Lymphangioma- Lymphatic Malformations of the Head and Neck

The nature of lymphatic malformations (LM) has sparked great interest since they were first described by Redenbacher in 1828. Many of the early researchers believed that lymphatic malformations were neoplasms. Currently, most researchers agree that LM are not neoplastic and have adopted the term "lymphatic malformation" to emphasize this fact.

Two major theories of the development of the lymphatic system have been proposed to explain the origin of LM. Sabin proposed that the lymphatic system develops from five primitive sacs which sprout from the venous system. In the head and neck, endothelial outbuddings from the jugular sac spread centrifugally to form the lymphatic system. McClure and Huntington proposed that the lymphatic system develops from mesenchymal clefts in the venous plexus reticulum and spread centripetally toward the jugular sac. LM develop from sequestration or congenital blockage of the primitive lymphatic anlage.

The most popular classification system was proposed by Landing and Farber in 1956. Lymphangioma simplex is composed of three walled lymphatic channels. Cavernous lymphangioma consists of dilated lymphatic spaces with increased fibrous advance. They also tend to invade surrounding tissue. Cystic lymphangioma is made up of endothelial lined cysts varying in size from mm to several cm. Histologically, there is no significant difference between the various clinically described types of lymphatic malformations according to Batsakis.

LM are considered rare entities. Bill and Sumner reported 5 new cases per 3000 first admissions per year. Gupta reported 1 in 4000 live births. TCH had 137 cases over a 13 year period from 1972-1985. In general, 50-60% of LM present at birth and 80-90% present by 2 years of age. In the adult population 45% cervical LM occur in the 40-60 year age range.

In most series, the head and neck are the most common sites for LM followed by the trunk, axilla, and extremities. In the head, the oral cavity and face area are the most common sites. In the neck, the posterior triangle is often quoted as the most common site. However, many series including that at TCH, have reported no difference between the anterior and posterior triangles. There does seem to be a predominance of right sided lesions. Laryngeal involvement is not uncommon.

The site of the lesion determines the symptomology. Many patients have no symptoms other than a soft tumor mass. Oral cavity and laryngeal involvement can produce stridor, dyspnea, and feeding difficulties.

Rapid expansion can occur with hemorrhage into the cyst or infection. Trauma can be associated with rapid development of cellulitis, and significant swelling of the LM. In cervicofacial LM, this can lead to life threatening airway obstruction.

Diagnosis in the case of large cervical LM is straightforward. The mass is soft, flaccid, fluctuant, with a multilobulated consistency. Imaging studies such as USG, CT, and MRI can be useful in treatment planning. In particular, CT and MRI are most useful in determining proximity to vital structures and mediastinal involvement preoperatively.

Many clinical entities have been reported to be associated with LM, including congenital glaucoma Klippel-Trenauny syndrome. In addition, Garden et al, reported on antenatal diagnoses using USG. Ten of 24 patients were diagnosed with Turner's syndrome.

LM represents <5% of all congenital neck masses and an even smaller number of neck masses in the adult. The differential diagnosis includes branchial cleft cyst, thyroglossal duct cyst, congenital retention cysts, dermal cyst, foregut cysts, deep hemangiomas, lymphadenitis, lipoma, malignancy and ranula.

Many alternative treatments have been proposed for LM. Most authors feel that spontaneous regression only rarely occurs. Broomhead reported a 15% spontaneous regression rate, but most authors do not report this experience. Steroids have been shown to cause regression in selected cases. Aspiration of the cystic contents was used as a temporizing measure in the past to relieve airway obstruction. Most authors discourage this practice because of the risk of infection. Sclerosing agents have been used in the past, but have fallen into disfavor due to the close proximity of major vascular structures and the brachial plexus. Ogita et al in Japan have described using OK-432 an immune modulator derived for low virulence strep pyogenes to stimulate an inflammatory response in the LM with resulting regression. Radiation therapy was used in the past, but is characterized by a high failure rate, and risk of lymphangitis, lymphedema, and lymphangiosarcoma.

Laser treatment is popular for the treatment of oral cavity and airway lesions. The advantages are less post-op edema, less tissue trauma and less bleeding. In these areas conservative therapy may result in frequent recurrence, while aggressive resection may lead to unacceptably high morbidity. The use of the CO2, Nd:YAG, and the Argon laser has been described. Multiple treatments are often necessary.

Surgical extirpation is usually the treatment of choice in cervical and facial LM. Complete exam is desirable, but can be difficult to achieve. Current mortality rates are about 3%. Incomplete resection is associated with higher recurrence rates. In the TCH series, the recurrence rate after complete exam was 6%; after incomplete exam, the rate was 35%. Ricciardeli and Richardson looked at recurrence rates of cervico-facial LM. They found that extraparotid, suprahyoid were associated with the highest recurrence rates. Lesions involving more than one site also had significantly higher recurrence rates. No infrahyoid lesions caused feeding difficulties or airway compromise, whereas suprahyoid lesions were associated with delayed feeding and respiratory difficulty. Children with infrahyoid or posterior cervical lesions can expect to have a low surgical complication rate and low recurrence. Patients with suprahyoid or tongue involvement are at severe risk for sudden swelling, airway obstruction, and feeding difficulties. When diagnosed at birth, surgical treatment of suprahyoid LM should be carefully considered. Tracheotomy and gastrostomy should be performed if needed. Attempts at surgical extripation should be delayed until adequate growth occurs to allow good anatomic dissection with less subsequent morbidity.

