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Objective: The aim of this study is to share our experience in treating patients with lymphatic malformations (LMs) over a span of 14 years, evaluating the efficacy and safety of sclerotherapy, particularly with the use of ethanol as sclerosant of choice., Methods: A retrospective review of pediatric patients diagnosed and later treated for LMs between 2008 and 2022 was conducted. We collected patient demographics, LM characteristics, treatment strategies, and outcomes, including response to treatment and complications., Results: The cohort included 36 patients (24 male patients), first presenting clinically at a median age of 5 months (range: 0-12 years). LMs were macrocystic (17), microcystic (3), and mixed types (16). In most patients (22), the malformation involved the cervicofacial area. Twenty-five patients underwent 54 procedures, averaging 2 procedures per patient (range: 1-13). Sclerotherapy resulted in 90% of patients exhibiting some response of the LM (P = .005). Ethanol was used in most procedures (31) and proved most efficacious, facilitating partial or complete response of the malformations in all cases compared with 72% with other sclerosants (P = .06). Sclerotherapy exhibited low complication rates among all sclerosants used (7%, P = .74)., Conclusions: Sclerotherapy is a safe and effective intervention for pediatric LMs. Ethanol demonstrated comparable efficacy and safety to other sclerosants, highlighting its potential as a preferred treatment option. This study supports the tailored use of sclerotherapy, guided by a thorough understanding of the risks and benefits, to provide optimized care for patients with LMs., Competing Interests: Disclosures None., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objectives: Lymphatic malformations (LMs) are abnormal lymphatic vessels with cystic characteristics, categorized as macrocystic, microcystic, or a combination of both. They represent the second most common vascular malformations, and their management involves multidisciplinary approaches based on clinical assessments and imaging studies. LMs manifest as a challenge to medical professionals in the head and neck, posing functional and aesthetic concerns. Our systematic review aims to compare the efficacy of sclerotherapy and surgery for LMs, identifying optimal treatment modalities for each scenario., Methods: We searched four electronic databases for related studies. Data were extracted from the included studies. We calculated the pooled rate ratios with 95% confidence intervals (CIs). The I 2 test was used to detect heterogeneity. The inclusion of the studies required the following prerequisites: 1- Studies focusing on any lymphatic malformations in the head and neck, whether microcystic, macrocystic, or a mix of both; 2- Studies performed on more than ten patients; 3- All interventions used as surgery, sclerotherapy, or both., Results: We included 58 studies in our systematic review, of which 45 were eligible for the meta-analysis. For macrocystic LMs, sodium tetradecyl sulfate (STS) mixed with ethanol and excision achieved the highest complete response rates at (92.9%) and (92.5%), respectively. Surgical excision showed the lowest poor response rate. Polidocanol microfoam had the highest poor response rate (11.1%). In microcystic LMs, combining sclerotherapy with excision showed the highest complete response rate (70.3%) and the lowest poor response rate (1.3%). Picibanil had the lowest complete response rate (9.1%) and the highest rate of poor response (61.4%). In mixed LMs, surgical excision had the highest complete response rate (70.3%)., Conclusion: Both surgical excision and STS combined with ethanol are highly effective for treating macrocystic LMs, achieving similar complete response rates. The combination of sclerotherapy and surgical excision demonstrated the best outcomes in microcystic LMs. Surgical excision demonstrates superior efficacy over sclerotherapy for mixed LMs. These findings suggest that excision is generally more effective in achieving complete and excellent responses across all LM subtypes. Further high-quality studies are necessary to standardize and optimize treatment protocols., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To discuss the usefulness of ultrasonography (US) in the diagnosis and management of pediatric head and neck lymphatic malformations (HNLMs)., Methods: We conducted a retrospective analysis of 140 children who were referred to our hospital for the treatment of HNLMs., Results: The median age at presentation was 12 months (1 day-171 months; 66.4% under 2 years old; 35.7% neonatus). The majority clinical presentations were asymptomatic mass (65.7%, 92/140) and cosmetic deformity (25.7%, 36/140). HNLMs involved the neck accounting for 65.7% (92/140), especially posterior cervical trigone (22.1%, 31/140), and submandibular (20.0%, 28/140). The US diagnostic accuracy was 91.4% (128/140). Their boundary with the surrounding tissues was usually clear (87.9%, 123/140), whereas the shape was mostly irregular (97.1%, 136/140). Based on surgical findings, there were 67 pure HNLMs and 73 intracystic hemorrhage. Between the two groups, there were statistical differences in capsule contents (χ 2  = 7.8299, p = 0.0051), flocculent echo floating (χ 2  = 21.2964, p < 0.0001), overlying skin (χ 2  = 9.0498, p = 0.0026), and palpation (χ 2  = 13.4058, p = 0.0003)., Conclusions: US typically reveals the lesion with clear boundary, irregular morphology, anechoic contents, no blood flow signal, and echoic intracapsular septum with blood flow signal. In contrast, bluish appearance, tensional palpation, and capsule contents with low/mixed echo or flocculent echo floating may indicate intracystic hemorrhage., (© 2024 Wiley Periodicals LLC.)

