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Volume 7, Number 4 (December 2012) , 263-266

Case Report

Lymphangioma Circumscriptum A Rare Form Of Lymphangiomas

Ibrahim Salih Elkheir*, Omar Alamin M.K, Mohamed AM Ibnouf, Hanaa Mohamed,

Hashim Mohamed


 

 

 

 

Superficial lymphangiomas are either cavernous type, diffuse or circumscribed (circumscriptum). Lymphangima circumscriptum (LC) is a rare benign condition characterized by the appearance of persistent clusters in the skin and subcutaneous tissue which may contain fluctuant cysts1. The first reported description ofLC was done by Fox and Fox2 in1878. They named it as lymphangiectodes2. The term LC is first used by Morris3 in1889. Complete surgical excision is the main treatment. Here we are reporting a case of lymphangioma circumscritum of the thigh in a six-year old girl.

 


 
 

Lymphangiomas are congenital malformations of the lymphatic system that may involve skin and subcutaneous tissue. They are hamartomatous malformations which account for 25% of benign lympho-vascular tumours in children4. They are not familial5.Lymphangioma circumscriptum, cavernous lymphangiomas, and cystic hygromas are the common forms of this condition. LC is a lesion that leads to superficial dilations of lymphatic vessels, which communicates with deeper subcutaneous lymphatic cisterns via small channels. These abnormal malformations are not connected to normal lymphatic6.Vesicles of LC discharge clear fluid and are prone to super-infection. They can appear over any part of the body and may have a verrucous appearance6.LC may look like a number of disorders including herpes zoster, viral warts and molluscum contagiosum7,8.

Surgical excision is the cornerstone in treatment but recurrence is common, with a reported5,9 first-time cure rate of 75%.It is recommended to surgically excise to the level of the deep fascia and obtain clear margins on frozen section to avoid recurrence10.

Case report:

A six-year old girl presented with two swellings in the medial aspect of the right thigh for one year. One swelling was above the other. The upper one was excised but soon recurred. Physical examination revealed a residual soft tissue mass with unhealed part of the wound and multiple clusters of vesicles which discharge colorless smelly fluid. There was no other swelling in any other part of the body. The lower swelling which was not operated before was 12x9cm.The upper one which was operated was about 18x10cm.

Complete excision of the recurrent mass was done and sent for histopathology. Figures1,2 and 3 show intra operative views. Histopathology revealed Lymphangioma circumscreptum as in figures 4 and 5. Follow up at three months revealed no recurrence of the lesion.

Figure1: Skin appearance before excision.

Figure 2: Specimen during excision.

Figure 3: Look at the clean uncut tissue and muscle after excision

 

Discussion:

Originally LC was diagnosed as a soft- tissue mass. LC the skin and subcutaneous tissue is uncommon. The dilated vessels cause expansion of the papillary dermis. There is some acanthosis and hyperkeratosis of the overlying epidermis. The lymph fluid within the vesicles may contain erythrocytes, lymphocytes, macrophages, and/or neutrophils. It is a benign disorder affecting lymphatic channels. Its diffuse and cavernous subtypes are inter-related, and may occur in association with each other14 since the basic pathogenesis is almost the same in all types.

In LC, there is dilated muscle coated lymphatic cistern in the subcutaneous plane, communicating with large dermal lymphatics upwards, and finally erupting as superficial vesicles on the surface of the skin as blow-out phenomenon. There may be one or more abnormal lymphatic cistern and vessels.

Lymphatics form closed system, neither communicate with each other nor with the main normal lymphatic channels11. The sequestration of cisterns is considered as developmental defects. It is reported after radiation therapy for malignant tumours12.

Lymphoedema is absent in the affected area, the reason being that the presence of normal lymphatic drainage. Also LC can be divided into smaller lesions (less than seven centimetres) and extensive lesions (more than that)10. The incidence of LC is higher in the first and second decade of life has preponderance females10,13. The proximal part of limbs, axillary folds, adjacent chest wall and flanks are the common sites13.

Figure  4: histology slide that shows dilated lymph vessels in the upper dermis that extend into the subcutis.

Figure 5: Histology slide, high power.

