Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

Treatment of orbital lymphatic malformation with oral sirolimus: a case report / Malformação linfática orbital tratada com sirolimo oral: relato de caso

ABSTRACT It is estimated that lymphatic malformations in children account for 6% of all benign vascular malformations. New medical therapies have been developed for the management of lymphatic orbital disease. The purpose of this article was to describe a clinical case of orbital venolymphatic malformation in a 10-year-old boy, causing proptosis and palpebral edema. The lesion was initially treated with local sclerotherapy. However, the lesion relapsed, and was successfully treated with oral sirolimus. Prospective studies are warranted to determine the appropriate dose and extend the indications of sirolimus in these patients.

RESUMO A incidência de malformações linfáticas em crianças é estimada em 6% de todas as malformações vasculares benignas. Têm sido desenvolvidos novos tratamento para doenças linfáticas orbitárias. Nosso objetivo é descrever um caso clínico de malformação venolinfática orbitária em um menino de 10 anos de idade, causando proptose e edema palpebral. A lesão foi tratada inicialmente com escleroterapia local. No entanto, a lesão teve recidiva e foi tratada com sucesso com sirolimo oral. Ainda são necessários estudos prospectivos para estabelecer a dose apropriada e a duração do tratamento com sirolimo nesses pacientes.

Clinical efficacy and safety of topical sirolimus in the treatment of 19 cases of superficial vascular malformation in children / 中华皮肤科杂志

Objective:To investigate efficacy and safety of topical sirolimus cream in the treatment of superficial vascular malformation in children.Methods:A single-center prospective study was carried out. Children with superficial vascular malformation were enrolled into this study from Vascular Anomalies Clinic, Beijing Children′s Hospital, Capital Medical University from September 2019 to September 2020, and treated with 0.1% sirolimus cream. The efficacy was evaluated according to an international four-level classification system through imaging examination, dermoscopy and subjective evaluation, and adverse reactions during the treatment were monitored. Statistical analysis was carried out by t test, univariate analysis of variance or Fisher′s exact test. Results:A total of 19 children with superficial vascular malformations were enrolled, including 12 males and 7 females, aged 1 - 11.5 years. Fourteen children were diagnosed with vascular and lymphatic malformations, 3 with lymphatic malformations, and 2 with venous malformations. Sixteen children presented with lesions on the lower extremities, 8 were accompanied by pain, 2 presented with ulceration, and 6 had previous treatment history. After 6-month treatment, 3 patients achieved improvement of level Ⅰ, 4 of level Ⅱ, 4 of level Ⅲ, and 8 of level Ⅳ; 16 achieved improvement, and 12 achieved marked improvement. Six patients showed significantly decreased length, thickness and width of lesions after 6 months of treatment (1.83 ± 0.84 cm, 1.00 ± 0.55 cm, 2.25 ± 1.25 cm, respectively) compared with those before treatment (2.40 ± 0.95 cm, 1.35 ± 0.61 cm, 2.50 ± 1.34 cm, t = 5.22, 10.25, 3.73, respectively, all P < 0.05) . Gender, age, medical history and pain sensation did not significantly affect the therapeutic effect (all P > 0.05) , while diagnostic classification of vascular malformations significantly affected the therapeutic effect ( P = 0.008) . Among the 19 children, 2 had mild local burning sensation after the treatment. After 1- and 6-month treatment, the blood concentrations of sirolimus were both below 1.0 ng/ml. Conclusion:Topical sirolimus is effective and safe in the treatment of superficial vascular malformation in children.

