Cystic Hygroma 

  • Author: Jason L Acevedo, MD; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP   more...
 
Updated: Jul 22, 2011
 

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. CH usually affects the head and neck (approximately 75%), with a left-sided predilection. Within the neck, the posterior triangle tends to be most frequently affected. Approximately 20% of CHs occur in the axilla; more infrequent 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: a failure of lymphatics to connect to the venous system, abnormal budding of lymphatic tissue, and 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 congential development, lymphangiomas can be acquired. They can arise from trauma (including surgery), inflammation, or obstruction of a lymphatic drainage pathway.

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Epidemiology

Frequency

International

The incidence of CH is estimated to be 1 case per 6,000-16,000 live births.

Mortality/Morbidity

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

As 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, although a decreased incidence in African Americans has been described.

Sex

The sex distribution is equal.

Age

Most cystic hygromas (50-65%) are evident at birth, with 80-90% of CHs presenting by age 2 years. Some authors believe that all CHs are present at birth, although they may have not yet fully manifested at that time.

CH can be visualized using abdominal ultrasonography by 10 weeks' gestation, although transvaginal ultrasonography provides superior detail. Fast-spin 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  Assistant Professor of Surgery, Uniformed Services University of the Health Sciences, Department of Otolaryngology, Fort Sill, Oklahoma

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

Disclosure: Nothing to disclose.

Coauthor(s)

Rahul K Shah, MD, FACS, FAAP  Assistant Professor of Otolaryngology and Pediatrics, 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 College of Physician Executives, American College of Surgeons, Massachusetts Medical Society, Phi Beta Kappa, and Triological Society

Disclosure: Nothing to disclose.

Holly L Neville, MD  Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami 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, and Association of Women Surgeons

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.

Specialty Editor Board

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.

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 School

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, and American Medical Association

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Glenn C Isaacson, MD, FACS, FAAP  Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine

Glenn C Isaacson, MD, FACS, FAAP 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 Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Covidien Honoraria Consulting

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Charles S Cox, Jr, MD, to the development and writing of this article.

References

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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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