Angioosteohypertrophy Syndrome

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Lucian Cracana - One of the best experts on this subject based on the ideXlab platform.

  • Klippel-Trenaunay-Weber Syndrome – A case report and review of the literature
    Archive of Clinical Cases, 2014
    Co-Authors: Florentina Anca Danciu, Catalina Elena Bistriceanu, Lucian Cracana
    Abstract:

    Klippel-Trenaunay-Weber Syndrome (KTW Syndrome), also known as congenital dysplastic angiopathy or Angioosteohypertrophy Syndrome, is a rare, sporadic, complex malformation that involves congenital malformations of veins, capillaries, and/or lymphatics, which leads to soft tissue hypertrophy and port-wine stains. We report a case of a 37 year old woman with congenital Klippel Trenaunay Weber Syndrome. She had many vascular abnormalities from birth which increased in time such that she had many surgical interventions. These abnormalities of veins and capillaries were leading to hypertrophy of the extremities – finger macrosomia. She had orthopedic comorbidities like Chopart amputation of the anterior part of her left foot and dorsal kyphoscoliosis because of the external scars. Studying similar cases from the literature, we found that Klippel-Trenaunay-Weber Syndrome could be associated with brain abnormalities. We admitted the patient for additional investigations because some of these patients could have cerebral malformations in the absence of neurologic abnormalities. We considered at that time that it was necessary to look for any unknown vascular abnormality. Management of this Syndrome should aim to correct any of the abnormalities present, if technically possible, and if the abnormality is causing symptoms.

Florentina Anca Danciu - One of the best experts on this subject based on the ideXlab platform.

  • Klippel-Trenaunay-Weber Syndrome – A case report and review of the literature
    Archive of Clinical Cases, 2014
    Co-Authors: Florentina Anca Danciu, Catalina Elena Bistriceanu, Lucian Cracana
    Abstract:

    Klippel-Trenaunay-Weber Syndrome (KTW Syndrome), also known as congenital dysplastic angiopathy or Angioosteohypertrophy Syndrome, is a rare, sporadic, complex malformation that involves congenital malformations of veins, capillaries, and/or lymphatics, which leads to soft tissue hypertrophy and port-wine stains. We report a case of a 37 year old woman with congenital Klippel Trenaunay Weber Syndrome. She had many vascular abnormalities from birth which increased in time such that she had many surgical interventions. These abnormalities of veins and capillaries were leading to hypertrophy of the extremities – finger macrosomia. She had orthopedic comorbidities like Chopart amputation of the anterior part of her left foot and dorsal kyphoscoliosis because of the external scars. Studying similar cases from the literature, we found that Klippel-Trenaunay-Weber Syndrome could be associated with brain abnormalities. We admitted the patient for additional investigations because some of these patients could have cerebral malformations in the absence of neurologic abnormalities. We considered at that time that it was necessary to look for any unknown vascular abnormality. Management of this Syndrome should aim to correct any of the abnormalities present, if technically possible, and if the abnormality is causing symptoms.

Catalina Elena Bistriceanu - One of the best experts on this subject based on the ideXlab platform.

  • Klippel-Trenaunay-Weber Syndrome – A case report and review of the literature
    Archive of Clinical Cases, 2014
    Co-Authors: Florentina Anca Danciu, Catalina Elena Bistriceanu, Lucian Cracana
    Abstract:

    Klippel-Trenaunay-Weber Syndrome (KTW Syndrome), also known as congenital dysplastic angiopathy or Angioosteohypertrophy Syndrome, is a rare, sporadic, complex malformation that involves congenital malformations of veins, capillaries, and/or lymphatics, which leads to soft tissue hypertrophy and port-wine stains. We report a case of a 37 year old woman with congenital Klippel Trenaunay Weber Syndrome. She had many vascular abnormalities from birth which increased in time such that she had many surgical interventions. These abnormalities of veins and capillaries were leading to hypertrophy of the extremities – finger macrosomia. She had orthopedic comorbidities like Chopart amputation of the anterior part of her left foot and dorsal kyphoscoliosis because of the external scars. Studying similar cases from the literature, we found that Klippel-Trenaunay-Weber Syndrome could be associated with brain abnormalities. We admitted the patient for additional investigations because some of these patients could have cerebral malformations in the absence of neurologic abnormalities. We considered at that time that it was necessary to look for any unknown vascular abnormality. Management of this Syndrome should aim to correct any of the abnormalities present, if technically possible, and if the abnormality is causing symptoms.

B. Kovacs - One of the best experts on this subject based on the ideXlab platform.

  • Klippel-Trénaunay-Weber Syndrome associated with abdominal aortic aneurysm in childhood.
    Journal of Vascular Surgery Cases and Innovative Techniques, 2015
    Co-Authors: Dittmar Böckler, Philiipp Erhart, Ingrid Haußer-siller, Elena Ellert, Hagen Meredig, B. Kovacs
    Abstract:

    Klippel-Trenaunay-Weber Syndrome (KTWS), also known as Angioosteohypertrophy Syndrome, is a rare congenital malformation with unknown etiology characterized by the combination of capillary malformations (port-wine strain), venous varicosities, and a soft tissue or bony hypertrophy of the affected limb. It is known to be rarely associated with abdominal aortic aneurysm (AAA) in adults. We report the first published case of KTWS and a rapidly progressing symptomatic AAA undergoing open repair in a child. This underlines the importance of AAA screening and treatment rather than surveillance in patients with KTWS.

Dittmar Böckler - One of the best experts on this subject based on the ideXlab platform.

  • Klippel-Trénaunay-Weber Syndrome associated with abdominal aortic aneurysm in childhood.
    Journal of Vascular Surgery Cases and Innovative Techniques, 2015
    Co-Authors: Dittmar Böckler, Philiipp Erhart, Ingrid Haußer-siller, Elena Ellert, Hagen Meredig, B. Kovacs
    Abstract:

    Klippel-Trenaunay-Weber Syndrome (KTWS), also known as Angioosteohypertrophy Syndrome, is a rare congenital malformation with unknown etiology characterized by the combination of capillary malformations (port-wine strain), venous varicosities, and a soft tissue or bony hypertrophy of the affected limb. It is known to be rarely associated with abdominal aortic aneurysm (AAA) in adults. We report the first published case of KTWS and a rapidly progressing symptomatic AAA undergoing open repair in a child. This underlines the importance of AAA screening and treatment rather than surveillance in patients with KTWS.