Cervical lymphangioma in adults: case report and current treatment

ABSTRACT Introduction: Cystic tumors of the neck are rare in adults. Some of them include metastatic nodes, branchial cysts, thyroglossal cysts and lymphangiomas, among others. Clinically speaking, lymphangiomas are slow-growing soft masses located in different spaces of the neck. Case report: This paper reports the case of a 36-year-old woman presenting with lymph-angioma, who consulted due to a right lateral mass in the neck of 20 days of evolution without associated systemic symptoms. Although relapse is frequent, the patient was successfully treated with surgery, without evidence of recurrence at 12 months of follow-up. Discussion: When cystic tumors of the neck occur in children, surgical urgencies may arise due to obstruction of the airway. However, lymphangioma in adults only produce contour deformity and rarely require urgent intervention, which allows for conservative management such as observation, repeated drainage or sclerotherapy that can be done using OK-432 (Picibanil). Nevertheless, surgery remains a good treatment option, but some complications may occur. Conclusion: Different treatment options were reviewed, which led to conclude that surgical resection of lymphangiomas continues to be a good treatment for this complex neck lesion.

RESUMEN Introducción: Los tumores quísticos del cuello son inusuales en los adultos. Sin embargo, se pueden encontrar metástasis a ganglios, quistes branquiales, quistes tiroglosos, linfangiomas, entre otros. Clínicamente, estos últimos son masas blandas de crecimiento lento que se localizan en diferentes espacios del cuello. Reporte de caso: Se reporta un caso de linfangioma en una mujer de 36 años, quien consultó por masa lateral derecha del cuello con evolución de 20 días sin síntomas sistémicos asociados. A pesar de que la recaída es frecuente, la paciente fue tratada con cirugía exitosa sin evidencia de recidiva durante 12 meses de seguimiento. Discusión: Cuando se presentan en niños, los tumores quísticos del cuello se pueden convertir en urgencias quirúrgicas debido a obstrucción de la vía aérea; no obstante, en los adultos solo producen deformidad de contorno y rara vez requieren una intervención apremiante, lo que permite conductas conservadoras como la observación, el drenaje repetido o la escleroterapia. Esta última puede hacerse con el OK-432 (Picibanil); sin embargo, la cirugía es una buena opción de tratamiento sin estar exenta de complicaciones. Conclusión: Se realizó revisión de las diferentes opciones de tratamiento y se concluyó que la resección quirúrgica de los linfagiomas continúa siendo la opción más adecuada para el manejo de esta compleja lesión del cuello.

Experiencia con OK-432 en malformaciones vasculares linfáticas en un hospital del norte de México / Experience with OK-432 in lymphatic vascular malformations in a hospital from northern Mexico

Resumen Introducción: Las malformaciones vasculares linfáticas (MVL), anteriormente llamadas linfangiomas, son malformaciones congénitas que se presentan en uno de cada 6,000 a 16,000 nacimientos. El sistema de clasificación más útil para el manejo del linfangioma se basa en el tamaño de los quistes. La resolución espontánea es infrecuente, por lo que el tratamiento expectante no se recomienda. El tratamiento clásico es la cirugía de escisión, pero puede afectar a estructuras vecinas o haber recidivas, por lo que se empezaron a estudiar sustancias esclerosantes, como el OK-432. La mayoría de los estudios incluyen pocos pacientes; los más grandes realizados en México se enfocan a lesiones específicas (macroquísticas) o únicamente a una región anatómica. Hasta la fecha, no existen estudios del uso de este fármaco en la población del norte de México. Método: Se describe la experiencia con OK-432 mediante un estudio retrospectivo, descriptivo, en los pacientes con MVL, de 2011 a 2016, en un hospital de referencia del norte de México. Resultados: Veintiséis pacientes con MVL recibieron tratamiento con OK-432. La mayoría fueron macroquísticos (69%), microquísticos (19%) y mixtos (12%). Del total de pacientes, 11 presentaron curación total. El 72% de la población estudiada tuvo una reducción de > 50% del tamaño de las lesiones con solo dos aplicaciones de tratamiento; no se presentaron recidivas. Se reportaron complicaciones en dos pacientes (hiperpigmentación de la piel). Conclusiones: El manejo con OK-432 demostró ser efectivo para el tratamiento de las MVL en un hospital de referencia del norte de México.

