Neck Lymphatic Malformations Research Articles

Healthcare utilization in patients with head and neck lymphatic malformations with the introduction of sirolimus

ObjectivesHistorically, head and neck lymphatic malformations (HNLM) have been managed through surgical and interventional procedures. Sirolimus was introduced in 2016 and has aided in symptomatic control of HNLM. The study objective was to assess healthcare utilization with the introduction of sirolimus for HNLM. MethodsAn observational cohort study of LM patients treated between 2008 and 2022 at a tertiary care children's hospital was performed. 588 charts were reviewed; patients with isolated, non-syndromic HNLM and at least 2 years of follow-up were included (n = 45). Data included sirolimus use, complications, presence of tracheostomy and/or gastrostomy-tube, and number and costs of HNLM-related sclerotherapies, procedures, hospitalizations, and emergency room visits. For patients who received sirolimus, encounters two years prior to and after sirolimus initiation were recorded. For the non-sirolimus group, encounters two years after the initial clinic visit for HNLM were recorded. Statistical analysis was used to compare the groups. ResultsMedian age at first clinic visit was 1.8 years (range 2 days–41 years). Tracheostomy was present in 43 % of sirolimus patients compared with 3 % of the non-sirolimus group (OR: 24.0, 95%CI: 1.55–1490, p = 0.02). Patients on sirolimus experienced significantly fewer sclerotherapy visits (z = 2.08, p = 0.03) compared to the non-sirolimus group. Minimal sirolimus-related side effects were reported. Total HNLM-related costs were significantly less in the sirolimus group during treatment (median $448.13, range $0–$7041.28) compared with before treatment (median $17,069.24, range $1999.16–$211,848.50, z = 2.20, p = 0.03). Median costs associated with sclerotherapy were less for the sirolimus groups during treatment compared with the non-sirolimus group (z = 1.97, p = 0.04). In the sirolimus group, costs associated with HNLM-related hospitalizations were significantly less during sirolimus treatment compared with before (z = 2.20, p = 0.03). ConclusionSirolimus has improved the clinical course for HNLM patients by decreasing number of procedures and healthcare costs, with limited side effects. Larger cohorts matching type of HNLM and age are needed to assess healthcare utilization benefits of sirolimus.

A multi-step approach to overcome challenges in the management of head and neck lymphatic malformations, and response to treatment

BackgroundLymphatic malformations are vascular developmental anomalies varying from local superficial masses to diffuse infiltrating lesions, resulting in disfigurement. Patients’ outcomes range from spontaneous regression to severe sequelae notwithstanding appropriate treatment. The current classification guides, in part, clinicians through the decision-making process, prognosis prediction and choice of therapeutic strategies. Even though the understanding of molecular basis of the disease has been recently improved, a standardized management algorithm has not been reached yet.ResultsHere, we report our experience on five children with different lymphatic anomalies of the head and neck region treated by applying a multidisciplinary approach reaching a consensus among specialists on problem-solving and setting priorities.ConclusionsAlthough restitutio ad integrum was rarely achieved and the burden of care is challenging for patients, caregivers and healthcare providers, this study demonstrates how the referral to expert centres can significantly improve outcomes by alleviating parental stress and ameliorating patients’ quality of life. A flow-chart is proposed to guide the multidisciplinary care of children with LMs and to encourage multidisciplinary collaborative initiatives to implement dedicated patients’ pathways.

Imaging manifestations of head and neck lymphatic malformations: A single-center experience of 170 surgical cases.

