Hội chứng Herlyn - Wener - Wunderlich và biến chứng ứ mủ âm đạo: báo cáo ca lâm sàng và tổng quan y văn

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Tác giả: Xuân Nghiêm Huỳnh, Bảo Trị Nguyễn, Trung Hiếu Nguyễn

Ngôn ngữ: vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y Dược học Phạm Ngọc Thạch, 2024

Mô tả vật lý: tr.215

Bộ sưu tập: Báo, Tạp chí

ID: 444466

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Tổng quan: Hội chứng Herlyn - Werner - Wunderlich (HWW) là một dị tật bẩm sinh hiếm gặp của đường tiết niệu - sinh dục nữ, bao gồm các bất thường của ống Mullerian và ống Mesonephric, đặc trưng bởi tam chứng: tử cung đôi, vách ngăn âm đạo một bên gây tắc nghẽn và bất sản thận cùng bên (hay còn gọi là hội chứng OHVIRA). Hội chứng này có thể được phân loại dựa trên việc tắc nghẽn hoàn toàn hoặc không hoàn toàn của một bên âm đạo. Triệu chứng thường xuất hiện ngay sau thời kỳ kinh nguyệt đầu tiên hoặc có thể biểu hiện muộn tùy thuộc vào loại bất thường. Triệu chứng phổ biến nhất là đau bụng dưới, thống kinh, và khối bất thường bụng dưới phát sinh do tử cung - âm đạo một bên bị tắc nghẽn, tích tụ máu (hematometra) hoặc tụ máu âm đạo (hematocolpos). Báo cáo ca lâm sàng: Chúng tôi trình bày về một bệnh nhân 15 tuổi, độc thân chưa quan hệ, mắc hội chứng OHVIRA điển hình đến bệnh viện Hùng Vương với tình trạng đau bụng dưới khi hành kinh nhiều và qua thăm khám phát có tình trạng ứ mủ nặng một bên vách ngăn âm đạo. Qua ca lâm sàng, chúng tôi mô tả vai trò của phương pháp hình ảnh trong chẩn đoán hội chứng Herlyn - Werner - Wunderlich và phương pháp điều trị phá ngăn âm đạo ứ mủ và may tái tạo cho bệnh nhân. Trên siêu âm và MRI, bệnh nhân có bất sản thận bên phải và có sự phì đại bù trừ của thận trái độc nhất, tử cung đôi với âm đạo bên phải có vách ngăn tắc nghẽn dẫn đến ứ căng mủ. Kết luận: Chẩn đoán và điều trị sớm hội chứng OHVIRA là cần thiết. Siêu âm và MRI là các phương pháp chẩn đoán hình ảnh tin cậy và chính xác để đánh giá các bất thường niệu dục ở phụ nữ. Abstract Background: Herlyn - Werner - Wunderlich syndrome (HWW) is a rare congenital anomaly of the female urogenital tract, involving abnormalities of the Mullerian ducts and Mesonephric ducts, characterized by a triad: double uterus, unilateral obstructed hemi - vagina, and ipsilateral renal agenesis (also known as OHVIRA syndrome). This syndrome can be classified based on complete or incomplete obstruction of one side of the vagina. Symptoms often manifest soon after the first menstrual period or may present later depending on the specific type. The most common symptoms include lower abdominal pain, menstrual pain, and an abnormal mass in the lower abdomen due to obstruction of one side of the uterus - vagina resulting in the accumulation of blood (hematometra) or vaginal blood accumulation (hematocolpos). Case report: We present a typical case of OHVIRA syndrome in a 15 - year - old unmarried patient who visited Hùng Vương Hospital with recurrent lower abdominal pain during menstruation and found to have a significant collection of pus in one side of the obstructed vaginal septum. In our clinical case report, we describe the role of imaging techniques in diagnosing Herlyn - Werner - Wunderlich syndrome and the treatment approach to relieve the obstructed vaginal pus collection and reconstruct the affected area for the patient. Imaging through ultrasound and MRI revealed unilateral renal agenesis and compensatory hypertrophy of the contralateral kidney, double uterus with an obstructed hemi - vagina leading to pus accumulation. Conclusion: In Herlyn - Werner - Wunderlich syndrome, early diagnosis and management is essential. Ultrasonography and magnetic resonance imaging are reliable and accurate imaging methods for evaluation of female urogenital anomaly. DOI: 10.59715/pntjmp.3.1.26

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