Abstract Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied. The majority of the studies are small in number of patients and are from Japan; the largest studies in Mexico are focused on specific lesions (macrocystic) or a determined anatomical region. To date, there are no studies of the population of the north of Mexico. Methods: The experience with OK-432 was described through a retrospective, descriptive study in patients with LVM, from 2011 to 2016, in a reference hospital of northern Mexico. Results: A total of 26 patients with LVM were treated with OK-432. The majority of the lesions were macrocystic (69 %), microcystic (19 %) and mixed (12 %). From the total number of patients, 11 fully healed, and 72 % of the study population had >50 % reduction in lesion size, with only 2 applications. There were no recurrences. Complications were reported in 2 patients who had skin hyperpigmentation. Conclusions: OK-432 probed to be an effective treatment for LVM in a reference hospital in the north of Mexico.

The Treatment Experience of Lymphatic Malformations in Pediatric Patients

PURPOSE: The management of lymphatic malformation (LM) in pediatric patients is challenging. Complete excision of LM is difficult to achieve in some cases. We reviewed our experience how to manage LM. METHODS: We retrospectively reviewed the patients who were treated for LM between 2010 and 2017. Medical records were reviewed about age of diagnosis, age of treatment age, gender, symptom, location of tumor, treatment modality, response and complication. RESULTS: Sixty-three patients (39 boys and 24 girls) were included. Mean age at diagnosis was 14.5±28.0 months (range, neonate-10 years). The involved lesion were head and neck in 27 patients (42.9%), abdominal cavity in 7 patients (11.1%), chest wall and abdominal wall in 11 patients (17.5%), buttock in 7 patients (11.1%), and extremities in 11 patients (17.5%). The treatment options were including surgical resection in 32 patients, sclerotherapy in 7 patients, surgical resection combined sclerotherapy in 19 patients, and close observation in 5 patients. We achieved complete remission in 39 patients. Fourteen patients showed partial remission and 6 showed recurrences. CONCLUSION: Despite surgical difficulty, meticulous excision with supportive treatment, and adjuvant sclerotherapy could get a favorable outcome without fatal complication. Decision should be based on surgeon's experience, location of LM, related symptoms, and consultation with patient's parents.

Percutaneous Drainage and Povidone-Iodine Sclerotherapy of Cervical Lymphatic Malformation

Lymphatic malformations in cases with macrocystic lesions can be treated with surgical excision or sclerotherapy using alcohol, bleomycin, doxycycline, or OK-432. We report a case of a 24-year-old woman who underwent percutaneous drainage and povidone-iodine sclerotherapy as primary treatment for cervical lymphatic malformation. The patient underwent povidone-iodine sclerotherapy for 3 consecutive days. After 8 months, ultrasonography of the lesion in the neck revealed complete resolution of the cervical lymphatic malformation without any complication. Povidone-iodine sclerotherapy can be a safe and cost-effective treatment option for cervical lymphatic malformation.

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.

Avaliação de consumo alimentar, medidas antropométricas e tempo de menopausa de mulheres na pós-menopausa / Food consumption in postmenopausal women and its relation with anthropometric measurements and time since menopause

