Management Of Lymphatic Malformations Research Articles

Sclerosing agents in the management of lymphatic malformations in children: A systematic review

PurposeSclerotherapy is frequently employed in treating lymphatic malformations (LMs), and multiple agents, practitioners and strategies exist. This review investigates the reported efficacy and safety of sclerosants in the pediatric population. MethodsAdhering to PRISMA guidelines, multiple databases were queried without linguistic or temporal restriction. Inclusion criteria were patients aged 0–18 exclusively receiving injection sclerotherapy for the treatment of LMs with follow-up data. Data abstracted included agent, dose, anatomic site and key outcome measures including complications (major/minor) and resolution rates (>95% reduction in volume). Critical appraisal was undertaken using the MINORS tool. ResultsForty-eight studies met the inclusion criteria with a mean MINORS score of 0.65 ± 0.08. Included studies yielded 886 patients, across nearly 30 years. The overall observed rate of success was 89%, with variable follow-up across publications (6 weeks - 10 years). Most reported LMs were macrocystic (82%) and had a higher resolution rate than mixed/microcytic variants (89%, 71%, 34%, p<0.01) For head/neck LMs, rates of complete regression for OK-432, bleomycin, and doxycycline were 67% ± 27% (n = 26), 91% ± 53% (n = 34) and 85% ± 16% (n = 52) respectively. Major complications were most commonly reported with OK-432, including airway compromise or subsequent operation. ConclusionsIn pediatric patients treated for LM by sclerotherapy, complication rates were low. Macrocystic lesions respond well but success rates were modest at best for microcystic disease. Differences in agent utilization were noted between high and low resourced contexts; despite its lack of federal approval, OK-432 was the most reported agent. Further prospective research is warranted. LOE3a

Lymphatic and Mixed Malformations.

Lymphatic and mixed malformations are rare and variable in presentation. They arise due to errors in vascular and lymphatic formation during early embryonic development. This leads to persistent infiltration of lymph fluid into soft tissues and causes a locally invasive mass with pathologic sequelae. Departing from historically descriptive terminology, such as "cystic hygroma," lymphatic malformations are now categorized as macrocystic, microcystic, or mixed lesions, based on size. Advances in imaging modalities, such as ultrasonography and magnetic resonance imaging, have made accurate characterization of these lesions possible and ultimately allow for early diagnosis and implementation of appropriate treatment based on the morphology of the lymphatic malformation. Management of lymphatic malformations can be quite challenging, and a multidisciplinary approach is most effective for optimum aesthetic and functional outcomes. New discoveries in the molecular biology of lymphatic malformations have provided treatment targets and established a role for pharmacotherapy. Sclerotherapy, laser, and radiofrequency ablation have all proven to be effective as minimally invasive treatment options for lymphatic malformations. Surgical intervention has a role in the treatment of focal lesions recalcitrant to these less invasive techniques. Operative planning is dictated by clinical goals, size, anatomic location, characteristics, and extent of infiltration.

Surgical resection of macrocystic lymphatic malformations of the head and neck: Short and long-term outcomes

