Cystic Hygroma Clinical Presentation

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

History

The presenting signs and symptoms of the cystic hygroma (CH) vary depending on the lesion's location.

  • The microcystic form of lymphangioma tends to predominate over CH in the oral cavity and oropharynx. Microcystic lymphangiomas commonly appear as clusters of clear, black, or red vesicles on the buccal mucosa or tongue.
  • CHs tend to predominate below the mylohyoid muscle and can involve both the anterior and posterior triangles of the neck.
  • The cysts are typically large and thick walled and have little involvement of surrounding tissue. The overlying skin can take on a bluish hue or may appear normal.
  • CHs often present after a sudden increase in size secondary to infection or intralesional bleeding. Spontaneous decompression or shrinkage is uncommon.
  • Rarely, children with CH display symptoms of newly onset obstructive sleep apnea syndrome (OSAS). This situation may involve children with CH or other space-occupying lesions of the supraglottis or paraglottic region. Suprahyoid lymphangiomas tend to cause more breathing difficulties than infrahyoid lesions.
  • Potentially life-threatening airway compromise that manifests as noisy breathing (stridor) and cyanosis is a possible symptom of lymphangiomas.
  • Feeding difficulties, as well as failure to thrive, may alert the clinician to a potential lymphangioma. This is especially true when the lesion affects structures of the upper aerodigestive tract.
  • Rare locations, such as the middle ear, have been reported.[2]
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Physical

Physical findings may include the following:

  • CHs are typically soft, painless, compressible (doughy) masses.
  • A CH typically transilluminates.
  • In children who present with CH of the neck, closely evaluate for tracheal deviation or other evidence of impending airway obstruction.
  • Closely inspect the tongue, oral cavity, hypopharynx, and larynx because any involvement may lead to airway obstruction.
  • Referral to an airway expert (otolaryngologist) to potentially perform a fiberoptic airway evaluation is justified in patients with a lymphatic malformation.
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Causes

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, although these are not considered a cause.

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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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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