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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 1  |  Issue : 3  |  Page : 161-163

Hemolymphangioma of neck in a child


Department of Oral and Maxillofacial Surgery, KVG Dental College and Hospital, Sullia, Karnataka, India

Date of Web Publication8-Dec-2014

Correspondence Address:
Sumana Devadiga
Department of Oral and Maxillofacial Surgery, KVG Dental College and Hospital, Sullia, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-2915.146500

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  Abstract 

Hemolymphangiomas are benign hamartomatous hyperplasia of lymphatic vessels, which are filled with blood. Although they are benign, they can undergo progressive growth with compression and infiltration of adjacent structures. The management of lymphangiomas in the head and neck region is still challenging. Surgery has been the main form of treatment, but total removal is not possible in all cases because of the extent of the lesions, which sometimes involve vital structures. This was a case report of 7-year-old female patient presented with a nontender swelling in the submandibular region, which was clinically diagnosed as branchial cleft cyst, but the microscopic examination revealed the presence of hemolymphangioma.

Keywords: Branchial cleft cyst, haemolymphangioma, neck swelling


How to cite this article:
Devadiga S, Ramaraj P N, Rai M, Patel KB. Hemolymphangioma of neck in a child. J Dent Res Rev 2014;1:161-3

How to cite this URL:
Devadiga S, Ramaraj P N, Rai M, Patel KB. Hemolymphangioma of neck in a child. J Dent Res Rev [serial online] 2014 [cited 2022 Nov 27];1:161-3. Available from: https://www.jdrr.org/text.asp?2014/1/3/161/146500


  Introduction Top


Lymphangiomas are malformations of the lymphatic system that most frequently affect the head and neck. Their precise pathogenesis is still unknown. [1] They are believed to be caused by congenital obstruction of lymphatic drainage. Histologically, lymphangiomas have been classified into three types on the basis of the size of the lymphatic channels: (a) Simple-made up of capillary-size lymphatic channels; (b) cavernous-containing larger lymphatic channels; and (c) cystic hygromas-which are multilocular cystic masses. Since separation of these types is difficult pathologically and all three types often coexist in the same lesion, these masses are collectively called lymphangiomas. [2]

Lymphangiomas are slow-growing tumors, and in the head and neck usually present as an obvious swelling, which is classically cystic and can be trans illuminated. [3] They are most commonly seen as cervical masses in children, but can also be found in other sites. About 75% of lymphangiomas that occur in the neck, generally found in the posterior triangle, and 20% occur in the axillary region. Other locations include the mediastinum, retro peritoneum, bones, scrotum, and abdominal viscera. About 3-10% of the cervical lymphangioma extend into the superior mediastinum. [2]

In the pertinent literature, their incidence in children is estimated to amount to 6% of all benign tumors with an equal distribution among sexes and races. In about half of the patients, the disease is already obvious at the time of birth. During the 1 st and 2 nd year of life, the occurrence of lymphangioma is diagnosed in 80-90% of the cases due to clinical symptoms. [1] These are primarily fluid filled and are usually superficial in location. [2]

History and physical examination usually suggest the diagnosis of lymphangiomas At times, it is necessary to confirm the diagnosis or differentiate the lesion from lipoma, dermoid cyst, branchial cyst, thyroglossal duct cyst, teratoma, salivary gland or thyroidtumors, neurofibroma, and meningoencephalocele. In these instances, radiological evaluation may be required. Soft tissue radiographs are useful to evaluate the airway. Both computed tomography scan and Ultrasound are helpful in demonstrating the solid or cystic nature of the lesion. The differentiation of lymphangioma from other fluid-filled masses may require needle aspiration or biopsy. A recent report suggests that magnetic resonance imaging is the most useful modality to assess the extension. [4] Despite all the advanced imaging techniques, the diagnosis of adult lymphangiomas remains a challenge. A correct diagnosis is ensured only by histopathological examination of the surgical specimen. [5]

The management of lymphangiomas in the head and neck region is still challenging. [1] The treatment of lymphangioma should be complete surgical excision as these tumors do not disappear on their own. [3] However, total removal is not possible in all cases because of the extent of the lesions that sometimes involve vital structures. Moreover, the potential for recurrent infection, airway compromise, feeding difficulties and interference with the development of normal speech, and concerns for esthetics complicate treatment planning. [1]

Hemolymphangioma of the neck is a rare disease and basically a benign cystic tumor. We present a hemolymph angioma of the neck in a 7-year-old child.


