|Year : 2008 | Volume
| Issue : 2 | Page : 79-81
Recurrent cystic hygroma with calcification in an adult
Sudeendra Prabhu1, Punnya Angadi1, GS Kumar2, R Vandana3, K Rekha1
1 Department of Oral Pathology, S.D.M. College of Dental Sciences and Hospital, Dharwad, Karnataka, India
2 Department of Oral Pathology, K.S.R. Institute of Dental Science and Research, Tiruchengode, Tamil Nadu, India
3 Department of Oral Pathology, Narayana Dental College, Nellore, Andhra Pradesh, India
Department of Oral Pathology, S.D.M. College of Dental Sciences and Hospital, Dharwad - 580 009, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infants or children younger than 2 years. Herein, we present a case of recurrent cystic hygroma in a 22-year-old woman. History revealed that she had a similar lesion in the left submandibular region when she was 2 years old and in the thyroid region seven years back. This case has been reported for its unusual occurrence in an adult, showing recurrences at different sites and presence of calcification.
Keywords: Adult, calcification, cavernous lymphangioma, cystic hygroma, cystic lymphangioma, recurrence
|How to cite this article:|
Prabhu S, Angadi P, Kumar G S, Vandana R, Rekha K. Recurrent cystic hygroma with calcification in an adult. J Oral Maxillofac Pathol 2008;12:79-81
|How to cite this URL:|
Prabhu S, Angadi P, Kumar G S, Vandana R, Rekha K. Recurrent cystic hygroma with calcification in an adult. J Oral Maxillofac Pathol [serial online] 2008 [cited 2020 Jul 7];12:79-81. Available from: http://www.jomfp.in/text.asp?2008/12/2/79/44584
| Introduction|| |
Cystic hygroma, also known as cavernous lymphangioma, is a relatively rare type of lymphangioma, contributing to 5% to 6% of benign tumors. 
The classic clinical presentation is a slow-growing, painless, soft, compressible swelling which is translucent to illumination,  most often found in the posterior triangle of the neck.  Rarely, it can occur in the mediastinum, mesentery, colon, retroperitoneum, cheek, and groin. 
It can cause numerous complications like respiratory obstruction and dysphagia if they grow rapidly.  These lesions are most commonly present at birth (50%), and 90% are evident by the age of two.  Very few cases have been reported in adults. 
Cystic hygroma is considered either to be a hamartoma or a true neoplasm. , It has been postulated that cystic hygroma develops as a result of lymphatic sequestration of primitive jugular sac. 
This case report presents a case of recurring cystic hygroma with concentric lamination of calcifications, occurring in an adult female.
| Case Report|| |
A 22-year-old woman presented with a gradually progressive painful swelling of four years duration in the right submandibular region. She had undergone surgery twice for similar swellings, which had occurred in the left submandibular region when she was 2 years old and in the thyroid region seven years back.
On examination, a diffuse swelling measuring 3.5×4 cm, extending superiorly from the right preauricular region to the lower border of the mandible and inferiorly from the chin to little beyond the angle of the right mandible, was seen [Figure 1]. On palpation, two soft, unattached, tender masses with multiple small hard mobile nodules were observed.
MRI in T2-weighted images revealed a lobulated hyperintense lesion in the region of the angle of mandible with multiple, small round-to-ovoid hypointense structures within the lesion, which could represent calculi [Figure 2]. Lesion was anterior to sternocleidomastoid muscle and caused posterior displacement of the muscle. The lesion was free from both the right parotid and submandibular glands. A likely diagnosis of cystic hygroma was made and surgery was performed.
Through a submandibular incision, a flap was raised dissecting the superficial structures, following which two large, well-circumscribed masses attached to each other were observed [Figure 3].
Careful dissection of the masses anteriorly up to the midline, superiorly up to the lower border of the mandible, and posteriorly up to the tail of the parotid was carried out. The masses were separated from their attachment to sternocleidomastoid muscle. Hemostasis was achieved using cautery and ligation. Wound closure was done, by suturing the subcutaneous tissue followed by fascia and skin.
The surgical specimens measured 3.5×4 cm, appeared irregular in shape but were well circumscribed. Cut surface revealed multiple cystlike spaces separated by thin fibrous septae and filled with gelatinous material and few cystic spaces filled with blood. Multiple concentric areas of calcifications, appearing white in color, were seen filling several of the cystic spaces [Figure 4].
