eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

Cystic Hygroma: Treatment & Medication

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

Treatment

Medical Care

Although some authors have reported watchful waiting of cystic hygroma (CH), it should be considered only in patients who are asymptomatic. The medical treatment of CH consists of the administration of sclerosing agents. Sclerosing agents include OK-432 (an inactive strain of group A Streptococcus pyogenes), bleomycin, pure ethanol, bleomycin, sodium tetradecyl sulfate, and doxycycline.

  • OK-432: Although not currently approved by the US Food and Drug Administration (FDA), OK-432 (Picibanil) has been reported to successfully treat CH.6 The mechanism of action is proposed to be an inflammatory response to the inactive bacteria, leading to fibrosis of the hygroma. OK-432 may be a viable option for large unilocular cysts. Currently, OK-432 is available in the United States only by protocol. It does not work well for small cysts. Because the procedure for using OK-432 involves aspiration prior to injection of the sclerosant, some have hypothesized that the true effect is from the aspiration.
  • Bleomycin: Bleomycin is considered a poor choice because of its toxicity (pulmonary fibrosis) because CH is a benign disease and other treatment options are available.
  • Alcohol: Absolute alcohol as a sclerosing agent has been used with some success in some patients; alcohol works well in vascular malformations.
  • Interferon alfa-2a: This has been used in the treatment of hemangiomas, and its use has been proposed in lymphangiomas. However, its efficacy has never been documented and it carries a serious side effect profile.
  • Fibrin sealant: The use of a fibrin sealant after aspiration of CH has been reported in the literature.

An infected CH should be treated with intravenous antibiotics, and definitive surgery should be performed once the infection has resolved. Incision and drainage or aspiration results in only temporary shrinkage, and subsequent fibrosis can further complicate the resection. Radiotherapy has not been demonstrated to be effective. The preferred treatment of all CH is surgical resection. Only resection can truly offer the potential for cure.

Surgical Care

The mainstay of treatment is surgical excision. Although surgery is the criterion standard for treatment, both the operating team and the family of the patient should go forward with the knowledge that CH is a benign lesion. If acute infection occurs prior to resection, surgery should be delayed at least 3 months.

  • The surgical team should attempt to completely remove the lymphangioma or to remove as much as possible, sparing all vital neurovascular structures. Complete excision has been estimated to be possible in roughly 40% of cases.
  • CHs are ideally removed in one procedure because secondary excisions are complicated by fibrosis and distorted anatomical landmarks.
  • Microcystic lesions are much more difficult to remove because of their intimate association with nearby tissues. Laser therapy is a recent advancement in the treatment of microcystic lesions.
  • The exceptions to excision at the time of diagnosis are few and include premature infants who are small in size and those with involvement of crucial neurovascular structures that are small and difficult to identify (eg, facial nerve). If no airway obstruction is present, surgery can be delayed until the child is aged 2 years or older, especially when operating around the facial nerve in the parotid area.
  • Signs of airway obstruction require surgical evaluation at the time of diagnosis. In emergency situations, aspiration with an 18-gauge or 20-gauge needle may obviate the need for an emergency tracheostomy.
  • Although traditional wisdom has dictated not aspirating lymphatic malformations, a study by Burezq et al documented success with serial aspiration of CH.7 In their series, 14 patients were treated with aspiration alone (3 needed multiple aspirations), with a mean follow-up of 5.75 years. No failures were reported. This technique may hold promise for the future management of CH. Other authors contend aspiration has no role and believe that aspiration is often followed by recurrence, hemorrhage, or infection.
  • Radiofrequency ablation has been advocated for use with intraoral lymphatic malformations, especially microcystic lesions.
  • Magnetic resonance–controlled laser-induced interstitial thermotherapy is a novel therapy that has been proposed for treatment of lymphangiomas.
  • CH can present on routine prenatal ultrasonography as a large obstructing airway mass, as can other pathologic conditions such as a teratoma or rhabdomyosarcoma. If such a mass is visible on ultrasonography, MRI should be performed to further delineate the mass. In these cases, a multispecialty team including a high-risk obstetrician, pediatric otolaryngologist, pediatric surgeon, and neonatologist should be present at the ex utero intrapartum treatment (EXIT) procedure. A planned cesarean delivery is performed, and intubation or tracheostomy is used to establish an airway. Extracorporal membrane oxygenation (ECMO) should also be available. Excision of the CH is delayed until the child is stable. Intrauterine cyst aspiration to facilitate vaginal delivery has been reported in the literature.

Consultations

Depending on the anatomical location, referral to a surgeon or surgical specialist is appropriate. In patients with CH of the head and neck, referral to an otolaryngologist is appropriate.

Diet

No special dietary requirements are necessary for patients with CH.

Activity

Patients with CH should be directed to avoid direct trauma to the area because intralesional bleeding or infection can be precipitated by trauma.

Medication

Sclerosant therapy, as described in Treatment, is the only medical therapy available to treat cystic hygroma.

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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