Objective: To compare events of recurrent swelling between treated and untreated patients with macrocystic lymphatic malformations of the head and neck not involving the airway. The frequency and timing of emergency department (ED) visits related to the event were analysed to provide data on efficacy and ideal timing of treatment., Methods: A 5-year retrospective review of a hospital database was conducted reviewing 35 patients (15 female, 20 male; mean age 3.9 years) with macrocystic lymphatic malformations of the head and neck not involving the airway. Patients treated with oral medications were excluded. A survival analysis was performed comparing the incidence of recurrent swelling of the malformation. A Cox regression analysis was conducted using age, gender, diameter of lymphatic malformation at presentation, and echogenicity on US as covariates. Fisher's test and mean comparisons were performed to correlate the populations baselines and the number and frequency of ED visits between the 2 groups., Results: Thirteen patients underwent sclerotherapy soon after initial presentation and 22 elected for observation. The two baseline populations differed at presentation with the treatment group being younger (1.4 ± 2.4 vs. 5.4 ± 6.3 years, p = 0.03) and with larger lesions (5.7 ± 2.7 vs. 4.0 ± 1.7 cm p = 0.03). Mean follow-up time was 2.7 years. Survival analysis showed 1 or multiple recurrences affected 16 patients in the untreated group and 3 patients in the treated group. (p = 0.04). Age, gender, diameter of the lesion at presentation and increased echogenicity on US were not predictive factors of recurrence. Although the probability of visiting the ED at least once did not differ between the two groups (p = 0.42), patients from the non-treatment group were more likely to visit the ED more than once (p = 0.03)., Conclusions: Sclerotherapy treatment may reduce the chance of recurrent swelling or an event after initial presentation to the ED., (© 2024. The Author(s), under exclusive licence to American Society of Emergency Radiology (ASER).)

Aim: The aim of this study was to describe the technique of DCMRL to identify central lymphatic abnormalities in patients with primary lymphatic anomalies and discuss utility of the findings., Materials and Methods: Twenty-eight patients with primary lymphatic abnormalities underwent dynamic magnetic resonance imaging (MRI) following injection of gadolinium directly into inguinal lymph nodes at a tertiary lymphovascular referral center., Results: Technical success was achieved in 23 patients (82.1%). Pathological imaging findings included obstructed, hypoplastic, or absent lymphatic channels with collateralization/rerouting or reflux of flow, lymphangiectasia, lymphatic pseudoaneurysms, and lymph leaks. Protocol modifications for improved imaging are highlighted including technical aspects of lymph node injection, image acquisition and MRI parameters. In two patients, imaging findings warranted embolization of the abnormal lymphatic channels with subsequent symptomatic improvement., Conclusion: DCMRL has been shown to be a safe, reproducible technique in patients with primary lymphatic anomalies enabling imaging of the central lymphatic system., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Lymphatic malformations (LMs) are congenital abnormalities in the development of the lymphatic system. They can damage other structures including the airway, blood vessels, nerves, or bones. LMs of the face can result in malocclusion, and many patients with LMs of the face require orthognathic surgery to correct the malocclusion. Surgery-first is a treatment modality which should be considered for individuals with LMs and is a protocol in which orthognathic surgery is performed prior to orthodontic treatment. This case report describes surgery-first and subsequent orthodontic treatment in a patient with a class III malocclusion due to a large facial LM., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: Mediastinal cystic lymphatic malformation (MCLM) in children is prone to misdiagnosis as cystic teratoma. We compared the clinical and radiologic features between the two diseases and performed a cross-comparison with previous research on adult cases. This study aims to identify characteristic pediatric manifestations to improve diagnostic accuracy., Methods: A retrospective study of clinical and radiologic data was conducted on 12 MCLM and 20 cases of cystic teratomas confirmed by pathology or intervention biopsy. Clinical characters and radiology features (mass location and morphology, density, component, secondary complication) were recorded and compared. We reviewed clinical studies on MCLM published in the past decades, analyzing radiological differences and comparing pediatric MCLM cases at our hospital with those in the literature., Results: Group comparison in pediatrics between MCLM and cystic teratomas: There were significant age differences (p = 0.036), shape (p = 0.003), CT difference value (p < 0.001), CT difference ratio (p < 0.001), calcification (p < 0.001), fat (p < 0.001), and typing (p < 0.001) between the two diseases. An analysis of literature data on MCLM cases involved 16 studies., Conclusion: The absence of internal fat or irregular morphology, along with a minimal CT difference value, may suggest the diagnosis of MCLM. In pediatric cases, anterior mediastinal diseases tend to extend toward the neck, and the presence of the thymus can complicate the component analysis, thereby increasing the risk of misdiagnosis. Clinical diagnosis and differential diagnosis of pediatric MCLM rely heavily on imaging evaluation., (© 2024. The Author(s).)

Purpose: To systematically review current literature on the treatment of lymphatic malformations (LMs) of the head and neck to guide treatment strategy., Methods and Materials: A systematic review and meta-analysis of literature until 16 November 2021 was performed on treatments of LMs in the head and neck., Results: Out of 9044 articles, 54 studies were eligible for inclusion with 26 studies providing detailed participant data. A total number of 1573 patients with a mean age of 21.22 months were analysed. Comparative meta-analysis did not reveal significant differences two proportions of volume reduction (≥ 50% and 100%) between sclerotherapy and surgical treatment. Regression demonstrated that positive predictors for volume reduction were surgery 17 (95% CI 0.26-34; p = 0.047) and treatment of macrocystic lesions 19 (95% CI 5.5-32; p = 0.006). Treatment of mixed lesions also demonstrated a trend towards achieving a greater volume reduction (p = 0.052). A higher de Serres stage of the lesion had a negative effect on the amount of volume reduction - 3.7 (95% CI - 7.0 to - 0.35; p = 0.030)., Conclusion: This comprehensive meta-analysis demonstrated no significant difference in volume reduction between various treatment modalities at study level. However, individual patient data indicated that surgery and larger cyst types are associated with a significant higher percentage of volume reduction, whereas a higher de Serres stage negatively impacted the amount of volume reduction. These findings can be used for patient counseling and treatment planning based on cyst type and de Serres stage. However volume reduction constitutes just one objective within a more complex treatment spectrum., (© 2024. The Author(s).)

