Efficacy of OK-432 sclerotherapy for different types of lymphangiomas: a review and meta-analysis.

OBJECTIVE: This study aims to perform a meta-analysis to figure out the efficacy of OK-432 sclerotherapy between Macrocystic (MAC) lymphangiomas and Microcystic (MIC) lymphangiomas. METHODS: We conducted a systematic review and meta-analysis to clarify the relationship between OK-432 and lymphangiomas. PubMed and ISI Web of Science were searched from inception to May 2022. Joanna Briggs Institute (JBI) manual was used to evaluate the risk of bias. We calculated pooled Relative Risks (RR) and 95% Confidence Interval (95% CI) using random effects model to evaluate the relations between OK-432 and lymphangiomas. RESULTS: A total of 11 studies (including 352 cases) about OK-432 sclerotherapy for lymphangioma were included in the current meta-analyses. The results suggested that the efficacy of OK-432 was significantly in MAC lesions than in MIC (RR=1.51, 95% CI 1.298-1.764), with significant moderate degrees of heterogeneity among 11 studies (I2=51.2%, p=0.025). Subgroup analyses suggested that there was significant association in both retrospective studies (RR=1.26, 95% CI 1.03-1.53) and classification (by 1 cm) (RR=1.37, 95% CI 1.04-1.80) were associated with the efficacy of OK-432. CONCLUSION: To our knowledge, our study represents the first meta-analysis examining the efficacy of OK-432 in the treatment of different types of LMs. However, the regional differences and the age differences of the subjects are the main limitations of this study and should be avoided in further research. Our results suggested that OK-432 sclerotherapy for macrocystic lymphangiomas was more effective.

Review of diagnosis, differential diagnosis, and management of retroperitoneal lymphangioma.

Lymphatic malformation (LM) is the currently preferred term for what was previously known as lymphangioma. Retroperitoneal LMs are extremely rare, benign, cystic masses that arise from lymphatic vessels. They can be challenging to diagnose because they resemble other retroperitoneal cystic tumors. The development of treatment strategies for rare diseases, including retroperitoneal LM, requires the acquisition of new knowledge to enhance our understanding of the disease progression. Therefore, we present an update regarding fundamental and advanced issues associated with retroperitoneal LM. This review describes the epidemiology, histopathology, biomedicine, clinical manifestations, radiological features, differential diagnosis, and management of this lesion.

How we approach the diagnosis and management of complex lymphatic anomalies.

Complex lymphatic anomalies (CLA) are congenital diseases of the lymphatic circulation system that are associated with significant morbidity and early mortality. While guidelines for the comprehensive evaluation of the CLA were recently published, the diagnostic approach and medical management are not standardized. This article presents the clinical features of four CLA: Gorham-Stout disease, generalized lymphatic anomaly, kaposiform lymphangiomatosis, and central collecting lymphatic anomaly. We also offer three cases from the authors' practice and our views on diagnostic testing and disease management including supportive care, medical therapies, and other interventions.

A Long-term Follow-up of Mandibular Deviation Caused by Congenital Cervical Lymphangioma Treated with an Orthodontic Approach.

AIM: To report a treatment case of mandibular deviation caused by congenital cervical lymphangioma with traditional orthodontic techniques, following-up by 10-year retention. BACKGROUND: Lymphangiomas, developmental anomalies, can induce various disturbances of swallowing, mastication, speech, breathing, and skeletal deformities as well as psychological stress and anxiety for the patient and their family. Lymphangiomas are benign with virtually no possibility of turning into a malignant lesion, so clinical management aims to treat the patient functionally. CASE DESCRIPTION: A girl, aged 6 years and 4 months, complained about facial asymmetry and anterior crossbite caused by congenital cervical lymphangioma. Her facial profile was the straight type with an adequate lip position. Anterior and right-side posterior crossbites were observed. On the frontal cephalogram, the menton shifted 3.0 mm to the right. A functional appliance with an expander was placed to correct her dental midline deviation and posterior crossbite. After 2-year treatment, the anterior and right-side posterior crossbites were improved. Multibracket treatment began after the growth spurt. After 44-month active treatment, a functional occlusion, including a Class I molar relationship with a proper interincisal relationship, was achieved. A functional occlusion was maintained during a 10-year retention period, while a mandibular downward growth was observed through the retention period. CONCLUSION: Conventional orthodontic techniques enable functional and stable occlusion even in patients with mandibular deviation caused by congenital cervical lymphangioma, although only using early orthodontic management by itself may have some limitations. CLINICAL SIGNIFICANCE: The hybrid technique combining functional appliance and intermaxillary elastics proves to be an effective therapy for correcting occlusal cant and mandibular deviation caused by cervical lymphangioma.

Efficacy and Clinical Outcomes of Bleomycin in the Treatment of Lymphangiomas: A Multicenter Experience.

