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.
Abdominal Cavity ; Abdominal Wall ; Buttocks ; Diagnosis ; Extremities ; Head ; Humans ; Lymphangioma ; Medical Records ; Neck ; Parents ; Picibanil ; Recurrence ; Retrospective Studies ; Sclerotherapy ; Thoracic Wall

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.
Bleomycin ; Doxycycline ; Drainage* ; Female ; Humans ; Neck ; Picibanil ; Povidone-Iodine* ; Sclerotherapy* ; Ultrasonography ; Young Adult

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.
Carotid Arteries ; Child* ; Constriction, Pathologic ; Deglutition ; Drainage ; Gastrostomy ; Humans ; Lip ; Lymphangioma* ; Medical Records ; Neck ; Paralysis ; Picibanil ; Postoperative Complications ; Retrospective Studies ; Sclerotherapy ; Speech Disorders ; Suction ; Trachea ; Tracheostomy

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.
Cystic Adenomatoid Malformation of Lung, Congenital* ; Decompression ; Diagnosis ; Fetal Therapies ; Fetus ; Gestational Age ; Humans ; Hydrops Fetalis ; Infant, Newborn ; Lung* ; Picibanil ; Prenatal Diagnosis ; Retrospective Studies ; Sclerosing Solutions

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.
Acetic Acid ; Airway Obstruction ; Bleomycin ; Catheters ; Drainage ; Fever ; Fibrosis ; Head ; Inflammation ; Intubation ; Lung ; Lymphangioma ; Lymphangioma, Cystic* ; Neck ; Needles ; Picibanil ; Sclerotherapy* ; Tracheostomy ; Ultrasonography

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.
Branchioma ; Cicatrix ; Neck* ; Picibanil ; Ranula ; Recurrence ; Sclerosing Solutions ; Sclerotherapy* ; Thyroglossal Cyst ; Ultrasonography ; Vascular System Injuries

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.
Humans ; Magnetic Resonance Imaging ; Orbit ; Picibanil ; Retrospective Studies ; Sclerotherapy

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.
Chest Tubes ; Chylothorax ; Drainage ; Edema ; Humans ; Hydrops Fetalis ; Infant ; Infant, Newborn ; Infant, Premature ; Picibanil ; Pleural Effusion ; Pleurodesis ; Pregnancy ; Recurrence ; Streptococcus pyogenes

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.
Aromatase ; Ascites ; Breast ; Breast Neoplasms ; Drainage ; Estrogens ; Female ; Humans ; Lymph Node Excision ; Mastectomy, Segmental ; Neoplasm Metastasis ; Ovariectomy ; Picibanil ; Pleural Effusion ; Positron-Emission Tomography ; Recurrence

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.
Biopsy, Fine-Needle ; Chronology as Topic ; Cosmetics ; Ethanol ; Follow-Up Studies ; Humans ; Necrosis ; Picibanil ; Prospective Studies ; Sclerotherapy ; Thyroid Function Tests ; Thyroid Gland ; Thyroid Nodule ; Thyrotoxicosis ; Vocal Cord Paralysis