Objective: Evaluate pregnancy and neonatal outcomes with fetal cystic lymphatic malformations (LMs), excluding those arising from the posterior neck, to facilitate patient counseling., Method: A systematic review was performed in accordance with PRISMA guidance. Case series and case reports published between 2000 and 2022 were included., Results: Sixty-five studies (96 fetuses) met the inclusion criteria. The average gestational age at diagnosis was 25.5 weeks with the commonest location being the anterior neck (28%). All patients were diagnosed with LM using two-dimensional (2D) ultrasound. Prenatal progression in LM size, presence of intralesional bleeding, or fetal hydrops occurred in 70% (41/59), 9% (5/59), and 3% (2/59), respectively. Chromosomal and structural abnormalities were reported in 4% (2/52) and 2% (2/96), respectively. Overall livebirth rate was 94% (79/84); 12/96 resulted in termination and 5/84 in in utero demise. The average gestational age of delivery was 37.7 weeks. Exactly 19% (15/79) had a vaginal birth, of which shoulder dystocia occurred in one infant. Ex utero intrapartum treatment (EXIT) procedure was performed in 13% (10/79). Postnatal treatment commonly involved surgical excision 38% (30/79), sclerotherapy in 21.5% (17/79), or combination of both in 11.4% (9/79). Of those with reported follow-up, 4 died within 1 year, 1 developed heart failure at 2 years of life, and the remaining 44 had normal developmental outcomes., Conclusion: Fetal cystic LMs, excluding those in the posterior neck, are not commonly associated with chromosomal, or additional structural abnormalities. They usually increase in size before delivery with only a minority developing complications. The good developmental outcome was reported in all survivors., (© 2024 American Institute of Ultrasound in Medicine.)

Intraorbital lymphatic-venous malformations are rare lesions that represent a therapeutic challenge given their location and high rate of recurrence, with only a few cases in adult patients having been published in the literature. We present the case of a 30-year-old male with a right intraorbital lymphatic-venous malformation treated with sirolimus at a dose of 4 mg/day with complete clinical and radiologic remission. Mild cold-like symptoms ensued during the first week of treatment and elevation of liver function enzymes and D-dimer occurred in the context of acute SARS-CoV-2 pneumonia. No major adverse effects were documented. After 18 months of treatment, the patient remains asymptomatic and ophthalmologic examinations including optical coherence tomography and visual field test are within normal values.

Objectives: 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)., Methods: We performed a retrospective review of 170 children who were referred to our hospital in the past 9 years for the treatment of HNLMs., Results: 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 (χ 2  = 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 (χ 2  = 25.4152, p < 0.05)., Conclusions: 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., (© 2024 Wiley Periodicals LLC.)

We report on two neonates born the same day, both with an isolated cervical lymphatic malformation. Cervical masses were detected by ultrasound late in the third trimester. Following interdisciplinary case conferences, a caesarean section in the presence of a neonatal team was the chosen delivery mode in both cases. Delivery and transition of the newborns were uneventful. The suspected diagnosis was confirmed by postnatal MRIs, which demonstrated neither associated malformations nor compression of vital structures. Therefore, an expectant approach was chosen for the newborn with the smaller lesion. The other newborn featured a sizeable lymphatic malformation, and due to consecutive head tilt, sclerotherapy was initiated in its second week of life. Our case report outlines the challenges of a rare connatal malformation. Guidelines are often missing. Individual decisions regarding delivery mode, diagnostics and therapy have to be made on an interdisciplinary basis and patients as well as parents need counseling and support over a long period. All the more significant is good, interdisciplinary collaboration between the involved disciplines., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible. Lymphatic abnormalities were classified into four types based on severity and location of lymphatic vessels. Classifications were compared between images and related to clinical outcomes such as postoperative drainage and hospitalization, lymphatic complications, heart transplant, and death. Results Forty-three patients (median age, 10 years [IQR, 8-11]; 20 [47%] boys, 23 [53%] girls) were included in the study. Lymphatic abnormalities progressed in 19 individuals after Fontan completion (distribution of lymphatic classifications: type 1, 23; type 2, 11; type 3, 6; type 4, 3 vs type 1, 10; type 2, 18; type 3, 10; type 4, 5; P = .04). Compared with individuals showing no progression of lymphatic abnormalities, those progressing to a high-grade lymphatic classification had longer postoperative drainage (median time, 9 days [IQR, 6-14] vs 17 days [IQR, 10-23]; P = .04) and hospitalization (median time, 13 days [IQR, 9-25] vs 26 days [IQR, 18-30]; P = .03) after Fontan completion and were more likely to develop chylothorax (12% [three of 24] vs 75% [six of eight]; P < .01) and/or protein-losing enteropathy (0% [0 of 24] vs 38% [three of eight]; P < .01) during a median follow-up of 8 years (IQR, 5-9). Progression to any type was not associated with an increased risk of adverse events. Conclusion The study demonstrated that lymphatic structural abnormalities may progress in select individuals with single ventricle physiology after Fontan completion, and progression of abnormalities to a high-grade classification was associated with worse postoperative outcomes. Keywords: Congenital Heart Disease, Glenn, Fontan, Lymphatic Imaging, Cardiovascular MRI Supplemental material is available for this article. Published under a CC BY 4.0 license.

