3月29日Received: March 29, 2017

受理日：2017年8月18日Accepted: August 18, 2017

発行日：2017年12月1日Published: December 1, 2017

目的：先天性食道閉鎖症は羊水過多，胃胞の欠如または小さいという超音波所見からスクリーニングされるが，同様な所見を呈する疾患との鑑別が困難なため，胎児診断率は44%と低率である．我々は，ポーチサイン（PS）の描出率，食道閉鎖症のタイプや合併奇形の有無とPSとの関係性について検討した．

対象と方法：2011年1月～2015年4月までに神奈川県立こども医療センターで出生後に食道閉鎖症と診断された28例のうち，胎児期に詳細な超音波検査を施行し本症を強く疑った17例について，PS陽性群とPS陰性群の2群に分けて，Gross分類，胃胞の有無，染色体異常の有無，合併異常の有無，母体苦痛緩和のための羊水穿刺回数，妊娠期間中の最大の羊水量（AFI）を後方視的に検討した．

結果と考察：対象17例のGross分類はC型13例（76%），A型4例（24%）であった．対象例のPS陽性は9例（53%）であった．最も早くPSを描出し得た例は30週であった．Gross分類別のPS陽性率を比較したところ，C型では54%（7/13），A型では50%（2/4）であり，Gross分類間で有意差を認めなかったことから，PS所見の有無でGross分類を予測することは困難と考えられた．18トリソミーのPS陽性率は33%と低率であり，胎児の嚥下とPS描出の関連が示唆される．合併疾患としては，VATER連合では全例でPS所見を認めた．先天性心疾患，胃胞描出の有無などではPS陽性率に差はなかった．

結論：胎児診断された食道閉鎖症では約半数でPS所見を認めることから，食道閉鎖症を疑った場合，ポーチの存在を念頭に置き胎児頚部を経時的に観察，描出することにより胎児診断率が上昇すると考える．

Purpose: Congenital esophageal atresia can be screened by ultrasound findings such as presence of polyhydramnios and absence of or small stomach bubble. However, the prenatal diagnosis rate is low (44%) due to difficulty in distinguishing this condition from other diseases manifesting similar findings. Here, we examined the relationship between depiction rate of pouch signs (PS), esophageal atresia type, and comorbid defects.

Subjects and Methods: Overall, 28 cases were diagnosed with esophageal atresia after birth at the Kanagawa Children’s Medical Center between January 2011 and April 2015. We performed a detailed ultrasound examination during the fetal period in 17 of these cases, which were highly suspected of having this disease, and divided them into two groups: PS-positive and PS-negative. For each group, we retrospectively examined the Gross category, presence or absence of stomach bubble, chromosomal and comorbid abnormalities, number of amniocentesis procedures required to relieve the mother’s pain, and maximum amniotic fluid index during pregnancy.

Results and Discussion: The Gross category of 13/17 subjects was type C (76%), whereas four were type A (24%). Nine cases (53%) were PS-positive. The earliest diagnosis of PS was at 30 weeks. We compared the PS-positive rate based on Gross category. The PS-positive rates were 54% (7/13) and 50% (2/4), respectively, for types C and A. Because the difference between Gross categories was not significant, we believed that it was difficult to predict Gross category using PS findings alone. The PS-positive rate in trisomy 18 cases was low (33%), which suggested a relationship between deglutition of the fetus and diagnosis of PS. As for complications, PS were present in all cases with VATER Association. There was no difference in PS-positive rate in the depiction of congenital heart disease or presence of stomach bubbles.

Conclusion: PS were present in approximately half of the prenatally diagnosed cases of esophageal atresia. In cases with suspected esophageal atresia, the prenatal diagnosis rate may increase by considering the presence of a pouch and by observing and depicting the fetus’s neck over time.

Key words: Congenital esophageal atresia; Fetal diagnosis; Pouch sign

超音波検査技術

学術賞－研究Researcher's Award - Research Paper

胎児食道閉鎖症におけるポーチサインの描出Visualization of Pouch Sign in Congenital Esophageal Atresia Cases