What is lymphangioma? What are the theories of lymphangioma? What are its type? What are its clinical presentation?

Lymphangioma (LA) are degenerative lesions arising from the lymphatic and refers to a group of developmental anomalies in which the lymphatics fails to connect in the normal way to the venous channels. As a result there are large fluid filled spaces occupying the tissues and expanding tissue planes.

The lymphatic system arises from 5 primitive sacs developed from the venous system

– 2 Jugular sacs

– 2 posterior sciatic sacs

-Single retroperitoneal sac

From these, endothelial buds extends centrifugally to form the peripheral lymphatic system. Two principal theories have been postulated to explain the origin of other lymphangiomas.

• Sequestration of lymphatic tissue derived from segments of the primitive sacs which retain the proliferative growth potential bear no connection with the normal lymphatic system.
• Endothelial fibrillar membrane proliferation from the walls of the cyst, penetrate the surrounding tissue along the line of least resistance between muscles and vessels to canalize and produce more cysts.These lesion are congenital arising from localized lymphatic stasis caused by congenital blockage of regional lymphatic drainage.

  TYPES OF LYMPHANGIOMA

A) Morphological types:

1. Lymphangioma simplex (capillary lymphangioma) -40%
   1. composed of the walled, capillary sized lymphatic channels
   2. Usually asymptomatic
   3. Present as pole, small vesicle like lesions visible on the skin or oral cavity.
2. Cavernous lymphangioma (35 to 45%)
   1. composed of dilated lymphatic spaces
   2. Often has fibrous adventitia
   3. Typically occurring in the tongue, cheeks and lips
   4. Present as patient diffuse swelling.
3. Cystic hygroma (20-25%): Macro cystic lesion c well defined margin
   1.  composed of cysts and sinuses
   2. Varying in size from a few mm to several cm in diameter.
   3. Present as a cystic mass of varying diameter containing eosinophilic acellular lymph fluid.
   4. Thin walled sinuses are lined by flat endothelial cells.
   5. These larger cystic masses which may communicate or be isolated.

B. Histological classification

1. Macro cystic
   1. large and compressible
   2. frequently multilocular
   3. ususally infrahyoid
2. Microcystic
   1. smaller and firmer
   2. Suprahyoid usually
3. Combined

PRESENTATION

1. Although congenital and in majority of cases present at birth they become clinically evident by 2-3 year of life
2. M:F =1:1
3. R=L=1:1
4. They can appear anywhere in the head and neck and about 75% of  cases of lymphangiomas are in head and neck region.
5. Simple and cavernous lymphangioma occurs where the tissue planes are relatively tight eg. tongue, lips cheeks
6. Acytic hygroma aeise mainly in neck where it has more lax.
7. Most lesions appears in posterior triangle often occupying the supra  clavicular fossa.
8. Commonest salivary region involved in parotid gland area.
9. Oral: 35%
10. cervical: 25%
11. Axillary: 15%
12. 75% of all lymphomas are located in neck, usually in posterior triangle with 10% extending to mediastinum
13. Smooth, soft, cystic, trans illuminate and demonstrate ballotment
14. Usually asymptomatic and slow growing except a distressing cosmetic problem. Cystic hygroma and cavernous lymphangioma either grow progressively or remain static and there have also been reports of involution.(stell & marans)

Rapid growth leading to pain airway compromise occur due to:

• Trauma causing internal venous haemorrhage
• Infection
• Rapid growth may lead to enlargement of affected side of face obscuring eyes and distorting mouth.There may be hypertrophy of mandible or maxilla but not so apparent.
• Airway obstruction more with intraoral/ intralingual lymphangioma.
• Another danger of excessive growth is damage to bone by pressure, absorption and vascular compression.
• The facial nerve is very resistant and is almost never damaged.
• Brachial plexus if compressed- pain and hyperaesthesia

 

Natural history

• Cystic hygromas of head and neck do not involute spontaneously.
• Some progression may take place with enlargement and infiltrative growth into previously unaffected areas sometime.
• Recurrent episodes of acute infection may arise and the lesion will become larger painful and inflamed. As a result of this infection, related IV can become enlargement
• The activity in cystic hygroma may subside with increasing age.
• There is no potential for malignant change.
• Rarely sequestration of fluid within a cystic hygroma especially in neonates may result in hypovolemic shocks and widespread intravascular thrombosis.

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