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Case Reports
. 2025 Aug 20;20(11):5645-5652.
doi: 10.1016/j.radcr.2025.07.014. eCollection 2025 Nov.

Extensive venolymphatic involvement in infantile Klippel-Trenaunay syndrome: A rare case from Ethiopia

Frezer Terefe  1   2 , Messay Gebrekidan  3 , Michael A Negussie  4 , Enatnesh Terefe  5
Affiliations
Case Reports

Extensive venolymphatic involvement in infantile Klippel-Trenaunay syndrome: A rare case from Ethiopia

Frezer Terefe et al. Radiol Case Rep. .

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by a triad of clinical features: capillary malformations, venous varicosities, and hypertrophy of soft or bony tissues. This case report presents a 1-year-old infant diagnosed with KTS, exhibiting multifocal pelvic, gluteal, and thigh macrocystic lymphatic malformations alongside significant limb overgrowth. The diagnosis was confirmed through clinical evaluation and advanced imaging techniques, including Doppler ultrasound and CT angiography. KTS poses unique challenges in diagnosis and management, necessitating a multidisciplinary approach to address potential complications such as deep vein thrombosis and chronic pain. This case underscores the importance of early diagnosis and ongoing monitoring in improving patient outcomes and highlights the need for increased awareness of the multiple imaging spectra of KTS among healthcare professionals.

Keywords: Hemihypertrophy; Klippel-Trenaunay syndrome; Macrocystic lymphatic malformations; Persistent embryonic lateral marginal and sciatic veins; Port-wine cutaneous stains; Veno-lymphatic malformations.

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Figures

Fig 1:
Fig. 1
Clinical photograph showing hemihypertrophy of the left limb and multiple port-wine cutaneous stains.
Fig 2
Fig. 2
(A and B) CT angiography of lower limb axial bone window demonstrating cortical thickening and bony enlargement.
Fig 3:
Fig. 3
CT Angiography axial image demonstrating arterial phase rim enhancement of macrocystic lymphatic malformation over the left psoas muscle.
Fig 4
Fig. 4
Axial delayed venous phase images show, the upper image: macrocytic lymphatic malformation in the presacral space (thick arrow) extending into left gluteal muscles (thin arrow), Lower image: lymphatic malformation in the anterior proximal thigh subcutaneous tissue.
Fig 5:
Fig. 5
Sagittal venous phase image of distal thigh showing a focally dilated distal segment of superficial femoral vein.
Fig 6
Fig. 6
(A) Delayed venous axial image demonstrating persistent /embryonic lateral marginal vein residing over the lateral aspect of the distal thigh. (B) the marginal vein crossing to the lateral aspect from the anatomic location of SSV.
Fig 7:
Fig. 7
Axial images showing dilated persistent sciatic vein (upper image), and dilated superficial and deep veins of the thigh (lower image).
See this image and copyright information in PMC

References

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