Fístula quilosa posoperatoria / Postoperative chylous fistula

Introducción: La fístula quilosa posoperatoria debida a lesión iatrogénica del conducto torácico es una complicación infrecuente y grave de la cirugía de cabeza y cuello. Objetivo: Describir las opciones de tratamiento de la fístula quilosa cervical posquirúrgica. Caso clínico: Se presenta un paciente de 18 años de edad con diagnóstico de linfangioma quístico de la región lateral izquierda del cuello, intervenido en el Servicio de Cirugía General del Hospital "Mnazi Mmoja", de Zanzíbar, Tanzania. Durante la evolución posoperatoria se constató una fístula quilosa de bajo débito la cual resolvió mediante tratamiento médico. El enfermo egresó curado a los 28 días después de la intervención quirúrgica. Conclusión: El tratamiento conservador es efectivo en la mayoría de las fístulas quilosas cervicales de bajo débito, mientras que en las de débito alto la cirugía ofrece una rápida resolución, aunque no existe consenso en torno al momento ideal para realizarla(AU)

Introduction: Postoperative chylous fistula due to iatrogenic thoracic duct injury is an infrequent and serious complication of head and neck surgery. Objective: To describe the treatment options of postoperative cervical chylous fistula. Clinical case: It was presented an 18-year-old patient with diagnosis of cystic lymphangioma of the left lateral region of the neck, which was removed in the General Surgery Service of the National Hospital Mnazi Mmoja of Zanzibar, Tanzania. During the postoperative evolution, a low-output chylous fistula was found and resolved by medical treatment. The patient withdrew cured at 28 days after the surgical intervention. Conclusion: Conservative treatment is effective in the majority of low-out put cervical chylous fistulas, while in high-debit, surgery offers a rapid resolution, although there is no consensus about the ideal time to perform it(AU)

Left-Sided Cisterna Chyli: a Case Report on a Rare Normal Anatomic Structure

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.

Congenital cutaneous pedicled macrocystic lymphatic malformation on the upper extremity: A rare case report and review of the literature

Congenital cystic lymphatic malformations on the extremities are very rare. The patient described in this case study presented with a cutaneous and pedicled macrocystic lymphatic malformation that was eliminated by electrocauterization. A 4-day-old female infant with a congenital cutaneous mass on the dorsal area of her left first metacarpophalangeal joint presented to an outpatient clinic. An electrocautery device was used to cut the pedicle gently with minimal bleeding to avoid mass rupture and to minimize morbidity. A simple wet dressing was applied for 1 week, and the wound subsequently healed completely. Cutaneous macrocystic lymphatic malformations are very rare, especially on the extremities, and no consensus exists on their treatment, which has not been previously described. This report presents this rare case, along with a review of the literature.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization / 대한소아소화기영양학회지

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Retrospective analysis of classification and treatment of microcystic lymphatic malformations of tongue / 中华口腔医学杂志

Objective@#To explore the clinical classification of microcystic lymphatic malformations of tongue and observe the treatment of microcystic lymphatic malformations of tongue by retrospective analysis, in order to provide reference for clinical practice.@*Methods@#From October 2005 to October 2015, the complete data of 220 cases of microcystic lymphatic malformations of tongue (115 males and 105 females) received and treated in Provincial Special Department of Vascular Anomalies, Linyi Tumor Hospital was analyzed retrospectively. The age ranged from 8 months to 52 years old, with a median age of 16 years old. All patients were followed up for 3 years, and according to their clinical manifestations, they were divided into three types: localized type of 23 cases, diffuse type of 161 cases, and megaloglossia type of 36 cases. Injection with pingyangmycin merely was performed on 58 cases, whereas merely surgery on 20 cases, injection with pingyangmycin combined with high frequency electrocoagulation on 55 cases, and surgery combined with injection with pingyangmycin on 87 cases. The therapeutic effect was evaluated according to the grade 4 standard. The χ2 test was used for statistical analysis of count data. Rank sum test was used for statistical analysis of ranked data.@*Results@#The percentage of surgery merely of localized type was 87.0% (20/23), significantly higher than that of other types of lesions [0% (0/197)] (χ2=178.060, P<0.001). The percentage of injection with pingyangmycin of diffuse type was 100% (161/161), significantly higher than that of other types of lesions [66.1% (39/59)] (χ2=60.034, P<0.001). The percentage of surgery combined with injection with pingyangmycin of megaloglossia type was 100% (36/36), significantly higher than that of other types of lesions [27.7% (51/184)] (χ2=65.800, P<0.001). After follow-ups for 3 to13 years, there were 0 cases of gradeⅠ, 11 cases of grade Ⅱ, 50 cases of grade Ⅲ, and 159 cases of grade Ⅳ. There were statistically significant differences in clinical efficacy among different clinical types (H=158.668, P<0.001). The percentage of level Ⅳ efficacy of localized type, diffuse type and megaloglossia type were 100% (23/23), 82.6% (133/161) and 8.3% (3/36) respectively. Local mucosa ulcer appeared in 45 cases and was cured through oral care and expectant treatment. Fever occurred in 28 cases and returned to normal within 24 h after expectant treatment. The lingual frenum was shortened in 16 cases. There were 12 cases with mild tongue swelling, 6 cases with influence on eating but without influence on breathing, and recovered spontaneously one week later.@*Conclusions@#The choice of different treatment methods of microcystic lymphatic malformations of tongue should be made according their clinical classification. The only surgical resection is recommended for localized protrude lesions. Injection with pingyangmycin in high tension combined with surgical resection or high frequency electrocoagulation is effective for diffuse lesions. Surgical resection combined with injection with pingyangmycin is suitable for the patients with megaloglossia type.