OBJETIVO: Avaliar o hábito alimentar e nutricional de mulheres na pós-menopausa e compará-los com o perfil antropométrico, faixa etária e tempo de menopausa. MÉTODOS: No período de junho a agosto de 2011, 148 mulheres na pós-menopausa residentes no Estado de São Paulo (região Sudeste do Brasil) foram avaliadas com um questionário estruturado contendo dados socioeconômicos, clínicos, antropométricos e alimentares. Avaliou-se nível de atividade física, variáveis bioquímicas, Índice de Massa Corporal (IMC), circunferência abdominal (CA) e consumo alimentar (energia, proteínas, carboidratos e gorduras, fibra, colesterol, vitaminas A e C, minerais, cálcio e ferro) de acordo com a faixa etária e o tempo de pós-menopausa (TPM). RESULTADOS: A média de IMC foi 29,0±5,6 kg/m2 e da CA, 95,7±12,9 cm. O consumo médio calórico diário atingiu 1.406,3±476,5 kcal. A ingestão e a adequação calórica foram significantemente mais apropriadas entre as mulheres eutróficas e com CA<88 cm. O mesmo ocorreu quanto ao consumo de proteínas (p<0,001 e p=0,006, respectivamente). Na análise por faixa etária ou TPM não houve diferenças significantes, exceto a média do consumo proteico, maior no grupo com 5 anos ou menos de menopausa (p=0,048). CONCLUSÃO: O perfil antropométrico de mulheres na pós-menopausa mostrou predominância de sobrepeso ou obesidade. O consumo alimentar apresentou-se adequado quanto às calorias e percentuais de macronutrientes, entre as eutróficas e com CA<88 cm. .

PURPOSE: To evaluate eating in postmenopausal women and its relation to anthropometry, age and time since menopause in São Bernardo do Campo residents. METHODS: During the period from June to August of 2011, 148 postmenopausal women residents in state of São Paulo (Southeast region of Brazil) were evaluated using a structured questionnaire containing socioeconomic, clinical, anthropometric and food data. The level of physical activity, biochemical variables, Body Mass Index (BMI), abdominal circumference (AC) and dietary intake (energy, protein, carbohydrates and fats, fiber, cholesterol, vitamins A and C, minerals, calcium and iron) were analyzed according to age and time after menopause. RESULTS: Mean BMI was 29.0≤5.6 kg/m2 and abdominal circumference was 95.7±12.9 cm. The average daily caloric consumption was 1,406.3±476.5 kcal. The calorie intake was significantly more appropriate in normal-weight women and women with AC<88 cm. The same was observed for protein intake (p<0.001 and p=0.006, respectively). No association was observed with age or duration of the postmenopausal period, except for average protein consumption that was higher in the group with five years or less of menopause (p=0.048). CONCLUSION: The anthropometry of postmenopausal women showed a predominance of overweight and obesity. Dietary intake was adequate in relation to the percentage of calories and macronutrients and calories among most normal-weight women and women with AC<88 cm. .

Surgical Treatment of Difficult Cervicofacial Lymphangioma in Children / 소아외과

PURPOSE: Cervical lymphangiomas are rare lymphovascular malformations arising in the neck, which form huge fluid-containing cysts. Treatment of the malformation consists of surgery and sclerotherapy. However, the optimal approach is still controversial. Here, we describe a series of cervical lymphangiomas which have been treated with surgical approaches. METHODS: We retrospectively investigated the medical records of 82 patients who had been diagnosed with cervicofacial lymphangioma from 2001 to 2012 in our center. A closed suction drainage with negative pressure was placed on the operative lesion following excision to prevent reaccumulation of lymphatic fluid and the drainage tube was removed after injecting OK-432 through the tube. RESULTS: Twelve patients underwent surgical excision of cervical lymphangioma. The median patient age was 3 months at the time of the operation. The patients have been followed-up over a period of 34 months. When lesions were located near vital organs such as the trachea or carotid artery or did not respond to repetitive OK-432 injections, surgical treatment might bring good outcomes. However, swallowing difficulty, lip palsy, or dyslalia due to adjacent nerve damage temporarily appeared as postoperative complications. Five children had tracheostomy due to tracheal or subglottic stenosis and 2 patients had gastrostomy due to aspiration while they eat after surgery. CONCLUSION: Surgery for cervicofacial lymphangioma should be conducted carefully in selective cases. A well thought-out surgical plan with a multidisciplinary surgical team approach and placement of closed suction drainage tube after surgery and adjuvant OK-432 sclerotherapy through drainage tube seem to be helpful for good outcome.

