Lymphangioma and mangement

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Management of Lymphangioma 2016/4/21 Surgical resident 蔡蔡蔡

Transcript of Lymphangioma and mangement

Page 1: Lymphangioma and mangement

Management of Lymphangioma

2016/4/21Surgical resident 蔡逸文

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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.

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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

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• 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.

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• 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

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• 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

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

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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.

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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)

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• 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.

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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

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• 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

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• 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

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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

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Laser• For Microcystic lesions, Remove mucosal microvesicles

• Carbon dioxide lasers, Neodymium:yttrium-aluminum-garnet (Nd:YAG) Lasers• lymphangioma circumscriptum

• pulsed dye lasers• cutaneous lesions

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• 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

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Take home message

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