Oral lymphangioma: A case report
Jeeva Rathan J1, Harsha Vardhan BG2, Muthu MS1, Venkatachalapathy1, Saraswathy K2, Sivakumar N11 Department of Pediatric dentistry, Meenakshi Ammal Dental College and Hospital, Maduravoyal, Chennai,Tamilnadu, India2 Department of Oral Medicine and Radiology, Meenakshi Ammal Dental College and Hospital, Maduravoyal, Chennai, Tamilnadu, India
Correspondence Address:Harsha Vardhan B GDepartment of Oral Medicine and Radiology,Meenakshi Ammal Dental College and Hospital,Alapakkam Main Road, Maduravoyal,Chennai - 600095, Tamilnadu IndiaAbstract
Lymphangiomas are congenital malformations of lymphatic vessels filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. Large lymphangioma extending into the tissue spaces of neck is referred to as cystic hygroma. Herewith, we present a case of cystic hygroma associated with lymphangioma of tongue leading to macroglossia in a 5-year-old boy.
Keywords: Cystic hygroma, Lymphangiomas, Macroglossia
Lymphangiomas are benign hamartomatous tumors of the lymphatic channels. They rarely represent as neoplasms; most likely present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels.
Lymphangiomas have a marked predilection for the head and neck region, which accounts for about 75% of all cases. About 50% of the lesions are noted at birth and around 90% develop by 2 years of age. They are known to be associated with Turner's syndrome, Noonan's syndrome, Trisomies, Cardiac anomalies, Fetal hydrops, Fetal alcohol syndrome, and Familial pterygium colli. Cystic hygroma in a child can grow suddenly due to infection or hemorrhage but can also shrink spontaneously.
Lymphangiomas have been classified into
1.Lymphangioma simplex (capillary lymphangioma), which consists of small, capillary sized vessels.
2.Cavernous lymphangioma, which is composed of large, dilated lymphatic vessels.
3.Cystic lymphangioma (cystic hygroma), which exhibits large macroscopic cystic spaces.
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. Cystic lymphangioma (cystic hygroma) mostly occurs in the neck and axilla as the loose adjacent connective tissues facilitate more expansion of the vessels.
Oral lymphangiomas may occur at various sites but they form most frequently on the anterior two-thirds of the tongue, which often results in macroglossia. It can also present in the palate, buccal mucosa, gingiva, and lip., The tumor is superficial in location and demonstrates a pebbly surface that resembles a cluster of translucent vesicles. The deeper lesions appear as a nodule or masses without significant change in surface texture or color.Case Report
A 5-year-old boy reported to the Department of Pedodontics with a chief complaint of pain in the right lower posterior tooth region for the past ten days, which increased at night. Extra orally he had a diffuse swelling on the left side that extended from the angle of mouth to the angle of mandible superiorly and 1 in. below the lower border of mandible inferiorly, which was soft and nontender to palpation [Figure - 1]. He had a surgical scar in the angle of mandible on the left side [Figure - 2]. While smiling deviation of the left angle of mouth to the right side was evident. His medical records revealed that he had cystic hygroma on the left side of the neck for which he was surgically treated at the age of 1 1/2 years. On palpation the submandibular group of lymph nodes on the right side was palpable, mobile and tender.
Intraoral hard tissue examination revealed caries in right lower second primary molar, left lower first primary molar and root stumps of left lower second primary molar. The intruded left upper central incisor gave a metallic sound on percussion suggesting ankylosis and right central incisor was mobile. He had gingival swelling in relation to right lower second primary molar. Tongue was large with numerous papillary and vesicle like projection on the dorsal surface [Figure - 3] extending as a growth on to the ventral aspect [Figure - 4]. There was also presence of a deep cleft extending posteriorly from foramen caecum to anterior 2/3rd of tongue roughly measuring 4 cm in length.
Orthopantamograph revealed caries in left lower first primary molar, right lower second primary molar involving pulp, and root stumps of left lower second primary molar. The lateral extra oral radiograph revealed maxillary intruded primary incisor whose root tip was not close to the succedaneous tooth.
Soft tissue ultrasonograph of the neck demonstrated multiloculated diffuse cystic lesion in the left side of neck with minimal vascularity on color Doppler studies. There was no vascularity within the sublingual cystic lesion. M-mode (Motion) Echomyography of tongue during swallowing of saliva demonstrated contraction of tongue muscles (mylohyoid, geniohyoid, and hyoglossus) with fluid containing sublingual hypoechoic lesion containing no muscle fibers [Figure - 5][Figure - 6][Figure - 7].
