CERVICOFACIAL LYMPHANGIOMAS

Author
Ted L Tewfik, MD Professor of Otolaryngology-Head and Neck Surgery, Professor of
Pediatric Surgery, McGill University Faculty of Medicine; Senior Staff, Montreal Children's
Hospital, Montreal General Hospital, and Royal Victoria Hospital

BACKGROUND
The natural history of cervicofacial lymphangioma is the presence of a mass in the
head and neck area. Cervicofacial lymphangioma is noted before birth, at birth, or in the first
few months of life in less than 50% of patients with this condition. The initial appearance of
cystic hygroma or lymphangioma in adulthood is less common. The growth rate of this
neoplasm is variable. Slow progression followed by pseudoregression eventually gives rise to
reappearance. Rapid growth or engorgement with lymph or blood is often associated with
direct infection, trauma, or a secondary respiratory or skin infection. Spontaneous regression
has been reported in as many as 15% of patients with cervicofacial lymphangioma. Malignant
change has not been reported.
An image depicting a lymphatic malformation can be seen below.

Reconstruction of CT in sagittal dimension. This patient was intubated because of

respiratory distress caused by the large lymphatic malformation.
PROBLEM
Lymphatic malformation or lymphangioma is a rare neoplasm or congenital rest that
presents in the head and neck region in more than 70% of cases. Whether patients with this
neoplasm present in childhood or adulthood, the identification of a mass requires a diagnosis
and management plan. Management may be complex and difficult because enlargement often
results in functional and cosmetic problems. Attempts at a classification have been created to
differentiate between the capillary, cystic, and cavernous forms of cervicofacial
lymphangioma; however, the histologic appearance is not always uniform, which suggests a
variable clinical and pathologic appearance of essentially the same disease. Determining
location, persistence, growth patterns, and functional and cosmetic problems associated with
lymphangioma usually requires a full diagnostic evaluation. Proactive therapy chosen on the
basis of a careful clinical assessment follows the diagnosis.

EPIDEMIOLOGY
Frequency

Cervicofacial lymphangioma is uncommon, representing fewer than 6% of benign

tumors of childhood. Incidence has been reported to be less than 2.8 per 1000 population. No
sex preponderance or side predilection has been reported.

ETIOLOGY
The origin of lymphangiomas is controversial. Theories include lymphangiomas as
true neoplasms, hamartomas, or congenital dysplasias of the lymphatics. To determine the
role of angiogenesis in the pathogenesis of lymphangioma, Sidle et al (2005) examined
patients' specimens for expression of angiogenic inducer vascular endothelial growth factor
(VEGF) and angiogenic inhibitor pigment epithelium-derived factor (PEDF) using
immunohistochemical analysis.[1] Staining patterns of VEGF and PEDF were evaluated.
Histological evidence of increased angiogenesis, including microvascular density, stromal
fibrosis, and inflammation, were graded in each group and correlated with recurrence.
Lymphangioma specimens demonstrated histological evidence of increased
angiogenic activity including multiple areas of increased VEGF staining combined with little
PEDF staining. Recurrent specimens had increased histological evidence of angiogenesis as
well as increased VEGF and decreased PEDF activity compared with nonrecurrent lesions.
 They concluded that lymphangiomas exhibit tumorlike pathogenesis owing to the
high expression of angiogenic inducers compared with the low expression of inhibitors.
Recurrence may be influenced by this imbalance of angiogenic mediators. Further research
with antiangiogenic therapy using agents such as PEDF analogues or anti-VEGF receptor
antibodies is indicated.
However, most physicians favor the theory that dysplastic lymphatic tissue is
sequestered in a target tissue space or organ during fetal development. Sabin (1909) proposed
that lymphatics are derived from primitive veins by budding endothelial cells.[2] Because
jugular sacs are the largest formations, the high incidence in the neck correlates well with
clinical studies and experience. Incomplete canalization of the lymphatics causes obstruction
of lymph flow and cyst or cavern development. These formations are analogous to blood-
containing cavernous hemangiomas. Target organs and tissues include the retroperitoneum,
chest wall, lung, mesentery, pancreas, scrotum, tongue, floor of the mouth, larynx, neck, lip,
mediastinum, parotid gland, adrenal gland, axilla, diaphragm, gallbladder, spleen, skull base,
colon, breast, subdural space, and pelvis.
A recent case series (2013) of 141 patients concluded that airway involvement of
lymphatic malformations predominantly affects the oral cavity and oropharynx and no cases
of glottis, subglottic, or tracheal involvement have been seen.[3]
Pathophysiology
Macroscopically, lymphangiomas are large cavernous, cystic, or complex multilocular
masses that extend into tissue fascial planes. Patients usually present with a painless
enlarging mass. Cystic types infiltrate into the surrounding tissue by fingerlike multiloculated
extensions. Within these multiloculated honeycombed cysts is a clear-to-cloudy, sometimes
blood-tinged, fluid with the consistency of uncooked egg whites. Cyst walls are indistinct;
penetration and decompression of the cyst is a common occurrence during surgical excision.
In many cases, cervical fetal lymphangi oma may be diagnosed before birth by means of
ultrasonography. Before 30 weeks' gestation, cervical lesion is almost always associated with
chromosomal abnormalities and congenital cardiac malformations usually in the septated
type. A high rate of spontaneous abortion is encountered in this type.
The different types of cervicofacial lymphangioma are classified as follows:
 Lymphangioma circumscriptum is a simplex superficial red macular or vesicular lesion of
mucous membranes or skin.
 Lymphangioma capillary type is a simplex lesion of dilated capillarylike channels.
 Lymphangioma cavernosa is a simplex lesion of dilated lymphatic channels with deep
extension and without cyst formation.
 Lymphangioma cystica (ie, cystic hygroma) is composed of large lymphatic cysts that
expand into adjacent soft tissue planes and are well defined, circumscribed, or lobulated.
 Lymphangioma complex is composed of multiloculated poorly defined cysts extending to
more than one anatomic area, tissue plane, or organ system.

