Lymphatic Vascular Malformations Treatment & Management
- Author: Meir Cohen, MD, MPS; Chief Editor: Gregory Caputy, MD, PhD, FICS more...
Medical Therapy
Local pressure
Elastic support stockings may help decrease the swelling and the functional handicap associated with lymphatic VMs of the extremities (see image below).
Lower limb lymphatic vascular malformation. Antibiotics
Viral or bacterial infection can cause acute infection of a lymphatic VM. Infection may be associated with acute enlargement, pain, local warmth, redness, and elevated systemic fever. Intravenous (IV) antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs) are indicated during such episodes.
Sclerotherapy
Transcutaneous injection of a sclerosant such as alcohol may help decrease a lymphatic VM.[7] It is mostly useful for macrocystic malformations and for combined venous-lymphatic lesions. The procedure is painful; therefore, it is performed by an invasive radiologist under general anesthesia.
Initially, 5 mL of contrast fluid is injected through a venous catheter to delineate the anatomy of the lesion and to detect escape of contrast to the systemic circulation (see the upper left portion of the image below). The upper middle portion of the image below shows injection of contrast into a facial venous-lymphatic malformation. The catheter was relocated when escape of contrast to the facial vein was detected (upper right). When a lumen filled with lymphatic fluid is detected, the lymph is aspirated. Alcohol (100%) mixed with a small amount of contrast fluid (alcohol-to-contrast ratio of 20:5) is then injected through the same venous catheter.
Complications of alcohol injection. The total amount of alcohol injected into a lesion of a full-sized male is approximately 50 mL. Exceeding a dose of 1 mL/kg is not advised. The catheter is left in place following alcohol injection in case some of the alcohol needs to be withdrawn. Injection is discontinued when skin changes such as peau d'orange, erythema, and bruising are observed. The upper left portion of the image above shows bruising and peau d'orange changes following injection with alcohol of a chest venous-lymphatic VM.
Additionally, doxycycline percutaneous image-guided sclerotherapy of macrocystic intra-abdominal lymphatic malformations has been shown to be safe and effective.[12]
To decrease the swelling, 4 mg of dexamethasone (PO/IV) is administered 3 times every day for 3 days after the procedure. NSAIDs are administered for pain control. The patient usually stays in the hospital overnight for pain control and for monitoring of possible vascular or neurologic limb compromise. Systemic leakage of alcohol may cause myocardial depression. Injection of alcohol close to the skin or mucosa may cause skin slough or skin necrosis. The lower right and left portions of the image above) shows necrosis of skin and mucosa following injection of alcohol to the forearm and tongue, respectively.
Bleomycin and OK-432
Intralesional bleomycin and OK-432 have recently been reported to have dramatic results.[11, 13]
Surgical Therapy
Surgery is the only way to "cure" a lymphatic malformation. It should be considered in the following situations:
- When intraoperative and postoperative bleeding can be controlled
- When surgery does not put another organ at risk (eg, injury to the eye or facial nerve)
The surgery must be well planned. Most lesions cannot be resected completely; therefore, the extent of the resection needs to be defined before the procedure. In certain situations, such as eyelid surgery, performing the surgery under local anesthesia is better. This facilitates intraoperative navigation. The image below shows an upper right eyelid lesion before and after resection under local anesthesia.
Eyelid lymphatic vascular malformation. Lymphangioma circumscriptum is a superficial lymphatic malformation of the skin. This often can be resected completely and the defect reconstructed with a skin graft (see image below). Administration of hemostatic agents such as recombinant factor VIIa (rVIIa) may decrease bleeding and improve surgical efficiency.[14]
Lymphangioma circumscriptum. Complications
Vision
Obstruction of vision during the first 6 months of life by a periorbital, cheek, forehead, or nasal VM may cause long-term visual damage. Early treatment with transcutaneous sclerosis and/or surgery is indicated in such patients.