The CT scans of the left side of neck revealed a large lobulated heterogeneous mass of 4.7 x 3.0 x 4.0 cm with cystic and solid components in the posterior cervical triangle. The mass extended anteriorly upto the buccal space and medially upto the Parapharyngeal space. On administration of IV contrast few enhancing tortuous canals were also seen. No significant enlargement of lymph nodes was seen. There was slight displacement of trachea to the right side. The carotid arteries, internal jugular vein, pharynx, epiglottis, and thyroid gland appeared normal [Figure - 8].
Incisional biopsy was performed on the ventral surface of tongue and tissue specimen sent for histopathological examination. Microscopic section revealed numerous dilated lymphatics lined by endothelial cells containing lymph giving a multilocular honey combed appearance. Some of the section showed the presence lymphatic channels filled with red blood cells. There were also plenty of aggregates of lymphocytes [Figure - 9][Figure - 10]. This histological picture can suggest a variant, which can be termed as 'lymphangiohemangioma.'
Lymphangiomas are hamartomatous, congenital malformations of the lymphatic system. They are the result of sequestration of lymphatic tissue that has retained its potential for growth and do not communicate with other lymphatic tissue. Embryologically it is derived from five primitive buds developing from the venous system which include paired jugular sacs, paired posterior sacs and a single retroperitoneal sac. Cystic hygroma in a child is congenital but their recurrence is due to secondary infection and trauma. Cervical lesions in a child can cause dysphagia and airway obstruction which is rare in an adult. In the present case, the swelling was noticed from birth and there was no evidence of any dysphagia and dyspnea. The CT scan showed only displacement of the airway and not compression.The anterior two-thirds on the dorsal surface of tongue is the most common site for intraoral Lymphangiomas leading to macroglossia., These patients tend to have speech disturbances, poor oral hygiene, and bleeding from tongue associated with oral trauma. In our case macroglossia occurred as a result of lesion on both dorsal and ventral surface of tongue. The patient presented with slurring of speech and poor oral hygiene.
The various treatment modalities for lymphangioma are surgical excision, radiation therapy, cryotherapy, electrocautery, sclerotherapy, steroid administration, embolization, and ligation,, laser surgery with Nd-YAG,, CO212],and radiofrequecncy tissue ablation technique. Surgical excision is the preferred treatment for cystic hygroma but complete removal is not possible because of the multiple fingerlike projections on to the surrounding tissues., On the other hand complete removal of intraoral Lymphangiomas mean complete removal of the affected organ.l In the present case, he was surgically treated at the age of 1/2 years for cystic hygroma but a recurrent lesion appeared.
Intralesional injection of sclerosing agents will result in inflammation, sclerosis and cicatrial contraction of the lesion, Sclerosants used previously diffuse through discontinuous basement membrane, which allowed the sclerosing agent to penetrate freely in to the connective tissues, diluting the concentration and rendering less effective. Some agents could affect the skin resulting in scar formation. Bleomycin was not used now-a-days as it r e sulted in pulmonary fibrosis leading to death. Presently OK-432 is the preferred intralesional sclerosants which is a lyophilized incubation mixture of the low-virulent Su strain of type III, group A Streptococcus pyogenes an origin with penicillin G potassium which has lost its streptolysin S- producing activity,, ,, when administered intralesionally; it evokes inflammation and infiltration of neutrophils and macrophages into the cystic spaces. There is also increase in activity of Natural Killer cells, Helper cells, and T-cells. In addition cytokines (Interlukin-6 and Tumor Necrosis Factor) are also produced. This results in interaction between these activated cells and cytokines directly on the endothelial cells to increase its permeability causing accelerated lymphatic drainage and increased lymph flow leading to shrinkage of the cystic spaces without skin damage and scar formation.,
To Prevent recurrence the rough oral care was carried out in, the Patient which included oral prophylaxis, restoration in left lower first primary molar, pulpectomy and stainless steel crown in right lower second primary molar and extraction of root stumps of left lower second primary molar to eliminate dental foci of infection. Since lymphangioma is primarily a disease of childhood, the pediatric dentist might be the first healthcare professional to be encountered with the lesion. An early diagnosis and intervention will help in reducing functional, psychological disturbances and cosmetic disfigurement.