Objective: This study is a systematic review and meta-analysis of the efficacy and safety of sirolimus in the management of pediatric cervicofacial lymphatic malformations (LMs)., Data Sources: EMBASE, Medline, Scopus, and Cochrane databases were searched, along with the reference list of all included articles., Review Methods: The study protocol was registered with PROSPERO and a systematic literature search strategy was designed and conducted with the aid of a medical librarian. All studies including case reports were included, with pooled analysis of raw data. A meta-analysis was conducted of magnetic resonance imaging (MRI), clinical, and airway outcomes., Results: Thirteen case series and five individual case reports were included. Meta-analysis showed 78% (95% CI 57%-94%) of 62 patients had a reduction in LM volume, on MRI criteria, by 20% or more, and 32% (95% CI 11%-57%) had a reduction of 50% or more. Further meta-analysis showed 97% (95% CI 88%-100%) of 78 patients reported some clinical improvement on sirolimus. Sirolimus may be of particular value in management of airway LMs; out of 27 tracheostomy-dependent patients, meta-analysis showed 33% (95% CI 1%-78%) were decannulated after starting sirolimus. Individual patient meta-analysis on 24 individuals showed a statistically significant better response to sirolimus when initiated under the age of 2 years., Conclusion: This review and meta-analysis support the efficacy of sirolimus in pediatric LMs of the head, neck, and airway. A large multi-center trial is needed to further explore its role and limitations. Laryngoscope, 134:2038-2047, 2024., (© 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: The first-line treatment of lymphatic malformations (LMs) is pharmacological or interventional; however, surgery is still necessary in selected cases. Our aim was to identify factors associated with the occurrence of permanent postoperative complications., Methods: This was a case series study of children operated on for LMs between 2001 and 2021 and followed-up in our institution. Patients who presented sequelae derived from surgical treatment (cases) and those who did not (controls) were compared., Results: We included 112 children who underwent surgery for LMs in different centers. Forty-nine cases and 63 controls were included (58% male), with a mean age of 34 months. Patients younger than 1 year presented more complications than older children, 59% (n = 29/49) vs 41% (n = 24/49), respectively (P = .02). LMs were in the cervicofacial region in seven patients in the control group compared with 30 of the cases (P ≤ .001), with microcystic malformations the most associated with sequelae (n = 11/15; P = .019). Concerning permanent complications, 88% were neurological (n = 43/49), mainly peripheral facial palsy (n = 17). There was greater postoperative residual disease in controls compared with cases (65% vs 14%, respectively; P ≤ .0001). However, following a second procedure in the control group, there was no significant difference in long-term cure rates (P = .38)., Conclusions: The risk of sequelae following surgery for LM increases significantly in patients younger than 12 months in cervicofacial and microcystic malformations. Because non-radical resections are associated with fewer complications and an optimal long-term cure rate, we consider that aggressive surgical approaches should be avoided if the absence of sequelae is not guaranteed., Competing Interests: Disclosures None., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Percutaneous sclerotherapy is an effective treatment for lymphatic malformations (LM) of the head and neck in adults. The purpose of this study was to examine the indications and efficacy of sclerotherapy for head/neck LM in the neonate and infant population., Methods: We retrospectively reviewed patients treated with percutaneous sclerotherapy for LM of the head/neck at age ≤12 months at a single vascular anomalies clinic. The clinical, anatomic, and technical aspects of each treatment, complications, and post-treatment clinical and imaging outcomes were analyzed., Results: 22 patients underwent 36 treatments during the first year of life. Median age at first treatment was 6.2 months (range 2-320 days). Severe airway compromise was the most frequent indication for treatment (31.8%). Sclerosants included doxycycline (80.5%), sodium tetradecyl sulfate (55.5%), bleomycin (11.1%) and ethanol (2.8%). There were no immediate procedure-related complications; sclerosant-related laboratory complications included transient metabolic acidosis (8.3%) and hemolytic anemia (5.5%). Median follow-up was 3.7 years (IQR 0.6-4.8). 47.6% of patients showed >75% lesion size reduction and 19.0% showed minimal response (<25% improvement). At last follow-up, 71.4% of children were developmentally normal and asymptomatic, 23.8% had recurring symptoms, and 4.8% required permanent tracheostomy. Patients with ongoing symptoms or limited response to percutaneous sclerotherapy (33.3%) were treated with long-term sirolimus., Conclusions: Percutaneous sclerotherapy is a safe and effective treatment for symptomatic LM of the head and neck in neonates and infants. Treatment strategy and management of recurrent symptoms requires consensus from an experienced, multidisciplinary team., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Percutaneous sclerotherapy is an effective technique for treating lymphatic malformations of the head and neck, with clinical success rates exceeding 84%.1 Sodium tetradecyl, which damages lipid membranes and stimulates free radical-induced local damage, and doxycycline, which inhibits angiogenesis, have emerged as the safest and most effective of several available sclerosants.2-4 Although severe periprocedural morbidity is rare, temporary local complications are reported in 14% and skin necrosis or scarring in up to 0.8-5.8% of sclerotherapy procedures.5 As these lesions are frequently located in the face and/or neck, even minor complications can be disfiguring and must be avoided. This technical video describes a 'dual-agent' approach for percutaneous sclerotherapy of macrocystic lymphatic malformations using sodium tetradecyl as a 'primer' followed by doxycycline as a definitive sclerosant (video 1). This technique emphasizes meticulous backtable preparation and effective use of ultrasound and fluoroscopy to minimize complications. neurintsurg;15/9/931/V1F1V1Video 1 ., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis., (Copyright © 2022 SERAM. Published by Elsevier España, S.L.U. All rights reserved.)

