Lymphangioma and mangement

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  • Management of Lymphangioma

    2016/4/21Surgical resident

  • Clinical scenario14 y/o femaleHistory of lymphangioma s/p excision in 2006 and left neck mass noted on 2012Sudden enlargement of the left neck with pain and some swallowing disturbance since about 4-5 days ago.

  • Cervicofacial lymphangiomaLymphatic malformationsLow-flow vascular anomaliesMacrocystic (diameter >1 cm) Microcystic (diameter
  • Failure of lymphatics to connect to the venous systemAbnormal budding of lymphatic tissueSequestered lymphatic rests that retain their embryonic growth potential

    Congenital developmentTrauma (including surgery)InflammationObstruction of a lymphatic drainage pathway.

  • 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 13Not 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

  • 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 limitation0: 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

    Preoperative complications reviewed include preoperative infection, respiratory embarrassment necessitating airway intervention, and feeding difficulties. Postoperative complications assessed were cranial nerve injury, wound infection, and seroma formation. Long-term sequelae included malocclusion, speech delay, and cosmetic deformity*

  • Department of Surgery, Section of Pediatric Surgery, Comer Childrens Hospital, The University of Chicago Medicine and Biological Sciences

  • SurgeryConcentrate on a defined anatomical regionLimit blood lossutilizing a staged approach as neededPerform a thorough dissectionPreserve critical vascular and neural structuresLeave a closed suction drainage system

    Mulliken JB, et al. Vascular anomalies. Curr Prob Surg. 2002.

    Mulliken JB, Fishman SJ, Burrows PE (2000) Vascular anomalies. Curr Prob Surg 37:517584*

  • SclerotherapySclerotherapy 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 sclerosantAdverse 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)

    Fevurly RD, Fishman SJ (2012) Vascular anomalies in pediatrics. Surg Clin North Am 92:769800Colletti G, Valassina D, Bertossi D et al (2014) Contemporary management of vascular malformations. J Oral Maxillofac Surg 72:510528Adams MT, Saltzman B, Perkins JA (2012) Head and neck lymphatic malformation treatment: a systematic review. Otolaryngol Head Neck Surg 147:62763958. Acevedo JL, Shah RK, Brietzke SE (2008) Nonsurgical therapies for lymphangiomas: a systematic review. Otolaryngol Head Neck Surg 138:41842459. Farnoosh S, Don D, Koempel J et al (2015) Efficacy of doxycycline and sodium tetradecyl sulfate sclerotherapy in pediatric head and neck lymphatic malformations. Int J Pediatr Otorhinolaryngol 79:88388760. Leung M, Leung L, Fung D et al (2014) Management of the low-flow head and neck vascular malformations in children: the sclerotherapy protocol. Eur J Pediatr Surg 24:97101*

  • 193 children with lymphangioma, 164 patients undergoing primary therapyTotal 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.

    . The diagnosis was established before 5 years of age in 87.0%. The distribution of lymphangiomas was: cervical (31.4%), craniofacial (18.9%), extremity (18.9%), trunk (9.2%), intraabdominal (9.2%), cervicoaxillothoracic (4.9%), multiple (3.8%), cervicomediastinal (2.2%), and intrathoracic (1.6%).*

  • 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

    Gilony D, Schwartz M, Shpitzer T et al (2012) Treatment of lymphatic malformations: a more conservative approach. J Pediatr Surg 47:18371842*

  • 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 SurgRetrospective cohort study, 174 patients from Cincinnati Childrens Hospital, Cincinnati, Ohio

    Balakrishnan K, Menezes MD, Chen BS et al (2014) Primary surgery vs primary sclerotherapy for head and neck lymphatic malformations. Otolaryngol Head Neck Surg 140:4145

    Cologne Disease Score (CDS). Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement towards progression(Int J Pediatr Otorhinolaryngol.2006 Jul;70(7):1205-12. Epub 2006 Feb 7.) 0 (worst) points to 10 (best) points. Each parameter yielded two, one or zero points. Two points were given when no limitation was seen in the patient concerning the respective item. One point was given at mild limitation and zero points were given when considerable limitation in the respective item could be observed.*

  • Ultrasound-guided liposuctionconjunction 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

  • AblationRadiofrequency ablation (RFA) for reducing mucosal lymphangiomatous lesions, especially microcystic LMsHigh frequency mode: Destruction of deep tissue without affecting adjoining structures or mucosa. Lesional size is diminished due to subsequent fibrosis. Low-frequency modeEnergy 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

    Berg EE, Sobol SE, Jacobs I (2013) Laryngeal obstruction bycervical and endolaryngeal lymphatic malformations in children:proposed staging system and review of treatment. AnnOtol Rhinol Laryngol 122:575581Kim SW, Kavanagh K, Orbach DB et al (2011) Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg 137:12471250*

  • LaserFor Microcystic lesions, Remove mucosal microvesicles

    Carbon dioxide lasers, Neodymium:yttrium-aluminum-garnet (Nd:YAG) Laserslymphangioma circumscriptumpulsed dye laserscutaneous lesions

  • SildenafilDecrease 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 studyRecommend caution before prescribingPropranololInfantile hemangiomasNot all patients respond to treatment, in case reportsSirolimusA 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

    Case reports show sildenafil can decreaseLM size and alleviate associated symptoms [99, 100]. In asmall, open-label study, sildenafil was shown to have atherapeutic response in six out of seven patients withoutsignificant side effects [101]. Despite promising results inmany case reports and small studies, other reports haveshown no effect of sildenafil in LMs, and recommendcaution before prescribing sildenafil empirically for LMsand other vascular anomalies

    Francis CS, Rommer EA, Kane JT et al (2012) Limited-incision surgical debulking of lymphatic malformations using ultrasound-assisted liposuction. Plast Reconstr Surg 130:920e922e*

  • Take home message

  • Preoperative complications reviewed include preoperative infection, respiratory embarrassment necessitating airway intervention, and feeding difficulties. Postoperative complications assessed were cranial nerve injury, wound infection, and seroma formation. Long-term sequelae included malocclusion, speech delay, and cosmetic deformity*Mulliken JB, Fishman SJ, Burrows PE (2000)