We express our sincere thanks to Prof. Rajaram PhD., DSc. Professor and Head, Department of Radiology and Imaging, Meenakshi General Hospital for a detailed radiological evaluation and to Dr. Malathi MDS, Professor, Department of Oral Pathology, Sri Ramachandra Dental College and Hospital for her meticulous and detailed histopathological analysis.
Neville BW, Damn DD, Allen CM. Oral and Maxillofacial Pathology. WB Saunders: Philadelphia; 1995. p. 395.
Morley SE, Ramesar KC, Macleod DA. Cystic hygroma in an adult: a case report. J R Coll Surg Edinb 1999;44:57-8.
Shafer WG, Hine MK, Levy BM. Developmental disturbances of the tongue. A textbook of Oral Pathology. 4th Edn. WB Saunders Co: 1983. p. 159-60.
Bernnan TD, Miller AS, Chen S. Lymphangiomas of the oral cavity: A Clinicopathologic, Immunohistochemical and Electron microscopic study. J Oral Maxillofac Surg 1997;55:932-5.
Chappius IIP. Current aspects of cystic lymphangioma in the neck. Arch De Pediatre 1995;1:186-92.
Guelmann M, Katz J. Macroglossia combined with lymphangioma: a case report. J Cli Pediatr Dent 2003;27:167-70.
Hartl DM, Roger G, Denoyelle F. Nicollas R, Triglia JM, Garabedian EN. Extensive lymphangioma presenting with upper airway obstruction. Arch Otolaryngol Head Neck Surg 2000;126:1378-82.
Hellman JR, Myer CM, Pregner EC. Therapuetic alternatives in the treatment of life threatening vasoformative tumors. Am J Otolaryngol 1992;13:48.
Suen J, Waner M. Treatment of oral cavity vascular malformations using the Meodymium: YAG laser. Arch Otolaryngol Head neck Surg 1991;115:1329.
Tasar F, Turner TF, Sener BC, Sencift K. Lymphangioma treatment with NdYAG laser. Turk J Pediatr 1995;37:253-6.
Dixon JA, Davis RK, Gilbertson JJ. Laser photocoagulation of vascular malformation of the tongue. Laryngoscope 1986;96:537-41.
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.
Barak S, Katz J, Kaplan I. The CO2 laser in surgery of vascular tumors of the oral cavity in children. J Dent Child 1991;58:293-6.
Cable BB, Mair EA. Radiofrequency ablation of lymphangiomatous macroglossia. Laryngoscope 2001;11:1859-61.
Ravitch MM, Rush BF Jr. Cystic hygroma. In : Welch KJ, Randolph JG, Ravitch MM, eds. Pediatric Surgery, Year Book: Chicago IL; 1986. p. 533-9.
Emery PJ, Bailey CM, Evans JN. Cystic hygroma of the head and neck: A review of 37 cases. J Laryngol Otol 1984;98:613-9.
Ogita S, Tsuto T, Deguchi E, Tokiwa K, Nagashima M, Iwai N. OK- 432 Therapy for Unresectable Lymphangiomas in children. J Pediatr Surg 1991;26:263-70.
Yura J, Hashimoto T, Tsuruga N, Shibata K. Bleomycin treatment for cystic hygroma in children. Arch Dis Child 1977;46:607-14.
Swenson O. Malformation of the neck. In : Swenson O, eds. Pediatr Surgery. CT. Appleton-Century-Crofts: East Norwalk; 1969. p. 307-20.
Chabner BA, Myers PO, Coleman CN, Johns DG. The clinical pharmacology of antineoplastic agents (second of two parts). N Engl J Med 1975;292:115968.
Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N. OK-432 therapy in 64 patients with lymphangioma. J Pediatr Surg 1994;29:784.
Ishida N, Hoshino T. A Streptococcal Preparation as a Potent Biological Response Modifier OK-432. Excerpta Medica: Amsterdam The Netherlands; 1985. p. l-5, 26-47,60-2.
Hanaue H, Kurosawa T, Miyakawa S, Horie F, Nemoto A, Yamoto H, et al . Immunohistological studies of the skin at the injection site of cases receiving the streptococcal agent OK-432. J Jpn Soc Cancer Ther 1985;20:2271-8.
Oral Lymphanagiomas - eMedicine