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Cystic Hygroma

  • Author: Jason L Acevedo, MD; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
 
Updated: Mar 11, 2015
 

Background

First described by Wernher in 1843, cystic hygroma (CH) is a cystic lymphatic lesion that can affect any anatomic subsite in the human body. It usually affects the head and neck (~75%), with a predilection for the left side. Within the neck, the posterior triangle tends to be most frequently affected. Approximately 20% of CHs occur in the axilla; less common subsites include the mediastinum, groin, and retroperitoneum.

CH is synonymous with cystic lymphangioma, which is also known as a macrocystic lymphatic malformation and was first described in 1828 by Redenbacker.

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Pathophysiology

Lymphangiomas are thought to arise from a combination of the following:

  • Failure of lymphatics to connect to the venous system
  • Abnormal budding of lymphatic tissue
  • Sequestered lymphatic rests that retain their embryonic growth potential

These lymphatic rests can penetrate adjacent structures or dissect along fascial planes and eventually become canalized. These spaces retain their secretions and develop cystic components because of the lack of a venous outflow tract. The nature of the surrounding tissue determines whether the lymphangioma is capillary, cavernous, or cystic.

CHs tend to form in loose areolar tissue, whereas capillary and cavernous forms of lymphangiomas tend to form in muscle. Studies using cell proliferation markers have demonstrated that lymphangioma enlargement is related more to engorgement than to actual cell proliferation. Molecular studies suggest that vascular endothelial growth factor C (VEGF-C) and its receptors may play an important role in the development of lymphatic malformations.[1]

In addition to congenital development, lymphangiomas can be acquired. They can arise from trauma (including surgery), inflammation, or obstruction of a lymphatic drainage pathway.

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Etiology

Karyotypic abnormalities are present in 25-70% of children with CH. CH has been noted to be more common in persons with Turner syndrome, Down syndrome, Klinefelter syndrome, and trisomy 18 and 13, though these are not considered to be causative.

In addition, several nonchromosomal disorders, including Noonan syndrome, Fryns syndrome, multiple pterygium syndrome, and achondroplasia, are associated with an increased incidence of CH. Intrauterine alcohol exposure has been associated with the development of lymphangiomas. Dissolution of bone caused by either lymphangiomas or hemangiomas is termed Gorham-Stout syndrome.

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Epidemiology

Frequency

International

The incidence of CH is estimated to be one case per 6000-16,000 live births.

Mortality/Morbidity

In some series, the reported mortality has been as high as 2-6%, usually secondary to pneumonia, bronchiectasis, and airway compromise. Obviously, this figure is pertinent in the larger-sized lesions.

As would be expected, morbidity depends on the anatomic location of the CH. In general, morbidity is related to cosmetic disfigurement and impingement on other critical structures, such as nerves, vessels, lymphatics, and the airway.

Race

Most series report no racial predominance, though a decreased incidence in African Americans has been described.

Sex

The sex distribution is equal.

Age

Most CHs (50-65%) are evident at birth, and 80-90% of them present by age 2 years. Some authors believe that all CHs are present at birth, even though they may not have fully manifested at that time.

CH can be visualized by means of abdominal ultrasonography by 10 weeks' gestation, though transvaginal ultrasonography provides superior detail. Fast-spin magnetic resonance imaging (MRI) can also be used to determine the extent of fetal CH. Elevated alpha fetoprotein levels in amniocentesis fluid has been reported in pregnancies with CH.

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Contributor Information and Disclosures
Author

Jason L Acevedo, MD Otoloaryngologist-Head and Neck Surgeon

Jason L Acevedo, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Rahul K Shah, MD, FACS, FAAP Associate Professor of Otolaryngology and Pediatrics, Associate Surgeon-in-Chief, Medical Director, Peri-operative Services, Children's National Medical Center, George Washington University School of Medicine and Health Sciences; Attending Physician, Department of Otolaryngology, Children's National Medical Center

Rahul K Shah, MD, FACS, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American College of Medical Quality, American Association for Physician Leadership, American College of Surgeons, Triological Society, Massachusetts Medical Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

Holly L Neville, MD Associate Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami, Leonard M Miller School of Medicine

Holly L Neville, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association of Women Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John E McClay, MD Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Dallas, University of Texas Southwestern Medical Center

John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Ravindhra G Elluru, MD, PhD Professor, Wright State University, Boonshoft School of Medicine; Pediatric Otolaryngologist, Department of Otolaryngology, Dayton Children's Hospital Medical Center

Ravindhra G Elluru, MD, PhD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, Association for Research in Otolaryngology, Society for Ear, Nose and Throat Advances in Children, Triological Society, American Society for Cell Biology

Disclosure: Nothing to disclose.

Acknowledgements

Orval Brown, MD Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas

Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

William K Chiang, MD Associate Professor, Department of Emergency Medicine, New York University School of Medicine; Chief of Service, Department of Emergency Medicine, Bellevue Hospital Center

William K Chiang, MD, is a member of the following medical societies: American Academy of Clinical Toxicology, American College of Medical Toxicology, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Michael D Poole, MD, PhD Consulting Staff, Georgia Ear Institute

Michael D Poole, MD, PhD, is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Society for Microbiology

Disclosure: Nothing to disclose.

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Reconstruction of CT in sagittal dimension. This patient was intubated because of respiratory distress caused by the large lymphatic malformation.
 
 
 
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