A 25 yearoldwoman with lymphangioma circumscriptum of the skin and tongue associated with cystic hygroma is reported for its rarity and unique presentation. Fifty percent of all lymphatic malformations are already obvious at the time of birth, and as many as 90% are diagnosed by the end of the second year of life owing to clinical symptoms. Conservative surgical excision is preferred in super. We present a 17yearold white male with lymphangioma of the right tonsil. Postoperatively, her tongue was located completely within her mouth with good cosmetic results. The clinical appearance of lymphangioma depends on the. Lymphangioma article about lymphangioma by the free dictionary. If the tumor is located in a deeper area, it may present as a submucosal mass11. Lymphangioma of the tongue is a common cause of macroglossia in children associated with difficulty in swallowing and mastication, speech disturbances, airway obstruction, mandibular prognathism, openbite and other possible deformities of maxillofacial structures. In infants, the growth rate gets accelerated and can be distinguished from the other types by the presence of clear fluid within the.
This article focuses on the general features of lymphangiomas. Oral lymphangioma is uncommon lesions that tend to involve the dorsal surface of the tongue. We describe our experience with a debilitated yearold girl who presented with severe macroglossia, secondary to. Bhayya h, pavani d, avinash tejasvi ml, geetha p 2015 oral lymphangioma. It appears as a cluster of small, firm blisters most commonly on the shoulders, neck, underarm area, limbs, and in the mouth, especially the tongue. Lymphatic malformations radiology reference article. It can occur in any region of the body, including the tongue and is composed of dilated lymphatic vessels. Lymphangioma is a benign tumor of the lymphatic vessels.
For example, a swollen tongue can cause speech and eating difficulties. Review article lymphangioma circumscriptum of the skin. It is usually bilateral and posterior, whereas lymphangiomas are lateral or anterior and are diagnosed in the second and third trimesters. Using a combination of a vshaped and central resection, we successfully treated a 6yearold girl who had massive lymphangioma of the tongue. Lymphangioma may be congenital or acquired, but is usually detected. A lymphangioma is a type of swelling that affects the neck, mouth, or head.
The treatment of recurrent lymphangioma in the oral buccal. Lymphangioma definition of lymphangioma by medical dictionary. Although tongue is a rare site for lymphangioma it is the most common cause for macroglossia 2. Surgical treatment of oral lymphangiomas with 2 laser. In contrast, capillary lymphangioma is usually confined to the skin and is clinically the least significant of the three types. Benign lymphangioendothelioma acquired progressive lymphangioma. Respiratory obstruction is a complication of head and neck lymphangiomas, especially in late presentations and can be due to the infiltration of the malformation in the tongue, pharynx, or larynx, or blockage of. Jul 19, 2017 the following are the complications of lymphangioma. Its pathogenesis is uncertain, but history, clinical examination, and histological examination should establish the diagnosis. This is an interesting case of lymphangioma of the tongue in a 2yearfemale child. Most lymphangiomas represent defects of development. Pdf lymphangiomas are developmental malformations and have a marked predilection. The most common location in the mouth is the dorsum of tongue, followed by lips.
After 3 months of treatment, the mass had decreased by. Lymphangioma is a benign, harmatomatous tumour of lymphatic vessels with a marked predilection for the head and neck region the aim of this report was to document a rare case of lymphangioma of the tongue. Lymphangioma causes, symptoms, diagnosis, treatment and. It is related to the lymphatic system and it is not.
Lymphangioma is a benign hamartomatous lesion caused by congenital malformation of the lymphatic system. Lymphangioma circumscriptum of the skin and tongue associated. It is usually present at birth or appears within the first 2 years. It appears as a skin colored, red or bluish rubbery inflammation under the skin. Redenbacher first described lymphangioma in 1828, and lymphangioma of the tongue was reported later in 1854 by virchow 2, 7. The mainstay of treatment for tongue lymphangioma is surgery15,16 on t h oehret hand sclerotherapy, simple electrocautery, vaporization with co2 laser, radiotherapy, pulsedye laser and radiofrequency could be used as a treatment. Case report a 16 year old female patient presented to the department oral and maxillofacial pathology with a painless swelling on the dorsal surface of anterior tongue. In 1976, whimster studied the pathogenesis of lymphangioma circumscriptum, finding lymphatic cisterns in the deep subcutaneous plane are separated from the normal network of lymph vessels. Lymphangiomas are benign lesions of vascular origin that show lymphatic differentiation. The lymphangiomas are localized in the head and neck area in about 75% of cases and about 80% of these cases are children less than 2 years old1. They communicate with the superficial lymph vesicles through vertical, dilated lymph channels.