Objective: To review the clinical characteristics and treatment outcomes of head and neck lymphatic malformations (HNLMs) in children., Methods: A retrospective study of 91 patients with HNLMs was performed., Results: The 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., Conclusions: HNLMs 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., Competing Interests: Conflict of interest None of the authors has any conflict of interest or otherwise., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background In the palliative pathway of single-ventricle physiology, lymphatic abnormalities on T2-weighted magnetic resonance imaging have been shown after the Glenn operation. It is believed that postsurgical hemodynamic changes contribute to the lymphatic changes.However, little is known about how early these abnormalities occur. Our purpose was to determine if lymphatic abnormalities occur as early as before the Glenn operation. Methods and Results We retrospectively reviewed patients with single-ventricle physiology and a T2-weighted magnetic resonance imaging scan before their Glenn operation (superior cavopulmonary connection) at The Children's Hospital of Philadelphia from 2012 to 2022. Lymphatic perfusion patterns on T2-magnetic resonance imaging were categorized from type 1 (no supraclavicular T2-signal) to type 4 (supraclavicular, mediastinal, lung parenchymal T2-signal). Types 1 and 2 were considered normal variants. Distribution of lymphatic abnormalities were tabulated, as well as secondary outcomes including chylothorax and mortality. Comparison was done using analysis of variance, Kruskal-Wallis test, and Fisher's exact test. Seventy-one children were included: 30 with hypoplastic left heart syndrome and 41 with nonhypoplastic left heart syndrome. Lymphatic abnormalities were present before Glenn operation in 21% (type 3) and 20% (type 4), and normal lymphatic perfusion patterns (type 1-2) were seen in 59% of patients. Chylothorax was present in 17% (only types 3 and 4). Pre-Glenn mortality and mortality at any time was significantly increased when having a type 4 lymphatic abnormality compared with types 1 and 2 ( P =0.04). Conclusions Lymphatic abnormalities can be found on T2-weighted magnetic resonance imaging in children with single-ventricle physiology before their Glenn operation. Mortality and chylothorax were more prevalent with advancing grade of lymphatic abnormality.

Background: Generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), kaposiform lymphangiomatosis (KLA), and central conducting lymphatic anomaly (CCLA) are rare, multisystem lymphatic disorders, referred to as complex lymphatic anomalies (CLAs). Their etiology remains poorly understood; however, somatic activating mutations have recently been discovered, and the results of targeted treatments are promising. This study aimed to elaborate on the phenotypic description of CLA. Methods: Thirty-six consecutive patients were recruited for the "GLA/GSD Registry" of the University Hospital of Freiburg, Germany (2015-2021). Clinical data were prospectively collected provided that a signed informed consent form was obtained. The latest proposed diagnostic guidelines were retrospectively applied. Results: Thirty-two patients (38% males) were included in the study; 15 GLA, 10 GSD, 3 KLA, and 4 CCLA patients were identified. Eighty-four percent already had symptoms by the age of 15 years. Osteolysis and periosseous soft-tissue infiltration were associated with GSD ( p  < 0.001 and p  = 0.011, respectively), ascites and protein-losing enteropathy with CCLA ( p  = 0.007 and p  = 0.004, respectively), and consumption coagulopathy with KLA ( p  = 0.006). No statistically significant differences were found in organ involvement, distribution of osteolytic lesions, number of affected bones and fractures. Twenty-five patients had complications; one patient with GLA died despite multimodal treatment. Spontaneous regression was seen in one patient with untreated KLA. Conclusions: CLA are rare, and their overlapping clinical presentations make differential diagnosis difficult. The characterization of our case series contributes to the phenotypic description and differentiation of these four clinical entities. A further understanding of their pathogenesis is crucial for evaluating targeted therapies and optimizing medical care.

Objective: Lymphatic malformations in the submandibular neck pose unique challenges to treatment that elevate their risk of recurrence. This case series provides a review of five patients, previously treated with sclerotherapy or with a history of multiple infections, who were treated in a novel fashion: single-stage resection using preoperative n-butyl cyanoacrylate (n-BCA) glue embolization., Methods: We performed a retrospective medical record review of five patients who underwent single-stage n-BCA embolization by Interventional Radiology followed by surgical resection by Otolaryngology, including a review of their symptoms, previous treatments, and post-treatment surveillance, with follow-up ranging from 4 to 24 months after the treatment of interest., Results: All study subjects had unremarkable perioperative courses, and four patients did not demonstrate any evidence of disease recurrence or persistence during the follow-up period. One patient was found to have a small area of persistent disease on post-treatment imaging, but has remained symptom free., Conclusions: Treatment of submandibular lymphatic malformations with n-BCA embolization followed by surgical resection can be performed in a single stage. This case series demonstrates that this approach can yield durable relief of symptoms, even in patients whose lesions were refractory to previous treatments., Competing Interests: Declaration of competing interest The authors report no conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: In the last few decades there has been development of innovative therapies for the treatment of craniofacial lymphatic malformations. Percutaneous sclerotherapy has demonstrated efficacy in the management of macrocystic lymphatic lesions, but it is less suitable for microcystic lesions given their size. The gravity-dependent technique is a novel augmentation of standard percutaneous sclerotherapy: the technique enables a sclerosing agent to permeate the small microchannels seen in microcystic lesions that would otherwise be difficult to treat., Methods: Between 2005 and 2021, 124 patients with microcystic or mixed lymphatic malformations were treated using a novel gravity-dependent sclerotherapy technique. Bleomycin at a maximum dose of 15 IU per session was used as the main sclerosing agent. Fluoroscopy and ultrasound were used to ensure proper positioning of the catheter prior to injecting the sclerosing agent. The response to treatment was assessed clinically and with cross-sectional imaging., Results: Nearly all cases showed significant improvement after the gravity-dependent technique. There have been no permanent complications from sclerotherapy treatment. There was a mild transient adverse effect from bleomycin in one case of erythema and tenderness that lasted several weeks then ameliorated., Conclusions: The gravity-dependent sclerotherapy technique is a suitable treatment option for microcystic lymphatic malformations., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate the safety and efficacy of percutaneous doxycycline sclerotherapy of head and neck lymphatic malformations (LM) with a 40-month follow-up of 27 cases., Materials and Methods: Twenty-seven consecutive patients with head and neck LM who underwent doxycycline sclerotherapy from 2010 to 2019 were retrospectively reviewed. Pre- and peri-interventional data collection included patients' demographics, clinical and radiological presentation, number of treatment procedures, amount of doxycycline used for each session of sclerotherapy. Postinterventional clinical outcome data were assessed based on the electronic, medical reports with special attention to clinical improvement, measurement of the lesion on ultrasound and magnetic resonance imaging after the last session of sclerotherapy and complications of sclerotherapy., Results: Twenty-seven patients underwent a mean of four doxycycline sclerotherapy sessions (range 1-23). The mean total dose of injected doxycycline per session was 170 mg. Maximal diameter of the LM decreased from mean 59.7 mm (median 58 mm; 25-130 mm) before treatment to mean of 29.6 mm (median 30 mm; 0 mm 64 mm) after the last session (P < 0.0001). Over a mean follow-up period of 40 months, complete or incomplete disappearance of symptoms was found in 23 (85%) and 3 (11%) of patients, respectively. In one patient, sclerotherapy did not result in better clinical outcome. Two out of 27 patients presented with minor post-procedural complications, and one patient with a post-procedural intralesional bleeding considered as a major complication., Conclusion: Ultrasound-guided, percutaneous doxycycline sclerotherapy is a safe and effective method of managing symptomatic LM of the head and neck., (© 2022. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

