eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

Cystic Hygroma: Differential Diagnoses & Workup

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

Differential Diagnoses

Teratomas and Other Germ Cell Tumors

Other Problems to Be Considered

Branchial cleft cyst
Thyroglossal duct cyst
Ranula
Goiter
Soft tissue tumors
Neck abscess

Workup

Laboratory Studies

Studies have suggested that fluorescent in situ hybridization (FISH) can be used to evaluate for cystic hygroma (CH) in prenatal chromosomal analysis. Chromosomes 13, 18, 21, X, and Y are specifically mentioned.1

Imaging Studies

MRI, CT scanning, and ultrasonography are all helpful in delineating the nature of a cystic neck mass. CT scanning and MRI reveal ringlike margin enhancement with sharp demarcation of cystic areas. The cystic areas tend to appear circumscribed and discrete. A poorly defined isodense mass that obscures muscle and fatty planes is more consistent with a microcystic lymphatic malformation than a CH.

  • MRI: MRI is the consensus study of choice. It provides the best soft tissue detail and can delineate the relationship of the lesion to underlying structures. Contrast can be used to differentiate hemangiomas from lymphangiomas. On MRI, CHs appear hyperintense on T2-weighted images and hypointense on T1-weighted images.
  • CT scanning: CT scanning is faster and may be more readily available than MRI. CT scanning carries the risk of radiation exposure, and detail is lost if the CH is surrounded by tissue of similar attenuation. Contrast helps to enhance cyst wall visualization and the relationship to surrounding blood vessels. On CT scans, CHs appear isodense to cerebrospinal fluid (CSF).
  • Ultrasonography: This is the least invasive study. It is very useful in demonstrating the relationship of CH to the surrounding structures. Ultrasonography has limited ability in assessing mediastinal and retropharyngeal structures. It can be used to detect CH in utero. Echographic visualization of multiple septae in fetal CH has been postulated to be a poor prognostic indicator.
  • Plain radiography: With any large mass of the head and neck, airway radiography (high-kilovolt anteroposterior and lateral neck radiographs or airway fluoroscopy) can be helpful in delineating possible airway compromise. Plain radiography is a reasonable initial imaging modality in the evaluation of a neck mass with a potential airway manifestation.
  • Lymphoscintigraphy: A case report highlighted the ability to visualize CH using lymphoscintigraphy.

Procedures

Several recent studies have documented the use of ultrasonography-guided endoscopic biopsy for diagnosis of GI lymphatic malformations.2

Histologic Findings

CHs are composed of large irregular sinuses with a single layer of flattened epithelial lining and fibrous adventitial coats. The thickness of the vessel wall varies, with both striated and smooth muscle components. CHs are identical to cavernous forms under a microscope; only the gross appearance of the cyst can be used to differentiate them.

Although most CHs are multicystic, in approximately 10% of cases, a unilocular cyst is found. Cysts can range from 1 mm to several centimeters in size and are filled with clear-to-straw–colored fluid, which is eosinophilic and protein rich. Individual cysts may be isolated or may freely communicate. The surrounding stroma is fibrous or fatty and may contain lymphoid aggregates, smooth muscle, or other local tissues.

Lymphangiomas may contain multiple subtypes (ie, capillary and cavernous) and, in these instances, are categorized based on the predominant subtype. In addition, lesions with a hemangiomatous component are considered hemangiolymphangiomas. Hemorrhage into the cyst is common and can be secondary to trauma or spontaneous bleeding.

Capillary lymphangiomas contain capillary-sized lymphatic channels that involve the epidermis. Cavernous lymphangiomas infiltrate surrounding structures and are dilated lymphatic channels. CHs (cystic lymphangiomas) are cystic masses lined by a single layer of endothelium with a connective tissue stroma.

Staging

Classification has been marred by a historical lack of conformity. In 1877, the first system was proposed by Wegener. In 1982, Mulliken and Glowacki presented a cell-based classification that is currently used by many authors.3 Their system stratifies lesions into hemangiomas or vascular malformations. CHs fall into the latter category. The World Health Organization (WHO) recognizes 3 types of lymphangiomas: capillary, cavernous, and cystic.

Central in a discussion of CH is the understanding that it is synonymous with macrocystic lymphatic malformation and cystic lymphangioma.

Giguere et al have proposed categorization of lymphangiomas based on the size of the cystic component, as follows:4

  • Macrocystic - Cystic spaces at least 2 cm
  • Microcystic - Spaces less than 2 cm
  • Mixed lesions

de Serres et al have proposed the following system for staging of CH of the head and neck:5

  • Stage I - Unilateral infrahyoid (17% complication rate)
  • Stage II - Unilateral suprahyoid (41% complication rate)
  • Stage III - Unilateral and both infrahyoid and suprahyoid (67% complication rate)
  • Stage IV - Bilateral suprahyoid (80% complication rate)
  • Stage V - Bilateral infrahyoid and suprahyoid (100% complication rate)

More on Cystic Hygroma

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

  1. Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations. Curr Opin Otolaryngol Head Neck Surg. Dec 2004;12(6):500-4. [Medline].

  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].

  9. Albanese CT, Wiener ES. Cystic hygroma. In: Spitz L, Coran AG, eds. Pediatric Surgery. London: Chapman & Hall; 1995:94-9.

  10. Barnes L. Tumors and tumor-like lesions of the soft tissues. In: Surgical Pathology of the Head and Neck. Vol 2. 2001:904-7.

  11. Cummings CW. Vascular tumors and malformations of the head and neck. In: Cummings Otolaryngology Head and Neck Surgery. Vol 4. 1998:4020-1.

  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.

  14. Mulliken JB, Glowacki J. Classification of pediatric vascular lesions. Plast Reconstr Surg. Jul 1982;70(1):120-1. [Medline].

  15. Peters DA, Courtemanche DJ, Heran MK, et al. Treatment of cystic lymphatic vascular malformations with OK-432 sclerotherapy. Plast Reconstr Surg. Nov 2006;118(6):1441-6. [Medline].

  16. Tran-Ngoc-Ninh, Tran-Xuan-Ninh. Cystic hygroma in children: a report of 126 cases. J Pediatr Surg. Apr 1974;9(2):191-5. [Medline].

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

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Further Reading

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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

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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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