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Klippel-trénaunay syndrome - a very rare and interesting syndrome.

Sharma D, Lamba S, Pandita A, Shastri S - Clin Med Insights Circ Respir Pulm Med (2015)

Bottom Line: Klippel-Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy.We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS.Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Neonatology, Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh, India.

ABSTRACT
Klippel-Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.

No MeSH data available.


Related in: MedlinePlus

The left foot shows oligodactyly and syndactyly.
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Related In: Results  -  Collection


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f4-ccrpm-9-2015-001: The left foot shows oligodactyly and syndactyly.

Mentions: A 12-year-old boy presented to us with the symptoms of verrucous plaques over both lower limbs of sizes 12 cm × 6 cm × 11 cm; certain areas over the plaque showed ulceration and occasional bleeding on minor trauma. In summation, the patient had multiple fluid-filled lesions intermingled with verrucous papules and plaques with enlargement of the left lower limb. On detailed physical examination, he had severe anemia (hemoglobin 6 mg/dL). His heart rate was 100/minute, which was regular, and blood pressure was 100/68 mmHg in the right upper arm. He had normothermia, with the absence of any jaundice, cyanosis, clubbing, and lymph node enlargement. He had marked hypertrophy of the left lower limb. There was a large port-wine stain on most of the left lower limb, except the upper medial portion of the thigh (Fig. 1). Detailed examination showed multiple distinct red to bluish black papulonodular angiokeratomas present over the port-wine stain. Similar lesions were also present over the right lower half of thigh extending over the knee joint and left buttock (Fig. 2). Gross hypertrophy was evident over the right knee joint. A few lesions were on the right buttock with involvement of the perianal area (Fig. 3). Lymphangiectatic lesions were abundant in the perianal area. The left foot had oligodactyly and syndactyly (Fig. 4). In measuring both the lower limbs, they were found to be of equal length. The mental condition according to the index event was as per age, and he did not have any intellectual deficits.


Klippel-trénaunay syndrome - a very rare and interesting syndrome.

Sharma D, Lamba S, Pandita A, Shastri S - Clin Med Insights Circ Respir Pulm Med (2015)

The left foot shows oligodactyly and syndactyly.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4356473&req=5

f4-ccrpm-9-2015-001: The left foot shows oligodactyly and syndactyly.
Mentions: A 12-year-old boy presented to us with the symptoms of verrucous plaques over both lower limbs of sizes 12 cm × 6 cm × 11 cm; certain areas over the plaque showed ulceration and occasional bleeding on minor trauma. In summation, the patient had multiple fluid-filled lesions intermingled with verrucous papules and plaques with enlargement of the left lower limb. On detailed physical examination, he had severe anemia (hemoglobin 6 mg/dL). His heart rate was 100/minute, which was regular, and blood pressure was 100/68 mmHg in the right upper arm. He had normothermia, with the absence of any jaundice, cyanosis, clubbing, and lymph node enlargement. He had marked hypertrophy of the left lower limb. There was a large port-wine stain on most of the left lower limb, except the upper medial portion of the thigh (Fig. 1). Detailed examination showed multiple distinct red to bluish black papulonodular angiokeratomas present over the port-wine stain. Similar lesions were also present over the right lower half of thigh extending over the knee joint and left buttock (Fig. 2). Gross hypertrophy was evident over the right knee joint. A few lesions were on the right buttock with involvement of the perianal area (Fig. 3). Lymphangiectatic lesions were abundant in the perianal area. The left foot had oligodactyly and syndactyly (Fig. 4). In measuring both the lower limbs, they were found to be of equal length. The mental condition according to the index event was as per age, and he did not have any intellectual deficits.

Bottom Line: Klippel-Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy.We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS.Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Neonatology, Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh, India.

ABSTRACT
Klippel-Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.

No MeSH data available.


Related in: MedlinePlus