Microcystic Lymphatic Malformation Research Articles

Intralesional injection of Pingyangmycin for vascular malformations in oral and maxillofacial regions: An evaluation of 297 consecutive patients

The purpose of the study was to investigate the indications and treatment results of intralesional injection of Pingyangmycin for vascular malformations in oral and maxillofacial regions. Between June 2002 and July 2007, 297 patients with oral and maxillofacial vascular malformations (except venular malformation and arteriovenous malformation) were treated with percutaneous injection of Pingyangmycin in our department. There were 159 males and 138 females aged from 8 months to 72 years (mean age 20.6 years). The types of the disease included 98 lymphatic malformations (66 microcystic and 32 macrocystic malformations), 179 venous malformations, and 20 mixed lymphatic-venular or venous malformations. The lesions were located in the oral and maxillofacial and head and neck regions, and measured 0.8 cm x 1.0 cm -12.4 cm x 10.5 cm in size. Two hundred and forty-nine patients underwent intralesional injection of Pingyangmycin alone, 30 patients had laser therapy, and 18 patients underwent surgical excision as well. The number of injection sessions was 1-5, with an average of 3.5 times. Pingyangmycin, dexamethasone, and lidocaine were mixed for injection at concentrations of 1.0mg of Pingyangmycin per milliliter for microcystic lymphatic malformation and 2.0mg of Pingyangmycin per milliliter for venous malformation and macrocystic lymphatic malformation. The patients were followed up for 1-6 years after treatment. The results were evaluated based on B-type ultrasound examinations before and after treatment. The clinical cure rate of venous malformation, macrocystic lymphatic malformation, microcystic lymphatic malformation and mixed malformation was 74.86%, 84.38%, 38.88%, and 60.0%. Five patients had fever. No ulceration, necrosis, or anaphylactic reaction was found. Intralesional injection of Pingyangmycin at an adequate concentration can be used as the therapy of choice for small and medium-sized venous malformations, macrocystic lymphatic malformations, microcystic lymphatic malformations, and mixed malformations. The advantages included a high cure rate, safety and reliability, short course, easy manipulation, and fewer side effects with satisfactory recovery of appearance and function.

Giant Lymphangioma of the Tongue

This report presents the treatment of an extensive lymphatic malformation of the tongue. Sclerosing agents are now widely used as the first-line treatment of lymphatic malformation. However, treatment of lymphatic malformation involving the face and the vital structures such as the airway remains to be challenging. A 4-year-old boy underwent a total of 15 OK-432 injection sclerotherapy treatments over a 2-year period, having slow progress until sudden enlargement of the tongue was noted shortly after the last injection. Partial excision of the lesion was performed. This case demonstrates the risk in treating large microcystic lymphatic malformation of the tongue with sclerotherapy and provides an insight in the management protocol.

Low power radiofrequency ablation for symptomatic microcystic lymphatic malformation of the tongue

Although many head and neck surgeons agree that complete surgical excision is the treatment of choice for lymphatic malformation, the infiltrating nature of microcystic lymphatic malformations and the involvement of vital structures of the head and neck make total removal nearly impossible in most cases. Recently radiofrequency ablation was introduced for the treatment of microcystic lymphatic malformations of the oral cavity; it destroys lesion tissues at low temperature (40-70 degrees C) with minimal damage to adjacent structures. However, high energy (800-1200 J) and general anesthesia for radiofrequency ablation can hinder the easy and repetitive application of radiofrequency to patients. To overcome this limitation of radiofrequency ablation for microcystic lymphatic malformations, we used the same technique with a lower power (6 W, less than 100 J per site) radiofrequency ablation in an office-based setting under local anesthesia in a child with a microcystic lymphatic malformation of the whole tongue, that was associated with recurrent bleeding and swelling. The repetitive low power energy radiofrequency ablation of the microcystic lymphatic malformation of the tongue was safe and effective; it provided the patient with symptomatic relief without recurrence at follow-up. Therefore, our modification of radiofrequency ablation of the microcystic lymphatic malformations could be technically feasible and easily applicable; our result suggests that it can be a useful alternative treatment option to relieve symptoms from microcystic lymphatic malformation of the oral cavity.

Lymphangioma circumscriptum (microcystic lymphatic malformation): Palliative coagulation using radiofrequency current

Lymphangioma circumscriptum (LC, microcystic lymphatic malformation), a hamartomatous lymphatic malformation, is a difficult condition to treat. Different treatments such as surgical excision, lasers, sclerotherapy etc have all been tried with varying success. We report here the efficacy of a radiofrequency current in two patients with lymphangioma circumscriptum. The radiofrequency technique is a safe, economical, and commonly available technique for the treatment of LC; the surgical safety and outcome were satisfactory in our patients.

