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Klippel-Trénaunay syndrome, pregnancy and the liver: an unusual interplay.

Samonakis DN, Oustamanolakis P, Manousou P, Kouroumalis EA, Burroughs AK - Ann Gastroenterol (2012)

Bottom Line: There is a well-established risk for thrombotic complications in these patients.A case of a young patient diagnosed post partum with the very rare liver involvement is presented.The complex clinical course, the multidisciplinary management and the long-term outcome are discussed.

View Article: PubMed Central - PubMed

Affiliation: The Royal Free Sheila Sherlock Liver Centre and University Department of Surgery, Royal Free Hospital Hampstead, London UK (Dimitrios N. Samonakis, Penelope Manousou, Andrew K. Burroughs) ; Department of Gastroenterology and Hepatology, University Hospital of Heraklion, Crete, Greece (Dimitrios N. Samonakis, Pantelis Oustamanolakis, Elias A. Kouroumalis).

ABSTRACT
Klippel-Trénaunay syndrome is a rare congenital syndrome characterized by capillary malformations, soft tissue and bone hypertrophy, and varicose veins. There is a well-established risk for thrombotic complications in these patients. A case of a young patient diagnosed post partum with the very rare liver involvement is presented. The complex clinical course, the multidisciplinary management and the long-term outcome are discussed.

No MeSH data available.


Related in: MedlinePlus

Limb asymmetry with right-side predominance
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Figure 1: Limb asymmetry with right-side predominance

Mentions: At referral, she was normotensive without encephalopathy. Pyrexia (38.8oC), mild tachycardia and moderately distended and tender abdomen were present. There were no cutaneous markers of chronic liver disease and no cardiac, respiratory or neurological abnormalities. However, she had limb asymmetry with the right side (length 95 cm, circumference 35 cm), larger than the left (length 93 cm, circumference 29 cm) (Fig. 1), together with right sided enlargement of the face compared to the left. She had capillary (port-wine) hemangiomas in the right cervical area and right upper limb (Fig. 2). She was diagnosed with the KTS.


Klippel-Trénaunay syndrome, pregnancy and the liver: an unusual interplay.

Samonakis DN, Oustamanolakis P, Manousou P, Kouroumalis EA, Burroughs AK - Ann Gastroenterol (2012)

Limb asymmetry with right-side predominance
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3959411&req=5

Figure 1: Limb asymmetry with right-side predominance
Mentions: At referral, she was normotensive without encephalopathy. Pyrexia (38.8oC), mild tachycardia and moderately distended and tender abdomen were present. There were no cutaneous markers of chronic liver disease and no cardiac, respiratory or neurological abnormalities. However, she had limb asymmetry with the right side (length 95 cm, circumference 35 cm), larger than the left (length 93 cm, circumference 29 cm) (Fig. 1), together with right sided enlargement of the face compared to the left. She had capillary (port-wine) hemangiomas in the right cervical area and right upper limb (Fig. 2). She was diagnosed with the KTS.

Bottom Line: There is a well-established risk for thrombotic complications in these patients.A case of a young patient diagnosed post partum with the very rare liver involvement is presented.The complex clinical course, the multidisciplinary management and the long-term outcome are discussed.

View Article: PubMed Central - PubMed

Affiliation: The Royal Free Sheila Sherlock Liver Centre and University Department of Surgery, Royal Free Hospital Hampstead, London UK (Dimitrios N. Samonakis, Penelope Manousou, Andrew K. Burroughs) ; Department of Gastroenterology and Hepatology, University Hospital of Heraklion, Crete, Greece (Dimitrios N. Samonakis, Pantelis Oustamanolakis, Elias A. Kouroumalis).

ABSTRACT
Klippel-Trénaunay syndrome is a rare congenital syndrome characterized by capillary malformations, soft tissue and bone hypertrophy, and varicose veins. There is a well-established risk for thrombotic complications in these patients. A case of a young patient diagnosed post partum with the very rare liver involvement is presented. The complex clinical course, the multidisciplinary management and the long-term outcome are discussed.

No MeSH data available.


Related in: MedlinePlus