They have poor communication with the normal lymph systems and therefore collect lymph. The presence of a 12 cm sized mass in the mouth or on the lips. The lesion is often asymptomatic and is detected with the appearance of a slow growing painless cystic mass covered by healthy mucosa 9. A diffusely enlarged tongue was present at birth and continued to grow as the child. Congenital macroglossal angiodysplasia archives of pathology. Lymphangioma is a benign proliferation of lymphatic vessels and represents hamartomas of malformed lymphatics. They are also seen on the liver, spleen, kidneys and retroperitoneum. Pdf anaesthetic consideration in macroglossia due to. The following are the complications of lymphangioma. Whimster theorized the cisterns might come from a primitive lymph sac that failed to connect with the rest of.
It is a a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue. It causes mechanical discomfort and functional and psychological disturbances. In the oral cavity, lymphangioma is a rare, nonodontogenic, benign. Lymphangioma could affect any part of the body with the exemption of the brain, but is most common in the neck and head. Multifocal and multicentric cavernous lymphangioma like and hemangiomalike areas merge with benign angioendotheliomatouslike foci in a background of variable muscle degeneration and marked. Tongue, palate, gingiva, lip, alveolar ridge and buccal mucosa are the most commonly affected sites in the oral cavity.
Pdf lymphangioma of the buccal mucosa semantic scholar. Oral lymphangioma is a benign tumor of the lymphatic system, which mostly occur in the oral cavity mouth. More than 50% affect anterior conjunctival and adnexal structures. Most lymphangiomas are present at birth 60% and, by the age of 2 years, 80% to 90% are present. Lymphangioma is the most common cause of secondary macroglossia in infancy. A rare entity supriya m kheur1, samapika routray2, yashwant ingale3, desai r s4 email for correspondence.
We present the case of a yearold female with lymphangioma of tongue. Lymphangioma circumscriptum is a microcystic lymphatic malformation. In cases that are not severe draining the fluid could give you shortterm relief. Lingual lymphangioma usually involves the dorsum of the tongue and the tip of the tongue is a rare location. Lymphatic malformations are rare malformations of the lymphatic system that occur most frequently in the first decade of life. Sep 20, 2014 lymphangioma of the tongue is a common cause of macroglossia in children associated with difficulty in swallowing and mastication, speech disturbances, airway obstruction, mandibular prognathism, openbite and other possible deformities of maxillofacial structures. Dec, 2018 many researchers believe that an oral lymphangioma is a malformation during the embryological development, rather than a true tumor. Macroglossia secondary to lymphangioma of the deep neck. About 4050% of lymphatic malformations are occurs in the tongue, which is the preferred site of intraoral involvement followed by buccal mucosa which is the second most common site. Intramuscular lipomatosis of tongue postgraduate medical. Here we present a case of lymphangioma of tongue in a 4yearold female patient. Lymphangioma of tongue dental courses linkedin slideshare. Respiratory obstruction is a complication of head and neck lymphangiomas, especially in late presentations and can be due to the infiltration of the malformation in the tongue, pharynx, or larynx, or blockage of a normal airway by large cysts.
Lymphangioma lymphangiomas are benign tumours of lymphatic vessels showing marked predilection for head and neck region. It is considered the lymphatic equivalent of a hemangioma of blood vessels. If the cyst forms behind the eye, it pushes the eye forward. They are common among the tumors occurring in the head and neck region but intraoral lymphangioma are rare in occurrence. The child had difficulty in chewing and swallowing solid food with impairment of speech. In tongue it mostly occurs on the dorsal surface and lateral border. A majority of the tumors are diagnosed before a child reaches 2 years of age. When a lymphangioma occurs in the oral cavity, there is a marked predilection for the anterior twothirds of the tongue 3, which often results in macroglossia 4. Lymphangioma of the tongue is a common cause of macroglossia in. Here we present a case of lymphangioma of tongue in a 4 yearold female patient. Goswami m, singh s, gokkulakrishnan s, singh a 2011 lymphangioma of the tongue. He had no obvious signs of surface bleeding, paroxysms of lesion expansion, or repeated respiratory infections or respiratory distress. Neither a borderline nor an overtly malignant vasoformative neoplasm was present.
What are the signs and symptoms of oral lymphangioma. The signs and symptoms of oral lymphangioma include. Jan 06, 2016 about 4050% of lymphatic malformations are occurs in the tongue, which is the preferred site of intraoral involvement followed by buccal mucosa which is the second most common site. The common site of occurrence for lymphangioma in the oral cavity is the anterior dorsum and lateral border of tongue. For a specific discussion in other locations, please refer to the articles.
Intralesional sclerotherapy with bleomycin in lymphatic malformation. A number of lymphangioma can be large in size and some are not but despite the size, the mass could still cause functional impairment of certain. Lymphangioma of the tongue is relatively rare and may cause facial structural deformity. When a lymphangioma does need treatment, the therapies are very successful. Lymphangioma of the tongue pia lopez jornet md, dds, phd oral medicine, university of murcia. The tumor may be localized in a small area of the tongue or floor of the mouth, or it may diffusely infiltrate these areas6.
