eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

Cystic Hygroma

Author: Jason L Acevedo, MD, Resident Physician, Department of Otolaryngology-Head and Neck Surgery, Walter Reed Army Medical Center
Coauthor(s): Rahul K Shah, MD, FAAP, Assistant Professor of Otolaryngology and Pediatrics, George Washington University, Children's National Medical Center; Attending Physician, Department of Otolaryngologoy, Children's National Medical Center; Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine; Michael D Poole, MD, PhD, Consulting Staff, Georgia Ear Institute
Contributor Information and Disclosures

Updated: Jul 18, 2008

Introduction

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.

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.

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

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.

Clinical

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.

Physical

  • 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.

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, and trisomy 18 and 13, although these are not considered a cause.

In addition, several nonchromosomal disorders, including Noonan syndrome, Klinefelter 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.

More on Cystic Hygroma

Overview: Cystic Hygroma
Differential Diagnoses & Workup: Cystic Hygroma
Treatment & Medication: Cystic Hygroma
Follow-up: Cystic Hygroma
Multimedia: Cystic Hygroma
References

References

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  2. de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg. May 1995;121(5):577-82. [Medline].

  3. Kuang-Tao Y. Detection of chylothorax and cervical cystic hygroma in hydrops fetalis using lymphoscintigraphy. Clin Nucl Med. Apr 2006;31(4):205-6. [Medline].

  4. Giguere CM, Bauman NM, Smith RJ. New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol. Dec 2002;111(12 Pt 1):1066-75. [Medline].

  5. Wheeler JS, Morreau P, Mahadevan M, Pease P. OK-432 and lymphatic malformations in children: the Starship Children's Hospital experience. ANZ J Surg. Oct 2004;74(10):855-8. [Medline].

  6. Ozen IO, Moralioglu S, Karabulut R, et al. Surgical treatment of cervicofacial cystic hygromas in children. ORL J Otorhinolaryngol Relat Spec. 2005;67(6):331-4. [Medline].

  7. Burezq H, Williams B, Chitte SA. Management of cystic hygromas: 30 year experience. J Craniofac Surg. Jul 2006;17(4):815-8. [Medline].

  8. Acevedo JL, Shah RK, Brietzke SE. Nonsurgical therapies for lymphangiomas: a systematic review. Otolaryngol Head Neck Surg. Apr 2008;138(4):418-24. [Medline].

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  12. Dries AM, McDermott J. Diagnosis of cystic lymphangioma of the pancreas with endoscopic ultrasound-guided fine needle aspiration. Am J Gastroenterol. Apr 2008;103(4):1049-50. [Medline].

  13. Feins NR, Raffensperger JG. Cystic hygroma, lymphangioma, and lymphedema. In: Raffensperger JG, ed. Swenson's Pediatric Surgery. 5th ed. Norwalk: 1990:167-72.

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  17. Wesley JR. Pediatric head and neck. In: Greenfield LJ, Mulholland M, Oldham KT, et al, eds. Surgery. 1997:1999.

Further Reading

Keywords

cystic hygroma, CH, cystic lymphatic lesion, macrocystic lymphatic malformation, hemangiomas, microcystic lymphangioma, cystic lymphangioma, lymphatic rests, capillary lymphangioma, cavernous lymphangioma, suprahyoid lesions, infrahyoid lesions, Turner syndrome, Down syndrome, trisomy 18, trisomy 13, Noonan syndrome, Klinefelter disease, Fryns disease, multiple pterygium disease, achondroplasia, Gorham-Stout syndrome

Contributor Information and Disclosures

Author

Jason L Acevedo, MD, Resident Physician, Department of Otolaryngology-Head and Neck Surgery, Walter Reed Army Medical Center
Jason L Acevedo, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Rahul K Shah, MD, FAAP, Assistant Professor of Otolaryngology and Pediatrics, George Washington University, Children's National Medical Center; Attending Physician, Department of Otolaryngologoy, Children's National Medical Center
Rahul K Shah, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Massachusetts Medical Society, Phi Beta Kappa, Society of University Otolaryngologists-Head and Neck Surgeons, 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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, 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.

CME Editor

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

Maureen Strafford, MD, Arnold P Gold Foundation Associate Professor, Departments of Anesthesiology and Pediatrics, Tufts University and Tufts-New England Medical Center
Maureen Strafford, MD is a member of the following medical societies: American Medical Women's Association, American Pain Society, American Society of Anesthesiologists, International Anesthesia Research Society, Society for Education in Anesthesia, Society for Pediatric Anesthesia, and Society of Cardiovascular Anesthesiologists
Disclosure: Nothing to disclose.

 
 
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