Breathing
Lymphatic VM, which invades the neck, may compress the soft tracheal rings of infants and present as stridor. Direct excision of the lesions is impossible in most patients because of the close proximity of the lesion to vital structures. Consider tracheostomy in patients in whom pharmacologic treatment has failed.[9]
Psychosocial complications
The presence of a VM psychosocially affects both the patient and his or her parents. Parents may be subjected to comments, questions, and unsolicited advice from friends, family, and complete strangers. Early psychosocial support by primary caregivers with the help of a dedicated vascular birthmark clinic team is mandatory.[15] Early surgery may be indicated in patients with visible facial VMs.
Outcome and Prognosis
Lymphatic lesions gradually enlarge and worsen with time.[3] Surgery is the only complete cure for this problem. However, this is not always possible, and the goal of treatment in many patients is improvement rather than cure.
Future and Controversies
A significant contribution to the understanding of vascular lesions is the introduction of a classification method by Mulliken and associates.[2] This made diagnosis and treatment more accurate and predictable. However, confusing and occasionally misleading terms used by different subspecialties are still found.
Improvement in patient monitoring and anesthesia during and after surgery made early excisions and transcutaneous sclerosis safer and more acceptable.
A major controversy is the timing of operative procedures. No clear-cut answer exists to this question. The authors believe that decisions should be made according to the individual patient. The psychosocial consequences of growing up with a facial deformity always should be taken into consideration.
Future research of specific genes and their angiogenic growth factor products will contribute to the understanding of the mechanism underlying the formation of lymphatic VMs and may provide new modalities of treatment at the gene level.[5]
Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases. Pediatr Dermatol. Dec 1991;8(4):267-76. [Medline].
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. Mar 1982;69(3):412-22. [Medline].
Mulliken JB. Vascular anomalies. In: Aston SJ, Beasley RW, Thorne CHM, eds. Grabb and Smith Plastic Surgery. 5th ed. NY: Lippincott Raven Publishers; 1997.
Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg. Jun 1993;91(7):1216-30. [Medline].
Breugem CC, van Der Horst CM, Hennekam RC. Progress toward understanding vascular malformations. Plast Reconstr Surg. May 2001;107(6):1509-23. [Medline].
Carmeliet P, Collen D. Vascular development and disorders: molecular analysis and pathogenic insights. Kidney Int. Jun 1998;53(6):1519-49. [Medline].
Alomari AI, Karian VE, Lord DJ, Padua HM, Burrows PE. Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement. J Vasc Interv Radiol. Oct 2006;17(10):1639-48. [Medline].
Suzuki Y, Obana A, Gohto Y, Miki T, Otuka H, Inoue Y. Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. Jun 2000;84(6):614-7. [Medline].
Padwa BL, Hayward PG, Ferraro NF, Mulliken JB. Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy. Plast Reconstr Surg. May 1995;95(6):951-60. [Medline].
Armstrong DC, ter Brugge K. Selected interventional procedures for pediatric head and neck vascular lesions. Neuroimaging Clin N Am. Feb 2000;10(1):271-92, x. [Medline].
Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. Jan 2005;32(1):99-116, ix. [Medline].
Chaudry G, Burrows PE, Padua HM, Dillon BJ, Fishman SJ, Alomari AI. Sclerotherapy of Abdominal Lymphatic Malformations with Doxycycline. J Vasc Interv Radiol. Aug 6 2011;[Medline].
Ernemann U, Kramer U, Miller S, Bisdas S, Rebmann H, Breuninger H, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J Radiol. May 11 2010;[Medline].
Buckmiller LM, Richter GT, Waner M, Suen JY. Use of recombinant factor VIIa during excision of vascular anomalies. Laryngoscope. Apr 2007;117(4):604-9. [Medline].
Sandler G, Adams S, Taylor C. Paediatric vascular birthmarks--the psychological impact and the role of the GP. Aust Fam Physician. Mar 2009;38(3):169-71. [Medline]. [Full Text].
Zuker MR, Cohen M. Congenital hand anomalies (discussion). In: Goldwyn RM, Cohen MN, eds. The Unfavorable Result in Plastic Surgery Avoidance and Treatment. 3rd ed. Philadelphia: Lippincott Williams and Wilkins; 2000:710-713.
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