To discuss the imaging manifestations and the utility of preoperative ultrasonography (US), contrast-enhanced computed tomography (CE-CT) and contrast enhanced magnetic resonance imaging (CE-MRI) in diagnosing the pediatric head and neck lymphatic malformations (HNLMs). We performed a retrospective review of 170 children who were referred to our hospital in the past 9 years for the treatment of HNLMs. The diagnostic rates of US, CE-CT and CE-MRI were 93.0% (146/157), 94.7% (143/151) and 100% (45/45), respectively. As in multilocular cases, intracystic septa detection rate was 91.5% (130/142), 50.4% (68/135) and 88.1% (37/42), and which had a statistical difference (χ2 = 25.8131, p < 0.05). US showed capsule contents anechoic in 51.0% (80/157) cases, hypoechoic or mixed echoic in 49.0% (77/157) cases, and flocculent or dotted echo floating in 36.9% (58/157) cases. CT showed low density of the capsule contents without enhancement in 69.5% (105/151) cases and mixed density with enhancement in 30.4% (46/151) cases. Liquid-liquid levers were seen in 8.6% (13/151) cases. MRI showed T1WI high signal and T2WI low signal of the capsule contents without enhancement in 28.9% (13/45) cases and mixed density in 71.1% (32/45) cases. Liquid-liquid levers were seen in 46.7% (21/45) cases. There were statistically significant differences between pure HNLMs and intracystic hemorrhage in capsule content (echo, density, signal), enhancement, and liquid-liquid lever (all p < 0.05). Among US, CE-CT and CE-MRI, intracystic hemorrhage diagnostic accuracy had a statistical difference (χ2 = 25.4152, p < 0.05). For clinical diagnosis and evaluation of HNLMs, we suggest that US combined with CE-CT for acute cases, and for stable cases, US combined with CE-MRI.

The value of MRI in the diagnosis of pediatric head and neck lymphatic malformations: A study of 46 surgical cases

Objective To discuss the value of MRI in diagnosing and evaluating the pediatric head and neck lymphatic malformations (HNLMs). Methods We performed a retrospective review of 46 children who were referred to our hospital in the last decade for the treatment of HNLMs. Results About 34 cases confirmed with intralesional hemorrhage while the capsule contents were dark red or light bloody liquid. The remaining 12 pure HNLMs were filled with yellow clear or watery liquid. The multilocular HNLMs accounted for 95.7 % (44/46). The accuracy of contrast enhanced MRI (CE-MRI) diagnosis of HNLMs was 100 %. On MRI, the HNLMs appeared as irregular shape [95.7 % (44/46)], clear boundary [91.3 % (42/46)], infiltrative growth [91.3 % (42/46)] cystic masses. The cystic wall and septa were hyperintense on T1WI and hypointense on T2WI (100 %), and displayed enhancement. The capsule contents had hypointense on T1WI and hyperintense on T2WI in 18 cases (pure HNLMs,12; intracystic hemorrhage,6), while that of mixed signal in 28 cases (pure HNLMs,0; intracystic hemorrhage,28). Capsule contents were enhanced in 22 cases (pure HNLMs,1; intracystic hemorrhage,21), while the remaining 24 without enhancement (pure HNLMs,11; intracystic hemorrhage,13). Liquid-liquid levers were found in 21 cases (pure HNLMs,0; intracystic hemorrhage,21). There were statistical differences in capsule contents signal, enhancement, and liquid–liquid levels between the two groups (P < 0.05). Conclusions On MRI, HNLMs typically show a thin-walled, well-circumscribed, irregularly shaped, infiltrative, unenhanced, multilocular cystic mass with hypointense on T1WI and hyperintense on T2WI. The capsule wall and septa are hyperintense on T1WI, hypointense on T2WI, and display enhancement. Changes in the signal of capsule contents or appearance of liquid–liquid levels indicate intracystic hemorrhage.

Outcome of Sclerotherapy in a Preterm Infant with a Giant Fetal Neck Lymphatic Malformation: A Case Report.

