Repetitive cerebral bleeding in an adult with Klippel-Trénaunay Syndrome

Petzold, Axel; Bischoff, Christian; Conrad, Bastian

doi:10.1007/s004150050609

Cited by 4 publications

(10 citation statements)

References 20 publications

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“…KTWS is diagnosed when at least two of these cardinal features are met. Other signs in patients with KTWS include bleeding, thrombophlebitis, lymphatic malformation and lymphedema, hyperpigmentation, ankle ulcers and other dermal findings of varicosis [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Glioblastoma multiforme in Klippel-Trenaunay-Weber syndrome: a case report

et al. 2015

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IntroductionKlippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome in which patients usually present with cutaneous hemangiomas, venous varicosities, and bone and soft tissue hypertrophy of the affected limb. Intracranial lesions in patients with KTWS are extremely rare, and are generally reported as single cases in the literature. We describe a rare case, where a patient with KTWS was found with a hemorrhagic grade IV astrocytoma. Although central nervous system abnormalities such as intracranial aneurysms and cerebral and spinal cord cavernomas have been described in patients with KTWS, to the best of our knowledge, this is the first report of an association between glioblastoma multiforme (grade IV astrocytoma) and KTWS in the English-language medical literature.Case presentationA 61-year-old white Caucasian man with a history of KTWS presented with seizures. Left upper and lower extremity hypertrophy, left foot, leg and ear gigantism and left-sided abdominal capillary hemangiomas were noted in the physical examination. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) were obtained, showing a heterogeneous lesion in the cingulate gyrus, with peripheral and central areas of T1 hyperintensity and layering T2 hypointensity consistent with a hemorrhage. A right parasagittal frontal craniotomy was performed with an interhemispheric approach. We had difficulty controlling the bleeding with bipolar electrocautery during surgery and finally were able to stop the bleeding using surgicel and gelfoam. Postoperative cranial CT and MRI scans showed intraparenchymal hemorrhage centered within the medial right frontal lobe. There was no increase in hematoma size in consecutive CT scans.ConclusionsCo-occurrence of vascular abnormalities with KWTS should be taken into consideration to avoid perilous preoperative and postoperative complications.

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Section: Discussionmentioning

confidence: 99%

Glioblastoma multiforme in Klippel-Trenaunay-Weber syndrome: a case report

et al. 2015

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“…Central nervous system involvement in Klippel Trenaunay Syndrome is considered to be extremely uncommon [ 3 , 7 ]. Arteriovenous malformation and cavernous angioma of brain and spinal cord, intracranial aneurysms, anomalies of the circle of Willis, macrocephaly, microcephaly, hemihypertrophy, hemimegalencephaly, etc.…”

Section: Discussionmentioning

confidence: 99%

“…Occasionally it may result in life threatening bleeding from gastrointestinal tract, genitourinary tract and central nervous system [ 2 ]. Intraparenchymal and subarachnoid hemorrhages are the two forms of intracranial hemorrhage reported in Klippel Trenaunay Syndrome [ 3 ]. Unlike in other locations, intracranial hemorrhage in Klippel Trenaunay Syndrome has been traced mainly to an underlying arteriovenous malformation [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…Intraparenchymal and subarachnoid hemorrhages are the two forms of intracranial hemorrhage reported in Klippel Trenaunay Syndrome [ 3 ]. Unlike in other locations, intracranial hemorrhage in Klippel Trenaunay Syndrome has been traced mainly to an underlying arteriovenous malformation [ 3 ]. To the best of our knowledge no report exists in the English literature on subdural hemorrhage and a venous source of intracranial bleed in Klippel Trenaunay Syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Subdural Hemorrhage in a Child with Klippel Trenaunay Syndrome

Thamburaj¹,

Nguyen²,

Choudhary³

et al. 2008

Radiology Case Reports

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A 10-year-old girl with features of Klippel Trenaunay Syndrome developed a large left frontoparietal subdural hemorrhage. CT angiography and cerebral angiography identified prominent subependymal veins and deep venous system predominantly in the left cerebral hemisphere in association with a dilated left vein of Labbe and hypoplastic superior sagittal sinus. No arteriovenous malformation or fistula was identified. Intracranial hemorrhage in Klippel Trenaunay Syndrome is extremely rare and usually traced to an underlying arteriovenous malformation or fistula. To the best of our knowledge neither a venous source of intracranial bleed nor subdural hemorrhage in Klippel Trenaunay Syndrome has been described in the English literature.

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“…Occasionally spontaneous intracranial haemorrhage may occur in the apparent absence of arteriovenous malformations or aneurysms. Some authors argue that such cases (often described as Klippel-Trenaunay with Sturge-Weber, or Klippel-Trenaunay-Weber syndrome) are, in fact, misdiagnosed cases of Sturge-Weber syndrome with limb involvement 4,12,14,[30][31][32] , as is likely in case 3.…”

Section: Klippel-trenaunay Syndromementioning

confidence: 99%