- Lymphangiomas are low-flow vascular anomalies that result from abnormal lymphatic development. They most commonly occur in the neck and can cause swelling, pain, and difficulty swallowing. - Treatment options include surgical excision, sclerotherapy, ablation techniques like radiofrequency ablation, and lasers. Complete surgical excision has the lowest recurrence rate but may not always be possible. Sclerotherapy is effective for macrocystic lymphangiomas. - Studies have found no difference in effectiveness between primary surgery and sclerotherapy. Adjuvant therapies like liposuction and sclerotherapy can help treat multi-cystic lesions. Experimental drug therapies are also being explored but require more

1. Management of
Lymphangioma
2016/4/21
Surgical resident 蔡逸文

2. Clinical scenario
• 14 y/o female
• History of lymphangioma s/p excision in 2006 and left neck mass
noted on 2012
• Sudden enlargement of the left neck with pain and some swallowing
disturbance since about 4-5 days ago.

3. Cervicofacial lymphangioma
• Lymphatic malformations
• Low-flow vascular anomalies
• Macrocystic (diameter >1 cm)
Microcystic (diameter <1 cm)
• Cystic hygroma(CH) is the term
historically used for cervical
macrocystic LMs.
• Neck (75%), axilla (20%), and
inguinal areas (2%)
Sabin FR. Am J Anat. 1909

4. • Failure of lymphatics to connect to the venous system
• Abnormal budding of lymphatic tissue
• Sequestered lymphatic rests that retain their embryonic growth
potential
• Congenital development
• Trauma (including surgery)
• Inflammation
• Obstruction of a lymphatic drainage pathway.

5. • Karyotypic abnormalities are present in 25-70% of children with CH.
• more common in persons with Turner syndrome, Down syndrome, Klinefelter
syndrome, and trisomy 18 and 13
• Not considered to be causative.
• Several non-chromosomal disorders(Noonan syndrome, Fryns
syndrome, multiple pterygium syndrome, and achondroplasia…) are
associated with an increased incidence of CH.
• Intrauterine alcohol exposure has been associated with the
development of lymphangiomas.
• Gorham-Stout syndrome: Dissolution of bone caused by either
lymphangiomas or hemangioma

6. • Cologne Disease Score (0(worst)-10(best))
• Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement
towards progression 10 (best) points.
• 2: no limitation was seen in the patient concerning the respective item.
• 1: mild limitation
• 0: considerable limitation in the respective item could be observed.
de Serres Lm, et al. Arch Otolaryngol Head Neck Surg. (1995)
Wittekindt C, et al. Int J Pediatr Otorhinolaryngol. 2006
17%
41%
67%
80%
100%
Complications
• Preoperative: preoperative infection, respiratory
embarrassment necessitating airway intervention,
and feeding difficulties.
• Postoperative: cranial nerve injury, wound
infection, and seroma formation.
• Long-term sequelae: malocclusion, speech delay,
and cosmetic deformity

8. Department of Surgery, Section of Pediatric Surgery, Comer Children’s Hospital, The University of Chicago
Medicine and Biological Sciences

9. Surgery
• Concentrate on a defined anatomical region
• Limit blood loss
• utilizing a staged approach as needed
• Perform a thorough dissection
• Preserve critical vascular and neural structures
• Leave a closed suction drainage system
Mulliken JB, et al. Vascular anomalies. Curr Prob Surg. 2002.

10. Sclerotherapy
• Sclerotherapy is effective in treating and resolving macrocystic LMs,
with much less efficacy in microcystic LMs
• Entering the cystic cavity with a direct puncture under radiographic
guidance, aspirating the cystic fluid, and finally injecting the
sclerosant
• Adverse reactions after sclerotherapy include soft tissue edema
resulting in airway obstruction, skin necrosis, and neuropathy
Ann M. Defnet, et al. Pediatr Surg Int (2016)
Manning SC, et al. Curr Opin Otolaryngol Head Neck Surg(2013)

12. • 193 children with lymphangioma, 164 patients undergoing primary
therapy
• Total excision (77.4%) with recurrence rates of 11.8%
• partial excision(20.7%) with recurrence rates of 52.9%.
• Sclerotherapy with D50W used as an adjunct in 9.5%.
• 17.6% recurrences in use of D50W after total resection; 40.0% Seroma
formation
• 11.8% recurrences in after total resection; 3.5% Seroma formation
• Sclerotherapy with 50% dextrose is not beneficial in the management
of recurrent disease or postoperative seromas.
• Local drains and perioperative antibiotics do not appear to diminish
the incidence of seromas and infectious complications, respectively.
38% of all operations for resection of
recurrent or residual disease.

13. Gilony D, et al (2012) J Pediatr Surg.
• Surgery for cases requiring a histologic diagnosis, microcystic disease,
patients with an urgent clinical problem (eg, airway obstruction), and
sclerotherapy failures
Retrospective study(1999 -2010) from Schneider Children's Medical Center of Israel, Petach Tikva, Israel

14. • Primary surgery versus primary sclerotherapy
• No difference in effectiveness of treatment of less complex head and
neck LMs after the first intervention or at one year
• Lower-stage LMs were often treated successfully after a single
intervention with either treatment type
Balakrishnan K, et al (2014) Otolaryngol Head Neck Surg
Retrospective cohort study, 174 patients from Cincinnati Children’s Hospital, Cincinnati, Ohio

15. • Ultrasound-guided liposuction
• conjunction with sclerotherapy to better treat multi-cystic LMs of the neck by
rupturing cyst walls with good effect and no complications, case report
Mitsukawa N, et al. J Craniofac Surg. 2012

16. Ablation
• Radiofrequency ablation (RFA) for reducing mucosal
lymphangiomatous lesions, especially microcystic LMs
• High frequency mode:
• Destruction of deep tissue without affecting adjoining structures or mucosa.
• Lesional size is diminished due to subsequent fibrosis.
• Low-frequency mode
• Energy to be transmitted through a conductive medium, such as isotonic
saline, for removal of a thin superficial layer with minimal injury to nearby
tissue
Berg EE, et al(2013) Ann Otol Rhinol Laryngol.
Kim SW, et al (2011) Arch Otolaryngol Head Neck Surg

17. Laser
• For Microcystic lesions, Remove mucosal microvesicles
• Carbon dioxide lasers, Neodymium:yttrium-aluminum-garnet
(Nd:YAG) Lasers
• lymphangioma circumscriptum
• pulsed dye lasers
• cutaneous lesions

18. • Sildenafil
• Decrease LM size and alleviate associated symptoms, in case report
• A therapeutic response in 6 of 7 patients without significant side effects in a
small, open-label study
• Recommend caution before prescribing
• Propranolol
• Infantile hemangiomas
• Not all patients respond to treatment, in case reports
• Sirolimus
• A tongue lesion that did not respond to surgery or propranolol in case report
Danial C, et al (2014). J Am Acad Dermatol
Swetman GL, et al (2012) N Engl J Med

19. Take home message