1. Topic review lymphangio-hemangioma
Intern 黃維立
2006/03/28

2. lymphangioma RARE congenital malformation of the lymphatic system
Incidence : 5.6% of all benign lesions of infancy + childhood

3. Male-female ratio: equal Age:
Most, about 50%, at birth
% by the end of 2nd year

4. Pathogenesis Failure of drainage from primordial lymph sacs into veins
Sequestration of lymphatic tissue with failure to joint central lymphatic channels
Abnormal budding of lymph vessels with loss of connection with lymphatic primordia
Sequestration隔離

5. Histology findings
These lesions are composed of dilated lymphatic channels with one or two endothelial layers ± an adventitial layer.
These dilated lymphatics can vary in size depending on the location and surrounding tissues ~bases for their classifications.

6. Four categories

7. location

8. Posterior triangle of neck (most common)
bordered by the posterior border of the SCM, trapezius, and clavicle
Posterior triangle of neck (most common)

9. Etiolgy Congenital lymphatic malformation! Acquired cause?
Traumatic: facial trauma
Infectious:
Iatrogenic: mediastinoscopy
Neoplastic:lymphoma

10. Clinical manifestation

11. Clinical manifestation
The presence of a mass
Small or unnoticed ---- large with mass effect
Airway obstruction: dyspnea
Problems with feeding: dysphagia
Complications:
Infection or inflammation of cystic neck structures
Spontaneous or traumatic hemorrhage into the cystic structure

12. How to diagnose? Characteristic appearance Image studies
Soft, doughy mass
Image studies
Ultrasonography CT
MRI  more important!
麵團似的
和相鄰structure間的關係，牽涉到之後surgery的考量

13. Different diagnosis ~ neck mass in infants and children ~
Soft
Lipoma
Hemangioma
Lymphangioma
Firm
Cyst: thyroglossal, branchial cleft, lingual , thymic
Abscess
Hematoma
Inflammatory lymphadenopathy
Neoplastic lymphadenopathy
Neoplasia, eg. Fibroma, neuroblastoma
Thyroid

14. MRI findings ~ lymphangioma ~
Low signal intensity on T1WI
High signal intensity on T2WI

18. Management
Surgery as the primary management option for all lymphangiomas
~the site of lesion: suprahyoid region ↑recurrence rate, morbidity and complication rate

19. Management Surgery Aspiration Injection of sclerosing agents
Picibanil (OK-432)
Diathermy(透熱療法:短波、微波、超音波 )
Radiation

20. Management Surgery Aspiration Injection of sclerosing agents Diathermy
Radiation
Observation?!

21. Observation Spontaneous regression or resolution (10-15%)
Selected cases: asympatomatic except for the presence of a mass