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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 2  |  Issue : 1  |  Page : 39-42

Recurrent bilateral cystic hygroma of the submandibular space in an adult patient


1 Department of Oral Pathology and Microbiology, Marata Mandal's NGH Institute of Dental Sciences and Research Centre, Belgaum, Karnataka, India
2 Department of Oral and Maxillofacial Surgery, KLE VK Institute of Dental Sciences and Hospital, Belgaum, Karnataka, India

Date of Web Publication2-Feb-2015

Correspondence Address:
Vijayalakshmi S Kotrashetti
Department of Oral Pathology and Microbiology, Marata Mandal's NGH Institute of Dental Sciences and Research Centre, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-6816.150595

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  Abstract 

Cystic hygroma is an uncommon type of benign neoplasm of lymphangioma commonly seen in children, rarely reported in adults. Most of the cases occur in head and neck region. The etiology and pathogenesis of this entity are still unclear. Clinically, they present as painless, soft, compressible, progressively enlarging mass. Various treatment methods have been described, but surgery remains the treatment of choice. Incomplete excision results in recurrence. We present a unique case of bilateral submandibular area cystic hygroma in an adult female patient with a history of recurrence twice.

Keywords: Cystic hygroma, head and neck, lymphangioma, submandibular area


How to cite this article:
Kotrashetti VS, Kotrashetti SM. Recurrent bilateral cystic hygroma of the submandibular space in an adult patient. Saudi J Oral Sci 2015;2:39-42

How to cite this URL:
Kotrashetti VS, Kotrashetti SM. Recurrent bilateral cystic hygroma of the submandibular space in an adult patient. Saudi J Oral Sci [serial online] 2015 [cited 2020 Jan 18];2:39-42. Available from: http://www.saudijos.org/text.asp?2015/2/1/39/150595


  Introduction Top


Lymphangiomas are benign neoplasm of lymphatic vessels, with most of the cases occurring in the head and neck region. [1] Cystic hygroma is a subtype of lymphangioma, first described by Redenbacker in 1828 who termed it as ranula congenital. [2],[3],[4] Cystic hygroma amounts to 1.2% of all benign and malignant tumors and 4% of all vascular tumors. [5] They mainly present at birth (50-65%) and more than 90% of these congenital malformations are found in children younger than 2 years. Occurrence in adulthood is rare. [1],[4] In adult patients they present as a painless gradually increasing mass, which may have precipitating factor or can occur as a suddenly arising, enlarging mass. [1],[4],[6] Complications are generally associated with a large mass, which can cause respiratory obstruction, interference with normal breathing and swallowing. [3] The lesion is transilluminable and is not attached to the skin, but to deeper structures. Computed tomography (CT) scan is most commonly used to know the exact extension of the lesion if it is large. Magnetic resonance imaging (MRI) and ultrasonography has also been advocated. [1],[3],[4],[7],[8] The most accepted treatment for cystic hygroma is surgical excision, however many sclerosing agents and the aspiration therapy have been used but the results are not conclusive. Recurrence is common if complete excision of the lesion is not carried out. [3],[4],[9] Here we report a rare case of cystic hygroma involving submandibular space bilaterally with history of recurrence twice in an adult female.


  Case Report Top


A 25-year-old female patient reported to the outpatient department of the institute with the chief complaint of asymptomatic progressive swelling in the neck since 3 months. On extra oral examination, the mass was seen diffusely occupying bilateral submandibular region and submental region measuring about 10-15 cm. On palpation the lesion was soft, compressible and cystic in nature. Transillumination was positive. The overlying skin was normal and appeared stretched [Figure 1]a and b. Intraoral examination showed, lesion extending into the floor of the mouth and appeared bluish red in few areas. Tongue appeared slightly raised and showed hypertropic papillae [Figure 1]c. Patient gave history of similar swelling in the same region when she was 1-year old and later at 10 years of age for which she was operated.
Figure 1: (a) Extraoral photograph showing extension of the lesion involving bilateral submandibular space. (b) Extraoral photograph showing stretched skin overlying the swelling. (c) Intraoral photograph showing lesion extended into floor of the mouth. (d) Computed tomography scan of the neck showing extension of the lesion to the symphysis menti, submandibular region and the proximal neck


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Based on this clinical differential diagnosis of recurrent cystic hygroma and hemangioma was made. A CT scan of the neck revealed a diffuse swelling with loss of intramuscular and fat planes in the symphysis menti, submandibular region and the proximal neck [Figure 1]d. A diagnostic, therapeutic aspiration with an 18 gauge needle was performed, which revealed straw colored fluid. Cytological examination depicted the proteinacious material with no cells.

