副島 周子¹, 日高 庸博¹, 佐藤 麻衣¹, 北代 祐三¹, 住江 正大¹, 中並 尚幸¹, 楠田 剛², 金城 唯宗², 黒木 愛³, 村上 信哉³

Chikako SOEJIMA¹, Nobuhiro HIDAKA¹, Mai SATO¹, Yuzo KITADAI¹, Masahiro SUMIE¹, Naoyuki NAKANAMI¹, Takeshi KUSUDA², Tadamune KINJO², Ai KUROGI³, Nobuya MURAKAMI³

¹福岡市立こども病院産科, ²福岡市立こども病院新生児科, ³福岡市立こども病院脳神経外科

¹Department of Obstetrics, Fukuoka Children's Hospital, ²Department of Neonatology, Fukuoka Children's Hospital, ³Department of Neurosurgery, Fukuoka Children's Hospital

キーワード : fetal myelomeningocele, fetal surgery, prenatal diagnosis

目的：脊髄髄膜瘤（myelomeningocele：MMC）胎児の神経学的予後改善を目的とした妊娠26週未満での胎児治療の早期安全性試験が本邦でも始まった．本研究では当院で管理したMMC胎児の出生前診断背景と生後予後を検討し，今後の課題を明らかにする．方法：2014年11月から2022年8月に当院で妊娠管理した胎児MMC症例を対象とし後方視的に検討した．出生前診断時期，診断契機となった超音波所見，生命・運動予後を主要評価項目とした．結果：対象は26例あり，紹介週数の中央値は妊娠25週（11‐34週），11例（42％）は26週以降に紹介されていた．5例は22週未満で診断され，うち3例は人工妊娠中絶となった．紹介契機はMMC単独が11例，脳室拡大単独が5例，脊椎所見および脳室拡大が6例，レモンサイン・バナナサインが1例，他の合併疾患が3例であった．中絶例を除いた23例の分娩週数中央値は38週（26‐40週）で，生後フォローアップ期間の中央値23か月（0‐67か月）において全例生存していたが，14例（61％）で運動機能障害を指摘された．生後18か月以上を経過した児15例中10例（67％）が神経因性膀胱と診断，自己導尿を必要とした．結論：胎児診断されたMMC症例は生後に運動機能障害を呈するものも多い．胎児診断の時期が現状では十分早いとは言えず今後診断時期を早める必要性が認識された．

Purpose: A safety trial for early fetal treatment before 26 gestational weeks to improve the neurological prognosis of fetuses with myelomeningocele (MMC) was conducted in Japan. The aim of the study was to examine the prenatal diagnostic background and postnatal outcomes of fetuses with MMC at our institution and identify future challenges for fetal treatment. Methods: We retrospectively reviewed the medical records of MMC cases in our institution encountered between November 2014 and August 2022. The timing of prenatal diagnosis, ultrasound findings leading to diagnosis, and the vital/motor prognosis were evaluated. Results: The study included 26 cases, all of which were referred from other institutions. The median gestational age at referral was 25 weeks (range: 11・34 weeks), with 11 cases (42%) being referred after 26 weeks. Five cases were diagnosed before 22 weeks, of which three opted for elective termination. The referral indications were isolated MMC in 11 cases, isolated ventriculomegaly in five cases, combined spine and ventricular abnormalities in six cases, lemon and banana signs in one case, and other associated conditions in three cases. Excluding pregnancy terminations, the median gestational age at delivery was 38 weeks (range: 26・40 weeks). At a median follow-up of 23 months (range: 0・67 months), all cases survived; however, 14 cases (61%) exhibited motor function impairment. Ten of 15 children (67%) who were at least 18 months of age were diagnosed with neurogenic bladder or required self-urination. Conclusion: While the vital prognosis of fetuses with MMC is favorable, many exhibit motor function impairment postnatally. The current timing of prenatal diagnosis is not sufficiently early, highlighting the need to expedite the diagnosis.