Lymphatic malformation (LM), most commonly present in the neck area, is benign vascular malformations of the lymphatic system. In an infant, however, LM poses a high risk of adverse outcomes. We present a case with a giant fetal LM. Through ultrasonography, at 23+ weeks of gestation, a septate cystic mass 7.2×6.5×6.3 cm in size was found on the right side of the fetus's neck. After extensive counseling by the multidisciplinary team, the parents chose to continue the pregnancy. Severe fetal tracheal compression was observed at 29 weeks by magnetic resonance imaging (MRI). At 31 weeks and 5 days, owing to suspected fetal distress, an emergency cesarean section was performed and a male baby weighing 1720 g was delivered. The mass was 10×16×8 cm in size and ex utero intrapartum treatment (EXIT) was implemented. Due to progressive growth of the mass secondary to intralesional bleeding, an intralesional injection of bleomycin was administered three days later. This injection was repeated at the age of 1 month and 8 days. The baby was followed up and, by a year after his birth, LM had disappeared. The baby has since been in good health. Accurate prenatal diagnosis and regular monitoring of a fetus with LM may improve prognosis. It is essential to have a trained multidisciplinary team to evaluate the condition of the fetus and the neonate and to provide treatment based on the evaluation. Our experience with intralesional bleomycin injection for the treatment of a giant fetal neck LM in a preterm infant had a favorable outcome. Long-term follow-up by a multidisciplinary team is needed in such cases.

Impact of puberty on procedure frequency for treatment of simple head and neck lymphatic and venous malformations

ObjectivePuberty has been shown to accelerate growth of vascular malformations, including lymphatic (LM) and venous malformations (VM). This study aims to compare the number of procedures performed before and after puberty in patients with LM and VM to assess whether the onset of puberty results in higher treatment frequency. MethodsA retrospective review of head and neck LM and VM patients who were evaluated between January 2009 and December 2019 was performed. Patient demographics, lesion characteristics, and procedural details were recorded. For the purposes of this study, 11 years or older in females and 12 years or older in males were the established cut-offs for the onset of puberty. ResultsAfter initial screening of 357 patients, 83 patients were included in the study based on inclusion criteria. There were 34 patients with LM (41 %) and 49 with VM (59 %). The mean age at diagnosis was 6.1 ± 10.9 years (LM: 4.2 ± 7.0, VM: 7.4 ± 12.9, p = 0.489). 68 patients underwent treatments, which included sclerotherapy, surgical excision, and/or laser. For all patients, the average number of lifetime treatments when initiated before puberty was 3.78 ± 2.81 and when initiated after puberty was 2.17 ± 1.37 (p = 0.022). Patients diagnosed pre-puberty were more likely to undergo treatments vs. those diagnosed after puberty (OR 10.00, 95 % CI: 2.61–38.28, p < 0.001). ConclusionWe found that the number of treatments was fewer in those who started treatment after puberty. This finding suggests that providers may elect to proceed with observation in asymptomatic patients, given that waiting until after the onset of puberty has not shown an increase in the procedural load on patients.

Management of the head and neck lymphatic malformations in children: A 7-year experience of 91 surgical cases

ObjectiveTo review the clinical characteristics and treatment outcomes of head and neck lymphatic malformations (HNLMs) in children. MethodsA retrospective study of 91 patients with HNLMs was performed. ResultsThe age ranged from 1 day to 14 years, of which 82.4 % (75/91) were under 2 years old and 45.1 % (41/91) were diagnosed at birth. The diagnostic rates of ultrasound, CT and MRI were 80.2 % (73/91), 90.1 % (82/91) and 100 % (8/8) respectively. There were 2 cases of complete excision, 8 of bleomycin sclerotherapy, and 81 of subtotal resection combined with bleomycin irrigation. Followed up for 3–93 months, all 91 cases were cured. ConclusionsHNLMs mostly occur within 2 years old, and nearly half of them are present at birth. Characteristic imaging findings can assist clinicians in diagnosis and treatment plan. Subtotal resection combined with bleomycin irrigation may be an appropriate first-line therapy for HNLMs involving the vital anatomical structures.

Primary targeted medical therapy for management of bilateral head and neck lymphatic malformations in infants.