  Materials and Methods Top


A 7-year-old female presented to the Department of oral surgery with the chief complaint of a swelling of the right side of jaw region since 3 months. On examination, patient had a solitary -defined oval swelling in the submandibular region measuring approximately 4 cm ×5 cm. Over lining skin was smooth. On palpation, the swelling was soft in consistency, nontender and fluctuant. A provisional diagnosis of branchial cleft cyst was made [Figure 1].

Ultrasound showed a superficial hypo echoic mass in the submandibular region without involvement of the submandibular gland and any vital structures.

Diagnostic aspiration was performed, and about 2 ml of reddish brown fluid was aspirated. On the basis of the clinical and radiographic findings, the differential diagnosis of branchial cleft cyst, lymphadenopathy, and lymphangioma were made.
Figure 1: Preoperative photo of the patient

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The patient underwent surgery under general anesthesia. Submandibular incision was given. Skin, platysma were reflected. Cyst along with the lymph node was excised [Figure 2]. Wound was sutured with 3-0 vicryl suture material. The specimen was then submitted for histopathological examination.

Histopathological sections showed connective tissue with large multiple cystic spaces lined by flattened endothelial cells filled with eosinophilic material or empty. Surrounding connective tissue is loose contains numerous adipose tissues infiltrated with lymphocytes and plasma cells. Numerous red blood cells filled spaces of various sizes lined by endothelial cells are observed. Another section shows lymphoid tissue with peripherally numerous lymphatic vessels filled with lymph and lymphocytes are also seen. Showing the impression of hemolymph angioma of the neck [Figure 3]a and b.
Figures 2: Intra operative site of the lesion

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Figure 3: Histopathological photo showing multiple cystic spaces with lymphatic channels (a), histopathological photo showing numerous red blood cells filled spaces(b)

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Healing was uneventful, and 1-year follow-up period showed no signs of recurrence.


  Discussion Top


Lymphangioma is a benign congenital malformation seen almost exclusively in children <2 years of age. There is a controversy on the best definition of lymphangioma, a condition that was first described by Redenbacher in 1828. Some authors consider it as a congenital malformation of the lymphatic system, others as a hamartoma, and others as a vascular benign tumor. [4] They consist in localized centers of abnormal development of the lymphatic system. Three theories have been proposed to explain the origin of this abnormality. The first suggests that a blockage or arrest of normal growth of the primitive lymph channels occurs during embryogenesis, [5],[6] the second that the primitive lymphatic sac does not reach the venous system, while the third advances the hypothesis that, during embryogenesis, lymphatic tissue lays in the wrong area. [6] The most widely accepted theory about the development of lymphangioma is that they arise from sequestrations of the primitive embryonic lymph sacs. [5]

Although they are benign, they can undergo progressive growth with compression and infiltration of adjacent structures. [1]

Lymphangiomas are classified as microcystic (capillary lymphangiomas), macrocystic (cavernous lymphangiomas) and cystic hygromas according to the size of the lymphatic cavities incorporated. Bill and summer, in 1965, presented the concept that cystic hygromas and lymphangiomas are variations of a single entity and that its classification is determined by its the location in the head and neck. A commonly used classification classifies these lesions into the capillary lymphangioma or lymphangioma simplex, cavernous lymphangioma, and cystic lymphangioma or cystic hygroma. When a lymphangioma is confined to fairly dense tissue, such as the tongue, it presents as a cavernous lymphangioma, but when it develops in the relatively loose fascia of the neck, acystic lesion occurs. These three types are frequently found together in the same patient, depending on the severity of the disease. [6],[7]

Overall, lymphangiomas account for about 6% of the benign tumors in the pediatric population. Both sexes are affected equally. Sixty percent are present at birth, and 90% are detected by the end of the 2 nd year. They neither become malignant nor have a familial tendency. Nearly 50% of lymphangiomas occurs in the head and neck, at submandibular and parotid area, [8] 42% in the trunk and extremities, and 10% in the internal organs. The most common single site was the neck with 30% of the lesions. [4] Localized cystic lesions are more inclined to occur in the neck, whereas diffuse involvement of the lower third of the face much more common. [6] Most lymphangiomas present with a mass or diffuse swelling, which initially grows slowly with the child and after sometime might slowly regress. Due to their size and location, they are sometimes a real therapeutic challenge. [4]