BACKGROUND: Bleomycin sclerotherapy became a popular nonsurgical option for the management of lymphangiomas. However, its efficacy has not been thoroughly evaluated. The purpose of this study was to assess the clinical outcomes and the effectiveness of bleomycin injection for the treatment of lymphangioma. METHODS: This retrospective study was conducted in 4 centers and included 47 infants and children. All patients had bleomycin sclerotherapy between November 2005 and September 2020. Men presented 53.2% of the study sample (n = 25), and the most common site was the head and neck (n = 29, 61.7%). RESULTS: Two injections were required in 11 patients (23.4%), and 7 patients (14.9%) required 3 or more injections. Excellent response was achieved in 63.8% (n = 30), 14 patients (29.8%) had a good response, and 4 had a poor response (8.5%). There was no difference in the response according to the site of the lesion (p = .75). The most frequent complication was recurrence (n = 11, 23.4%), and swelling occurred in 5 patients (10.6%). No patient had facial or phrenic nerve palsy or hoarseness. Two patients had persistent pain, and 2 had an infection (4.3%). CONCLUSION: Intralesional bleomycin injection could be an effective therapy for lymphangiomas. The procedure has a low complication profile, and long-term study is recommended to evaluate the systemic and late bleomycin injection complications.

Bleomycin sclerotherapy in lymphangiomas of the head and neck region: a prospective study.

Intralesional sclerotherapy for lymphatic malformations (LMs) has become a modality of choice because of the high morbidity and recurrence rates with surgical excision. Traditionally, the macrocystic variant has shown good results with sclerotherapy. This prospective study was performed to evaluate the role of bleomycin sclerotherapy in the management of different radiological variants of LM. A total of 142 patients were included in this study. The lesions were classified as macrocystic, microcystic, or mixed LMs on the basis of ultrasonography. All patients were managed by intralesional injection of bleomycin and were recalled after 4 weeks for evaluation. Colour photographs of the patients were taken before the onset of treatment and at each monthly visit, and were utilized to assess the response. Following the second, third, and fourth doses, the response was better in patients with the macrocystic variant than in those with the other two variants. However, after the completion of six doses, 80.3% of patients with the macrocystic variant, 67.4% with the microcystic variant, and 71.4% with the mixed type had a complete response. There was no difference in the overall response between the three types (P=0.28). Oedema, erythema, and local induration with fever were the most common adverse effects and were more common in younger children.

Thoracic duct lymphangioma and chylous ascites in a 24-year-old female

Lymphangiomas are rare and correspond to 0.7% to 4.0% of mediastinal tumors, and isolated mediastinal location occurs in 1% of cases. They are benign tumors that originate from a congenital malformation of the lymphatic vessels and are diagnosed more frequently in children less than 2 years of age. Chylous ascites is a clinical manifestation of thoracic duct lymphangioma and is composed of lymph accumulation caused by dilation of this lymphatic channel. It appears milky in the peritoneal cavity, containing triglyceride levels higher than 200 mg/dl. We report the case of a young patient with chylous ascites and lymphangioma of the thoracic duct, who was conservatively treated with octreotide and a low-fat diet with medium-chain triglycerides.

A Case of Lymphangiomatosis With Infected Lymphangiomas Effectively Treated by Thoracoscopic Debridement and Drainage.

A 40-year-old woman with lymphangiomatosis also had an intrathoracic lymphangioma infection. Since the age of 8 years, the patient had undergone repeated abdominal and mediastinal cyst surgeries and had received a diagnosis of lymphangiomatosis. At this time, she showed a high fever of 38.5°C. Cultures of both blood and fluid aspirated from the cyst were positive for Staphylococcus aureus. Chest CT imaging revealed an enlarged right-sided mediastinal cystic lymphangioma with new septa in it. A chest tube was inserted into the cyst to remove effusion. The patient was then started with the antibacterial drug cefazolin at 3 g/d. But effective drainage was difficult because of the high viscosity of the effusion and septa working as barriers. We removed these components with endoscopic surgical instruments and via a pulsed-lavage system under general anesthesia. Postoperative CT images showed reexpanded lung structure and reduced cyst size. The patient has taken a favorable course for 2 years.

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment.

Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.

Efficacy Of Intralesional Bleomycin Injection Sclerotherapy In Macrocystic Lymphangioma In Paediatric Patients.

BACKGROUND: This study is aimed to assess the efficacy of intralesional Bleomycin sclerotherapy in macrocystic lymphangioma in children. METHODS: This prospective observational study included 40 children diagnosed with macrocystic lymphangioma and treated with intralesional injection of Bleomycin from March 2016 to Dec 2018. We excluded all the patients above 12 years of age, lesions with size less than 2 cm, and post-surgical recurrent lesions. The response to the treatment was monitored clinically by assessing length, breadth and area of the lesion and ultrasonographically. Mean follow-up period was 2 years. Study variables were analysed by simple descriptive statistics. Mean and standard deviation were calculated for numerical variables (age). Frequency and percentage were calculated for gender, site, size and type of cystic lymphangioma and total number of treatment sessions. RESULTS: The mean age of patients was 3.51±2.98 years. Out of 40 patients, there were 26 (65%) male and 14 (35%) females with female to male ratio of 1:1.8. The commonest site and type of cystic lymphangioma was neck and macrocystic, i.e., 77.5% and 85% respectively. The pre and post procedure size of lesion was 9.71±3.95cm and 3.11±1.02 cm, respectively. Outcome was excellent in 20% patients, good in 72.5% and poor in 7.5% patients. CONCLUSIONS: Intralesional Bleomycin sclerotherapy is effective in the treatment of macrocystic lymphangioma and is found to be harmless as there was no grave complication observed in this study.