Lymphatic malformations are rare benign developmental anomalies of the lymphatic system that can be diagnosed by prenatal ultrasound. Depending on their anatomical site and size, the lesions can cause a variety of aesthetic and functional deficits. Several treatment options are available, the most suitable is still under debate. The experience gained at our Centre and the review of the literature can be useful to improve prenatal counseling, that is challenging due to the heterogeneity of clinical presentation and treatment.

Objective: To investigate the clinicopathological features, clinical manifestations and different diagnosis of patients with complicated lymphatic anomaly. Methods: The clinical and pathologic data of four patients with complicated lymphatic anomaly diagnosed and treated in Peking Union Medical College Hospital from January 2000 to December 2021 were collected and analyzed. Results: One Gorham-Stout disease case and three generalized lymphatic anomaly cases were included in this cohort. Patients' ages ranged from 7 to 32 years. There were three males and one female. The positions of biopsy included three bone biopsy and one bronchus biopsy. Microscopically, all cases showed diffuse enlarged lymphatic channels. At the same time, osteogenesis was obvious in Gorham-Stout disease case. Radiologically, cortical loss was seen in Gorham-Stout disease, and lytic bone confined to the medullary cavity presented in generalized lymphatic anomaly. The three generalized lymphatic anomaly cases also had coagulopathy, and two had effusion. Conclusions: The histologic feature of complicated lymphatic anomaly was diffuse lymphatic malformation, and the diagnosis depends on clinical and pathologic information. The treatment and prognosis of these diseases are different, and therefore it is necessary to understand their clinical and pathologic features and make the correct diagnosis.

Background: Sclerotherapy is the first-line therapeutic method for lymphatic malformations (LMs). This retrospective cohort study evaluated the effectiveness and safety of a novel combined foam sclerosant: polidocanol and pingyangmycin foam (PPF), for treating cervicofacial macrocystic LMs. Methods and Results: From July 2018 to October 2020, 51 patients with cervicofacial macrocystic LMs were enrolled in this study. All patients received intralesional 3% polidocanol or PPF injections. The outcome was evaluated regarding demographic and clinical characteristics, degree of treatment response, and post-treatment complications. Overall, 16 patients (31.4%) underwent PPF sclerotherapy. All these patients (100%) showed remarkable reduction in lesion size within three sessions. Excellent responses were shown in 88.5% of these patients within three sessions, which is higher than single polidocanol sclerotherapy (80%). The average sessions (duration) of PPF sclerotherapy were 2.5, which was significantly shorter than the single foam sclerotherapy ( p  < 0.05). Treatment duration was significantly associated with age, lesion location, lesion size, and number of cysts ( p  < 0.05). No severe complications were noted in this study. Local or systemic complications, such as swelling and mild fever occurred but subsided without any specific treatment. Conclusions: PPF is a safe, and effective combined foam sclerosant for the treatment of cervicofacial macrocystic LMs. This combined foam can improve treatment response and reduce treatment duration compared with a single sclerosant. It can be broadly used if further large-scale clinical trials verify its efficacy and safety.

