Circumscribed, soft, slowly increasing circumscribed swelling on the right abdominal wall and flank. Two old scars after unsuccessful open partial resection. Circumscribed pain in the area of the swelling, the overlying skin is not discolored.
Coronal, T2-weighted, fat-suppressed MRI of the lower abdomen at the level of the groin. Normal inguinal lymph nodes. The lymphatic malformation of the abdominal wall presents as large, dysplastic, balloon-like, septated cysts that are grossly hyperintense (white).
Transverse T1-weighted, fat-suppressed MRI after contrast administration shows only minimal enhancement of the thin walls of the lymphatic malformation. This is typical of a lymphatic malformation, as the liquid in the cysts (lymph) does not enhance.
Overview X-ray in the area of the right pelvic crest after injection of contrast medium via the needle directly located in the lymphatic malformation. Large parts of the lymphatic malformation are contrasted from this needle position, representing an optimal needle position for sclerotherapy.
Additional puncture approximately 5 cm cranially into another cystic compartment of the lymphatic malformation. This compartment does not communicate with the lower part of the malformation still visible caudally. Therefore, it must be sclerosed separately in order to successfully occlude the entire lymphatic malformation.
New sclerotherapy in a second session using direct puncture technique under ultrasound guidance into the remaining cystic areas of the lymphatic malformation (X-ray overview image after contrast injection via the needle). After injection of the contrast medium, the punctured area is then evacuated of fluid as completely as possible. Then the sclerosing agent, here picibanil (OK-432), is injected via the same needle position.
In the ultrasound examination 6 months after the 2nd sclerotherapy, only very small cystic parts of the lymphatic malformation are detectable. The larger cysts are occluded. As the patient is asymptomatic, there is no further treatment of these residual parts in an intrinsically benign lesion.
Macrocystic lymphatic malformations (LM) located directly on the skin can lead to lymphatic fluid oozing out via small skin vesicles (lymphatic vesicles) due to the underlying high local lymphatic pressure. These areas are called “lymphangioma circumscriptum”. Sclerotherapy (here with the agent picibanil = OK-432) closes the lymphatic channels via a strong local inflammation and seals the skin. A favorable aspect in this case were the large parts of the lymphatic malformation communicating with each other (visible after injection of contrast agent via the puncture needle). Therefore, only a few punctures were necessary to sclerose the entire lymphatic malformation. This minimally invasive procedure is usually gentler in the long run than open surgical measures. Individual smaller cysts in the often irregularly shaped lymphatic malformation can be easily reached with the liquid sclerosant.
All images © Wohlgemuth