eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Cervicofacial Lymphangiomas: Workup

Author: Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Coauthor(s): Richard L Fabian, MD, Former Director, Former Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Contributor Information and Disclosures

Updated: Oct 17, 2008

Workup

Imaging Studies

  • Barium swallow and/or upper and lower GI series, when indicated
  • Chest radiography
  • Computed tomography
    • CT identifies a multiloculated cyst with smooth septa, which enhances uniformly after contrast injection.
    • CT fluid density ranges from -4 to 34 HU, depending on the lipid content and presence of blood.
    • CT contrast is unpredictable because enhancement occurs in only 50% of cases.
  • Magnetic resonance imaging: MRI is the study of choice in the evaluation of head and neck lymphangiomas.
  • Ultrasonography: For cervical lymphangiomas, the ultrasonogram depicts a cystic mass with smooth, thin, or irregular walls.
  • Arteriography: This study is indicated in cases in which a hemangioma, an arterial vascular (A/V) malformation, paragangliomas/glomus tumors, or vascular schwannomas are strongly suggested by means of conventional imaging studies.
  • Screening ultrasonography and MRI
    • Screening ultrasonography has been a valuable tool to evaluate for fetal abnormalities, such as cystic hygroma, diaphragmatic hernia, bronchopulmonary sequestration, fetal airway obstruction, and other life-threatening birth defects.
    • MRI is helpful to corroborate these findings and refine the diagnosis.
    • This combination of imaging techniques is also useful when evaluating the abdomen in children and adults.
  • Lymphoscintigraphy: This technique may help identify the source of lymph flow but is not available as a standard clinical study.
  • Nuclear magnetic imaging (NMR) and MRI
    • These imaging studies delineate lymphangiomas more accurately with excellent identification of extent because of T2-weighted sequences. Images on T2 have greater signal intensity than muscle, cerebrospinal fluid, and fat.
    • NMR is the study of choice before surgical excision.
    • Gadolinium adds no additional imaging characteristics.

Other Tests

  • Depending on the anatomic location, other diagnostic studies may include the following:
    • Full neuro-ophthalmic examination for orbital or central lesions
    • Laryngoscopy, bronchoscopy, and/or esophagoscopy for lesions of the upper aerodigestive system
    • Abdominal endoscopy and/or colonoscopy for lower GI lesions

Diagnostic Procedures

  • Slim-needle biopsy usually yields straw-colored fluid, not the cellular material necessary for histologic diagnosis.
  • Tissue diagnosis, as a direct and final correlate to imaging studies, is the best and most consistent confirmation of lymphangioma.

Histologic Findings

Excluding simplex circumspecta, lymphangiomas may histopathologically be classified into 2 types—the cavernous type and the cystic type. However, histologic characteristics of both types are the usual finding. The cavernous variety tends to involve the lips, tongue, and floor of the mouth. On histologic examination, these masses are composed of dilated cystic spaces containing blood or lymph lined by endothelial cells with a scant-to-dense fibrous stroma. Cell types in the stroma include lymphocytes, lymphatic tissue, fat, or muscle. Spaces vary in size, from capillary to cavernous channels often resembling a Swiss-cheese pattern. Indiscreet margins with no encapsulation correlate with the gross appearance in lobulated cystic types, which have multiple fingerlike extensions into the surrounding tissue.

More on Cervicofacial Lymphangiomas

Overview: Cervicofacial Lymphangiomas
Workup: Cervicofacial Lymphangiomas
Treatment: Cervicofacial Lymphangiomas
Follow-up: Cervicofacial Lymphangiomas
References
[ CLOSE WINDOW ]

References

  1. Sidle DM, Maddalozzo J, Meier JD, et al. Altered pigment epithelium-derived factor and vascular endothelial growth factor levels in lymphangioma pathogenesis and clinical recurrence. Arch Otolaryngol Head Neck Surg. Nov 2005;131(11):990-5. [Medline].

  2. Sabin FR. The lymphatic system in human embryos, with a consideration of the morphology of the system as a whole. Am J Anat. 1909;9:43-91.

  3. Orvidas LJ, Kasperbauer JL. Pediatric lymphangiomas of the head and neck. Ann Otol Rhinol Laryngol. Apr 2000;109(4):411-21. [Medline].