Congenital heart disease can lead to various lymphatic complications including traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema. Advancements in the imaging of central lymphatics and guided interventions have improved outcomes in these children. Dynamic contrast-enhanced magnetic resonance (MR) lymphangiography allows for the assessment of abnormal lymphatic drainage. This technique is preferred for evaluating lymphatic conditions such as plastic bronchitis, chylothorax, chyloptysis, chylopericardium, protein-losing enteropathy and chylous ascites, among other lymphatic disorders. In this review, we discuss lymphatic abnormalities encountered on MRI in children with congenital heart disease. We also briefly review treatment options., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To evaluate the safety and efficacy of bleomycin infusion sclerotherapy using a syringe pump in microcystic and mixed (microcystic components with the presence of a cyst over 1 cm) lymphatic malformations (LMs)., Materials and Methods: Patients who received bleomycin sclerotherapy with a syringe pump for microcystic or mixed LMs were reviewed. Cystic components of LMs were accessed under sonographic guidance, followed by injection of an opacified bleomycin solution using a syringe pump (infusion rate, 10-20 mL/h) under fluoroscopic guidance. Imaging outcomes were graded as complete (> 90% size reduction), partial (25-90%), or no response (< 25%). Clinical outcomes and procedure-related complications were also reviewed., Results: Forty-nine patients with 81 sclerotherapies were analyzed. The mean age was 17 years (range 0.1-65 y). Thirty-one (63%) patients had microcystic LMs, and 18 (37%) had mixed. A mean of 1.7 sessions (range 1-4) of sclerotherapy was performed using a mean cumulative dose of bleomycin of 10.8 U (range 1.5-39 U). The mean infusion time was 39 min (range 14-130 min). Regarding imaging outcomes, there was a complete response in 29% (n = 14), a partial response in 57% (n = 28), and no response in 14% (n = 7). Regarding clinical outcomes, there was a complete response in 39% (n = 19), a partial response in 51% (n = 25), and no response in 10% (n = 5). According to the CIRSE classification, no major complications were identified., Conclusions: Bleomycin slow infusion sclerotherapy provides gradual filling of sclerosant to target microcystic components. This technique is safe and feasible for the management of microcystic or mixed LMs., Level of Evidence: Level 4, Case series., (© 2022. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

Mesenteric lymphatic malformations result from abnormal proliferation of disorganized mesenteric lymphatic channels. Sclerotherapy is often preferred over surgery as it is less invasive and has lower post-procedure morbidity. Sclerotherapy has been described as durable and effective with a low complication rate. We describe a serious complication from sclerotherapy of a lymphatic malformation extending from the mesentery through the bowel wall, highlighting the spectrum of this pathology and the need for multidisciplinary management of complex cases., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Noonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with Noonan syndrome to further elucidate this complex disease spectrum. A retrospective evaluation of patients with confirmed Noonan syndrome and clinical signs of lymphatic dysfunction undergoing DCMRL between 01/2019 and 04/2021 was performed. MRL included T2-weighted imaging (T2w) and DCMRL. Clinical history/presentation and genetic variants were recorded. T2w-imaging was evaluated for central lymphatic abnormalities and edema distribution. DCMRL was evaluated regarding the presence of cisterna chyli/thoracic duct, lymphatic leakages, pathological lymphatic reflux and abnormal lymphatic perfusion. The time from start of contrast-injection to initial enhancement of the thoracic duct venous junction was measured to calculate the speed of contrast propagation. Eleven patients with Noonan syndrome with lymphatic abnormalities (5 female, 6 male; 7 infants, 4 adults; mean age 10.8 ± 16.4 years) were identified (PTPN11 n = 5/11 [45.5%], RIT1 n = 5/11 [45.5%], KRAS n = 1/11 [9%]). Patients had a chylothorax (n = 10/11 [91%]) and/or pulmonary lymphangiectasia [dilated pulmonary lymph vessels] (n = 9/11 [82%]). Mediastinal/pulmonary edema was depicted in 9/11 (82%) patients. The thoracic duct (TD) was (partially) absent in 10/11 (91%) cases. DCMRL showed lymphatic reflux into intercostal (n = 11/11 [100%]), mediastinal (n = 9/11 [82%]), peribronchial (n = 8/11 [73%]), peripheral (n = 5/11 [45.5%]) and genital lymphatics (n = 4/11 [36%]). Abnormal pulmonary/pleural lymphatic perfusion was seen in 8/11 patients (73%). At infancy peripheral/genital edema was more prevalent in patients with RIT1 than PTPN11 (n = 3/5 vs. n = 0/5). Compared to patients with PTPN11 who had fast lymphatic enhancement in 4/5 patients, enhancement took markedly longer in 4/5 patients with RIT1-mutations. Thoracic duct dysplasia, intercostal reflux and pulmonary/pleural lymphatic perfusion are characteristic findings in patients with Noonan syndrome presenting with chylothorax and/or pulmonary lymphangiectasia. Central lymphatic flow abnormalities show possible phenotypical differences between PTPN11 and RIT1-mutations., (© 2022. The Author(s).)

Competing Interests: Competing interests: None declared.

Purpose: The purpose of our study was to evaluate the efficacy and safety of ultrasound-guided iodine tincture cauterization combined with postoperative intralesional negative pressure in the management of cervicofacial cystic lymphatic malformation (cLM)., Method: From January 2019 to July 2021, indocyanine green lymphography was performed preoperatively to confirm the lymph inflow, and this treatment was administered in 71 patients with cervicofacial cLM in our center. All cases were evaluated by curative effects, treatment frequency, and adverse events. The duration of posttreatment follow-up was from 12 to 14 months., Results: Indocyanine green lymphography indicated at least one lymphatic inflow in each cLM lesion. Excellent resolution was observed in 87.3% of cases, and good improvement of the treated cLM occurred in 9.9% of cases, and 2 cases with fair outcomes required subsequent treatment. It is noteworthy that no case was treated more than 3 times. Some minor adverse effects, including localized itch and scar, were managed by symptomatic treatment., Conclusions: Because of satisfactory outcomes and low treatment frequency, ultrasound-guided iodine tincture cauterization combined with intralesional negative pressure represents an efficacious, safe, and feasible method for the management of macro-cLM in the cervicofacial region., Competing Interests: Conflicts of interest and sources of funding: This study was supported by Nanjing Medical Science and Technology Development Project (grant no. YKK20134). None of the authors have any financial arrangements or potential conflicts of interest related to this article., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Lymphatic anomalies comprise a spectrum of disorders ranging from common localized microcystic and macrocystic lymphatic malformations (LMs) to rare complex lymphatic anomalies, including generalized lymphatic anomaly, Kaposiform lymph-angiomatosis, central conducting lymphatic anomaly, and Gorham-Stout disease. Imaging diagnosis of cystic LMs is generally straightforward, but complex lymphatic anomalies, particularly those with multiorgan involvement or diffuse disease, may be more challenging to diagnose. Complex lymphatic anomalies are rare but associated with high morbidity. Imaging plays an important role in their diagnosis, and radiologists may be the first clinicians to suggest the diagnosis. Furthermore, radiologists are regularly involved in management given the frequent need for image-guided interventions. For these reasons, it is crucial for radiologists to be familiar with the spectrum of entities comprising complex lymphatic anomalies and their typical imaging findings. In this article, we review the imaging findings of lymphatic anomalies, including LMs and complex lymphatic anomalies. We discuss characteristic imaging findings, multimodality imaging techniques used for evaluation, pearls and pitfalls in diagnosis, and potential complications. We also review recently discovered genetic changes underlying lymphatic anomaly development and the advent of new molecularly targeted therapies.

