Klippel-Trenaunay Syndrome: a case report with brief review of literature

Zea, Mohammad Iqbal; Hanif, Mohammad; Habib, Mohammad Asadul; Ansari, Ahmed

doi:10.3315/jdcr.2009.1038

Cited by 23 publications

(50 citation statements)

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“…The prevalence of deep venous aplasia or hypoplasia, as detected with venographic techniques (ascending venography and varicography), ranges from 18% [27] to 40% [26]. Lymphatic malformations have also been common in up to 70% of cases [1], that include primary lymphaedema, cystic hygroma or lymphangiectasia associated with reflux of chyle [29].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Multidisciplinary management approach for KTS is warranted [3]. The paediatrician, internist, phlebologist, orthopaedic, plastic and vascular surgeons, interventional radiologist, cardiologist, vascular internist and a physical therapy physician can all be involved in KTS patient's management [1].…”

Section: Managementmentioning

confidence: 99%

“…Rarely, patients with KTS can have intraosseous vascular malformations [1]. Genitourinary system involvement includes the penis, scrotum, vagina, vulva, and bladder.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…In patients with a history of recurrent cellulitis, intermittent or prophylactic antibiotics may be considered [1].…”

Section: Managementmentioning

confidence: 99%

“…It has three major features: 1) vascular naveus, 2) hypertrophy of soft tissue and bony overgrowth, and 3) varicose veins. It has a wide spectrum of presentation, from truncular involvement to extratruncular and from infiltrating to limited form of presentation [1][2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%

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Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

Hannawi¹

2013

J Clin Exp Dermatol Res

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Introduction: Klippel Trenaunay Syndrome (KTS) is a rare, congenital malformation. Several theories have been postulated to describe its pathogenesis. However, the exact etiology is not known. It's characterized by a triad of (1) haemangioma due to capillary malformation, (2) bone and soft tissue hypertrophy, and (3) varicose veins. Interestingly, lipoatropy rather than hypertropy of the involved limbs had been described in some cases. The clinical presentation of this syndrome is variable ranging from minimal disease to sever presentation such as significant cosmetic disfiguring, life threatening bleeding and embolism. The KTS is classified according to severity. This is important step in away to educate patients, to predict prognosis and to set treatments, especially in severe cases. Physicians should not only be attentive to the physical aspects but also to the psychological and social aspects of KTS. Case presentation:A 16-year-old boy presented with multiple port-wine stain type vascular anomalies and varicose veins. Also, there was a marked deforming enlargement of his right foot, right knee and number of his left toes with, striking gigantism of both lower limbs. This is consistent with clinical presentation of KTS. Furthermore, there were additional features matching myotonic dystrophy outlook; face showed a remarkable lipodystropy with bilateral upper limbs wasting and atrophy. This could represent the Inverse-Klippel Trenaunay Syndrome. According to our knowledge, this had never been reported to be associated with Klippel Trenaunay Syndrome Conclusion: Klippel Trenaunay and Inverse Klippel Trenaunay Syndrome can be seen in the same patient.

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Section: Clinical Presentationmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

“…Rarely, patients with KTS can have intraosseous vascular malformations [1]. Genitourinary system involvement includes the penis, scrotum, vagina, vulva, and bladder.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…In patients with a history of recurrent cellulitis, intermittent or prophylactic antibiotics may be considered [1].…”

Section: Managementmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

Hannawi¹

2013

J Clin Exp Dermatol Res

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Klippel-Trenaunay syndrome: a rare cause of disabling pain after a femoral fracture

Notarnicola

Pesce

Maccagnano

et al. 2012

Arch Orthop Trauma Surg

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Klippel-Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, as well as bone and soft tissue hypertrophy of the extremity and venous malformations. We present the case of a 52-year-old man with a femoral fracture and a history of haemangiomas, limb bone hypertrophy and varicosity. The patient was finally diagnosed with KTS and treated in mini-invasive surgery by endomedullary fracture nailing in general anaesthesia. Clinical management was particularly demanding, not only because of the need to monitor the risk of haemorrhage and thrombosis but also because of the onset of a rare picture of neuropathic pain with hyperalgesia and allodynia, never previously reported in patients affected by KTS, that required the administration of major opioid drugs. According to our knowledge, this is the second case of KTS managed for femoral fracture. Unlike the previous report in literature, in this case a severe disabling neuropathic pain complicated the clinical management.

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