Malformación linfática facial mixta: Caso clínico / Mixed facial lymphatic malformation: Case report

Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions. Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up. Case report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension. Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

El linfangioma corresponde a una malformación vascular de los vasos linfáticos, preferentemente de la región de cabeza y cuello. La mayoría de los casos son de evolución progresiva y requieren un manejo multidisciplinario. Actualmente la primera opción terapéutica es la esclerosis, reservando la cirugía para el tratamiento de las lesiones remanentes. Objetivo: Presentar un caso de malformación linfática (ML) facial, tratado con escleroterapia, cirugía y ortodoncia en un seguimiento a 15 años. Caso clínico: Paciente de sexo femenino que consulta al año de edad por aumento de volumen progresivo de partes blandas en su mejilla derecha. El estudio de imágenes confirmó el diagnóstico de Malformación Linfática microquística. Se manejó con esclerosis seriada con OK-432 y Bleomicina. A los 2 años de edad se consideró que la respuesta era adecuada, y se procedió a realizar extirpación quirúrgica intralesional submandibular, con resección parcial de la lesión. La biopsia confirmó el diagnóstico de ML microquística. Seis meses después se planificó nueva resección utilizando el mismo abordaje y extirpando lesión remanente, con evolución favorable hasta la edad de 9 años en que requiere cirugía y manejo por ortodoncia, por recidiva de lesión a nivel intraoral. Evolución favorable hasta que a la edad de 13 años se planifica nueva cirugía y manejo por ortodoncia para suspender la comisura bucal derecha. Conclusión: El manejo de la ML mediante escleroterapia, cirugía, y ortodoncia muestra en este caso las ventajas de un tratamiento multidisciplinarion a largo plazo.

Cross-sectional study comparing different therapeutic modalities for cystic lymphangiomas in children

OBJECTIVE: Here, we describe our experience with different therapeutic modalities used to treat cystic lymphangiomas in children in our hospital, including single therapy with OK-432, bleomycin and surgery, and a combination of the three modalities. METHODS: We performed a retrospective, cross-sectional study including patients treated from 1998 to 2011. The effects on macrocystic lymphangiomas and adverse reactions were evaluated. Twenty-nine children with cystic lymphangiomas without any previous treatment were included. Under general anesthesia, patients given sclerosing agents underwent puncture of the lesion (guided by ultrasound when necessary) and complete aspiration of the intralesional liquid. The patients were evaluated with ultrasound and clinical examinations for a maximum follow-up time of 4 years. RESULTS: The proportions of patients considered cured after the first therapeutic approach were 44% in the surgery group, 29% in the bleomycin group and 31% in the OK-432 group. These proportions were not significantly different. Sequential treatment increased the rates of curative results to 71%, 74% and 44%, respectively, after the final treatment, which in our case was approximately 1.5 applications per patient. CONCLUSION: The results of this study indicate that most patients with cystic lymphangiomas do not show complete resolution after the initial therapy, regardless of whether the therapy is surgical or involves the use of sclerosing agents. To achieve complete resolution of the lesions, either multiple operations or a combination of surgery and sclerotherapy must be used and should be tailored to the characteristics of each patient. .

Ultrasound-guided sclerotherapy for benign non-thyroid cystic mass in the neck

Surgical excision has traditionally been the treatment of choice for benign non-thyroid cystic neck masses, including lymphatic malformation, ranula, branchial cleft cyst, thyroglossal duct cyst, and parathyroid cyst. However, there is a tendency toward recurrence after surgery, and surgery may be accompanied by complications, including nerve injuries, vascular injuries, and scar formation. Ultrasound-guided sclerotherapy using various agents has been challenged and successfully applied as an alternative treatment for benign non-thyroid cystic neck masses. This report reviews the available sclerosing agents and describes the applications of sclerotherapy to the treatment of benign cystic masses in the neck.