Case Presentation

A 2-year-old Latin American boy presented with a 2 to 3 month history of a slowly enlarging, non-tender, right neck mass. His birth history was unremarkable. He had no prior history of significant medical illness or hospitalizations. Physical exam revealed a 3.5cm x 3.5cm discrete mass anterior to the right sternocleidomastoid and inferior to the mandible. A CT of the neck confirmed the clinical impression of a cystic lesion of the anterior right neck consistent with a lymphatic malformation. Subsequently, the patient was taken to the operating room where the mass was excised without difficulty. Microscopic examination confirmed the diagnosis of cystic lymphangioma. Postoperatively, the patient was noted to have slight weakness of the right lower lip depressor. At his one month follow-up visit, the patient had resolution of the weakness. His incision was well healed, without evidence of recurrent disease.

April MM, Rebeiz EE, Friedman EM, Healy GB, Shapshay SM. Laser therapy for lymphatic malformations of the upper aerodigestive tract. Arch Otolaryngol Head Neck Surg 1992;118:205-208.

Balakrishnan A, Bailey CM. Lymphangioma of the tongue: a review of pathogenesis, treatment and the use of surface laser photocoagulation. J Laryngol Otol 1991;105:924-930.

Batsakis JG. Tumors of the Head and Neck: Clinical and Pathological Considerations, 2nd ed. Baltimore: Williams & Wilkins, 1979:301-304.

Bill AH, Sumner DS. A unified concept of lymphangioma and cystic hygroma. Surg Gynecol Obstet 1965;120:79-81.

Brock MR, Smith RJH, Parey SE, Mobley DL. Lymphangioma: an otolaryngologic perspective. Int J Pediatr Otorhinolaryngol 1987;14:133-140.

Brooks JE. Cystic hygroma of the neck. Laryngoscope 1973;83:117-128.

Broomhead IW. Cystic hygroma of the neck. Br J Plast Surg 1964;17:225-244.

Cohen SR, Thompson JW. Lymphangiomas of the larynx in infants and children: a survey of pediatric lymphangioma. Ann Otol Rhinol Laryngol Suppl 1986;127:1-20.

Dixon JA, Davis RK, Gilbertson JJ. Laser photocoagulation of vascular malformations of the tongue. Laryngoscope 1986;96:537-541.

Emery PJ, Bailey CM, Evans JNG. Cystic hygroma of the head and neck: a review of 37 cases. J Laryngol Otol 1984;98:613-619.

Evans DA, Baugh RF, Gilsdorf JR, Heidelberger KP, Niparko JK. Lymphangiomatosis of skull manifesting with recurrent meningitis and cerebrospinal fluid otorrhea. Otolaryngol Head Neck Surg 1990;103:642-646.

Garden AS, Benzie RJ, Miskin M, Gardner HA. Fetal cystic hygroma colli: antenatal diagnosis, significance, and management. Am J Obstet Gynecol 1986;154:221-225.

Goodman P, Yeung CS, Batsakis JG. Retropharyngeal lymphangioma presenting in an adult. Otolaryngol Head Neck Surg 1990;103:476-479.

Goshen S, Ophir D. Cystic hygroma of the parotid gland. J Laryngol Otol 1993;107:855-857.

Hamoir M, Remacle M, Youssif A, Moulin D, Francois G, Claus D, et al. Surgical management of parapharyngeal cystic hygroma causing sudden airway obstruction. Head Neck Surg 1988;10:406-410.

Karmody CS, Fortson JK, Calcaterra VE. Lymphangiomas of the head and neck in adults. Otolaryngol Head Neck Surg 1982;90:283-288.

Kennedy TL. Cystic hygroma-lymphangioma: a rare and still unclear entity. Laryngoscope 1989;99:1-5.

Mahboubi S, Potsic WP. Computed tomography of cervical cystic hygroma in the neck. Int J Pediatr Otorhinolaryngol 1989;18:47-51.

Marentette LJ. Craniofacial surgery for congenital and acquired deformities. In: Cummings CW, Krause CJ. Otolaryngology - Head and Neck Surgery. St. Louis: Mosby: 242-245.

Ninh TN, Ninh TX. Cystic hygroma in children: a report of 126 cases. J Pediatr Surg 1974;9:191-195.

Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Arch Otolaryngol Head Neck Surg 1991;117:546-553.

Schefter RP, Osen KD, Gaffey TA. Cervical lymphangioma in the adult. Otolaryngol Head Neck Surg 1985;93:65-69.

Singh S, Baboo ML, Pathak IC. Cystic lymphangioma in children: report of 32 cases including lesions at rare sites. Surgery 1971;69:947-951.

Stal S, Hamilton S, Spira M. Hemangiomas, lymphangiomas, and vascular malformations of the head and neck. Otolaryngol Clin North Am 1986;19:769-796.

Suen JY, Waner M. Treatment of oral cavity vascular malformations using the neodymium:YAG laser. Arch Otolaryngol Head Neck Surg 1989;115:1329-1333.

Ward PH, Harris PF, Downey W. Surgical approach to cystic hygroma of the neck. Arch Otolaryngol 1970;91:508-518.

White B, Adkins WY. The use of the carbon dioxide laser in head and neck lymphangioma. Lasers Surg Med 1986;6:293-295.

Wright GL, Smith RJH, Katz CD, Atkins JH Jr. Benign parotid diseases of childhood. Laryngoscope 1985;95:915-920.

Yuh WTC, Buehner SL, Kao SCS, Robinson RA, Dolan DK, Phillips JJ. Ann Otol Rhinol Laryngol 1991;100:737-742.