  4. Peral Cagigal B, Serrat Soto A, Calero H, et al. [OK-432 therapy for cervicofacial lymphangioma in adults]. Acta Otorrinolaringol Esp. May 2007;58(5):222-4. [Medline].

  5. 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].

  6. Anadol AZ, Oguz M, Bayramoglu H, et al. Cystic lymphangioma of the spleen mimicking hydatid disease. J Clin Gastroenterol. Jun 1998;26(4):309-11. [Medline].

  7. Anderson NG, Kennedy JC. Prognosis in fetal cystic hygroma. Aust N Z J Obstet Gynaecol. Feb 1992;32(1):36-9. [Medline].

  8. Antoniades K, Kiziridou A, Psimopoulou M. Traumatic cervical cystic hygroma. Int J Oral Maxillofac Surg. Feb 2000;29(1):47-8. [Medline].

  9. Baer S, Davis J. Cystic hygroma presenting in adulthood. J Laryngol Otol. Oct 1989;103(10):976-7. [Medline].

  10. Bagwell CE. CO2 laser excision of pediatric airway lesions. J Pediatr Surg. Nov 1990;25(11):1152-6. [Medline].

  11. Borecky N, Gudinchet F, Laurini R, et al. Imaging of cervico-thoracic lymphangiomas in children. Pediatr Radiol. 1995;25(2):127-30. [Medline].

  12. Boxen I, Zhang ZM, Filler RM. Lymphoscintigraphy for cystic hygroma. J Nucl Med. Apr 1990;31(4):516-8. [Medline].

  13. Bronshtein M, Bar-Hava I, Blumenfeld I, et al. The difference between septated and nonseptated nuchal cystic hygroma in the early second trimester. Obstet Gynecol. May 1993;81(5 (Pt 1)):683-7. [Medline].

  14. Caro PA, Mahboubi S, Faerber EN. Computed tomography in the diagnosis of lymphangiomas in infants and children. Clin Imaging. Jan-Mar 1991;15(1):41-6. [Medline].

  15. Chappuis JP. [Current aspects of cystic lymphangioma in the neck]. Arch Pediatr. Feb 1994;1(2):186-92. [Medline].

  16. Charabi B, Bretlau P, Bille M, et al. Cystic hygroma of the head and neck--a long-term follow-up of 44 cases. Acta Otolaryngol Suppl. 2000;543:248-50. [Medline].

  17. Chong KT, Ong CL. Cystic hygroma in adulthood. Singapore Med J. Jun 1997;38(6):261-2. [Medline].

  18. Claesson G, Gordon L, Kuylenstierna R. [A revolutionary Japanese method for treatment of lymphangioma]. Lakartidningen. Apr 29 1998;95(18):2074-7. [Medline].

  19. Duman L, Karnak I, Akinci D, et al. Extensive cervical-mediastinal cystic lymphatic malformation treated with sclerotherapy in a child with Klippel-Trenaunay syndrome. J Pediatr Surg. Jan 2006;41(1):e21-4. [Medline].

  20. Edwards PD, Rahbar R, Ferraro NF, et al. Lymphatic malformation of the lingual base and oral floor. Plast Reconstr Surg. Jun 2005;115(7):1906-15. [Medline].

  21. Emery PJ, Bailey CM, Evans JN. Cystic hygroma of the head and neck. A review of 37 cases. J Laryngol Otol. Jun 1984;98(6):613-9. [Medline].

  22. Emran MA, Dubois J, Laberge L, et al. Alcoholic solution of zein (Ethibloc) sclerotherapy for treatment of lymphangiomas in children. J Pediatr Surg. May 2006;41(5):975-9. [Medline].

  23. Fageeh N, Manoukian J, Tewfik T, et al. Management of head and neck lymphatic malformations in children. J Otolaryngol. Aug 1997;26(4):253-8. [Medline].

  24. Farmand M, Kuttenberger JJ. A new therapeutic concept for the treatment of cystic hygroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Apr 1996;81(4):389-95. [Medline].

  25. Figi FA. Radium in treatment of multilocular lymph cysts of neck in children. AJR Am J Roentgenol. 1929;21:473-80.

  26. Filston HC. Hemangiomas, cystic hygromas, and teratomas of the head and neck. Semin Pediatr Surg. Aug 1994;3(3):147-59. [Medline].

