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两例产妇产前脾破裂及低血压与子代的默比厄斯综合征和脑瘫相关。默比厄斯综合征及部分脑瘫病例存在子宫胎盘血管病因的进一步证据。

Two cases of maternal antenatal splenic rupture and hypotension associated with Moebius syndrome and cerebral palsy in offspring. Further evidence for a utero placental vascular aetiology for the Moebius syndrome and some cases of cerebral palsy.

作者信息

Lipson A H, Gillerot Y, Tannenberg A E, Giurgea S

机构信息

Department of Clinical Genetics, New Children's Hospital, Parramatta, Sydney, NSW, Australia.

出版信息

Eur J Pediatr. 1996 Sep;155(9):800-4. doi: 10.1007/BF02002911.

Abstract

UNLABELLED

We wish to report two cases of congenital abnormality after antenatal car accidents resulting in ruptured spleen and severe hypotension in the mothers at 8 and 14 weeks gestation. The first case had the classical Moebius syndrome with 6th and 7th cranial nerve palsy with abnormal brain stem evoked responses, presumably due to hypoxic/ischaemic brain stem damage and the second case had severe retardation and hypertonic cerebral palsy which at post mortem was found to be due to old hypoxic/ischaemic lesions to the caudate nucleus putamen and striatum.

CONCLUSION

The cases described provide evidence that severe maternal hypotension during pregnancy can be associated with lesions to the midbrain and brain stem of offspring. The mechanism is probably utero-placental insufficiency, and extrapolation from these two unusual cases would support utero-placental insufficiency as a cause of Moebius syndrome and limb deficiency after chorionic villus sampling.

摘要

未标注

我们希望报告两例产前车祸后出现先天性异常的病例,两名母亲在妊娠8周和14周时分别出现脾破裂和严重低血压。第一例患有典型的莫比乌斯综合征,伴有第6和第7颅神经麻痹以及异常的脑干诱发反应,推测是由于缺氧/缺血性脑干损伤所致;第二例有严重智力发育迟缓及高张力型脑瘫,尸检发现是由于尾状核、壳核和纹状体陈旧性缺氧/缺血性病变所致。

结论

所描述的病例提供了证据,表明孕期母亲严重低血压可能与子代中脑和脑干病变有关。其机制可能是子宫-胎盘功能不全,从这两例罕见病例推断,子宫-胎盘功能不全可能是莫比乌斯综合征以及绒毛取样后肢体缺陷的一个原因。

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