Klippel-Trenaunay-Type Syndrome in Pregnancy

Stein, Susan; Perlow, Jordan H.; Sawai, Shirley K.

doi:10.1097/01.ogx.0000201924.87671.90

Cited by 41 publications

(49 citation statements)

References 37 publications

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“…Disseminated intravascular coagulation has also been reported in patients with KTS after minor and major episodes of bleeding. 10 Large hemangiomas and varicosities can trap large amounts of blood as well as sequester and destroy platelets, activating the coagulation cascade, eventually resulting in intravascular coagulopathy, as seen in our patient. Surprisingly, our patient had undergone an uneventful cesarean section 9 years earlier.…”

Section: Discussionmentioning

confidence: 74%

“…Stripping the superfi cial varicosities and orthopedic surgery for limb discrepancies can be done if it becomes symptomatic. 10 Elective splenectomy might be recommended as a treatment option for KTS patients with hemangiomas of the spleen or splenomegaly causing compression symptoms. 5 Although visceral involvement is rare, gastrointestinal, genitourinary, vaginal, and intraabdominal bleeding may occur in the presence of a visceral malformation.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: Report of a case

Karakayalı

Başaran²,

Soy³

et al. 2010

Surg Today

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We report a case of Klippel-Trenaunay syndrome (KTS) with serious morbidity caused by the rupture of hemangiomas of the spleen and inferior epigastric artery (IEA). A 40-year-old woman, who had suffered from edema and varicose veins in her left leg and toes since birth, underwent emergency laparotomy and splenectomy for a spontaneous splenic rupture. Pathological examination revealed hemangiomatosis of the spleen. She presented again 40 days later with a rectus muscle hematoma, which computed tomography revealed to be actively bleeding. Arteriography confirmed a bleeding IEA, which was then embolized. Hematological investigation revealed a heterozygous form of factor VIII and fibrinogen deficiency. The patient recovered well and was asymptomatic at her 1-year follow-up. We report this case to reinforce that investigations for KTS should involve all organ systems, and include detailed hematologic tests. By defining coagulation and vascular abnormalities, life-threatening bleeding episodes may be prevented.

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Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 99%

Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: Report of a case

Karakayalı

Başaran²,

Soy³

et al. 2010

Surg Today

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“…AVM are lined by dysplastic cells exposed to stagnated blood, which can trigger chronic localized intravascular coagulation [21]. DIC has been reported in Klippel-Trenaunay patients; a syndrome of slowflow vascular malformations and skeletal and soft tissue hypertrophy [22,23]. Nevertheless, DIC triggered by AVM was never reported in CLOVES.…”

Section: Discussionmentioning

confidence: 99%

Arteriovenous Malformation in CLOVES Syndrome

Abu-Haniyeh¹,

Alkukhun²,

Al-Natour³

et al. 2017

Med Rep Case Stud

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CLOVES syndrome (Congenital Lipomatous Overgrowth, Vascular malformations, and Epidermal nevi) is a rare and recently discovered syndrome. It is associated with vascular malformations and abnormal fatty tissue and skeletal growth. In this case, we report the clinical manifestations overtime in a patient with CLOVES syndrome who presented a unique arteriovenous malformation that led to severe neurological impairment by compressing the spinal cord. We also discuss the visceral findings in our patient, such as double ureter and splenomegaly, and describe the finding of corneal hydrops in both eyes and Chiari I malformation on brain imaging.

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“…Sie werden auch als «Portweinfleck» bezeichnet und sollten nicht mit den «fading capillary stains» verwechselt werden, welche bei vielen Neugeborenen beobachtet werden (ca. 40% [39]. Trotzdem ist eine venöse Malformation keine Kontraindikation für eine Schwangerschaft.…”

Section: Kapilläre Malformationunclassified

Vaskuläre Malformationen – Diagnostik und Behandlung

2014

Cardiovasc Med

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Summary Diagnosis and treatment of vascular malformationsVascular malformations are congenital vessel anomalies that may affect the entire vascular tree. They may be isolated, such as venous malformations, or occur in combination, such as arterio-venous malformations. Depending on their size and localization, vascular malformations can be associated with marked morbidity. As these disorders are rare and frequently misdiagnosed, they often result in inappropriate treatment.

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Klippel-Trenaunay-Type Syndrome in Pregnancy

Cited by 41 publications

References 37 publications

Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: Report of a case

Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: Report of a case

Arteriovenous Malformation in CLOVES Syndrome

Vaskuläre Malformationen – Diagnostik und Behandlung

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