Small intestinal hemolymphangioma treated with enteroscopic injection sclerotherapy: A case report and review of literature.

BACKGROUND: Hemolymphangiomas are rare malformations composed of both lymphatic and vascular vessels and are located in the pancreas, spleen, mediastinum, etc. Small intestinal hemolymphangioma is extremely rare and often presents as obscure gastrointestinal bleeding. It is rarely diagnosed correctly before the operation. Endoscopic injection sclerotherapy is usually used as a management of bleeding in esophageal varices and was occasionally reported as a treatment of vascular malformation. The treatment of small intestinal hemolymphangioma with enteroscopic injection sclerotherapy has not been reported. CASE SUMMARY: A 42-year-old male complained of recurrent episodes of melena and dizziness, fatigue and reduced exercise capacity for more than 2 mo. Gastroduodenoscopy and blood test revealed a gastric ulcer and anemia. Treatment with oral proton-pump inhibitors and iron did not improve symptoms. We then performed a capsule endoscopy and anterograde balloon-assisted enteroscopy and revealed a hemolymphangioma. Considering it is a benign tumor without malignant potential, we performed enteroscopic injection sclerotherapy. He was discharged 4 days later. At follow-up 3 mo later, the melena disappeared. Balloon-assisted enteroscopy revealed an atrophied tumor atrophied and no bleeding. Argon plasma coagulation was applied to the surface of the hemolymphangioma to accelerated healing. When he returned for follow-up 1 year later, anemia was resolved and the tumor had been cured. CONCLUSION: Balloon-assisted enteroscopy and capsule endoscopy are effective methods for diagnosis of hemolymphangioma. Enteroscopic injection sclerotherapy is an effective treatment.

Japanese Clinical Practice Guidelines for Vascular Anomalies 2017.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety and systematizing treatment, employing evidence-based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQ) were decided based on the important clinical issues. For document retrieval, key words for published work searches were set for each CQ, and work published from 1980 to the end of September 2014 was searched in PubMed, Cochrane Library and Japana Centra Revuo Medicina databases. The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System technique. A total of 33 CQ were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies.

Lymphangiomas: Rare presentations in oral cavity and scrotum in pediatric age group.

The incidence of lymphangioma is 1.2 to 2.8/1000 newborns. They present at birth/before 2 years, with predilection for the head and neck (50%-70%). The buccal mucosa is the second most common site reported (14 cases reported) after the anterior two-thirds of tongue. The scrotum is a rare site with less than 50 cases reported (till 2002). Involvement of vital structures, aesthetic, and functional requirements may necessitate treatments such as surgical excision, radiation, cryotherapy, electrocautery, sclerotherapy, embolization, ligation, and laser. Two rare cases - the first being primary, late-onset buccal lymphangioma, with vesicular presentation, and the second being genital lymphangioma involving the right side of scrotum, thigh, and groin with extension to the left groin - are highlighted.

Peripelvic Lymphatic Malformation Presenting With Gross Hematuria and Renal Colic: A Case Report.

Renal lymphangectasia is a benign malformation of the lymphatics which can be perirenal or peripelvic in location. Diagnosis is made on imaging including ultrasound, computed tomography , or MRI. We present an unusual case of renal lymphangectasia presenting with gross hematuria and flank pain diagnosed on computed tomography.

[Orbital Disorders in Childhood]. / Orbitaerkrankungen im Kindesalter.

Orbital disorders in childhood include various pathologies. Whereas neoplastic lesions and structural anomalies (which frequently cause strabismus) predominate in newborns and infants, vascular lesions become more common in toddlers. Inflammatory lesions are less common than in adults and affect mostly older children and adolescents. The most common orbital mass in infants and young children is a dermoid cyst, a benign lesion.Rhabdomyosarcoma as the most common primary orbital malignancy and neuroblastoma as a common metastasis to the orbit should be considered in children with new onset orbital space-occupying lesions.Capillary hemangioma and lymphangioma (lymphatic malformation) are relatively common vascular orbital lesions in children. Novel therapeutic options include systemic betablockers for capillary hemangioma and sirolimus for lymphangioma. In contrast to adults, external signs of an orbital fracture may be absent or mild in children. If ocular motility restriction after orbital trauma is present in a child without obvious external signs, a "white-eye blowout fracture" of the orbit should be considered and requires prompt surgical repair if present.Inflammatory disorders of the orbit such as thyroid-related orbitopathy and idiopathic orbital inflammation are uncommon in young children and occur more frequently in adolescents. In young children, amblyopia may complicate any orbital disease. It should be treated using part-time occlusion or pharmacologic penalisation of the fellow eye and the underlying cause should be addressed.