Under general anesthesia, an incision was made from mastoid to mastoid the region. An apron flap was raised; the mass superficial to strap muscle, digastric, and the salivary gland was excised. The bony excess was removed by an angle to angle inferior cortectomy [Figure 2]a-c. Wound was closed in layers with a suction ring in situ [Figure 2]d. The resected specimen was soft and fragile showing multiple cysts of various sizes [Figure 3]a. The healing was uneventful.
Figure 2: (a) Operative photograph showing the extent of incision made. (b) Photograph showing surgically opened site with the lesion. (c) Photograph showing excised excess bone by cortetomy. (d) Postoperative photograph with sutures


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Figure 3: (a) Photograph showing gross appearance of the soft tissue with multiple small and large cystic spaces. (b) Photomicrograph showing lymphatic spaces lined by single layer endothelial cells (H and E, ×4). (c) Photomicrograph showing lymphatic and cavernous spaces with lymph and lymphocytes in the vessels (H and E, ×4). (d) Photomicrograph showing aggregates of lymphocytes showing germinal centers (H and E, ×4)


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Microscopic examination showed large thin endothelial lined cystic spaces few containing an eosinophillic material and varying number of lymphocytes. Areas of cavernous and capillary components with red blood cells were present [Figure 3]b and c. Aggregates of lymphoctes were present with areas of germinal center [Figure 3]d. Based on these features final diagnosis of "cystic hygroma" was made.


  Discussion Top


The name hygroma is derived from Greek word hygros, meaning moist and oma meaning tumor. [4] Over the past century, the term hygroma has been used interchangeably with cystic lymphangioma, because of confusion regarding the etiology of these lesions. Thus, it is still a debatable issue that whether lymphangiomas are true neoplasm, hamartoma or lymphangiaectasis. [10]

Adolph Wernher proposed the lesion to be a true neoplasm [4] and Virchow agreed to the view of Wernhers, later neoplastic position was generally accepted. [4],[11] Literature suggested that Koester (1872) first described the lesions as lymphangiomas after demonstrating endothelial lining in the cyst. [4] Later Sabin in 1901 described the development of lymphatic system which develops from five primitive lymphatic sacs that sprout from the venous system that is, paired jugular sacs, paired posterior or sciatic sacs and a single retroperitoneal sac. Based on this Sabin proposed that sprouting of endothelial channels extend centrifugally into the embryo to form the peripheral lymphatic system, whereas McClure and Huntington proposed the centripetal theory. [3],[4],[8]

Later Goetsch (1938) supported the centrifugal theory and theorized that hygromas arise from sequestrations of developing lymphatic tissue derived from the primordial jugular sacs. The sequestration possesses properties of lymphatic anlage and has the ability to grow independently and inseminate within tissue analogous to developing lymphatic. [4],[8],[9],[10]

Cystic hygromas are primarily found in children and occurrence in adults is rare. In adults, they are seen at 4 th or 6 th decade of life [6] and mean the age of 45 years. [4] The male to female ratio appears to be equal in most reports. [8] This lesion can occur in any location but seen commonly in head and neck area (75%). 56% of cases occur in posterior triangle and 44% in the anterior triangle, [1] but can occur anywhere in the neck. Right and left side of the neck is affected equally. [4] Occurrence of this lesion in the submandibular area is 6.25% and cause difficulty in breathing or swallowing. Mediastinal extension occurs in 10% of cases. [9] The present case showed bilateral involvement of submandibular area. On literature search no such case of bilateral involvement of the submandibular space is reported, thus making this case unique.

The etiology for occurrence of this lesion in adults is unknown. Many develop after surgical procedures, chronic inflammation, irradiation, minor trauma, infection or obstruction of lymphatic drainage. [1],[4],[6] Our case had no history of trauma or infection but had a history of recurrence.

Diagnosis of cystic hygroma is done mainly by history and physical examination. [3] Clinically they are soft, compressible, painless cystic mass gradually increasing in size. [7],[9] On aspiration, they produce straw colored serous fluid. There is often no precipitation factor but rapid expansion has been seen following trauma and infection. [4] Complications associated with cystic hygroma include airway obstruction and dysphagia which is common in children, but often rare in adults. [1]

Radiographic and laboratory examination are of little diagnostic aid. [11] But for larger lesion to determine the accurate extension CT, MRI and ultrasound are used prior to surgery. [1],[3],[4],[7] In the present case CT scan was carried out to determine the nature and extent of the lesion. The differential diagnosis for cystic hygroma includes brachial cleft cyst, haemangioma, thyroglossal duct cyst, lymphoma, hamartoma, teratoma, lipoma, dermoid cyst, thyroid mass. [1],[6]

Microscopically cystic hygroma shows cystic spaces lined by single layer of flattened endothelial cells. Few lymphocytes with eosinophilic lymph fluid can be seen in the lymphatic spaces. Occasionally collections of lymphocytes are found forming true germinal centers. The lesions may surround and destroy normal tissue with the traversing septae often containing thrombosed vessels or degenerate muscle remnants. [1],[5]

Lymphangiomas have been classified by several authors based on histology, clinical findings, and both. Faber (1956) first classified lymphangiomas into three groups based on histological appearance as:

  1. Lymphangioma simplex or capillary lymphangioma,
  2. Cavernous lymphangioma, and
  3. Cystic lymphangioma or cystic hygroma. [1],[4],[10]


Grossly the excised tumor mass shows multilocular cysts of various sizes some interconnected and some are isolated, [2] which is similar to our findings. The recommended treatment for cystic hygroma is complete surgical excision [12] Complete surgical excision with cortectomy was done for the present case. Various other treatment modalities have been used like injection of sclerosing agents, intralesional and systemic steroids, systemic cyclophosphamide, intralesional injection of fibrin, bleomycin, repeated aspiration, OK-432, incision and drainage, radiotherapy and laser therapy for superficial lesions. The efficacy of these treatment modalities is not well known and their role in the treatment of difficult or unresectable cases is undetermined. [4],[6],[12]

Recurrence rate is related to histologic type and site of presentation, with impact respectability and not to patient age or size of the lesion. [1] Invasive lymphangiomatous lesions of the oral cavity show recurrence rate of 89% has reported by Ricciardelhi and Richardsons. Recurrence is common if not excised completely. Riechelmann et al. have found 0% recurrence after complete excision. 56-86% of recurrence after subtotal and partial removal respectively and 100% recurrence rate after aspiration. [4] The recurrence rate is 10-15% and usually recurs within 1 year of surgery. [13] Recurrence in our case can be attributed to incomplete removal. Literature suggests recurrence of the lesion will be within 1 year of surgical treatment, but can present delayed recurrence as long as 10 years after initial operation. Our case also showed recurrence twice at the interval of 2 years and 16 years. Thus suggests that recurrence can also occur later in life after first operation. The mortality and the morbidity for this disease are most likely related to the size of the hygroma and the age of the patient. [8] Mortality rates are between 3% and 7% with the largest lesions having higher mortality rates. [14]

 
  References Top

1.
Woolley SL, Smith DR, Quine S. Adult cystic hygroma: Successful use of OK-432 (Picibanil). J Laryngol Otol 2008;122:1260-4.  Back to cited text no. 1
    
2.
Jose MT, Fermin GD, Javier DM, Gaston GZ, Heddie OS. A congenital neck mass. Oral Surg Oral Med Oral Pathol Oral Radiol Endo 1996;83:363-4.  Back to cited text no. 2
    
3.
Giguère CM, Bauman NM, Smith RJ. New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol 2002;111:1066-75.  Back to cited text no. 3
    
4.
Sherman BE, Kendall K. A unique case of the rapid onset of a large cystic hygroma in the adult. Am J Otolaryngol 2001;22:206-10.  Back to cited text no. 4
    
5.
Christopher DM. Vascular tumors. Diagnostic Histopathology of Tumors. 3 rd ed., Vol. 1. St. Louis: Elsevier Publishing; 2007. p. 67-8.  Back to cited text no. 5
    
6.
Antoniades K, Kiziridou A, Psimopoulou M. Traumatic cervical cystic hygroma. Int J Oral Maxillofac Surg 2000;29:47-8.  Back to cited text no. 6
    
7.
Ozen IO, Moralioglu S, Karabulut R, Demirogullari B, Sonmez K, Turkyilmaz Z, et al. Surgical treatment of cervicofacial cystic hygromas in children. ORL J Otorhinolaryngol Relat Spec 2005;67:331-4.  Back to cited text no. 7
    
8.
Kennedy TL. Cystic hygroma-lymphangioma: A rare and still unclear entity. Laryngoscope 1989;99:1-10.  Back to cited text no. 8
    
9.
Mehta MR. Cystic hygroma: Presentation of two casfes with a review of the literature. Indian J Otolaryngol Head Neck Surg 2000;52:319-22.  Back to cited text no. 9
    
10.
Wiess's E. Tumors of lymph vessels. In: Weiss SW, Goldblum JR. editors. Soft Tissue Tumor. 4 th ed. Vol. 2. Philadelphia: Mosby Inc.; 2001. p. 956.  Back to cited text no. 10
    
11.
Bauer RA, Hardman FG. Intra-oral surgical management of cystic hygroma. Br J Oral Surg 1976;14:36-40.  Back to cited text no. 11
    
12.
Farmand M, Kuttenberger JJ. A new therapeutic concept for the treatment of cystic hygroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;81:389-95.  Back to cited text no. 12
    
13.
Riechelmann H, Muehlfay G, Keck T, Mattfeldt T, Rettinger G. Total, subtotal and partial surgical removal of cervicofacial lymphangiomas. Arch Otolaryngol Head Neck Surg 1999;125:643-8.  Back to cited text no. 13
    
14.
Douglas RG. Cysts of the neck, unknown primary tumor and neck dissection. In: Luna MA, Pfaltz M, editors. Diagnostic Surgical Pathology of the Head and Neck. 1 st ed. Philadelphia: Elsevier Publising; 2000. p. 665.  Back to cited text no. 14
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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