BackgroundControversy exists on management of lymphatic malformations, with schools of thought advocating for observation, surgery or sclerotherapy. This study sought to examine outcomes after surgical resection of pediatric cervicofacial macrocystic lymphatic malformations (MLM). MethodsCase series with planned data collection on pediatric patients with cervicofacial MLM who underwent surgical resection at a tertiary referral center for vascular anomalies from January 1995 to June 2016. For consistency in patient population analysis, patients who had pre-surgical sclerotherapy or had mixed or microcystic disease were excluded. The main outcome was complete response rate (CR) and long-term recurrence-free survival (RFS). ResultsSixty-three patients who underwent excision of MLM were included, 52.4% were female, 77.8% Caucasian. The majority had de Serres stage I-III (96.8%) affecting the neck (71.4%). Patients were discharged the same day (28.6%), or had a 1 day median length-of-stay (interquartile range (IQR) = 2). Surgical complications included seroma/hematoma (9.5%), transient nerve weakness (facial nerve, sympathetic chain, or phrenic nerve, 6.3%), and infection (1.6%). On long-term follow-up (median: 12 months, IQR 1–43 months), a single surgery achieved CR in 90.5% of patients. RFS was achieved in 86% of patients in our observation period of up to 15 years. Most patients requiring a second intervention failed within 6-months of initial procedure (4/5 patients, 90%); associated factors included bilaterality, advanced staging, and partial response at first-follow-up (p = 0.0051, 0.0051, and <0.0001, respectively). ConclusionsSurgery is safe and effective as first line treatment for selected MLM. For stage I-III MLM CR and long-term RFS can be achieved with a single surgery. A direct and randomized comparison of treatment modalities is needed.

Intralesional sclerotherapy with bleomycin in lymphatic malformation of tongue an institutional experience and outcomes

Introduction:The management of lymphatic malformations (LMs) continues to improve with advancement in molecular genetics, imaging, and treatment options. However, the management of tongue LMs remains a challenge due to the location, function involved, and long-term disabilities. We propose injection sclerotherapy with bleomycin in the management of spectrum of tongue LMs.Methods:Children with LMs involving the tongue were prospectively treated with bleomycin sclerotherapy. Outcome measured was the efficacy of sclerotherapy, complications, and functional outcome.Results:A total of 11 children underwent sclerotherapy with bleomycin for varying tongue lesions. Excellent outcome was seen in children with macroglossia. Eight children with isolated (focal) lesions had a resolution of symptoms with a clearance of lesions. Specific complications related to bleomycin toxicity were not encountered in our series during the follow-up of 4 years.Conclusion:In our series, children with macroglossia had an excellent outcome with normalization of tongue size and function. Children with focal tongue lesions also had good to excellent outcome. We recommend treatment of tongue LM with bleomycin sclerotherapy as the first line of management. Ease of treatment, early intervention, and excellent response makes it a favorable treatment option.

Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review.

Extensive lymphatic malformations are low-flow vascular malformations that can cause devastating complications. Treatment of these malformations is challenging. This systematic review presents current use of sirolimus in patients with extensive lymphatic malformations. MEDLINE and Google scholar search was conducted for studies on sirolimus treatment of lymphatic malformations up to July 2017. Search items included "lymphatic malformation," "lymphangioma," "cystic hygroma," "vascular malformation," "low-flow malformation," "sirolimus," "rapamycin," and "mTOR inhibitor." Twenty studies, including 71 patients receiving sirolimus, were included into this review. Forty-five patients had lymphatic malformations, eight patients venolymphatic malformations, and 19 patients capillary-lymphatico-venous malformations. Sirolimus led to a partial remission of disease in 60 patients, three patients had a progressive disease, and the outcome of eight patients was not reported. Dosing, target trough level, and duration of treatment differed between the studies. Common adverse effects were hyperlipidemia and neutropenia. Available literature indicated that sirolimus therapy might be effective for lymphatic malformations. However, further randomized controlled studies are required to analyze the efficacy and long-term adverse events and to clarify the potential role for sirolimus in the management of lymphatic malformations.

Nursing Implications for the Management of Lymphatic Malformation in Children [Formula: see text

Lymphatic malformation (LM) is a benign congenital childhood growth that presents primarily at birth, with the remainder becoming evident by age 2 years. LM can cause devastating complications such as respiratory failure, dyspnea, dysphagia, organ compression, and nerve compression. Surgery is the preferred treatment option, although resection is not always an option due to the anatomic location of the malformation. Other treatments that have been tried with success include sclerotherapy, radiofrequency ablation, propranolol, and sirolimus. Nurses have an important role in caring for the child with LM, as with any complex disease. Nurses provide care at the bedside, education to the family, case coordination, and psychosocial support. LM is a rare disease in infancy necessitating pediatric nurses to support families through prolonged treatments and potential complications.

