lymphatic malignancy management details.pptx

plastic surgery

1. Case Discussion on Lymphatic Malformation Amina U (PRSR4) March, 2024 GC

2. Introduction Vascular anomalies are blood vessels that have developed incorrectly Vascular anomalies are divided into neoplastic and nonneoplastic Vascular tumors – endothelial neoplasms Vascular malformations – abnormal development of vascular elements during embryognesis

3. A 2yr2mo old male child who presented with progressively increasing swelling of the right UE, neck and back areas since birth PE – Soft compressible nontender swelling over the right hand, forearm, arm extending to right side of neck and upper chest and right side of upper back, swelling increases in dependent position Has erythematous nonblanching patch over the swelling at the back US – ill marginated different sized and multiloculated cystic lesions at the right lateral neck extending to forearm and hand along the subcutaneous tissue with internal echocomplex collection with different echogenicity, no flow on color Doppler DDx: lymphangioma Xray – no bony lesion

4. Lymphatic malformation aberrant collections of lymphatic fluid-filled spaces Truncular Vs Extratruncular (lymphangioma) Can also occur with other VM Based on size of its channels: Macrocystic – cysts large enough to be treated by sclerotherapy (5 mm or larger) Microcystic – cysts too small to be cannulated by a needle Combined ( macrocystic /microcystic) Most commonly on the head and neck Cause site-specific morbidity Lymphatic malformations progress over time Complications - bleeding and infection

5. soft and compressible overlying skin may be normal, have a bluish hue, or be studded with pink–red vesicles

6. Diagnosis Clinical – 90% of lymphatic malformations are diagnosed by history and physical examination US – confirm Dx, differentiate between macrocystic and microcystic lesions MRI – Large or deep lymphatic malformations confirm the diagnosis, define the type of malformation ( macrocystic , microcystic, or combined), determine the extent of disease and plan treatment CT – to delineate osseous involvement, particularly if resection is planned Histopathology – if imaging is equivocal or if a malignant process is suspected

7. Management Observation – Small or asymptomatic lesions Sclerotherapy – first-line for large or problematic macrocystic /combined LM doxycycline, sodium tetradecyl sulfate (STS), ethanol, bleomycin, and OK-432 Ethanol is an effective sclerosant but has the highest complication rate Resection – significant morbidity symptomatic microcystic LM symptomatic macrocystic /combined LM with all macrocysts already treated with sclerotherapy small, well-localized LM (microcystic or macrocystic ) that may be completely excised Radiofrequency ablation – problematic mucosal vesicles in the oral cavity

8. Management Intervention for a lesion causing a visible deformity should be considered before 4 years of age to limit psychological morbidity Sclerotherapy is the first-line mode of treatment for a large or problematic macrocystic or combined lymphatic malformation Almost all macrocystic lesions will have an excellent response to sclerotherapy Under GA using ultrasound and/or fluoroscopic guidance Doxycycline – safe, effective, may prevent infectious complications, solution of 10 mg/ml is injected (up to 50 ml (500 mg) may be used for infants and small children) Sodium tetradecyl sulfate (STS) is our second-line sclerosant Bleomycin – for difficult anatomic areas or for lesions that have not been responsive to other agents Ethanol – can be used for small lesions, but large volumes should be avoided to reduce the risk of local and systemic toxicity

9. Sclerosant Under GA using ultrasound and/or fluoroscopic guidance Periprocedueral antibiotics After fluid is aspirated from the cyst, the sclerosant is injected Large cysts occasionally require placement of a pigtail catheter and sequential drainage and injection over several days. Resolution of macrocysts may occur within days, but can take 6-8 wks Additional injections every 6-8 wks might be required Lymphatic malformations may reexpand over time; patients often need repeat sclerotherapy over their lifetime

10. Resection For symptomatic macrocystic lymphatic malformation sclerotherapy is no longer possible because all of the macrocysts have been treated small and well localized lesions where resection is curative Excision is usually subtotal, because they typically involve multiple tissue planes and important structures so recurrence is common Associated with significant morbidity: major blood loss, iatrogenic injury, and deformity Patients and families are counseled that lymphatic malformations can expand after any intervention, and thus additional treatments may be required.

11. References Neligan - Craniofacial, Head and Neck Surgery, 5 th edition Arin K. Greene – vascular anomalies, classification, diagnosis and management Mulliken and Young’s vascular anomalies, 2 nd edition