PRESENTATION
Children or, less commonly, adults usually present with a mass in the head and neck area.
Approximately 90% of cases of cervicofacial lymphangioma become clinically apparent by
the patient's third year of life. Common head and neck sites in childhood are the cervical area,
floor of the mouth, and the tongue. Orofacial manifestations include mandibular or maxillary
deformation, rotation, and malocclusion (ie, crossbite). In a study by Orvidas et al, the most
common location involved was the submandibular region, followed by the parotid and
cheek.[4]
 Obtain a careful history.
 Perform a complete head and neck examination as well as a general examination.
 Lymphangioma in the head and neck generally involves a swelling or mass that is soft to
palpation and well circumscribed or diffuse. It may have defined or ill-defined borders and
is often associated with a bluish discoloration.
 Regional venous congestion and the absence of pain are common.
 Common additional symptoms and findings include diplopia, proptosis, respiratory
distress, dysphagia, and dysphonia.
 Children with massive head and neck and chest lymphangiomas have higher mortality and
morbidity. The involvement of the airway is an important prognostic factor. [5]
Differential diagnosis consideration

Differentiation from other cervical congenital masses or malignancy is necessary in

children and adults. The presence of a mass in the larynx or trachea is usually associated with
airway compromise and may require differentiation from a subglottic hemangioma. The
presence of a mass in an anatomic location generates a differential diagnosis, particularly
when combined with diagnostic studies. The cystic nature of the process and the bluish hue
often associated with this growth frequently resemble characteristics of a hemangioma.
Lesions in the floor of the mouth, particularly when bulging in the submental or submaxillary
region, may simulate a plunging ranula. Other differential considerations in the head and
neck/axillary region include a branchial cleft cyst, a thyroglossal duct cyst, an axillary
lymphocele, a varix, a lipoma, a schwannoma, a parotid cyst, malignant tumors, or other
growths capable of arising in this anatomic area.
Kura and Rane (2011) documented a case of cervicofacial actinomycosis mimicking
lymphangioma circumscriptum. It presented as "lumpy jaw" with draining sinuses that
discharged the characteristic "sulfur granules." It responded well to penicillin.[6]
Location of different classifications of cervicofacial lymphangioma

Cystic lymphatic malformation is more common in the neck, while the cavernous type
usually appears in the lips, tongue, cheek, and floor of the mouth. The anterior two thirds of
the tongue is the most common intraoral site, producing macroglossia. Enlargement of this
congenital rest in the upper aerodigestive tract can result in airway distress, dysphagia,
aspiration, and difficulty breastfeeding in a child. Cystic hygroma may be a solitary or
complex opaque neck mass that spreads along multiple tissue planes.
Cavernous lymphangioma may be well circumscribed or diffuse. Extension into the
skin produces a bluish discoloration. The diffuse form tends to infiltrate into the surrounding
tissue planes, around blood vessels and nerves with poorly defined borders. An attempt at
staging has been reported. This system stages lymphangioma from early T1 to late T4 disease
and is based on cosmetic changes, functional deficits, age of patient at diagnosis, and the
number of sites involved.

INDICATIONS
Indications for therapy in the neonate with cervical lymphangioma are complex.
Severe cosmetic changes do not necessitate immediate intervention unless functional
impairment is occurring. Failure to thrive because of ineffective breastfeeding in a child and
aspiration and airway compromise during the first 3 months of life are clear indications for
immediate intervention. Rapid growth at any age is another indication for immediate
intervention. Assess persistence or recurrence of disease in older age groups according to
location, functional loss, and cosmetic impairment. Lymphangioma is best treated by means
of surgical resection. However, surgical resection may be associated with a significant
neurologic deficit and high rate of recurrence of the lymphangioma, which is reported to
approach 81% in the cystic type arising above the hyoid bone.
CONTRAINDICATIONS
The principle contraindication to surgery is the circumstance in which further
intervention will result in serious functional or cosmetic consequences rendering the quality
of life below the standards of a reasonable functional life. This problem often arises in
clinical situations in which previous therapy has resulted in significant complications that are
often neurologic. Multiple recurrences usually indicate that the probability of cure from
additional surgery is low. These recurrences are usually associated with lymphangiomas
diagnosed in patients younger than 1 year or with lymphangiomas involving the lip,
hypopharynx, larynx, tongue, and floor of the mouth.
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