When lymphangioma occurs in the mouth, the anterior two thirds of the tongue is the most commonly affected region. In this report report, a rare case of lymphangioma of the left buccal mucosa of retromlar area in a. Deeper lesions on the tongue present as soft, illdefined masses resulting in macroglossia 12. Lymphangioma of the orbit new york eye cancer center. The sub types are probably variants of the same pathologic process and depend on the size of the vessel and nature of the surrounding tissues. Overall, people generally recover without any problems.
Lymphangioma of the head and neck with tongue involvement is more often seen than the isolated involvement of the tongue. We present a rare case of lymphangioma tongue which presented to us as macroglossia and wide excision of the lesion was done. The condition may be reported under several names, including. Pdf lymphangiomas are congenital malformations of lymphatic vessels. Mri imaging is typical with lesions isointense on t1, high signal on t2, with no co. Generalized lymphangioma of tongue is a rare cause of macroglossia in children.
Lymphangioma of the tongue is a rare condition, but it is reported to be the most common cause of congenital macroglossia. Lymphangioma of the tongue extensive and complex defects of the scalp, middle third of the face, and palate. The majority of tumours of the tongue are malignant. Cavernous lymphangioma forms an illdefined, spongy, compressible mass and is found most commonly in the tongue, cheek, floor of mouth, and lips. When the mass is congenital, the malformation is spongy, soft, and not tender. Lymphangioma circumcript lymphangioma proliferation affecting the skin cavernous lymphangioma lymphatic proliferation characterized by large, dilated lymphatic channels progressive lymphangioma rare lymphatic proliferation occurring in the skin characterized by dissecting vascular channels usually affect age 40. Skin tumors are called lymphangioma circumscriptum. Presenting here is a case of localized lymphangioma of the tongue in a. Virchow 1854 gave the first accurate description of lymphangioma. Typically, the lymphangioma bleeds into itself causing cysts of blood called chocolatecysts within the tumor.
Lymphangioma tends to start in the superior and nasal orbital quadrants. Lymphangioma, macroglossia, lymphangioma tongue, sleep apnea, vglossoplasty full text the full text of this article is available as a pdf 670k. They are benign and do not require medical treatment, although some patients may choose to have them surgically removed for cosmetic reasons. Its occurrence on the palate, gingiva, oral mucosa and lips is very rare 2,3. Mix type is combining of microcystic and macrocystic types 9,10. Lymphangioma is a benign, hamartomatous tumor of lymphatic vessels with a marked predilection for the head, neck and oral cavity.
Oral lymphangioma of the buccal mucosa a rare case report. Lymphangiomas affecting the chest may result in difficulty. Lingual lymphangioma presents specific therapeutic pro. The treatment of lymphangioma in the buccal mucosa by surgical. Treatment of a tongue lymphangioma with sirolimus after. Children with focal tongue lesions also had good to excellent outcome. Researchers believe that an oral lymphangioma occurs as an abnormality during embryological growth while in the womb.
Lymphangioma is a benign tumor involving lymphatic channels. Collection of photos, images and pictures of lymphangioma lymphangioma treatment. Herewith, we present a case of cystic hygroma associated with lymphangioma of tongue leading to macroglossia in a 5yearold boy. Many of these cysts and masses can be removed or reduced in size. Although tongue is a rare site for lymphangioma it is the most common cause for macroglossia. Sep 05, 2017 it is a a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue. Lymphangioma is a rare, benign, congenital disease of unknown etiology that originates from lymph vessels and this entity was first described by virchow in 1854. They are believed to arise from lymph sac sequestration and.
Lymphangiomas are rare congenital tumors of the lymphatic system, and tonsillar lymphangioma is an extremely rare occurrence. Bone, breast, kidney tumor, lymph nodes, mediastinum, skin nonmelanocytic tumors, skin nontumor, spleen. Surgical treatment of oral lymphangiomas with co2 laser. A risk of secondary growth is classically described after. They may be localized in any part of the body and its organs and tissues, but they are found most often in the neck, skin, subcutaneous connective tissue, or retroperitoneal space less frequently, in the liver, spleen, or kidneys. Presenting here is a case of localized lymphangioma of the tongue in a five year. Successful airway management of an infant or child with macroglossia prerequisites recognition of a potential airway problem. Lymphangioma pictures, symptoms, diagnosis, treatment. From christie hospital and holt radium institute, manchester simple tumours of the tongue are comparatively rare, but diffuse lymphangioma is the most common cause of macroglossia and localised growths also occur. Occurrence in other areas such as cheeks, lips, floor of the mouth, palate and gingiva has also been reported 46. Lymphangioma circumscriptum of the skin and tongue. Lymphangioma circumscriptum, a microcystic lymphatic malformation, resembles clusters of small blisters ranging in color from pink to dark red.
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