Hội chứng Herlyn-Wener-Wunderlich: Báo cáo ca lâm sàng

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Tác giả: Vũ Hà Phúc Nguyễn, Nhật Thăng Trần, Thị Nhật Vy Trần

Ngôn ngữ: Vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y học TP. Hồ Chí Minh 2021

Mô tả vật lý: 211-216

Bộ sưu tập: Metadata

ID: 410698

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Hội chứng Herlyn-Werner-Wunderlich (HWW) là dị tật bẩm sinh hiếm gặp của đường tiết niệu sinh dục liên quan đến bất thường Mullerian, đặc trưng bởi tam chứng: tử cung đôi, nửa âm đạo tắc nghẽn và bất sản thận cùng bên. Chúng tôi trình bày một ca lâm sàng hội chứng HWW trong thai kỳ. Bệnh nhân nữ 25 tuổi, G0P0, tiền căn tử cung đôi và thận trái độc nhất đã biết trước. Bệnh nhân đang mang thai ở tuần thứ 32 nhập viện vì các triệu chứng của dọa sinh non. Chẩn đoán hội chứng HWW dựa trên siêu âm, MRI, và thám sát vách ngăn âm đạo trên lâm sàng. Bệnh nhân được điều trị với cầm máu, giảm gò, hỗ trợ phổi, Magnesium sulfate bảo vệ não và xuất viện sau 4 ngày. Bệnh nhân bị vỡ ối lúc thai 37 tuần 3 ngày, sinh một bé gái 2950g Apgar 8/9 với phương pháp mổ lấy thai do chuyển dạ đình trệ. Chẩn đoán và điều trị sớm là cần thiết. Công cụ chẩn đoán không xâm lấn thường là siêu âm và MRI. Trong thai kỳ, phần lớn phôi thai thường làm tổ ở buồng tử cung có âm đạo không bị tắc nghẽn. Biến chứng sản khoa thường gặp là sẩy thai tái phát, bất thường kiểu thế và cấu trúc thai nhi, thai chậm tăng trưởng trong tử cung, sinh non, tăng tỷ lệ mổ lấy thai, nhau bong non, sót nhau, ối vỡ non., Tóm tắt tiếng anh, Herlyn-Werner-Wunderlich (HWW) syndrome is a rare female urogenital congenital anomaly, characterized by a classic triad of uterus didelphys with blind hemivagina and ipsilateral renal agenesis. We presented a case of pregnancy with Herlyn-Werner-Wunderlich syndrome. A 25-year-old-women, G0P0, was admitted to our OB/GYN department at 32 weeks of gestational age with symptoms of premature labor. Before pregnancy, she was diagnosed with a bicorporeal uterus and unilateral renal agenesis. Herlyn-Werner-Wunderlich syndrome was diagnosed despite advance pregnancy using ultrasound, magnetic resonance imaging and visually observed of a longitudinal vaginal septum through a speculum. She was managed with tocolytic agent and antenatal corticosteroid. After 4 days of hospitalization, she was dischaged and readmitted again at 37+3 weeks due to rupture of membrane. Cesarean section was perfomed and she delivered a healthy girl. In Herlyn-WernerWunderlich syndrome, early diagnosis and management is essential. Ultrasonography and magnetic resonance imaging are reliable and accurate imaging methods for evaluation of female urogenital anomaly. In most pregnancy with HWW syndrome, implantation occurs in the hemi-uterus with unobstructed hemivagina. Obstetrics complications include recurrent pregnancy loss, fetal stuctural anomalies, malpresentation, intrauterine growth restriction, preterm rupture of membrane, preterm birth, placenta abruption, retained placenta, and increase risk of cesarean delivery.

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