Abstract：
UNASSIGNED: Vascular Ehlers-Danlos syndrome also referred to as Ehlers-Danlos Type IV is an uncommon autosomal dominant genetic disorder linked to connective tissue abnormality. Its evolution is marked by the occurrence of severe vascular, digestive and obstetrical complications. The current case highlights the importance of early diagnosis and physician awareness about this disorder as it can improve the patient\'s prognosis.
METHODS: We present the case of a 34-year-old woman, who presented at 36 weeks of amenorrhea with labor pain. The labor evolution was marked by an increased fluctuating abdominal pain, a sudden loss of the fetal station detected during cervical examination and decelerations to 60 beats per min, leading to an emergency caesarean section. During the laparotomy, the patient presented a spontaneous bilateral extension of the cutaneous incision requiring the realization of stopping stitches. The fetus and placenta had been expelled via a 9 cm long uterine wall rupture also known as an open book uterine rupture. A live male infant weighting 2890 g was promptly delivered and transported to NICU for respiratory distress. Physical features typical of EDS-IV allowed us to suspect this disorder and genetic analysis identified the presence of COL3A1 gene mutation, confirming the diagnosis.
UNASSIGNED: Early recognition of Vascular Ehlers-Danlos syndrome is of paramount importance to improve the prognosis of affected patients, who often present themselves with life-threatening situations. Clinicians should maintain a high index of suspicion for the clinical signs of this inherited connective tissue disorder that is characterized by distinctive features.
METHODS: We present the case of a 34-year-old woman, who presented at 36 weeks of amenorrhea with labor pain. The labor evolution was marked by an increased fluctuating abdominal pain, a sudden loss of the fetal station detected during cervical examination and decelerations to 60 beats per min, leading to an emergency caesarean section. During the laparotomy, the patient presented a spontaneous bilateral extension of the cutaneous incision requiring the realization of stopping stitches. The fetus and placenta had been expelled via a 9 cm long uterine wall rupture also known as an open book uterine rupture. A live male infant weighting 2890 g was promptly delivered and transported to NICU for respiratory distress. Physical features typical of EDS-IV allowed us to suspect this disorder and genetic analysis identified the presence of COL3A1 gene mutation, confirming the diagnosis.
UNASSIGNED: Early recognition of Vascular Ehlers-Danlos syndrome is of paramount importance to improve the prognosis of affected patients, who often present themselves with life-threatening situations. Clinicians should maintain a high index of suspicion for the clinical signs of this inherited connective tissue disorder that is characterized by distinctive features.
摘要：
未经证实:血管Ehlers-Danlos综合征也称为Ehlers-Danlos综合征IV型是一种与结缔组织异常相关的不常见的常染色体显性遗传病。它的进化以严重血管的发生为标志，消化和产科并发症。目前的病例强调了早期诊断和医生对这种疾病的认识的重要性，因为它可以改善患者的预后。
方法：我们介绍一个34岁女性的案例，在闭经36周时出现分娩疼痛。分娩演变的特点是腹部疼痛的波动性增加，在子宫颈检查期间检测到胎儿站的突然丢失，并减速至每分钟60次，导致紧急剖腹产.在剖腹手术中,患者表现为皮肤切口的自发双侧延伸，需要实现停止缝合。胎儿和胎盘是通过9厘米长的子宫壁破裂而排出的，这也被称为开放式子宫破裂。体重为2890g的活男婴被迅速运送并运送到NICU进行呼吸窘迫。EDS-IV典型的身体特征使我们怀疑这种疾病，遗传分析确定了COL3A1基因突变的存在，确认诊断。
UNASSIGNED：早期识别血管Ehlers-Danlos综合征对改善患者预后至关重要，他们经常面临危及生命的情况。临床医生应该对这种以独特特征为特征的遗传性结缔组织疾病的临床症状保持高度怀疑。
方法：我们介绍一个34岁女性的案例，在闭经36周时出现分娩疼痛。分娩演变的特点是腹部疼痛的波动性增加，在子宫颈检查期间检测到胎儿站的突然丢失，并减速至每分钟60次，导致紧急剖腹产.在剖腹手术中,患者表现为皮肤切口的自发双侧延伸，需要实现停止缝合。胎儿和胎盘是通过9厘米长的子宫壁破裂而排出的，这也被称为开放式子宫破裂。体重为2890g的活男婴被迅速运送并运送到NICU进行呼吸窘迫。EDS-IV典型的身体特征使我们怀疑这种疾病，遗传分析确定了COL3A1基因突变的存在，确认诊断。
UNASSIGNED：早期识别血管Ehlers-Danlos综合征对改善患者预后至关重要，他们经常面临危及生命的情况。临床医生应该对这种以独特特征为特征的遗传性结缔组织疾病的临床症状保持高度怀疑。
