Cystic Hygroma Workup

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

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.[3]

Next

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). See the image below. Reconstruction of CT in sagittal dimension. This pReconstruction of CT in sagittal dimension. This patient was intubated because of respiratory distress caused by the large lymphatic malformation.
  • 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.
Previous
Next

Procedures

Several recent studies have documented the use of ultrasonography-guided endoscopic biopsy for diagnosis of GI lymphatic malformations.[4]

Previous
Next

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.

Previous
Next

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.[5] 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:[6]

  • 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:[7]

  • 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)
Previous
Proceed to Treatment & Management
 
 
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

  1. Perkins JA, Manning SC, Tempero RM, Cunningham MJ, Edmonds JL Jr, Hoffer FA, et al. Lymphatic malformations: current cellular and clinical investigations. Otolaryngol Head Neck Surg. Jun 2010;142(6):789-94. [Medline].

  2. Tanna N, Sidell D, Schwartz AM, Schessel DA. Cystic lymphatic malformation of the middle ear. Ann Otol Rhinol Laryngol. Nov 2008;117(11):824-6. [Medline].

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

  4. 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].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

  9. Niramis R, Watanatittan S, Rattanasuwan T. Treatment of cystic hygroma by intralesional bleomycin injection: experience in 70 patients. Eur J Pediatr Surg. May 2010;20(3):178-82. [Medline].

  10. Sainsbury DC, Kessell G, Fall AJ, Hampton FJ, Guhan A, Muir T. Intralesional bleomycin injection treatment for vascular birthmarks: a 5-year experience at a single United Kingdom unit. Plast Reconstr Surg. May 2011;127(5):2031-44. [Medline].

  11. Impellizzeri P, Romeo C, Borruto FA, Granata F, Scalfari G, De Ponte FS, et al. Sclerotherapy for cervical cystic lymphatic malformations in children. Our experience with computed tomography-guided 98% sterile ethanol insertion and a review of the literature. J Pediatr Surg. Dec 2010;45(12):2473-8. [Medline].

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

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

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

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

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

  17. 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].

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

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

  20. 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].

  21. 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].

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

 
Previous
Next
 
Reconstruction of CT in sagittal dimension. This patient was intubated because of respiratory distress caused by the large lymphatic malformation.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.