Lymphangiomas are a heterogenous group of benign malformations of lymphatic system. These are congenital malformations of lymphatic system. Lymphangiomas frequently occur in cervicofacial region followed by axilla, thorax and extremities1. Lymphangiomas are classified as microcystic, macrocystic and mixed type. Lymphangiomas are usually seen at birth but can manifest at any age or even antenatally detected by Ultrasound. The most common presentation is swelling followed by skin changes and associated pain in cases of infected Lymphangiomas. Ultrasound of the swelling is used for diagnosis of Lymphangiomas. MRI is sometimes done to know the extent and surrounding structure involvement. Lymphangiomas can be treated with sclerotherapy, surgical resection, or both. Sclerosants used are pure ethanol, sodium tetradecyl sulfate, doxycycline, bleomycin and OK-432. Surgical excision remains the gold standard treatment for Lymphangiomas2, 3,4. However, with the advent of new drugs like bleomycin and OK-432 intralesional sclerotherapy is gaining popularity now a days 5. Many centers are presently using intralesional bleomycin sclerotherapy as first line of management of Lymphangiomas with good results due to its easy availability and cost efficiency6. Ours is one such study. In the present study we have retrospectively studied regarding the efficiency of intralesional bleomycin as sclerosant in pediatric Lymphangiomas in a tertiary care center.

 After taking prior approval from Institutional Ethics and Institutional Scientific Committee the study is done. A retrospective review of 40 patients admitted in Department of Pediatric Surgery, Rangaraya Medical College, Kakinada with lymphangioma and treated with intralesional bleomycin from January 2021 to June 2024 was done. The data is collected from hospital and pediatric surgery medical records. Children less than 12 years, Lymphangiomas over head and neck, chest wall, abdominal wall and extremities were included in this study. Lesions around trachea, Mediastinal Lymphangiomas, infected type and lymphangioma size less than 1cm were excluded from this study. The diagnosis is made clinically and confirmed with ultrasound. The data regarding child age, gender, weight, symptoms, location, size, type of lymphangioma, number of doses, complications and response were collected for all patients. Photographs of the lesions before and after the procedure were also collected. The procedure was done under short general anesthesia under strict aseptic conditions in Operation Theater. The cyst fluid is first aspirated and then 0.5 IU/kg body weight of bleomycin was injected into the lesions at multiple sites. The maximum dose of bleomycin given in our study is 10 IU per dose irrespective of the baby weight. In case of multicystic Lymphangiomas, when more than one cyst was aspirated the calculated dose was divided by the number of cysts aspirated and the divided dose was injected into each cyst. The aspirated volume and bleomycin injection volume was 5:1 ratio. Patient was kept under observation for 24 hours and then discharged. The 1st follow up visit was after 2 to 4 weeks and evaluated for response and any complications.  Size of the lesion was taken and noted. If the lesion is still present child was re evaluated with ultrasound and planned for second and third dose if necessary. One month gap was given for each dose of bleomycin injection. In our study, the response to sclerotherapy was graded as: Excellent, Good and poor response depending on reduction in size of the swelling. Excellent response is >85% regression in size. Good response is 50-85% regression in size. Poor response is <50% regression in size. The minimum post operative follow up duration was 6 months. The data collected was processed