Management of lymphatic malformations in children.

To review the literature on lymphatic malformations and to provide current opinion about the management of these lesions. Current treatment options include nonoperative management, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New therapies are emerging, including sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management centers on the patient's quality of life. Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, in addition to knowledge gained from clinical practice. A patient-centered approach should guide timing and modality of treatment. Continued study of lymphatic malformations will increase and solidify a treatment algorithm for these complicated lesions.

Current treatment paradigms in the management of lymphatic malformations

Lymphatic malformations (LMs) are benign abnormalities of the lymphatic system that can be significantly infiltrative and intimately involve critical structures of the head and neck, making their management difficult. Historically, LMs have been managed by surgical excision, but this treatment approach results in significant morbidity and a high recurrence rate secondary to subtotal resection. As an alternative to surgery a variety of drugs have been used as intralesional sclerosants and immunotherapeutics. These agents offer improved outcomes with lower morbidity as compared to surgery when targeted to macrocystic LMs. However intralesional therapy is not effective in the treatment of microcystic LMs. The development of treatments that are effective for all types of LMs will require further understanding of lymphangiogenesis and the pathogenesis of LMs.

Management of lymphatic malformations and macroglossia: Results of a national treatment survey

Objective To determine current trends among American Society of Pediatric Otolaryngology members on the treatment of various stages of lymphatic malformation (LM) with an emphasis on tongue management. Methods We queried the members on practice demographics, number of LMs and LM-Mac treated, preferred treatment of different stages of LM and Lm-Mac, indications for LM-Mac tongue treatment, preferred method of surgical tongue reduction, and medical management of acutely enlarging LMs and LM-Mac. Results 39/329 (12%) American Society of Pediatric Otolaryngology members responded to the survey. Airway obstruction or obstructive sleep apnea (27/39, 69%) followed by recurrent tongue trauma with bleeding, pain or mucosal changes (11/39, 28%) were the most common indications for tongue management. 16/37 (43%) of respondents preferred staged tongue reduction followed by neck dissection (cervical approach to the LM), 8/37 (22%) preferred staged neck dissection followed by tongue reduction, and 13/37 (35%) preferred simultaneous treatment of the tongue and neck. The preferred methods of tongue reduction were superficial laser ablation (17/38, 45%) and surgical excision (14/39, 36%). The preferred methods of surgical tongue reduction were anterior wedge (18/38, 47%) and midline keyhole reduction (13/38, 34%). For rapidly enlarging lymphatic malformations involving the tongue, the majority of respondents indicated that they would admit and observe (34/38, 89%), give steroids (34/37, 92%) and administer antibiotics (35/38, 92%). Conclusions While providing insight into treatment patterns, this survey also helps to elucidate the need for multicenter trials for treatment of LM to develop a standard of care that can be recommended based on evidence based medicine rather.

New treatment options for lymphangioma in infants and children.

Lymphangiomas are congenital malformations of the lymphatic system. These lesions occur most often in the head and neck area, and their treatment continues to be a challenge. Fortunately, a number of advances have occurred in the diagnosis and management of lymphatic malformations in the past decade. The purpose of this article is to clarify the embryology, pathogenesis, histopathology, and classification of these lesions, as well as to describe their various forms of clinical presentation. We provide a complete review of the diagnostic measures available and thoroughly discuss new therapeutic interventions proposed to treat lymphangiomas.

Management of lymphatic malformations: If, when, and how

Lymphatic malformations are usually congenital in origin and often arise in the head and neck region. The size of lymphatic malformations varies markedly and treatment is tailored ascending to the degree of function and cosmetic compromise. Current common treatment options include observation, sclerosant therapy, somnoplasty, laser reduction/excision, and surgical removal. The classification, clinical presentation, diagnosis and treatment of lymphatic malformations are further discussed in this article.