Lymphatic malformation

Abstract

Background: The prevalence of lymphangiomas has been estimated to be 1.1 to 5.3 cases per 10,000 live births. However, the prevalence appears to be higher when the studies include live-births, terminated pregnancies, and still-births: then, the estimate is 30 cases per 10,000.1 Lymphangiomas represent around 4% of all vascular tumors and approximately 25% of benign pediatric vascular tumors.2 Orbital lymphangiomas have a prevalence of 1.1 to 5.3 cases per 10,000 live births with an equal incidence between males and females.1,3 Clinical case: E.N is 42 years old, female, a teacher and a citizen of the 3rd bag in Dornogobi. First, a lumpy, red source started to appear on the eyelid of the right eye when she was 6 months old. In 1986, she received medical advice from Orbis hospital, underwent steroid treatment, tissue analysis, and surgery. Since then, under medical supervision, she underwent a total of 5 surgeries and 10 radiation treatments in 1991, 1994, 2002 and 2006. Since 2021, her symptoms have been present such as loss of vision of her right eye and discharge. The enlarged blood vessels, numerous deep cysts, a small amount of discharge, and recurrence of tumor were revealed by examination. Then, she has been sent to the tertiary level clinics and treatment is in progress. 2021 was determined to be disabled and currently under supervision. Discussion: Complete surgical excision remains the best treatment for lymphatic malformations. Because incomplete resection can lead to recurrence5. Other treatments, such as sclerotherapy, have been suggested to reduce the effects and complications of surgery. Various products such as doxycycline, bleomycin, etibloc, and OK-432 have been used as sclerotherapeutic agents6. In addition to OK-432, other agents have been reported to cause perilesional fibrosis, thereby complicating surgical resection. When treating orbital lymphadenopathy with sclerosants, the risk of vision loss and orbital compartment syndrome due to expansion of the lesion after sclerosant injection should always be kept in mind. Therefore, the treatment of orbital lymphatic malformations is very difÏcult. Conclusion: 1. In terms of treatment, it can be observed in cases where there are no severe abnormalities in terms of anatomical structure without changes in vision. Scleratherapy – various types of drugs are injected into cysts under ultrasound control to cause scarring. These drugs include OK-432 (Picinibil), sodium tetradecyl sulfate, doxycycline, ethanol, pingyanmycin, and bleomycin. 2. Surgical treatment depends on the location and size of the tumor, and the risk of recurrence after surgery is high. 3. In the rare clinical case we are presenting this time, if re-surgery is performed, there is a high risk of complications during the surgery, bleeding, and compressive optic neuropathy due to the abnormal development of blood vessels in the retrograde, so we are currently monitoring it. 4. Therefore, in the future, depending on the progress, it is planned to carry out treatment with an anti-atherogenic injection (bleomycin) under ultrasound control. ­