  27. Giguere CM, Bauman NM, Sato Y, et al. Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: a prospective multi-institutional trial. Arch Otolaryngol Head Neck Surg. Oct 2002;128(10):1137-44. [Medline].

  28. 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].

  29. Goshen S, Ophir D. Cystic hygroma of the parotid gland. J Laryngol Otol. Sep 1993;107(9):855-7. [Medline].

  30. Grosfeld JL, Skinner MA, Rescorla FJ, et al. Mediastinal tumors in children: experience with 196 cases. Ann Surg Oncol. Mar 1994;1(2):121-7. [Medline].

  31. Hancock BJ, St-Vil D, Luks FI, et al. Complications of lymphangiomas in children. J Pediatr Surg. Feb 1992;27(2):220-4; discussion 224-6. [Medline].

  32. Hase T, Kodama M, Sano H, et al. Successful treatment of a recurrent large cystic hygroma in a child with intracystic injection of OK-432. Eur J Pediatr. May 1996;155(5):424. [Medline].

  33. Holland AJ. OK-432 and lymphatic malformations in children: The Starship Children's Hospital experience. J Pediatr Surg. Jul 2005;40(7):1214.

  34. Hutchinson I. Mandibular enlargement: an unusual complication of a cervical cystic hygroma. Int J Paediatr Dent. Jun 1996;6(2):123-7. [Medline].

  35. Ikarashi T, Inamura K, Kimura Y. Cystic lymphangioma and plunging ranula treated by OK-432 therapy: a report of two cases. Acta Otolaryngol Suppl. 1994;511:196-9. [Medline].

  36. Kennedy TL, Whitaker M, Pellitteri P, et al. Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope. Nov 2001;111(11 Pt 1):1929-37. [Medline].

  37. Lacosta JL, Zabaleta M, Sanchez Galan L. [Congenital cervical deformities]. An Otorrinolaringol Ibero Am. 1998;25(2):173-83. [Medline].

  38. Landing BH, Farber S. Tumors of the cardiovascular system. In: Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology; 1956:124-38.

  39. Mahboubi S, Potsic WP. Computed tomography of cervical cystic hygroma in the neck. Int J Pediatr Otorhinolaryngol. Sep 1989;18(1):47-51. [Medline].

  40. Mikhail M, Kennedy R, Cramer B, et al. Sclerosing of recurrent lymphangioma using OK-432. J Pediatr Surg. Aug 1995;30(8):1159-60. [Medline].

  41. Ogita S, Tsuto T, Nakamura K, et al. OK-432 therapy for lymphangioma in children: why and how does it work?. J Pediatr Surg. Apr 1996;31(4):477-80. [Medline].

  42. Ogita S, Tsuto T, Tokiwa K, et al. Treatment of cystic hygroma in children with special reference to OK-432 therapy. Z Kinderchir. Oct 1987;42(5):279-81. [Medline].

  43. Orford J, Barker A, Thonell S, et al. Bleomycin therapy for cystic hygroma. J Pediatr Surg. Sep 1995;30(9):1282-7. [Medline].

  44. Pia F, Aluffi P, Olina M. [Cystic lymphangioma in the head and neck region]. Acta Otorhinolaryngol Ital. Apr 1999;19(2):87-90. [Medline].

  45. Pui MH, Li ZP, Chen W, et al. Lymphangioma: imaging diagnosis. Australas Radiol. Nov 1997;41(4):324-8. [Medline].

  46. Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch Otolaryngol Head Neck Surg. May 1991;117(5):546-53. [Medline].

  47. Ruiz E Jr, Valera ET, Verissimo F, et al. [OK-432 therapy for lymphangioma in children]. J Pediatr (Rio J). Mar-Apr 2004;80(2):154-8. [Medline].

  48. Samuel M, McCarthy L, Boddy SA. Efficacy and safety of OK-432 sclerotherapy for giant cystic hygroma in a newborn. Fetal Diagn Ther. Mar-Apr 2000;15(2):93-6. [Medline].

  49. Sato K, Maekawa T, Yabuki K, et al. Cystic lymphangiomas of the colon. J Gastroenterol. Aug 1999;34(4):520-4. [Medline].

  50. Sermon A, Gruwez JA, Lateur L, et al. The importance of magnetic resonance imaging in the diagnosis and treatment of diffuse lymphangioma. Acta Chir Belg. Oct 1999;99(5):230-5. [Medline].