Background: Complex lymphatic anomalies (CLA) are a group of conditions that pose diagnostic and therapeutic challenges due to their rarity and overlapping clinical findings. This case series describes the complex pathology and novel combination therapies of three patients diagnosed with various types of CLA. Methods and Results: A retrospective review of medical records was performed for three patients treated for CLA between 2011 and 2019. Diagnostics, imaging, treatment, and follow-up were reviewed in the electronic medical record and combined with the literature review within the analysis. One patient had involvement of her skull base and ear canals, diagnosed after ear canal abnormalities were detected on computed tomography following meningitis. The second patient had involvement of her posterior ribs and T7-T12 vertebral bodies, with thoracic instability requiring a back brace. The third patient had involvement of his left lower extremity and hemipelvis, necessitating a left above the knee amputation. Case 1 progressed on sirolimus and pamidronate but responded to zoledronic acid (ZA). She developed flares of coagulopathy and cellulitis that required reinforcement with vincristine and steroid pulses. Similarly, case 2 progressed on sirolimus and ZA alone, but achieved stable disease with added vincristine. Upon further disease progression, stabilization was obtained by the reinforcement of ZA. Case 3 required a combination of surgery as well as medical management with sirolimus and pamidronate. All three patients now have stable disease. Conclusion: This case series depicts a multidisciplinary and multiagent approach to the management of CLA with severe bony involvement using sirolimus, bisphosphonates, vincristine, and steroids.

Background: Mesenteric lymphatic malformations (LMs) represent rare congenital anomalies that can include chylous or nonchylous content. The pathologic mechanisms explaining this phenomenon are poorly understood and not yet described. Furthermore, the current management approach does not consider the contents of the mesenteric LMs. In the present study, we have defined the relationship between the lymphatic mesenteric cyst content and the histologic evidence of LMs within the bowel wall., Methods: We retrospectively investigated all patients with mesenteric LMs treated surgically at our department from 1999 to 2018., Results: A total of 11 patients (6 girls and 5 boys) were included in our analysis. Seven patients had presented with LMs located in the jejunal mesentery, three in the ileocecal region, and only one in the mesocolon transversum and omentum. Of the 11 children, 7 had had LMs with nonchylous content and 4 had presented with chylous content LMs. Intestinal resection was performed in all 4 patients with chylous content LMs and 4 patients with nonchylous content LMs. Histopathologic evaluation of the surgical specimens determined that only the LMs with chylous content displayed malformed lymphatic channels throughout the bowel wall. The resected small bowel of four patients with nonchylous content showed no LM extension throughout the intestinal wall., Conclusions: LMs with chylous content seem to develop from malformed lymphatic channels within the bowel wall. In such cases, segmental intestinal resection is mandatory. In contrast, mesenteric LMs with nonchylous content can potentially be treated without bowel resection if the blood supply can be preserved. This finding is, to the best of our knowledge, reported in the present study for the first time., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background Dynamic contrast-enhanced MR lymphangiography (DCMRL) is the reference standard used to diagnose various thoracic lymphatic disorders, such as traumatic chylothorax and plastic bronchitis. However, accessibility and logistical challenges have prevented the wide dissemination of this technology. Purpose To evaluate the feasibility of intranodal CT lymphangiography (ICTL) in the diagnosis and planning of subsequent intervention in patients with thoracic lymphatic disorders. Materials and Methods In this retrospective review, five women suspected of having lymphatic abnormalities (ranging from traumatic chylothorax to plastic bronchitis) and with contraindications to MRI underwent ICTL from September 2019 to May 2020. Needles (25 gauge) were placed in the bilateral inguinal lymph nodes with US guidance, and water-soluble iodinated contrast material was injected. CT fluoroscopy was used to monitor the opacification of the cisterna chyli to determine the timing of CT. After ICTL, the thoracic duct was catheterized, and lymphangiography was performed through the thoracic duct catheter. The ICTL and subsequent lymphangiographic findings were then visually compared by using three-dimensional reconstructions. Results Intranodal injection of water-soluble contrast medium was successful in all patients evaluated (five women; mean age, 68 years ± 11 [standard deviation]; range, 53-83 years). The central lymphatics were opacified in four of the five women, demonstrating abnormal pulmonary lymphatic flow from the thoracic duct into the lung parenchyma. In one of the five women, thoracic duct injection showed successful ligation of the thoracic duct. The time elapsed from injection of contrast medium to visualization of the thoracic duct ranged from 2 to 27 minutes. ICTL and lymphangiographic findings matched well. Conclusion Intranodal CT lymphangiography sufficiently depicted central lymphatic anatomy in patients with lymphatic abnormalities, thereby demonstrating its use as a feasible alternative to more technically challenging methods, such as dynamic contrast-enhanced MR lymphangiography. © RSNA, 2021.