Sclerotherapy of cystic lymphangioma

Cystic lymphangioma is a congenital lymphatic malformation that occurs most commonly in the head and neck. Most lymphangiomas are asymptomatic, but progressive enlargement may cause feeding difficulty, airway, and cosmetic problems. According to the diameter of the majority of the cysts, lymphangiomas are divided into macrocystic (>1 cm), microcystic (<1cm), or mixed types. Sclerotherapy has been recommended as a primary treatment because surgery may result in severe tissue defect or functional complication. OK-432 is the most widely used sclerosant. Typical complications include fever, local inflammation, pain and swelling, which can usually be controlled conservatively. Bleomycin has potential risk of lung fibrosis, although there has been no such report in the literature. Acetic acid is a more potent sclerosant, which may be used as a secondary drug due to the risk of adjacent nerve damage in high concentration.The lesions are punctured with a 21 G needle under ultrasound guidance. In macrocystic lesions, we inserted 6-7 F catheters for drainage and instillation of sclerosant. While OK-432 and bleomycin are not removed, alcohol and acetic acid should be removed after indwelling for 20 minutes. Overall, an excellent response (over 90% resolution) rate of 48% was achieved. Response rate was higher in macrocystic type than microcystic type. There may be a risk of airway obstruction due to swelling and inflammation in cervical lesions. Preventive tracheostomy or intubation may be necessary in those lesions. In conclusion, sclerotherapy is a safe and effective for treatment of lymphangioma. It is recommended as a first line therapy.

Intrauterine therapy for macrocystic congenital cystic adenomatoid malformation of the lung

OBJECTIVE: To report on our experiences with thoracoamniotic shunting and/or the injection of a sclerosing agent (OK-432) to treat fetuses diagnosed with macrocystic congenital cystic adenomatoid malformation (CCAM) of the lung. METHODS: A retrospective study was undertaken in six fetuses with macrocystic CCAM at our institute that had been confirmed by postnatal surgery between August 1999 and January 2012. RESULTS: Six fetuses that had been diagnosed with macrocystic CCAM were analyzed. The median gestational age at diagnosis was 23.5 weeks (range, 19.5-31.0 weeks), and at the time of primary treatment was 24.0 weeks (range, 20.5-31.0 weeks). The mean size of the largest cyst at the initial assessment was 42.5+/-15 mm. Four fetuses were associated with mediastinal shifting, and one also showed fetal hydrops. All fetuses underwent a shunting procedure within the cysts, one case among them was also treated with OK-432. After the completion of all procedures, the mean size of the largest cyst was all decreased (14.2+/-12 mm). The median gestational age at delivery was 38.0 weeks (range, 32.4-40.3 weeks). All of the newborns underwent the surgical resection at a median age of 6 days (range, 1-136 days) and are currently doing well without any complications. CONCLUSION: We suggest that intrauterine decompression therapy to manage fetal macrocystic CCAM is recommendable treatment for good perinatal outcome.

Lymphatic Malformations of the Orbit

OBJECTIVES: Lymphatic malformations of the orbit are rare lesions that constitute approximately 1% to 8% of all orbital masses. They are difficult to treat since they do not remain within anatomic boundaries and tend to penetrate into normal orbital structures. The aim was to analyze clinical courses and therapy options in patients with lymphatic malformations of the orbit. METHODS: Thirteen patients with orbital lymphatic malformations confirmed by magnetic resonance imaging between 1998 and 2009 were enrolled in this study. Patients' charts were retrospectively reviewed to analyze clinical courses and treatment options. RESULTS: Four patients suffered from isolated intraorbital lymphatic malformations without conjunctival involvement, in three of them the masses were completely resected, in one patient close controls were performed. Three patients had isolated intraorbital lymphatic malformations with conjunctival involvement. Surgical volume reduction of the exterior parts of the lymphatic malformation were performed without any complications and satisfying outcome in these cases. Six patients suffered from intra- and periorbital lymphatic malformations. In 3 patients a watch-and-wait strategy was initiated. In the other 3 patients a surgical therapy was performed, one patient additionally received sclerotherapy with OK-432; however, these 3 patients suffered from residual lymphatic malformations. CONCLUSION: The presented cases underline the inconsistencies in the malformations behavior and underscore the inability to make specific recommendations regarding treatment. The treatment decision should be based on the size and location of the lymphatic malformation. The untreated patient must be watched for signs of visual detoriation, which may signal the need for therapeutic intervention.

