妊娠合并结节性硬化症相关肾错构瘤孕晚期自发性破裂大出血一例  

Spontaneous rupture and massive hemorrhage of tuberous sclerosis-related renal hamartoma in a pregnant woman in third trimester:a case report

作　　者：刘翠莲 王润芳 李思思 尹晓茜 霍琰[1] Liu Cuilian;Wang Runfang;Li Sisi;Yin Xiaoqian;Huo Yan(Department of Obstetrics,Hebei General Hospital,Shijiazhuang 050000,China)

机构地区：[1]河北省人民医院产科,石家庄050000

出　　处：《中华围产医学杂志》2020年第11期774-777,共4页Chinese Journal of Perinatal Medicine

摘　　要：本文报道1例妊娠合并结节性硬化症相关肾错构瘤孕晚期自发性破裂大出血患者的诊疗过程。患者入院时孕39周,先兆临产,但隐瞒了既往双侧肾错构瘤病史。入院时因持续性腰腹痛、腹肌紧张、子宫张力大、胎心率下降至90次/min,考虑胎盘早剥于孕39周+1行急诊手术。术中见腹腔不凝血约200 ml,行剖宫产术娩出一活男婴,胎盘母体面未见胎盘早剥迹象。缝合子宫后探查右侧腹膜后一巨大青紫色包块,肾动脉造影示肾错构瘤破裂出血,遂行选择性右侧肾动脉栓塞术。术后7 d恢复可,家属要求出院观察。新生儿出生体重2355 g,因重度窒息转新生儿科治疗8 d,生后查心脏彩超提示心脏横纹肌瘤可能,癫痫发作1次,一般情况良好出院。随访患者至产后30 d,除有血尿外一般情况良好,继续口服依维莫司治疗并定期随访,新生儿出院后未再次发作癫痫。本病例提示临床应重视妊娠合并肾错构瘤患者,若妊娠期出现腹部异常膨隆、血尿及腰痛等表现,需警惕肾错构瘤破裂大出血可能。The diagnosis and treatment of spontaneous rupture and massive hemorrhage of tuberous sclerosis-related renal hamartoma in a woman in the third trimester are reported.The patient was admitted at 39 weeks of gestation,with threatened labor and a history of bilateral renal hamartoma,which had been hidden.Placental abruption was considered due to persistent lumbago,abdominal pain,abdominal muscle tension,uterine tension and fetal heart rate dropping to 90 bpm,and an emergency cesarean section was performed at 39+1 weeks.About 200 ml of bloody ascites was found in the peritoneal cavity.A live boy was delivered and no blood clot was seen in the maternal face of the placenta.After the uterine incision was closed,a huge bluish purple mass was detected on the right-side retroperitoneum and the renal angiography showed rupture and hemorrhage of a right renal hamartoma.A selective right renal artery embolization was performed.The patient recovered after the operation and was discharged seven days later required by the family.The patient was in good condition except for hematuria during a 30-day postpartum follow-up,and oral everolimus treatment and regular follow-up were continued.The newborn with a birth weight of 2355 g was transferred to the neonatology department after birth due to severe asphyxia,and postnatal echocardiography suspected heart rhabdomyoma.The baby had one seizure but was otherwise well,and was discharged after eight days.The seizure did not recur to the neonate after discharge.Clinicians should pay attention to pregnant women with renal hamartoma.If abnormal abdominal distension,hematuria or lumbago occur during pregnancy,rupture of renal hamartoma and possible massive hemorrhage should be considered.

关 键 词：妊娠并发症 结节性硬化症 肾错构瘤破裂出血 胎儿心脏横纹肌瘤 

分 类 号：R714.2[医药卫生—妇产科学] R737.11[医药卫生—临床医学]