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胸腹联体双胎合并一胎心脏畸形临床精准诊治

Diagnosis and treatment of thoraco-omphalopagus conjoined twins with complex cardiac malformation in a fetus

  • 摘要:
    目的 介绍1对胸腹联体儿合并一胎心脏畸形的特殊治疗体会。
    方法 选择在复旦大学附属妇产科医院进行产前诊断并出生、在复旦大学附属儿科医院治疗的1对胸腹联体双胎,其中一胎合并心脏畸形,总结诊疗经过,分析特殊临床表现及治疗的个体化方案。
    结果 足月剖宫产,两患儿尿量差异大、体质量增加稍缓慢,且先心患儿在出生后1个月开始出现哭吵后青紫。于出生后38 d行分离术,术后9 d,先心患儿再次行B-T分流术。分离术后2周,无先心患儿顺利出院。先心患儿B-T分流术后20 d亦顺利出院。
    结论 胸腹联体虽常见,但合并心脏畸形增加手术风险,需结合心脏疾病程度与临床表现,制定个体化治疗方案,进而能提高救治成功率。

     

    Abstract:
    Objective To introduce the experience of the special treatment of the thoraco-omphalopagus conjoined twins, one of which has complex heart malformation.
    Methods A case of thoraco-omphalopagus conjoined twins, one was accompanied by congenital heart malformation, the twins who received the prenatal diagnosis and were born in the Obstetrics and Gynecology Hospital of Fudan University and received treatment in the Children's Hospital of Fudan University were selected. The related diagnosis, treatment, and specific clinical manifestation were summarized to provide individualized therapy.
    Results After the full-term cesarean section, two children were largely different in urine volume and gained weight relatively slowly. In addition, the child with heart malformation was observed cyanosis after noisy crying when she was 1-month old. The conjoined twins underwent separation on the 38th day after birth. Nine days after the separation, the child with heart malformation underwent Blalock-Taussig (BT) shunt. Two weeks after the separation, the child without heart disease was successfully discharged from the hospital. Twenty days after B-T shunts, the child complicated with congenital heart disease was also discharged successfully.
    Conclusions Although thoraco-omphalopagus is the most frequent type of conjoined twins, the risk of surgery increases when it is complicated with cardiac malformation. An individualized treatment plan should be formulated in consideration of the degree of heart disease and clinical manifestations, thus improving the success rate of treatment.

     

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