Cavernous lymphatic malformation involving the bilateral labia minora: a case report / 中华皮肤科杂志

Objective To improve the understanding of cavernous lymphatic malformation (CLM) of the vulva.Methods The clinical manifestations,histopathological and immunohistochemical features of a case of CLM involving bilateral labia minora were analyzed,and relevant literatures were reviewed.Results A 17-year-old unmarried female patient presented with gradually enlarged subcutaneous masses on the bilateral labia minora for 2 years.Physical examination showed that soft kidney-shaped subcutaneous masses were observed on the bilateral labia minora.After surgical resection,a yellow-white solid cut surface was observed,and there were scattered rice-sized cyst cavitics filled with clear colorless liquid.Histopathologically,a large number of dilated lymphatic vessels with different sizes of lumens were scatteredly distributed in the dermis.The lumens were lined by a single layer of flattened endothelial cells,and there were thick fibrous septa between the lumens.Endothelial cells in the lymphatic vessels were stained positive for D2-40 and CD31,but negative for CD34.Interstitial fibroblasts were stained positive for progesterone receptors,but negative for estrogen receptors.Finally,the patient was diagnosed with CLM of bilateral labia minora.After surgical resection of the masses on the bilateral labia minora,no relapse was observed during the follow-up of 6 months.Conclusion Histopathological and immunohistochemical features facilitate the diagnosis and differential diagnosis of CLM.

Management for lymphatic malformation in infants: a single center experience / 中华整形外科杂志

Objective@#To assess the therapeutic effect of management for lymphatic malformation(LM)in infants.@*Methods@#This retrospective study recruited clinical data of 996 patients with LM from June 2004 to July 2015 in our center. All patients were diagnosed as LM after ultrasound, CT or MR scan. All patients were divided into Group 1 (427 patients, treated by endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol), Group 2(239 patients, treated by combined pinyangmycin and dexamethasone injection), Group 3 (330 patients, treated by surgical resection only). The clinical effects were observed in three groups, and therapeutic effect differences in gender, age, maximum diameter, location, range, histological typing, lymph property and treatments were analysed in cervicofacial LM.@*Results@#Group 1: 333 patients were cured (78.0%), Group 2: 165 patients were cured(69.0%), Group 3: 238 patients were cured (72.1% ). The difference in cure rate between Group 1 and Group 2 or between Group 1 and Group 3 was significant(P<0.05). The number of patients with ≥2 treatments in Group 3 was significantly less than that in other two groups. The cure rate of LM in cervicofacial area was significantly lower than that in other parts of body (P<0.05). In cervicofacial LM patients, the therapeutic effect differences in maximum diameter, range, histological typing, lymph property were statistically significant (P<0.05). At the multivariable logistic regression analysis, LM range as well as histological typing were independent factors influencing the therapeutic effect (P<0.05).@*Conclusions@#Both treatment of endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol, and surgical resection have good therapeutic effect on LM in infants. LM range as well as histological typing are important factors independently influencing the therapeutic effect of cervicofacial LM in infants.