Lymphangiomas are benign, slow-growing tumor having a tendency to grow in an infiltrating manner into the surrounding tissues leading to compression, resulting functional disturbances. Clinically they have doughy feel, pitting edema and does not empty with compression. Most of the time diagnosis is made on the basis of history, clinical findings and confirmed by cytopathologically or histopathologically. [8] The lymphangioma consists of multiple, intervening lymph vessels in a loose fibrovascular stroma. These lymphatic channels may be filled with blood that is known as hemolymph angioma. [9] Imaging technique identifies the extension of the tumor. [8]

Management of lymphangioma in children is challenging because complete resection is difficult to achieve in some cases, and recurrences are common. Surgical excision of localized hemolymph angiomas, is the treatment of choice. [4] However, when it infiltrates vital neurovascular neck structures, complete excision is difficult, and if only partial, the recurrence rate is very high as well as high risk of surgical complications. [8] Several alternative techniques to the gold standard surgical excision have been reported namely aspiration, incision and drainage, use of steroids, chemotherapy, chemical sclerosis and use of laser. [10]

We presented a case of hemolymph angioma of the neck in a child, which was treated with surgical excision. Despite all the advanced imaging techniques, the diagnosis of lymphangiomas remains a challenge. A correct diagnosis is ensured only by histopathological examination of the surgical specimen.


  Conclusion Top


Hemolymphangioma is a change of lymphatic vessels that rarely affects the head and neck region. Superficial and localized lesions can be treated by conservative surgical excision with low relapse rates. Therefore, this condition knowledge is of fundamental importance for its correct diagnosis and proper therapeutic indication.

 
  References Top

1.
Wiegand S, Eivazi B, Zimmermann AP, Sesterhenn AM, Werner JA. Sclerotherapy of lymphangiomas of the head and neck. Head Neck 2011;33:1649-55.  Back to cited text no. 1
    
2.
Kapoor R, Saha MM, Talwar S. Sonographic appearances of lymphangiomas. Indian Pediatr 1994;31:1447-50.  Back to cited text no. 2
    
3.
Al-Salem AH, Qaisruddin S, Abusrair H, Qureshi SS, Varma KK. Giant mediastinal cystic hygroma in a child. Ann Saudi Med 1997;17:92-4.  Back to cited text no. 3
    
4.
Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years' experience with lymphangiomas in children. J Pediatr Surg 1999;34:1164-8.  Back to cited text no. 4
    
5.
Mathew M, Dil SK. Adult lymphangioma - A rare entity: A report of two cases. Turk Patoloji Derg 2012;28:80-2.  Back to cited text no. 5
    
6.
Baskota DK, Singh BB, Sinha BK. OK-432: An effective sclerosing agent for the treatment of lymphangiomas of head and neck. Kathmandu Univ Med J (KUMJ) 2007;5:312-7.  Back to cited text no. 6
    
7.
Aciole GT, Aciole JM, Soares LG, Santos NR, Santos JN, Pinheiro AL. Surgical treatment of oral lymphangiomas with CO 2 laser: Report of two uncommon cases. Braz Dent J 2010;21:365-9.  Back to cited text no. 7
    
8.
Vasconcelos MG, Santos BC, Lemos LC, Ribeiro BF, Iglesias DP, Vasconcelos RG, et al. Oral lymphangioma: Case report. RSBO 2011;8:352-6.  Back to cited text no. 8
    
9.
Rajendran R. Benign and malignant tumors of oral cavity. Lymphangioma. In: Rajendran R, Sivapathasundharam B, editors. Text Book of Oral Pathology. 7 th ed. New Delhi: Elsevier; 2012. p. 154.  Back to cited text no. 9
    
10.
Grasso DL, Pelizzo G, Zocconi E, Schleef J. Lymphangiomas of the head and neck in children. Acta Otorhinolaryngol Ital 2008;28:17-20.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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