  51. Shimizu K, Nishino M, Arita K. Cystic lymphangioma: its orofacial manifestations. ASDC J Dent Child. Nov-Dec 1999;66(6):377-82, 366. [Medline].

  52. Smith RJ, Burke DK, Sato Y, et al. OK-432 therapy for lymphangiomas. Arch Otolaryngol Head Neck Surg. Nov 1996;122(11):1195-9. [Medline].

  53. Stein M, Hsu RK, Schneider PD, et al. Alcohol ablation of a mesenteric lymphangioma. J Vasc Interv Radiol. Feb 2000;11(2 Pt 1):247-50. [Medline].

  54. Suzuki Y, Obana A, Gohto Y, et al. Management of orbital lymphangioma using intralesional injection of OK- 432. Br J Ophthalmol. Jun 2000;84(6):614-7. [Medline].

  55. Takinami S, Kaga M, Yahata H, et al. Radiation-induced hypoplasia of the teeth and mandible. A case report. Oral Surg Oral Med Oral Pathol. Sep 1994;78(3):382-4. [Medline].

  56. Tanigawa N, Shimomatsuya T, Takahashi K, et al. Treatment of cystic hygroma and lymphangioma with the use of bleomycin fat emulsion. Cancer. Aug 15 1987;60(4):741-9. [Medline].

  57. Thomas RL. Prenatal diagnosis of giant cystic hygroma: prognosis, counselling, and management; case presentation and review of the recent literature. Prenat Diagn. Nov 1992;12(11):919-23. [Medline].

  58. Tibesar RJ, Rimell FL, Michel E. Cystic hygroma of the skull base. Arch Otolaryngol Head Neck Surg. Dec 1999;125(12):1390-3. [Medline].

  59. Turner C, Gross S. Treatment of recurrent suprahyoid cervicofacial lymphangioma with intravenous cyclophosphamide. Am J Pediatr Hematol Oncol. Nov 1994;16(4):325-8. [Medline].

  60. Vigil DV, DiFiori JP, Puffer JC, et al. Arachnoid cyst and subdural hygroma in a high school football player. Clin J Sport Med. Jul 1998;8(3):234-7. [Medline].

  61. Watson WL, McCarthy WD. Blood and lymph vessel tumors. A report of 1,056 cases. Urg Gynecol Obstet. 1940;71:569-71.

  62. White CW, Wolf SJ, Korones DN, et al. Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr. Jan 1991;118(1):59-66. [Medline].

  63. Yang AD, Chang YL, Chaou WT. A huge cystic hygroma of the neck associated with intraventricular haemorrhage in a term neonate. Acta Paediatr. Mar 1999;88(3):344-6. [Medline].

  64. Yuh WT, Buehner LS, Kao SC, et al. Magnetic resonance imaging of pediatric head and neck cystic hygromas. Ann Otol Rhinol Laryngol. Sep 1991;100(9 Pt 1):737-42. [Medline].

  65. Zheng JW, Qin ZP, Zhang ZY. [Management of lymphatic malformations in oral and maxillofacial regions: the rationale according to the new classification]. Shanghai Kou Qiang Yi Xue. Dec 2005;14(6):553-6. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

lymphangioma, lymphangiomas, cystic hygroma, cervicofacial lymphangiomas, lymphangioma circumscriptum, lymphangioma capillary type, lymphangioma cavernosa, lymphangioma cystica, lymphangioma complex

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Ted L Tewfik, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Society of Pediatric Otolaryngology, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Richard L Fabian, MD, Former Director, Former Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Richard L Fabian, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Ophthalmology, American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Medical Association, American Society for Head and Neck Surgery, Association of Military Surgeons of the US, and Undersea and Hyperbaric Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Daniel J Kelley, MD, Consulting Staff, Eastern Shore ENT and Allergy Associates and Peninsula Regional Medical Center
Daniel J Kelley, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Laryngological Rhinological and Otological Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Karen Hall Calhoun, MD, William E Davis Professor and Chair, Department of Otolaryngology-Head and Neck Surgery, University of Missouri
Karen Hall Calhoun, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Medical Association, American Rhinologic Society, Association for Research in Otolaryngology, Society of University Otolaryngologists-Head and Neck Surgeons, Southern Medical Association, Texas Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.