The interventional treatment of complex lymphatic malformation in children / 中华放射学杂志

Objective To explore the interventional treatment method for complex and refractory lymphatic malformation in children.Methods The clinical data of 78 cases with complex and refractory lymphatic malformation during January 2013 to January 2016 in our department were retrospectively analyzed.The lesions involved the neck in 28 cases,maxillofacial regions in 19 cases,the chest and armpit in 8 cases,the limb in 7 cases,the pelvic cavity and retroperitoneal space in 6 cases,the superior mediastinum in 3 cases,the hypogloeeis in 3 cases and scrotum or perineum region in 4 cases.All the children underwent ultrasound or MRI imaging studies preoperatively.The interventional procedures included:(1) Percutaneous puncture of the LM for sclerotherapy.The lesions were punctured with 20 G needle under the guidance of DSA or ultrasound and the correct positions were confirmed with angiography.The liquid of the lesions was extracted as far as possible.The dosage of sclerosing agents was adjusted according to the size of lesion.The dose of Laurolacrogol injection was 1/10—1/5 of the amount of the liquid in the lesions and the maximum of Laurolacrogol foam was ≤8 ml(20 mg).The dose of Pingyangmycin was ≤ 8 mg.(2) The drainage catheter placement and sclerotherapy.Percutaneous catheter drainage under ultrasound guidance or by surgery was conducted.The liquid in the lesions was drained by retaining the catheter for 1-4 weeks,and sclerotherapy was applied for several sessions during this period.(3) Treatment for one time a week,2 times a session.The interval of every two sessions was 4 weeks.The sclerosing agents included:Laurolacrogol Injection or Pingyangmycin for the lesions with high tension,Laurolacrogol foam for the lesions with low tension,Pingyangmycin for the microcystic lesions.Statistical analysis was conducted using SPSS20.0 software.Results A total of 208 sessions of sclerotherapy for 78 LM patients were performed and average session was (3.0±0.8).Nine patients used Lauromacrogol foam,23 patients used Lauromacrogol Injection,39 patients used pingyangmycin,while combined treatment was conducted in 7 patients.Laurolacrogol injection was used in 20 cases,Pingyangmycin in 29 cases and combination therapy in 5 cases for the high tension lesions in the neck,maxillofacial,chest and armpit.The low tension lesions of pelvic cavity and retroperitoneal space in 6 cases and of superior mediastinum in 3 cases were placed with draining catheters,and treated with sclerotherapy with Laurolacrogol foam.Three cases with hypogloeeis LM was neonates,who were treated with Laurolacrogol injection.The lesions on the limb and scrotum or perineum region were almost microcystic,were treated with Pingyangmycin in 9 cases and with combination therapy in 2 cases.The total curative rate was 97.4％ (76/78),total effective rate was 100％ (78/78).Imaging examinations showed that the cavities were closed or only a small amount of residual sclerotic lesions were present.Clinical examinations showed that the surface masses almost disappeared.The follow up period was 6 months to 2 years.There were no serious complication and adverse reactions occurred.Conclusions Interventional treatment is a safe,effective,and minimally invasive treatment for the complex and refractory lymphatic malformation.In order to receive the best treatment effect,we should use targeted therapy for different region and type of LM.

Malformaciones vasculares: actualización en diagnóstico por imágenes y tratamiento / Vascular malformations: an update on imaging and treatment

Las malformaciones vasculares componen un amplio y heterogéneo espectro de lesiones, que frecuentemente se presentan como un desafío diagnóstico y terapéutico para el pediatra. El uso de una nomenclatura inadecuada durante mucho tiempo ha llevado a confusión. Dado que el tratamiento de esta patología depende de cada tipo de malformación vascular, su correcta clasificación e identificación es crucial. El objetivo es brindar la información necesaria sobre la clasificación y denominación actual de las malformaciones vasculares y los conceptos básicos sobre las herramientas disponibles para el diagnóstico y tratamiento de esta compleja patología.

Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

Cervicofacial Lymphatic Malformations: A Retrospective Review of 40 Cases

BACKGROUND: Lymphatic malformation (LM) is a form of congenital vascular malformation with a low incidence. Although LM has been studied, no consensus has emerged regarding its cause or treatment. METHODS: In this study, we retrospectively evaluated 40 patients who visited our vascular anomalies center for the treatment of cervicofacial LM, which is a common manifestation of LM. The medical records of patients over a period of 12 years were reviewed and analyzed for commonalities regarding the diagnosis and the results of treatment. RESULTS: Suspected cervicofacial LM was confirmed through imaging studies. No difference in incidence was observed according to sex, and 73% of patients first presented with symptoms before the age of two years. The left side and the V2-V3 area were most commonly affected. No significant differences in incidence were observed among the macrocystic, microcystic, and combined types of LM. A total of 28 out of 36 patients received sclerotherapy as the first choice of treatment, regardless of the type of lesion. Complete resolution was achieved in only 25% of patients. CONCLUSIONS: LM is important to confirm the diagnosis early and to choose an appropriate treatment strategy according to the stage of the disease and each individual patient's symptoms. When treatment is delayed or an incorrect treatment is administered, patient discomfort increases as the lesion gradually spreads. Therefore, more so than is the case for most other diseases, a team approach on a case-by-case basis is important for the accurate and appropriate treatment of LM.

Tratamento intralesional de malformações linfáticas com ênfase na escleroterapia com picibanil (OK-432): revisão sistemática / Intralesional treatment of lymphatic malformations with emphasis on Picibanil (OK-432) sclerotherapy: a systematic review

INTRODUÇÃO: Conduziu-se revisão sistemática retrospectiva da literatura incluindo estudos relatando o uso de picibanil para tratar malformações linfáticas (ML). MÉTODOS: A pesquisa foi realizada com estudos publicados no PubMed de janeiro de 1990 a 14 de abril de 2013. Na estratégia de busca, usou-se os descritores "OK-432" ou "Picibanil" e "lymphatic malformation". Os seguintes elementos foram comparados aos de outras modalidades relatadas e, então, compilados: mecanismo de ação, indicações, contraindicações, eficácia, administração, efeitos colaterais, complicações, vantagens e desvantagens. RESULTADOS: Foram encontrados 44 estudos, 27 dos quais atenderam aos critérios de inclusão. O picibanil é uma preparação liofilizada de uma cepa de baixa virulência de Streptococcus pyogenes inativada pela penicilina G. Seu mecanismo de ação ainda não definido claramente, mas especula-se que provoque uma resposta inflamatória controlada com adesão das paredes dos cistos. O picibanil é indicado quase que unanimemente para o tratamento da ML macrocística, cuja resposta é mais efetiva do que em lesões microcísticas ou mistas. Em geral, o picibanil é administrado por meio de punção com visualização direta ou guiada por ultrassonografia, com o paciente sob anestesia geral. A preparação comumente utilizada consiste em 0,1 mg de picibanil em 10 ml de soro fisiológico. Os efeitos colaterais são, em geral, leves; sendo dor, inchaço e febre os mais frequentemente relatados. CONCLUSÃO: Os estudos apresentam pouca evidência científica. A revisão sistemática identificou que o picibanil é útil no tratamento da ML de qualquer tipo, mas tem resultados melhores em lesões macrocísticas. A eficácia foi comparável à de outras terapias. Não foi apresentada nenhuma contraindicação específica. Embora o mecanismo de ação ainda não tenha sido determinado, o picibanil trata-se de opção de tratamento.

