Lymphatic Vascular Malformations Workup

  • Author: Meir Cohen, MD, MPS; Chief Editor: Gregory Caputy, MD, PhD, FICS   more...
 
Updated: Sep 30, 2011
 

Imaging Studies

  • The most important imaging tool is contrast-enhanced MRI.[10] This diagnostic test, which requires sedation or general anesthesia for children younger than 6 years, demonstrates the extent of the lesion and helps differentiate between hemangiomas and venous, lymphatic, and arterial lesions. It also may help to differentiate between a vascular lesion and a nonvascular lesion, such as those found in neurofibromatosis.
    • MRI scans have 3 basic images: T1-weighted spin-echo image, T2-weighted spin-echo image, and contrast-enhanced (gadolinium) T1-weighted spin-echo image. T refers to the time necessary for the protons to discontinue spinning (see the first image below). T1 refers to a value around 600 milliseconds, and T2 refers to a value around 4000 milliseconds. The typical findings in the 3 modes are presented in the second image below. MRI - MRI T1, T2. MRI - MRI T1, T2. Diagnosis – MRI. Diagnosis – MRI.
    • Lymphatic VMs have a typical solid appearance with low intensity on T1-weighted spin-echo image, which is equal to that of venous VMs and less intense than that of hemangiomas.[10] Contrast-enhanced T1-weighted images show a very low central intensity with a typical rim enhancement of the lymphatic lesion. The first image below shows a patient with cystic hygroma. The second image below (image on left) shows a moderate intensity at T2-weighted spin-echo image and very low central intensity with typical rim enhancement of the lymphatic lesion on contrast-enhanced T1-weighted image (image on right). Cystic hygroma. Cystic hygroma. MRI of cystic hygroma. MRI of cystic hygroma.
  • Ultrasonography helps differentiate a low-flow from a high-flow lesion. Prenatal ultrasonography can detect relatively large lesions as early as the second trimester.[11]
  • CT can detect calcifications, which are present in low-flow combined venous lymphatic lesions.
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Histologic Findings

Lymphatic malformations are composed of dysplastic vesicles or pouches filled with lymphatic fluid. They can be described as either microcystic, macrocystic, or combined forms. Lymphatic VMs have walls of variable thickness, composed of both striated and smooth muscle, with nodular collections of lymphocytes in the connective tissue stroma.[3]

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Contributor Information and Disclosures
Author

Meir Cohen, MD, MPS  Consulting Staff, Department of Plastic Surgery, Schneider Children's Medical Center of Israel, Tel Aviv University

Meir Cohen, MD, MPS is a member of the following medical societies: American Cleft Palate/Craniofacial Association and Plastic Surgery Research Council

Disclosure: Nothing to disclose.

Coauthor(s)

Shimon Maimon, MD  Head of Invasive Radiology Unit, Beilinson Campus, Rabin Medical Center, Israel

Disclosure: Nothing to disclose.

Dan Ben-Amitai, MD  Head of Pediatric Dermatology Service, Lecturer, Schneider Children's Medical Center of Israel

Dan Ben-Amitai, MD is a member of the following medical societies: Israel Medical Association

Disclosure: Nothing to disclose.

Eric Bensimon, MD, FRCS(C)  Assistant Professor, Department of Surgery, University of Montreal Faculty of Medicine, Canada

Eric Bensimon, MD, FRCS(C) is a member of the following medical societies: American Society of Plastic Surgeons, Canadian Society of Plastic Surgeons, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Shahin Javaheri, MD  Chief, Department of Plastic Surgery, Martinez Veterans Affairs Outpatient Clinic; Consulting Staff, Advanced Aesthetic Plastic & Reconstructive Surgery

Shahin Javaheri, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery and American Society of Plastic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Wayne Karl Stadelmann, MD  Stadelmann Plastic Surgery, PC

Wayne Karl Stadelmann, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Society of Plastic Surgeons, New Hampshire Medical Society, Northeastern Society of Plastic Surgeons, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Nicolas (Nick) G Slenkovich, MD  Director, Colorado Plastic Surgery Center

Nicolas (Nick) G Slenkovich, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, American Society of Aesthetic Plastic Surgery, American Society of Plastic Surgeons, and Colorado Medical Society

Disclosure: Nothing to disclose.

Chief Editor

Gregory Caputy, MD, PhD, FICS  Chief Surgeon, Aesthetica Plastic and Laser Surgery Center, Inc

Gregory Caputy, MD, PhD, FICS is a member of the following medical societies: American Society for Laser Medicine and Surgery, Canadian Medical Association, International College of Surgeons, International College of Surgeons US Section, Pan-Pacific Surgical Association, and Wound Healing Society

Disclosure: Syneron Corporation Salary Speaking and teaching

References

  1. 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].

  2. 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].

  3. Mulliken JB. Vascular anomalies. In: Aston SJ, Beasley RW, Thorne CHM, eds. Grabb and Smith Plastic Surgery. 5th ed. NY: Lippincott Raven Publishers; 1997.

  4. 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].

  5. Breugem CC, van Der Horst CM, Hennekam RC. Progress toward understanding vascular malformations. Plast Reconstr Surg. May 2001;107(6):1509-23. [Medline].

  6. Carmeliet P, Collen D. Vascular development and disorders: molecular analysis and pathogenic insights. Kidney Int. Jun 1998;53(6):1519-49. [Medline].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. Jan 2005;32(1):99-116, ix. [Medline].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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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Classification of vascular lesions.
Classification of vascular malformations.
Diagnosis - Medical history.
Lower limb lymphatic vascular malformation.
Diagnosis - Physical examination.
Lymphangioma circumscriptum.
Thigh lymphatic vascular malformation.
Orbital lymphatic vascular malformation.
Eyelid lymphatic vascular malformation.
MRI of eyelid lymphatic vascular malformation.
Right facial lymphatic vascular malformation with right open bite.
Gradual enlargement of a facial vascular malformation.
Tongue lymphatic vascular malformation.
Cystic hygroma.
MRI of cystic hygroma.
Neck lymphatic vascular malformation.
Axillary lymphatic vascular malformation.
Contrast-enhanced T1-weighted spin-echo image of a venous-lymphatic vascular malformation.
Large axillary venous-lymphatic vascular malformation.
Huge upper limb lymphatic malformation.
Lower limb lymphatic vascular malformation.
Panorex showing mandibular hypertrophy due to a lymphatic vascular malformation.
Venous or lymphatic lesion on T2-weighted spin-echo image.
Contrast-enhanced T1-weighted spin-echo image showing rim enhancement, which is typical of lymphatic vascular malformations.
Contrast-enhanced (gadolinium) T1-weighted spin-echo image showing areas with rim enhancement, which are typical of lymphatic vascular malformations.
MRI - MRI T1, T2.
Diagnosis – MRI.
Orbital hemangioma - Differential diagnosis.
Complications of alcohol injection.
 
 
 
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