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[血管平滑肌脂肪瘤、结节性硬化症与妊娠]

[Angiomyolipomas, tuberous sclerosis and pregnancy].

作者信息

Illescas Molina Tamara, Montalvo Montes Joaquín, Contreras Cecilia Esther, Muñoz Muñiz María del Mar, González González Antonio, Herraiz Martínez Miguel Angel

机构信息

Servicio de Ginecología y Obstetricia, Hospital Clínico San Carlos, Madrid, España.

出版信息

Ginecol Obstet Mex. 2009 Aug;77(8):380-6.

PMID:19902629
Abstract

Renal angiomyolipomas are uncommon tumours during pregnancy, but it's important to consider them in the differential diagnosis, for the increased risk of complications such as bleeding, specially during pregnancy. Their characteristics are different depending on if the tumour is associated or not with facomatosis. To show both possibilities, we describe two cases of pregnant women with angiomyolipomas: the first one presented with bleeding of the tumour (Wünderlich's syndrome), but surveillance without treatment was possible until 37th week of gestation, and embolization of the tumour was performed afterwards. The second patient had multiple well known angiomyolipomas, associated to tuberous sclerosis; even though she had required two previous embolizations, she had an uneventful pregnancy, without any episodes of bleeding. Both pregnancies ended successfully by means of a cesarean section. Since there are few papers available in the literature, there is not an agreement about therapeutic management when pregnant women present with bleeding angiomyolipomas (Wünderlich's syndrome). Treatment options include partial or total nephrectomy (with cesarean section simultaneously in patients at 28 weeks of gestation or later), transcateter selective arterial embolization (which can also be preventive), and sometimes, conservative management. If this last option is possible, there's still discussion about the risk of vaginal delivery compared with that of cesarean section, in terms of bleeding of the tumour.

摘要

肾血管平滑肌脂肪瘤在孕期并不常见,但在鉴别诊断时考虑到它们很重要,因为其存在出血等并发症风险增加,尤其是在孕期。根据肿瘤是否与错构瘤病相关,其特征有所不同。为展示这两种可能性,我们描述了两例患有血管平滑肌脂肪瘤的孕妇病例:第一例出现肿瘤出血(肾出血性梗死综合征),但在妊娠37周前可行观察等待而不进行治疗,之后对肿瘤进行了栓塞治疗。第二例患者有多个已知的血管平滑肌脂肪瘤,与结节性硬化症相关;尽管她之前已接受过两次栓塞治疗,但孕期顺利,未发生任何出血事件。两次妊娠均通过剖宫产成功结束。由于文献中可用的论文较少,对于患有出血性血管平滑肌脂肪瘤(肾出血性梗死综合征)的孕妇,在治疗管理方面尚无共识。治疗选择包括部分或全肾切除术(妊娠28周及以后的患者同时行剖宫产)、经导管选择性动脉栓塞(也可作为预防性措施),有时也采用保守治疗。如果可以选择最后一种方案,在肿瘤出血方面,关于阴道分娩与剖宫产的风险仍存在争议。

相似文献

1
[Angiomyolipomas, tuberous sclerosis and pregnancy].[血管平滑肌脂肪瘤、结节性硬化症与妊娠]
Ginecol Obstet Mex. 2009 Aug;77(8):380-6.
2
Conservative management of a bleeding renal angiomyolipoma in pregnancy.妊娠期出血性肾血管平滑肌脂肪瘤的保守治疗
Obstet Gynecol. 2006 Feb;107(2 Pt 2):490-2. doi: 10.1097/01.AOG.0000167392.59472.c3.
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[Bilateral renal angiomyolipoma associated with tuberous sclerosis. Right nephrectomy following arterial embolization].[双侧肾血管平滑肌脂肪瘤伴结节性硬化症。动脉栓塞后行右肾切除术]
Actas Urol Esp. 1994 Feb;18(2):145-8.
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Wunderlich's syndrome due to spontaneous rupture of large bilateral angiomyolipomas.双侧巨大血管平滑肌脂肪瘤自发性破裂所致的温德利希综合征。
Emerg Med J. 2009 Jan;26(1):72. doi: 10.1136/emj.2008.062091.
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Conservative management of pulmonary lymphangioleiomyomatosis and tuberous sclerosis complicated by renal angiomyolipomas in pregnancy.孕期肺淋巴管平滑肌瘤病和结节性硬化症合并肾血管平滑肌脂肪瘤的保守治疗
J Matern Fetal Neonatal Med. 2004 Feb;15(2):132-4. doi: 10.1080/14767050410001659879.
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The Wunderlich's syndrome secondary to massive bilateral angiomyolipomas associated with advanced tuberous sclerosis.继发于与晚期结节性硬化症相关的双侧巨大血管平滑肌脂肪瘤的温德利希综合征。
Saudi J Kidney Dis Transpl. 2011 May;22(3):534-7.
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Management of renal angiomyolipomas associated with tuberous sclerosis complex.与结节性硬化症相关的肾血管平滑肌脂肪瘤的管理
J Urol. 2004 Jan;171(1):102-5. doi: 10.1097/01.ju.0000100100.36354.61.
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[The clinical aspects and treatment of renal angiolipomas in patients with tuberous sclerosis].[结节性硬化症患者肾血管平滑肌脂肪瘤的临床特点与治疗]
Ned Tijdschr Geneeskd. 1990 Aug 11;134(32):1556-8.
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Conservative management of giant symptomatic angiomyolipomas in patients with the tuberous sclerosis complex.结节性硬化症患者巨大有症状血管平滑肌脂肪瘤的保守治疗
BJU Int. 2007 Oct;100(4):794-7. doi: 10.1111/j.1464-410X.2007.07059.x. Epub 2007 Jun 21.
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Renal angiomyolipoma: optimal treatment based on size and symptoms.肾血管平滑肌脂肪瘤:基于大小和症状的最佳治疗方法。
Clin Nephrol. 1998 May;49(5):281-6.

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Wunderlich's syndrome in pregnancy: a shocking triad.妊娠期温德利希综合征:一种令人震惊的三联征。
BMJ Case Rep. 2019 May 19;12(5):e229219. doi: 10.1136/bcr-2019-229219.
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Renal angiomyolipoma during pregnancy: Case report and literature review.妊娠期肾血管平滑肌脂肪瘤:病例报告及文献复习
Turk J Obstet Gynecol. 2015 Jun;12(2):118-121. doi: 10.4274/tjod.32848. Epub 2015 Jun 15.
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Successful Pregnancy Outcome in a Patient with Solitary Kidney Affected by Angiomyolipoma: A Rare Case.肾血管平滑肌脂肪瘤累及单肾患者的成功妊娠结局:1例罕见病例
J Clin Diagn Res. 2016 Oct;10(10):QD06-QD07. doi: 10.7860/JCDR/2016/21448.8670. Epub 2016 Oct 1.
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Giant bilateral renal angiomyolipomas and lymphangioleiomyomatosis presenting after two successive pregnancies successfully treated with surgery and rapamycin.巨大双侧肾血管平滑肌脂肪瘤和淋巴管平滑肌瘤病在两次连续妊娠后出现,经手术和雷帕霉素成功治疗。
ScientificWorldJournal. 2011;11:2115-23. doi: 10.1100/2011/263137. Epub 2011 Nov 2.