Successful pleurodesis with OK-432 in preterm infants with persistent pleural effusion / 소아과

OK-432 (picibanil) is an inactivated preparation of Streptococcus pyogenes that causes pleurodesis by inducing a strong inflammatory response. Intrapleural instillation of OK-432 has recently been used to successfully treat neonatal and fetal chylothorax. Here we report a trial of intrapleural instillation of OK-432 in two preterm infants who were born with hydrops fetalis and massive bilateral pleural effusion. Both cases showed persistent pleural effusion, refractory to conservative treatment, up to postnatal days 26 and 46, respectively. An average of 80 to 140 mL of pleural fluid was drained daily. In case 1, the infant was treated with OK-432 during the fetal period at gestation 28 weeks and 4 days of gestation, but showed recurrence of pleural effusion and progressed into hydrops. Within two to three days after OK-432 injection, the amount of pleural fluid drainage was dramatically decreased and there was no reaccumulation. We did not observe any side effects related to OK-432 injection. We suggest that OK-432 should be considered as a therapeutic option in infants who have persistent pleural effusion for more than four weeks, with the expectation of the early removal of the chest tube and a good outcome.

The Utility of Sono-Guided Sclerotherapy for Benign Thyroid Cyst: Prospective Study / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Ultrasonography (USG)-guided sclerotherapy using a sclerotherapic agent such as ethanol, OK-432, recently has gained popularity as a treatment for nonfunctioning benign thyroid nodules. The study evaluates the efficacy and safety of the USG-guided sclerotherapy for that purpose. SUBJECTS AND METHOD: Included in the study were 23 patients who had complaints of applied pressure or cosmetic problems due to cystic thyroid nodules, which had been diagnosed as benign and nonfunctioning by fine-needle aspiration biopsy and thyroid function test. Sclerotherapy was performed with OK-432 or 99.9% ethanol following nearly complete evacuation of the fluid from the cystic portion of thyroid nodule. At each USG follow-up, we measured the volume of cysts, symptom scores, cosmetic scores using a visual analog scale, and related complications were examined at postoperative dates of one and six months. RESULTS: The volume of thyroid cysts were significantly reduced (p<0.01) with mean volume reduction rates of 77.3+/-21.2% at postoperative 6 months. The cosmetic score and symptom score were significantly improved following sclerotherapy (p<0.01). Major complications related to sclerotherapy, such as vocal cord paralysis, thyrotoxicosis and tissue necrosis, did not happen during the follow-up observation. CONCLUSION: USG-guided sclerotherapy could be a simple and effective treatment modality for benign cystic thyroid nodules.

A Case of Pseudo-Meigs' Syndrome Associated with Ovarian Metastases from Breast Cancer / 한국유방암학회지

A 54-year-old woman with long-lasting pleural effusion developed abdominal distention due to ascites from bilateral ovarian tumors. The patient had undergone breast-conserving surgery and axillary lymph node dissection for left breast cancer in October 2000, and had developed left pleural effusion in July 2006. Cytological examination of the pleural effusion found no malignant cells. Thoracic drainage with intrathoracic administration of OK-432 (Picibanil) had failed to control the pleural effusion. Positron emission tomography taken at the abdominal distention showed bilateral ovarian tumors. After failure to control the ascites with systemic and intra-abdominal chemotherapy, bilateral oophorectomy resulted in normalization of elevated serum tumor-marker levels and the disappearance of both the ascites and pleural effusions (i.e., pseudo-Meigs' syndrome). Pathological examination showed the tumors to be estrogen receptor-positive metastatic ovarian tumors from her breast cancer. The patient remained well with no further recurrence for 40 months under aromatase inhibitor therapy.