Radiofrequency Ablation of Microcystic Lymphatic Malformation in the Oral Cavity

To determine the efficacy and safety of radiofrequency (RF) ablation of vesicles and the resulting symptomatic control of microcystic lymphatic malformation (LM) in the oral cavity. An institutional review board-approved retrospective study with follow-up telephone interview. Tertiary pediatric medical center. Patients Eleven children (6 girls and 5 boys), aged 4 to 16 years, presenting between August 1, 2002, and December 1, 2004. Intervention Radiofrequency ablation of LM in the oral cavity. Symptoms related to LM, postoperative oral intake, and postoperative antibiotic requirements. Eleven patients presented with microcystic LM involving the lips, tongue, floor of the mouth, or buccal mucosa. Complaints included bleeding, infection, swelling, vesicle formation, and malocclusion. Patients underwent RF ablation (coblation) of oral cavity lesions. Seven (64%) of the 11 patients were able to tolerate oral intake in the recovery room. The need for antibiotics was reduced after RF ablation. All patients related diminished bleeding, pain, infection, or vesicle formation, with more than half reporting a significant improvement (6 patients) or complete resolution (1 patient). Five (62%) of 8 parents stated that the improvement after RF ablation was superior to that following previous procedures. Subtotal RF ablation of LM appears to be safe, with early postoperative oral intake and minimal postoperative pain. Further studies are needed to determine long-term control of LM.

Lymphocytopenia in Children With Lymphatic Malformation

To determine whether an immunologic abnormality exists in patients with lymphatic malformation (LM). Retrospective case series. Tertiary care pediatric hospital. Twenty-one consecutive patients (11 male and 10 female) undergoing LM treatment. Clinical data (ie, age, clinical LM stage, radiographic appearance, and histologic findings) were correlated with complete blood cell count and detailed lymphocyte differential. Complete blood cell counts and lymphocyte subsets were measured in 21 and 18 patients, respectively. The average age at the time of testing was 67 months (range, 1-231 months). The patients were categorized according to LM stage, including 4 (19%) with stage 1, 4 (19%) with stage 2, 4 (19%) with stage 3, 7 (33%) with stage 4, and 2 (10%) with stage 5 disease. Radiographic LM appearance was macrocystic in 6 patients (29%), mixed macrocystic and microcystic in 8 (38%), and microcystic in 7 (33%). Complete blood cell count data demonstrated lymphocytopenia in 6 patients (29%). The results of the lymphocyte subset tests showed concomitant T-, B-, and natural killer (NK)-cell deficiency in 6 (33%) of 18 patients. All 6 patients with T-cell lymphocytopenia had normal neutrophil and platelet counts. Spearman rank and chi(2) analyses showed that LM stage 4 or 5 and microcystic LM were significantly associated with lymphocytopenia (P = .002 and P = .008, respectively). Histologic analysis did not demonstrate increased lymphocytes in any LM specimens. We found T, B, and NK lymphocytopenia in patients with large bilateral or microcystic LM. Although the relationship between lymphocytopenia and infection was not addressed in this study, the recognition of lymphocytopenia in patients with LM may have important clinical and prognostic implications.

Use of the deep inferior epigastric perforator flap in a 15-month-old boy after resection of lymphatic malformation

Lymphatic malformations are benign tumors that arise from congenital defects of the lymphatic system. Despite the fact that all lymphatic malformations develop embryologically, their presence may not be detected until the tumor enlarges from hemorrhage or infection. Typical of these tumors is a slow-growing, asymptomatic phase with the potential for rapid and massive expansion and a tendency to infiltrate into surrounding tissues. These tumors are associated with a high rate of recurrence, especially with incomplete excisions. To minimize the chance of recurrence, a complete resection of a groin microcystic lymphatic malformation was performed on this 15-month-old boy, creating a significant defect in the upper thigh. Immediate coverage was felt to be the best option for reducing any associated morbidity. We used a skin-soft tissue flap supplied by perforators of the deep inferior epigastric artery to repair the wound. Using this technique, the musculofascial structures in the abdominal wall were completely spared, and the skin and fat were transferred on a vascular leash to the desired location. The donor site was closed by approximating the fascial edges without tension. The defect on the thigh was filled with tissue of similar thickness and characteristics, avoiding potential contour irregularities and color mismatch. The deep inferior epigastric perforator (DIEP) flap is a useful option for groin defects and can be applied safely in the pediatric population.