Post partum haemorrhage secondary to Marfan's disease of the uterine vasculature

Irons, D. W.; Pollard, Kerry

doi:10.1111/j.1471-0528.1993.tb15244.x

Cited by 7 publications

(4 citation statements)

References 9 publications

(14 reference statements)

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“…During histopathological analysis, fragmentation of the elastic lamina of the dilated vessels and mucoid degeneration of the walls was noted. In this case, the postpartum hemorrhage was attributed to vascular modification secondary to the Marfan syndrome [14].…”

Section: Discussionmentioning

confidence: 80%

“…Few cases have been reported of postpartum hemorrhage in Marfan syndrome. Some authors suspect that degenerative vascular change may be present in the uterine vessels and that pregnancy may precipitate or exacerbate dilatation and dissection of already diseased vessels [14]. Recently, one case of postpartum hemorrhage was reported in a woman with Marfan syndrome presenting numerous dilated and tortuous blood vessels on the inner uterine aspect.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous Bilateral Broad Ligament Rupture Associated with a Connective Tissue Disorder

Weber¹

2017

JGWH

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Background: Hemoperitoneum secondary to spontaneous rupture of uterine vessels in the broad ligament during pregnancy usually results from trauma or varicose vein dilatation, leading to uterine vessel rupture.Case: A 33-year-old primigravida presented to the hospital for abdominal pain. Fetal distress led to an urgent caesarean. Abdominal exploration revealed hemoperitoneum secondary to bilateral lesion of the broad ligament and abnormal hysterotomia extension. The patient was referred for genetic evaluation of connective tissue disease. A fibrillin-1 (FBN-1) mutation was discovered; a mutation associated with Marfan syndrome. Conclusion:Although this patient had no other signs suggesting Marfan syndrome, the FBN-1 mutation may explain the bilateral broad ligament rupture without trauma. We recommend screening for connective tissue disease in patients with this complication.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Spontaneous Bilateral Broad Ligament Rupture Associated with a Connective Tissue Disorder

Weber¹

2017

JGWH

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“…Recently, in addition to the well-characterized aortic abnormalities, there have been reports in MFS patients of aneurysm, dissection, dilatation, or rupture of the iliac, subclavian, carotid, uterine, and pulmonary arteries [8][9][10]. In contrast, renal involvement in MFS has been reported sporadically and has focused on structural abnormalities such as ectopia and duplex kidney [4].…”

Section: Discussionmentioning

confidence: 98%

Renovascular hypertension in Marfan syndrome

Baum

Harris

Burrows

et al. 1997

Pediatric Nephrology

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A defect in fibrillin integrity predisposes patients with Marfan syndrome to vascular wall abnormalities, most notably aortic rupture and dissection. Renal vascular anomalies have not been described previously in children with Marfan syndrome. In this report, we detail data from a hypertensive 14-year-old girl with clinical stigmata of Marfan syndrome and a diagnostic evaluation significant for characteristic aortic root dilatation and aneurysm, as well as a disparity in renal size and function exacerbated by captopril administration. Renal arteriography confirmed a left main renal artery stenosis that was not amendable to balloon angioplasty. Surgical resection resulted in significant improvement in hypertension. Pathological examination of the resected renal artery segment revealed intimal proliferation, fragmentation of the elastic media, and inner medial dissection. This patient demonstrates that, in addition to the aorta, renal arteries can be affected with the characteristic vascular wall pathology of Marfan syndrome, resulting in systemic hypertension. These data suggest that children with Marfan syndrome and hypertension need to be evaluated carefully for the presence of renal anomalies.

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“…Die Sectio-Hysterektomie wurde in Absprache mit der Patientin und dem Ehemann aus prophylaktischen und therapeutischen Gründen durchgeführt. Zum einen sind postpartale Hämorrhagien im Zusammenhang mit MFS beschrieben worden [15,16,17]. Eine Erklärung könnte die mangelnde Kontraktilität der Spiralarterien sein, wie es auch bei anderen Bindegewebserkrankungen, z.…”

Section: Diskussionunclassified

Aortendissektion in der Gravidität bei Marfan-Syndrom - Fallbericht und Behandlungskonzept1

Preiß

Hösli²,

Holzgreve³

et al. 2001

Z Geburtshilfe Neonatol

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Post partum haemorrhage secondary to Marfan's disease of the uterine vasculature

Cited by 7 publications

References 9 publications

Spontaneous Bilateral Broad Ligament Rupture Associated with a Connective Tissue Disorder

Spontaneous Bilateral Broad Ligament Rupture Associated with a Connective Tissue Disorder

Renovascular hypertension in Marfan syndrome

Aortendissektion in der Gravidität bei Marfan-Syndrom - Fallbericht und Behandlungskonzept1

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