Patients born with bilateral head and neck lymphatic malformations (BHNLMs) often require multiple invasive treatments, including tracheostomy. We hypothesized that primary targeted medical therapy (pTMT) with diagnostic needle aspiration reduces the need for invasive therapy such as surgical resection and/or sclerotherapy. Retrospective case review was performed of infants with BHNLMs (Grade 2 or De Serres stage IV and V) treated only at our institution from 2000 to 2021. Patients were divided into two cohorts: those managed with pTMT and those managed with observation, sclerotherapy, or surgical intervention (non-pTMT). Data regarding interventions, clinical outcomes, morbidity, and mortality were analyzed with descriptive statistics. Nine children with BHNLMs met inclusion criteria. Three (33%) were in the pTMT cohort and six (66%) were non-pTMT. Eight (89%) malformations were genotyped, and all demonstrated hotspot PIK3CA variants. All pTMT patients had sirolimus initiated in the first month of life and underwent needle aspiration of malformation cyst fluid for cell-free DNA samples. All pTMT patients tolerated medical therapy. For the non-pTMT cohort, primary treatment included none (deceased, n=1, 17%), observation with needle aspiration (n=1, 17%), surgical resection (n=2, 33%), or combination surgery and sclerotherapy (n=2, 33%). Intubation duration, intensive care and initial hospital length of stay were not different between cohorts. Four non-pTMT patients (67%) required tracheostomy, and two (33%) died prior to discharge. All pTMT patients survived and none required tracheostomy. Non-pTMT patients required a median of two invasive therapies prior to discharge (IQR 1-4) and a mean total of 13 over the course of their lifetime (IQR 1-16), compared to the pTMT group who did not require any lifetime invasive therapy, even after initial pTMT and discharge home. This study compares patients with BHNLMs (Grade 2) treated with pTMT versus those treated with observation or invasive therapy. Patients treated with pTMT required no surgical or invasive procedural treatment of their malformations, no tracheostomy placement, no unplanned readmissions after discharge, and had no mortalities. Needle aspiration was useful as a therapeutic adjunct for cell-free DNA diagnosis of PIK3CA variants, which guided TMT.

The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations

Lymphatic malformations (LMs) are developmental defects of lymphatic vessels. LMs are histologically benign lesions, however, due to localization, size, and unexpected swelling, they may cause serious complications that threaten vital functions such as compression of the airways. A large swelling of the face or neck may also be disfiguring and thus constitute a psychological strain for patients and their families. LMs are also highly immunologically reactive, and are prone to recurrent infections and inflammation causing pain as well as chronic oozing wounds.The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe. There are only few available guidelines on management and follow up of LMs, which commonly focus on very specific situations, such as head and neck LM (Zhou et al., 2011). It is still unclear, what constitutes an indication for treatment of LMs and how to follow up the patients. The Vascular Anomalies Working Group (VASCA-WG) of VASCERN decided to develop a diagnostic and management pathway for the management of LMs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following 2 face-to-face meetings and multiple web meetings to facilitate discussion, and by mail to avoid the influence of most authoritative members.The VASCA-WG has produced this opinion statement reflecting strategies developed by experts and patient representatives on how to approach patients with lymphatic malformations in a practical manner; we present an algorithmic view of the results of our work.

Classification specific lymphatic malformations management on head and neck in children

The aim of this study was to retrospectively analyze the outcomes from surgical and nonsurgical treatments for head and neck lymphatic malformations (LMs) in children. Fifty-eight patients were divided into a surgical group (22) and a nonsurgical group (36). The surgical group contained microcystic LMs in the tongue treated by surgery or with sclerotherapy. The nonsurgical group contained macrocystic and mixed LMs in floor of the mouth, neck, face, submandibular region, maxillofacial region and neck treated by sclerotherapy or with microwave ablation (MWA). In the surgical group, the mean follow-up time was 44 ± 15.3 months. At last follow-up, 15 LMs (68.2%) were completely controlled, 3 LMs (13.6%) were stable without need for further treatment, and 4 LMs (18.2%) were persistent. In the nonsurgical group, the median follow-up time was 23.5 months (13.0, 32.8). At last follow-up, 28 LMs (77.8%,) have a volume reduction rate of >50%, and 6 LMs (16.7%.) have the complication. Surgery is suitable for microcystic LMs in the early stage with clear boundary and becomes limited for them in the late stages due to diffuse lesions. Sclerotherapy is effective for macrocystic and mixed LMs. Sclerotherapy with MWA is exceptionally effective for large cystic LMs with multiple compartments.