INTRODUCTION: We performed a retrospective systematic review of studies reporting the use of Picibanil for treatment of lymphatic malformations (LMs). METHODS: We searched the PubMed database for available studies, including those published between January 1990 and April 14, 2013. The search strategy involved the use of the keywords "OK-432" or "Picibanil" and "lymphatic malformation." Information was compiled regarding the reported mechanism of action, indications, contraindications, efficacy, administration, side effects, complications, and advantages and disadvantages compared to those of other modalities. RESULTS: Forty-four studies were found, of which 27 fulfilled the inclusion criteria. Picibanil is a lyophilized preparation of a low-virulence strain of Streptococcus pyogenes inactivated with penicillin G. Its mechanism of action is unclear, but it has been speculated that it causes a controlled inflammatory response with adhesion of cyst walls. Picibanil is almost unanimously indicated for the treatment of macrocystic LMs, which show a greater effectiveness response compared to that shown by microcystic or mixed LMs. Picibanil is usually administered by puncturing, either with direct visualization or guided by ultrasound, with the patient under general anesthesia. The most widely used preparation comprises 0.1 mg of Picibanil in 10 mL of saline. Side effects are mostly mild, with pain, swelling, and fever being the most frequently reported. CONCLUSION: The studies had low scientific evidence. A systematic review found that Picibanil is useful against any LM, with better results in macrocystic lesions. Efficacy was comparable to that of other therapies. No specific contraindication was presented. Although the mechanism of action has not been established, the inclusion of Picibanil as a treatment option is warranted.

Linfangioma microcístico acral: diagnóstico diferencial em lesões verrucosas de extremidades / Acral microcystic lymphangioma: differential diagnosis in verrucous lesions of the extremities

Linfangioma é uma má-formação originária da migração anormal de tecido linfático, levando a falhas na comunicação e na drenagem da linfa. Apresenta-se mais comumente como vésico-pápulas agrupadas de conteúdo translúcido na pele ou mucosas. A apresentação inicial na forma de placa verrucosa limitada a um único pododáctilo é atípica e reforça a relevância deste relato. Ainda que considerado lesão benigna, o linfangioma, a depender de sua localização e dimensão, pode apresentar dor local ou infecções recorrentes, com interferência substancial na qualidade de vida. Relatamos caso de linfangioma circunscrito a pododáctilo de surgimento tardio na idade adulta, na forma de placa verrucosa - apresentação atípica confirmada apenas após histopatológico. A excisão cirúrgica garantiu resposta estética e funcional satisfatória.

Lymphangiomas are a malformation caused by the abnormal migration of lymphatic tissue, leading to failures in the communication and drainage of the lymphatic system. They usually present as groups of translucent papules and vesicles in the skin or mucous membranes. Presentation as a verrucous plaque limited to a single toe is unusual and emphasizes the relevance of this case report. Although considered a benign lesion, depending on the site affected by the lymphangioma and its size, it may lead to localized pain or recurrent infections, substantially affecting the patient's quality of life. The present case report describes a patient with lymphangioma of late onset developing in adulthood in the form of a verrucous plaque confined to a single toe. Diagnosis of this unusual presentation could only be confirmed following histopathology. Surgical excision resulted in a satisfactory functional and cosmetic outcome.

Enfoque diagnóstico de los pacientes que presentan malformaciones linfáticas en cabeza y cuello: reporte de caso / Diagnostic approach to patients presenting with lymphatic malformations in head and neck: case report

La malformación linfática es una entidad poco frecuente que tiene características imaginológicas particulares. En este artículo presentamos el caso de una niña de seis años de edad con una masa en el cuello y en el piso de la boca, que ha consultado de forma recurrente desde los tres años por aumento en el tamaño de las lesiones y afectación de la vía aérea. En la actual hospitalización se presentó con reactantes de fase aguda elevados. Se le inició manejo médico con corticoesteroides y antibióticos y se le solicitó una tomografía computarizada y, posteriormente, una resonancia magnética de cuello, donde se documentaron lesiones con las características propias de las malformaciones linfáticas macroquísticas en el lado izquierdo del cuello y el piso de la boca, con desplazamiento de la línea media y compresión de la vía aérea. La biopsia confirmó el diagnóstico.

Lymphatic malformations are infrequent lesions that have specific imaging features. We report the case of a 6 years old patient with a mass in the neck and floor of the mouth. She had multiple consultations since 3 years of age due to increasing size of the lesions and involvement of the airway. In the last hospitalization, she had elevatedacute phase reactants. Medical management was started with steroids and antibiotics. CT and MRI of the head and neck f documented cystic lesions with features of macro cystic lymphatic malformations on the left side of the neck and floor of the mouth, with midlinedisplacement and compression of the airway. Biopsy confirmed the diagnosis.