During the study period 40 cases were selected for the study. Out of 40 patients in our study 22 were male (55%) and 18 were female (45%). 12 (30%) are between 1-2 years. Eighteen (45%) patients are between 2 to 6 years. Ten (25%) patients are between 6 to 12 years. The most common site of lymphangioma in our study is neck with 14 out of 40 patients (35%) followed by upper limb with 8 patients (20%), 7 in chest wall (17.5%), 6 in lower limb (15%)and 5 over the face (12.5%). Most common presentation was swelling only, seen in 28 patients (70%).  7 patients (17.5%) presented with swelling and skin changes, 5 patients (12.5%) presented with swelling and pain. The lesion size was between 1-2cm in 9 patients (22.5%), 2-5cm in 24 patients (60%) and more than 5 cm in 7 patients (17.5%). Out of 40 patients in our study 27 patients (67.5%) are of macrocystic variety, 4 are of microcystic variety (10%) and 19 patients (22.5%) are of mixed type. The maximum numbers of doses given in our study are three with one month gap. 17 patients (42.5%) were given 3 doses, 13 patients (32.5%) were given 2 doses and 10 patients (25%) got resolved with single dose. The response was excellent in 25 patients (62.5%), good response was seen in 9 patients (22.5%) and poor response was seen in 6 cases (15%). All macrocystic variety showed excellent response (100%). Out of 9 patients of mixed variety, excision was done for only 3 patients as they showed poor response even after 3 doses. In microcystic variety out of 4 patients, 3 cases did not show any response even after 3 doses and surgical excision was done for three of them, 1 patient showed partial response.

Complications included pyrexia, redness, and skin color changes. The swelling increased in size immediately after injection in some cases which was transient, lasted for 1 day to 1 week and got relieved with simple analgesics and antipyretics and hot fomentation.

Incidence of lymphangioma is 1 in 1000 to 16000 livebirths7,8. Lymphatic malformations/lymphangiomas are usually noted at birth but can manifest at any age. Etiology is unknown. LM are thought to have following etiology: (a) Failure of lymphatic sacs to communicate with central venous system (b) Retained embryonic growth potential in sequestered lymphatic rests (c) Atypical budding of lymphatic tissue These lymphatic rests can penetrate adjacent structures and later become canalized. These spaces develop cystic components due to lack of venous outflow. Depending on the surrounding tissue Lymphangiomas can be classified as Capillary, cystic and cavernous type. Capillary and Cavernous forms of lymphangioma tend to form in muscle. whereas cystic form occurs in loose areolar tissue.  Lymphangiomas are classified as Macrocystic, Microcystic and Combined macro and micro type. Most commonly present as ballotable masses with normal overlying skin, sometimes a blue hue is seen if large cysts are seen underlying it. Lymphangiomas can be treated with sclerotherapy, surgical excision or both. Intralesional sclerotherapy is more effective for macrocystic type. Various sclerosants used are pure ethanol, sodium tetradecyl sulfate, doxycycline, bleomycin and OK-432. In complex Lymphangiomas, and those with extension to the surrounding nerves and vascular structures excision is difficult. sclerotherapy is a viable alternative to surgery.  Bleomycin and OK-432 are most commonly used recently. Bleomycin is produced from streptomyces verticillus. It is an antineoplastic drug belonging to antibiotic class of drugs. It was first developed in 1966 and it acts by inhibition of DNA synthesis9. It is used in the treatment of malignant pleural effusion, during which its property of causing marked fibrosis and scarring was identified10. In 1977, the sclerosing property was first time used in the treatment of lymphangioma.  Studies are being conducted regarding its efficacy. Ours is one such study. In our study, intralesional injection have showed promising results. The results were comparable to the the other similar published papers. Various authors have published articles with success rate between 36-63% for complete regression, 88% for significant regression and poor/no regression 12-23%.  Recent study in 2010 by Rozman et al11 study has complete resolution in 63%, significant regression in 84% and poor/no response in 16%. In 2004, Mahajan et al study had success rate of 53.3%, 86.7% and 13.3% respectively12. Zulfiqar et al study in 1999 had result of 36%, 82% and 18% respectively13. Niramis et al had noted 83% response14 and Baskin et al15noted 95% response suggesting that bleomycin as a best alternative for surgery in lymphangioma. In our study the response rate is excellent in 62.5%, good response in 22.5% and poor response was seen in 15% of cases. In our short term follow up, no recurrence was encountered. Others have also not found recurrence to be of much concern.

Bleomycin seems to be a safe and effective intralesional treatment for lymphangiomas. This type of therapy can be used as a primary line of management for lymphangiomas in pediatric age group.

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