Alpelisib for the treatment of PIK3CA-related head and neck lymphatic malformations and overgrowth

PurposePIK3CA-related overgrowth spectrum (PROS) conditions of the head and neck are treatment challenges. Traditionally, these conditions require multiple invasive interventions, with incomplete malformation removal, disfigurement, and possible dysfunction. Use of the PI3K inhibitor alpelisib, previously shown to be effective in PROS, has not been reported in PIK3CA-associated head and neck lymphatic malformations (HNLMs) or facial infiltrating lipomatosis (FIL). We describe prospective treatment of 5 children with PIK3CA-associated HNLMs or head and neck FIL with alpelisib monotherapy. MethodsA total of 5 children with PIK3CA-associated HNLMs (n = 4) or FIL (n = 1) received alpelisib monotherapy (aged 2-12 years). Treatment response was determined by parental report, clinical evaluation, diary/questionnaire, and standardized clinical photography, measuring facial volume through 3-dimensional photos and magnetic resonance imaging. ResultsAll participants had reduction in the size of lesion, and all had improvement or resolution of malformation inflammation/pain/bleeding. Common invasive therapy was avoided (ie, tracheotomy). After 6 or more months of alpelisib therapy, facial volume was reduced (range 1%-20%) and magnetic resonance imaging anomaly volume (range 0%-23%) were reduced, and there was improvement in swallowing, upper airway patency, and speech clarity. ConclusionIndividuals with head and neck PROS treated with alpelisib had decreased malformation size and locoregional overgrowth, improved function and symptoms, and fewer invasive procedures.

Partial resection of a face and neck lymphatic malformation after tracheostomy: A case of difficult airway management

Lymphatic malformation (LM) is a congenital abnormality of lymphatic vessel formation. When it occurs in the face and neck, airway management may be required. We describe a 1-month-old boy with a large LM of the face and neck. This case required airway management due to large lesions, and a tracheostomy needed to be performed. After the tracheostomy, the patient was troubled with frequent ventilation problems and developed a tracheal ulcer because of tracheostomy tube instability that occurred due to twisting of the trachea caused by the large lesions. Accordingly, we performed a partial resection of the LM, and the airway was stabilized. After partial resection, the patient was able to receive sclerotherapy and an m-TOR inhibitor as radical treatment. Based on this, we believe partial resection of an LM can be a viable option to stabilize airway management in cases of large lesions that cause airway constriction and tortuosity.

Delaying Invasive Treatment in Unilateral Head and Neck Lymphatic Malformation Improves Outcomes.

Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number. Consecutive HNLM patients (n= 199) between 2010 and 2017, aged 0-18 years. ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ2 ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT). Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p< 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR=0.09, 95% CI 0.02-0.36, p< 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR=0.47, 95% CI 0.33-0.66, p< 0.001). Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs. 4 Laryngoscope, 133:956-962, 2023.

Prenatally Diagnosed Lymphatic Malformations of the Head and Neck

Objectives: To describe prenatal diagnosis and perinatal management of patients with head and neck lymphatic malformations (LMs) at a tertiary fetal diagnostic and vascular anomalies center. To identify prenatal characteristics correlating with the need for airway intervention at birth. To describe postnatal clinical course in patients with prenatally diagnosed head and neck LMs. Methods: A retrospective review of all patients with head and neck LMs diagnosed in utero at a tertiary prenatal diagnostic center from 1996 to 2020 was performed. Prenatal records of pregnant patients and postnatal records of patients were reviewed. Univariate and multivariate logistic regression analyses were conducted to identify correlates of need for extrauterine intrapartum treatment (EXIT) delivery and correlates of clinical outcomes. Results: Over the study period, 26 fetuses were diagnosed with LM of the head and neck, 15 of which survived the perinatal period. Outcomes in these survivors are reported including birth modality, DeSerres’ stage, duration and modality of treatment, complications, hospitalizations, and functional outcomes including need for tracheostomy and gastrostomy tube. The majority of patients (n = 8) were born via the EXIT modality. Almost half (n = 7) were DeSerres’ stage V. Prenatal imaging findings of neck vessel compression (p = 0.023) and tracheal deviation (p = 0.006) correlated with EXIT delivery. The most common treatment modalities were sclerotherapy (n = 14), surgery (n = 7), and sirolimus (n = 8). Conclusions: This study identified prenatal anatomic findings of tracheal deviation and neck vessel compression as factors associated with EXIT delivery. None of the patients in our series required tracheostomy during the EXIT procedure, although 20% eventually underwent tracheostomy. The majority of infants with prenatally diagnosed head and LMs required multimodality treatment with some combination of sclerotherapy, surgery, and medical management, as well as multidisciplinary care from our vascular anomalies team throughout the first years of life and beyond.

