Korean J Obstet Gynecol.  2010 Jul;53(7):647-651. 10.5468/kjog.2010.53.7.647.

Congenital lymphangiomatosis of the right lower limb

• We JS
• Shin JE
• Jang DG
• Choi SK
• Park IY
• Shin JC

• KMID: 2273946
• DOI: http://doi.org/10.5468/kjog.2010.53.7.647

Abstract

Lymphangiomatosis is a condition of lymphatic tissue malformation with multiple or diffuse involvement of soft tissues, visceral organs. Congenital abnormalities of the lymphatic system are very rare, and reports of congenital lymphangiomatosis are even fewer. We experienced a case of congenital lymphangiomatosis detected as edema of the right limb by prenatal ultrasonography and then diagnosed by magnetic resonance imaging. We describe this case with a brief review of the literature.

Keyword

Congenital lymphangiomatosis; Prenatal ultrasonography; Magnetic resonance imaging

MeSH Terms

Congenital Abnormalities
Edema
Extremities
Lower Extremity
Lymphatic System
Lymphoid Tissue
Magnetic Resonance Imaging
Ultrasonography, Prenatal

Figure

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  Fig. 1 Two dimensional and three dimensional ultrasonography images at 31+5 weeks of gestation showed fetal low limbs; right thigh (A), left and right thigh (B), left leg and foot (C) and right leg and foot (D). Right lower limb was edematous for left leg (D).

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  Fig. 2 Fetal MRI imaged showed edema of right lower limb; leg (A), thigh (B). Skin and subcutaneous tissue of right lower limb (a white arrow) was thicker for left lower limb (a white arrow head). A scan performed with techniques to study the accompanying abnormalities. There was no particular abnormality.

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  Fig. 3 Chest X-ray images of the neonatal boy showed no abnormal finding of the other side.

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  Fig. 4 Magnetic resonance imaging of newborn boy showed enlarged right lower limb. Diffuse edematous swelling of skin and subcutaneous soft tissue was showed with high signal on T2 weighted image (A) and T1 weighted image (B). After infusion of contrast media, thin reticular enhancements are seen in the subcutaneous fat layer (C). Photograph of the newborn boy showing enlargement and discoloration of the right lower limb (D).

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