The classical subtype is characterized by extensive swelling with thickened, hypertrophied, hyperpigmented, lichenified skin. Clusters of vesicles may be scattered over the involved skin. These vesicles may vary in appearance from pale white to reddish brown. Some of these vesicles may become oozing13,17. Lymphangiography was the procedure of choice to map the involved Lymphatics11,13. Magnetic resonance imaging (MRI) is the investigation of choice to see the extent of disease13.

The diagnosis is made on histopathology. Surgical excision is still the only option of treatment for extensive/classical subtype despite chances of recurrence11. Other modalities are intra-lesional sclerotherapy using doxycycline, Picibanil (OK-432)14, or vaporization by carbon dioxide laser15. The principle of surgery is based on the excision of all sequestrated lymphatic cisterns in the subcutaneous plane, since they are the main culprits. The diseased subcutaneous tissue is recognized by the presence of cysts, fibrous strands, and wetness in the deep fascia11,10. The prognosis is excellent as it is a non-neoplastic condition. However, squamous cell carcinoma has been reported in an existing LC16.

 

 

Conclusion:

Our patient   was a six-year old  girl, suffering from LC in the inner aspect of the proximal part of right thigh. Cure was obtained in the second surgical excision, with an acceptable cosmetic result. To minimize the chances of recurrence, wide local excision of diseased subcutaneous tissue is mandatory rather than   wide excision of skin.

 

 

References:

1. Browse NL, Whimster I, Stewart G, Helm CG, Wood JJ. Surgical management of lymphangioma circumscriptum. Br. J. Surg 1996; 73: 585-588.

2. Fox T, Fox TC. On the case of lymphangiectodes with an account of the histology of the growth. Trans Path Soc, Londaon 1879;30:470-6.

3. Morris M. Lymphangioma circumscriptum. In: International Atlas of Rare Skin Diseases. Unna PG, Morris M, Duhring LA, Leloir H, eds. London: Lewis 1889; 1-4.

4. Schwartz RA, Fernández G: Lymphangioma. eMedicine Dermatology [Journal serial online]. 2009. 5. Flanagan BP, Helwig EB. Cutaneous lymphangioma. Arch Dermatol. 1977;113:24–30.

6. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol. 1976;94:473–86.

7. Janniger C, Droano A. Zoster. E-Medicine. 2009 Sep 29. [cited 2009 Dec 9]

emedicine.medscape.com/article/909908-overview Adriat. 2002;11:101–4.

8. Erkilic S, Kocer NE, Mutaf M. Giant lymphangioma circumscriptum mimicking wart in a 13-year-old girl. J Dermatol. 2006;33: 501–3.

9. Bond J, Basheer MH, Gordon D. Lymphangioma circumscriptum: Pitfalls and problems in definitive management. Dermatol Surg. 2008; 34:271–5.

10. Browse NL, Whimster I, Stewart G et al. Surgical management of ‘lymphangioma circumscriptum’. Br J Surg. 1986;73: 585

11. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976; 94: 473-86.

12. Hamad AA, Provencher D, Ganjei P, Penalver M. Lymphangioma circumscriptum of the vulva: case report and review of the literature. Am J Obstet Gynaecol 1989;73: 496-9.

13. Edwards JM, Peachey RDG, Kinmoth JB. Lymphangiography and surgery in lymphangioma of the skin. Br J Surg 1972; 59(1):36-41

14. Mikhai IM, Kennedy R, Cramer B et al. Sclerosing of recurrent lymphangioma using OK-432. J Paed Surg 1995; 30: 1159-60.

15. Bailin PL, Kantor GR, Wheeland RG. Carbon dioxide LASER vapourisation of lymphangioma circumscriptum. J Am Acad Dermatol 1986; 14: 257-62.

16. Wilson GR, Cox NH, Mclean NR et al. Sqamous cell carcinoma arising within congenital lymphangioma circumscriptum. Br J Dermatol 1993; 129: 337-9.

17. Peachey RDG, Lim CC, WhimsterIW. Lymphangioma of Skin- A review of 65 cases. Br J Dermatol 1970; 83: 519-27.

 

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*Correspondence to: Mr. Ibrahim Salih Elkheir Consultant Pediatric Surgeon, Khartoum North Teaching Hospital. 

 

 

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