Quantifying Head and Neck Lymphatic Malformation Volume and Composition During Treatment

Objective: Head and neck lymphatic malformation (HNLM) treatment involves a combination of observation, surgery, sclerotherapy, and targeted medical therapy. Objective comparison of these differing treatments is unstandardized due to heterogeneity of HNLM location, size, and variable components. Our objective was to develop a protocol for standardized assessment of magnetic resonance imaging (MRI) image sets through novel semiautomated algorithms. We aimed to obtain reproducible multimodal tissue level data that can be analyzed for interval HNLM treatment response. Methods: Patients who were undergoing therapy for HNLM were queried from an institutional database between 2015 and 2020. MRI sequences were registered in 3D Slicer, and in MATLAB a multimodal tool was developed to quantitate HNLM volume and changes in composition. Volume measures were normalized to normal childhood growth using the nasion-basion distance. Reproducibility studies were conducted to evaluate interrater reliability. Results: HNLM undergoing excision (n = 3) had a 92.3%–96.7% decrease malformation volume. HNLM having medical treatment with sirolimus and aspirin had a 7.3%–36.4% decrease in normalized volume, the majority of which was due to a decrease in cystic fluid content (reduced by 27.0%–36.4%). HNLM treated with sclerotherapy had no normalized volume change following treatment. One HNLM first treated with sirolimus had a 27.8% decrease in normalized volume and then combined normalized volume reduction of 75.6% after resection. Conclusion: This proof-of-concept use of longitudinal HNLM MRI in pediatric patients undergoing treatment demonstrates that objective information can be obtained through this method. This information can be used to determine treatment efficacy and optimize lesion specific treatment strategies.

EP282: Alpelisib for the treatment of PIK3CA-related head and neck lymphatic malformations and overgrowth

PIK3CA-related overgrowth spectrum (PROS) and large PIK3CA-associated lymphatic malformations of the head and neck (HNLM) pose unique treatment challenges. Traditional therapies often include multiple invasive (ie, sclerotherapy or surgery) interventions with suboptimal functional and cosmetic outcomes. HNLM is the most common pathology in the head and neck with PROS, and additional pathology may include facial infiltrating lipomatosis (FIL), megalencephaly capillary malformation syndrome (MCAP), Klippel-Trenaunay-Weber syndrome and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis and spinal syndrome (CLOVES).

Application of surgery combined with bleomycin irrigation for complex cervical-facial lymphatic malformations in children