Uso de OK-432 (Picibanil) como alternativa no quirúrgica para el manejo de ránulas y mucoceles / The use of OK-432 (Picibanil) as a non-surgical alterantive for the management of ranula and mucoceles

Mucocele y ránula, son términos clínicos aplicados para describir un pseudoquiste asociado con extravasación mucosa en los tejidos circundantes, donde se hallen glándulas salivales menores. Estas lesiones ocurren como resultado de un trauma del conducto excretor de laglándula salival o por la obstrucción de flujo salival por otra lesión asociada, como un sialolito o un tapón bacteriano. Estas patologías se originan en una glándula salival menor y son también conocidos como fenómeno de retenciónmucosa o reacción de escape mucoso.El manejo de estas lesiones es quirúrgico en todos los casos, sin embargo, presenta algunas dificultades propias del evento, complicacionespostquirúrgicas y secuelas permanentes en el paciente; en la actualidad se ha manejado este tipo de lesiones con otro tipo de terapéutica, como escleroterapia, uso de toxina Botulínicatipo A, LASER, o el OK-432 (Picibanil), que es una mezcla de Streptococo Pyogenes del grupo A mermado en su virulencia y penicilina G en polvo liofilizado que funciona como agente esclerosante para linfangiomas y agenteantineoplásico. En este trabajo se presenta una revisión bibliográficaacerca de esta patología y una nuevaopción de tratamiento con este medicamento, sus indicaciones y contraindicaciones, así como su administración, reacciones secundarias y complicaciones durante el manejo de lesiones reactivas asociadas a las glándulas salivales menores.

Infantile Fibrosarcoma in Neonate

Infantile fibrosarcoma is a rare malignant soft tissue tumor occurring especially in newborn and young children under 2 years. We experienced three cases of infantile fibrosarcoma presenting in the neonatal period. Case 1 presented with a multiseptated cystic mass on his left thigh at birth that was diagnosed as lymphangioma. After picibanil injection, we noted the size of the mass doubled and a solid lesion was prominent in the magnetic resonance image. Case 2 was found to have a reddish mass on his lower back mimicking hemangioma. Over 2 weeks, the mass grew rapidly with internal hemorrhaging. Case 3 was noted to have an encircling mass around the splenic flexure, which developed into congenital bowel obstruction. All of the tumors were resected completely, but microscopic resection margin was not clear in two patients. The two patients received adjuvant chemotherapy and all patients are well without evidence of recurrence.

Successful Treatment of Steatocystoma Multiplex with OK-432 Sclerotherapy / 대한피부과학회지

Steatocystoma multiplex is a hamartomatous malformation of the pilosebaceous junction and this manifests as multiple, asymptomatic, yellowish or skin-colored cystic papules and nodules located most commonly on the upper anterior portion of the trunk, upper arms and axillae. Various treatment methods such as radical excision, simple surgery, CO2 laser, cryotherapy, needle aspiration and oral retinoids have been reported, but there is currently no standard treatment. OK-432 is a biological preparation consisting of lyophilized powder containing Streptococcus pyogenes Su strain cells (group A, type3) that were treated with benzylpenicillin potassium. This has been used for sclerotherapy in patients with lymphangioma. We herein report on a case of 36-year-old female with steatocystoma multiplex on the trunk and axillary area. She was successfully treated with intralesional infusion of OK-432 after aspiration of the oily contents without recurrence or any complications such as scarring, atrophy and skin necrosis. We suggest that this sclerotherapy is a cosmetically acceptable treatment option for the multiple lesions of steatocystoma multiplex.