Klippel-Trenaunay syndrome

Liguori, Andrew E.; Jorgensen, Scott A.; Towbin, Alexander J.; Towbin, Richard B.

doi:10.37549/ar2451

Cited by 3 publications

(5 citation statements)

References 1 publication

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The venous or veno-lymphatic malformation is extensive and does not involve high-flow arteriovenous malformation (AVM). Liguori et al (2) reported that 70% of KTS patients have a "vein of Servelle," also known as a lateral marginal vein, extending from the foot or ankle to the infra-inguinal region. Both malformations typical of KTS were present in our case, who had port-wine stain as well as the lateral marginal vein in the left lower extremity.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Klippel–Trénaunay syndrome with profound abdominal lymphatic-venous malformation in a three-day-old newborn: a case report and literature review

Wei,

Liu,

Lin

et al. 2024

Front. Pediatr.

View full text Add to dashboard Cite

BackgroundKlippel–Trénaunay syndrome, a kind of congenital limb-length-discrepancy disorder, is commonly associated with a variety of vascular anomalies.Case presentationWe present the case of a three-day-old newborn with a profound abdominal mass lesion during prenatal magnetic resonance imaging (MRI) examination. After delivery, physical examination revealed mild hemihypertrophy of the left lower extremity and red lesions on the left thigh. MRI of the abdomen showed a cyst-like lesion measuring 6.3 cm × 2.7 cm × 5.5 cm in the upper abdomen. Within the mass, there were also some possible calcified spots exhibiting high T1WI signals and low T2WI signals. A computed tomography (CT) scan of the abdomen was consistent with an ill-defined cystic tumor with small calcifications and encasement of mesenteric vessels. A MRI of the left lower extremity showed a tubular structure with a signal void and homogeneous strong enhancement located in the anterior subcutis of the left lower limb. The CT scan confirmed that the tubular structure was consistent with a venous malformation. This patient had features of Klippel–Trénaunay syndrome, including port-wine stains, a profound abdominal mass, and vascular malformations of the left lower extremity.ConclusionsIn this article, we presented a case of Klippel–Trénaunay syndrome, emphasizing both prenatal and confirmatory postnatal cross-sectional imaging findings. The rare presentation of an abdominal lymphatic-venous formation played a pivotal role as a crucial indicator for an early diagnosis of Klippel–Trénaunay syndrome.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The incidence of KTS is very low and is estimated to be about 1:100,000 (1). The disease has no apparent ethnic or sex predilection (2,3). We present a newborn case of KTS exhibiting the classical features and a rare profound abdominal mass.…”

Section: Introductionmentioning

confidence: 96%

Klippel–Trénaunay syndrome with profound abdominal lymphatic-venous malformation in a three-day-old newborn: a case report and literature review

Wei,

Liu,

Lin

et al. 2024

Front. Pediatr.

View full text Add to dashboard Cite

show abstract

“…The venous or veno-lymphatic malformation is extensive and does not involve high-ow arteriovenous malformation (AVM). Liguori [2] et al reported that 70% of KTS patients have a "vein of Servelle," also known as a lateral marginal vein, extending from the foot or ankle to the infra-inguinal region.Both malformations typical of KTS were present in our case, who had red spots as well as the lateral marginal vein in the left lower extremity.…”

Section: Discussionmentioning

confidence: 99%

“…Although no de nitive treatment for KTS has been approved, an early approach consisting of multidisciplinary management should be considered. Macrocystic lymphatic malformations can be treated with coil embolization or sclerotherapy, while microcystic malformation might be treated only with oral sirolimus or doxycycline [1,2]. In addition, laser therapy is used to reduce the port-wine stains.…”

Section: Discussionmentioning

confidence: 99%

Klippel–Trénaunay syndrome with profound abdominal lymphangiohemangioma in a three-day- old newborn: A case report and literature review

Wei

Liu²,

Lin

et al. 2023

Preprint

View full text Add to dashboard Cite

Background: The congenital limb-length-discrepancy disorders, such as Parkes Weber syndrome and Klippel–Trénaunay syndrome are commonly associated with a variety of vascular anomalies.Case presentation: We present the case of a three-day-old newborn with a profound abdominal mass lesion during prenatal magnetic resonance imaging (MRI)examination. After delivery, physical examination revealed mild hemihypertrophy of the left lower extremity and red spots on the left thigh. MRI of the abdomen showed a cyst-like lesion measuring 6.3 × 2.7 × 5.5 cm in the upper abdomen. Within the mass, there were also some possible calcified spots exhibiting high T1WI signals and low T2WI signals. A computed tomography (CT) scan of the abdomen was consistent with an ill-defined cystic tumor with small calcifications and encasement of mesenteric vessels. A MRI of the left lower extremity showed a tubular structure with a signal void and homogeneous strong enhancement located in the anterior subcutis of the left lower limb. The CT scan confirmed that the tubular structure was consistent with a venous malformation. This patient had features of Klippel–Trénaunay syndrome, including port-wine stains, a profound abdominal mass, and vascular malformations of the left lower extremity.Conclusions: Early distinction between Klippel–Trénaunay syndrome and Parkes Weber syndrome was essential based on the identification of significant hemodynamic arteriovenous fistulas. The rare presence of an abdominal lymphangiohemangioma served as an important clue for early diagnosis of Klippel–Trénaunay syndrome.

show abstract

“…[ 1 ] Klippel–Trenaunay syndrome (KTS) is a classical triad that consists of vascular malformations, varicosities at unusual distribution, and unilateral soft and skeletal tissue hypertrophy, usually of the lower extremity. [ 2 3 ] Children need corrective orthopedic surgeries of the lower limb. Apart from subcutaneous hemangiomas in the abdominal area, neuraxial hemangiomas are a known occurrence in KTS.…”

Section: Introductionmentioning

confidence: 99%

Sciatic nerve hemangioma, a challenge in klippel–Trenaunay syndrome for perioperative analgesia

et al. 2022

View full text Add to dashboard Cite

Children with Klippel–Trenaunay syndrome have vascular malformations, varicosities at unusual distribution, and unilateral soft and skeletal tissue hypertrophy of the lower extremity. Corrective surgery of the deformity is necessary to improve gait and wear acceptable footwear. Perioperative pain relief for debulking of the right great toe in a known case of KTS with sciatic hemangioma was achieved with ultrasound-guided sciatic nerve block in an avascular area.

show abstract

Klippel-Trenaunay syndrome

Cited by 3 publications

References 1 publication

Klippel–Trénaunay syndrome with profound abdominal lymphatic-venous malformation in a three-day-old newborn: a case report and literature review

Klippel–Trénaunay syndrome with profound abdominal lymphatic-venous malformation in a three-day-old newborn: a case report and literature review

Klippel–Trénaunay syndrome with profound abdominal lymphangiohemangioma in a three-day- old newborn: A case report and literature review

Sciatic nerve hemangioma, a challenge in klippel–Trenaunay syndrome for perioperative analgesia

Contact Info

Product

Resources

About