Objective:To present experience and evaluate the safety and efficacy of surgery combined with bleomycin irrigation for the management of head and neck lymphatic malformations in children. Methods:The medical records of all patients with cervical-facial lymphatic malformations who presented to Shanghai Children's Hospital from August 2014 to December 2020 were reviewed. 97 children were divided into surgery group（81 cases） and sclerotherapy group（16 cases）. Conventional contrast-enhanced magnetic resonance imaging（MRI） and B-ultrasound examinations were performed both preoperatively and postoperatively. The surgical group received lymphangioma resection combined with bleomycin irrigation. The sclerotherapy group was treated with B-ultrasound-guided percutaneous lymphangioma aspiration and bleomycin sclerotherapy. SPSS 21.0 software was used to evaluate the clinical cure rate and postoperative complications of lesions in both groups by chi-square test. Kaplan-Meier method was used to calculate the disease-free survival rate and draw survival curve. Results:In the surgery group of 81 children, 64 cases were cured and 17 cases were effective while in the sclerosis group, 8 cases were cured and 8 cases were effective. Cox proportional risk model found that children in the surgery group had a higher cure rate and a lower risk of 5-year recurrence than those in the sclerotherapy group, with statistically significant differences（χ²=5.814, P<0.05）. The risk of recurrence in the surgery group was 35.4% of that in the sclerotherapy group（HR=0.354, P<0.05）. In regards to postoperative complications, the surgical group had no higher rate of temporal facial paralysis and other nerve injuries compared to the sclerotherapy group（χ²=1.041, P=0.308）. Conclusion:Surgery combined with bleomycin irrigation in the complex cervical-facial lymphatic malformations was confirmed to be effectively and safely. The principle of the surgery was to protect the structure and function of normal tissue while excising the lesions as much as possible. When the lesions involved the posterior two-thirds of the tongue, the floor of the mouth, parapharynx, retropharynx, or hypopharynx spaces. Radiofrequency ablation was used in the surgical excision, which made the surgery more minimally invasive, accurate and personalized.

A neonatal large neck lymphatic malformation successfully treated with Eppikajyutsuto

Currently, there are various treatment methods for neck lymphatic malformations (LMs). In recent years, there have been reports of usage of Eppikajyutsuto (TJ-28), a traditional Japanese Kampo medicine for LMs. We report the case of a patient who was delivered art 38 weeks and 5 days of gestation, with a birth weight of 3958 g. A right neck cyst was identified during the fetal period. Contrast-enhanced CT and cytology revealed a right large neck LM (14.0 × 11.7 × 6.0 cm) after birth by cesarean delivery. First, we considered sclerotherapy and mTOR inhibitors because of the necessity to reduce the tumor size however, it was thought that there might be complications associated with their usage. Hence, TJ-28 was administered at 39 days of age. A sufficient tumor shrinkage effect was observed 3 months after administration, and therefore, surgical resection was performed 8 months after administration. TJ-28 is considered to be a useful treatment with few side effects for large neck LMs when sclerotherapy or mTOR inhibitors are contraindicated in patients.

A Radiologic Grading System for Assessing the Radiographic Outcome of Treatment in Lymphatic and Lymphatic-Venous Malformations of the Head and Neck.

Two-thirds of lymphatic malformations in children are found in the head and neck. Although conventionally managed through surgical resection, percutaneous sclerotherapy has gained popularity. No reproducible grading system has been designed to compare sclerotherapy outcomes on the basis of radiologic findings. We propose an MR imaging-based grading scale to assess the response to sclerotherapy and present an evaluation of its interrater reliability. A grading system was developed to stratify treatment outcomes on the basis of interval changes observed on MR imaging. By means of this system, 56 consecutive cases from our institution with formally diagnosed head and neck lymphatic malformations treated by sclerotherapy were retrospectively graded. Each patient underwent pre- and posttreatment MR imaging. Each study was evaluated by 3 experienced neuroradiologists. Interrater reliability was assessed using the Krippendorff α statistic, intraclass coefficient, and 2-way Spearman ρ correlation. The overall Krippendorff α statistic was 0.93 (95% CI, 0.89-0.95), denoting excellent agreement among raters. Intraclass coefficients with respect to consistency and absolute agreements were both 0.97 (95% CI, 0.96-0.98), illustrating low variability. Every combination of individual rater pairs demonstrated statistically significant (P < .01) linear Spearman ρ correlations, with values ranging from 0.90 to 0.95. The proposed radiographic grading scale demonstrates excellent interrater reliability. Adoption of this new scale can standardize reported outcomes following sclerotherapy for head and neck lymphatic malformation and may aid in the investigation of future questions regarding optimal management of these lesions.

Head and Neck Lymphatic Malformation Management Algorithm: An Experience from a Tertiary Centre in Tanzania

Head and Neck Lymphatic Malformation Management Algorithm: An Experience from a Tertiary Centre in Tanzania