Sections revealed varying proportions of large and small lymphatic channels containing lymph, few blood vessels, adipose tissue, fibrous tissue, and lymphoid tissue. Areas of hemorrhage were also seen [Figure 5] and [Figure 6]. Sections from submandibular gland were histologically unremarkable [Figure 7]. Few cystic spaces were distended, and concentric laminations suggestive of calcification were observed [Figure 8]. Based on the microscopic observations in correlation with clinical features, a final diagnosis of cystic hygroma was made.
| Discussion|| |
The clinical presentation of the present case showed the classical features of a cystic hygroma except for the presence of multiple hard nodules of calcification. The swelling was painful, which was elicited during neck movements like bending. This is probably due to pressure impingement of the nerve in that area.
In addition to the typical histopathological features of large dilated lymphatic spaces, vessels, nerves, and lymphoid tissue, an unusual finding in the present case was the presence of calcifications, which were seen in the gross specimen as concentric rings, and microscopically as partially calcified concretions. The calcification is probably due to dystrophic calcification of hemorrhagic areas.  This finding occurring in cystic hygroma of head and neck region is a very rare feature.
The differential diagnosis of cystic hygroma includes several conditions of the neck, presenting as soft, compressible mass like thyroglossal cyst, benign cystic lymph node, lipoma, and plunging ranula. 
A variety of treatment options have been tried, including surgical excision, aspiration, radiation, and sclerosing therapy.  The opinions on the management are divergent; the recent trend is towards wide excision to prevent recurrence. 
Prognosis is generally good, although recurrence is relatively common and seen in 80% of cases in the supra-hyoid region.  Forman reported that the recurrence is seen in sites where the tumor was not noticeable earlier.  Incomplete removal is thought to be the prime reason for recurrence because lymphangiomas have thin walls consisting of endothelium, which can be easily torn.  Follow-up should therefore be carried out for an extended period.
Recurrence was a major problem in the present case. When the patient was 2 years old, the lesion was first excised as a mass of the left submandibular region. A second surgery was carried out for a recurrent lesion in thyroid region seven years back, which subsequently recurred again after four years in the right submandibular region. Following this surgery, the patient is now being followed up regularly; and in the past one year, there has been no evidence of recurrence.
| Conclusion|| |
The present case is being reported as it presented with multiple recurrences occurring in varying sites, emphasizing the importance of complete surgical excision and long-term follow-up of congenital malformations like cystic hygroma. Furthermore, the present case presented with calcification, which appears to be extremely rare in the head and neck region.
| References|| |
|1.||Farmand M, Kuttenberger JJ. A new therapeutic concept for the treatment of cystic hygroma. Oral Surg Oral Pathol Oral Radiol Endod 1996;81:389-95. |
|2.||Osborne TE, Haller JA, Levin LS, Little BJ, King KE. Submandibular cystic hygroma resembling a plunging ranula in a neonate. Oral Surg Oral Pathol Oral Radiol Endod. 1991;71:16-20. |
|3.||Aantoniades K, Kiziridou A, Psimopoulou M. Traumatic cervical cystic hygroma. Int J Oral Maxillofac Surg 2000;29:47-8. |
|4.||Jose M. Toranzo, Guerrero F, Dibidox J, Gonzalez G, Sedano HO. A congenital neck mass. Oral Surg Oral Pathol Oral Radiol Endod 1996;82:363-4. |
|5.||Bauer RA, Hardman FG. Intraoral surgical management of cystic hygroma. Br J Oral Surg 1976;14:36-40. [PUBMED] |
|6.||Swischuk LE, Hoeffel JC, John SD. Primary intrathoracic lymphangioma masquerading as teratoma. Pediatr Radiol 1996;26:827-9. [PUBMED] |
|7.||Farman AG, Katz J, Eloff J, Cywes S. Mandibulo-facial aspects of the cervical cystic lymphangioma (cystic hygroma). Br J Oral Surg 1978;16:125-34. [PUBMED] |
|8.||Katsuno S, Ezawa S, Minemura T. Excision of cervical cystic lymphangioma using injection of hydrocolloid dental impression material. Int J Oral Maxillofac Surg 1999;28:295-6. [PUBMED] |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]