Conjunctival Lymphangiectasia - case report.

Conjunctival lymphangiectasia is a rare pathology that represents the enlargement of the lymphatic vessels localized in the conjunctiva. Patients may be asymptomatic or experience symptoms such as foreign body sensation, congestion, irritation, dryness, and blurry vision. There are various methods of therapy for patients with severe and symptomatic conjunctival lymphangiectasia. Surgical excision has the lowest rates of recurrence. We present a case of a 24-year-old woman with conjunctival lymphangiectasia and a history of left lower limb enlargement and bilaterally enlarged submandibular and upper jugular lymph nodes without an identifiable cause, who presented to the ophthalmology clinic accusing ocular discomfort, foreign body sensation and transparent conjunctival cystic lesions in the left eye for the last five months. Abbreviations: OD = right eye, OS = left eye, OCT = optical coherence tomography, VEGF = vascular endothelial growth factor.

Treating and preventing recurrence of recurrent genital acquired lymphangiectasia using lymphaticovenous anastomosis at genital area: A case report.

After the simple resection of genital acquired lymphangiectasia, a high recurrence rate is reported, as lymphatic fluid from the legs to the genital region continues. Here we report a case in which we treated and prevented recurrence of recurrent genital acquired lymphangiectasia with genital lymphaticovenous anastomosis (LVA). A 48-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cervical cancer at 30 years of age. The patient consulted our department for lymphedema in bilateral legs and acquired lymphangiectasia in the major labia. Despite nine attempted genital acquired lymphangiectasia resections and LVA in the lower limbs, which was aimed to prevent the recurrence, it recurred within 1-2 months postoperatively. Then we injected indocyanine green (ICG) to the bilateral edge of the anus before 10th LVA. ICG flowed from the anus to the genital area and we performed acquired lymphangiectasia resection and genital LVA on this lymphatic vessel (one anastomosis). After 6 months postoperatively, no recurrence of acquired lymphangiectasia, lymphatic leakage, or cellulitis had occurred. We found that a lymphatic flow from the anus into genital acquired lymphangiectasia occurred in one of the patients and the genital LVA, anastomosis of that responsible lymphatic vessel to the vein, might prevent genital acquired lymphangiectasia recurrence after resectional treatment.

Current and future role of fetal cardiovascular MRI in the setting of fetal cardiac interventions.

Over recent years, technical developments resulting in the feasibility of fetal cardiovascular magnetic resonance (CMR) have provided a new diagnostic tool for studying the human fetal heart and circulation. During the same period, we have witnessed the arrival of several minimally invasive fetal cardiac interventions (FCI) as a possible form of treatment in selected congenital heart diseases (CHDs). The role of fetal CMR in the planning and monitoring of FCI is not yet clear. Indeed, high-quality fetal CMR is not available or routinely offered at most centers caring for patients with prenatally detected CHD. However, in theory, fetal CMR could have much to offer in the setting of FCI by providing complementary anatomic and physiologic information relating to the specific intervention under consideration. Similarly, fetal CMR may be useful as an alternative imaging modality when ultrasound is hampered by technical limitations, for example, in the setting of oligohydramnios and in late gestation. In this review, we summarize current experience of the use of fetal CMR in the diagnosis and monitoring of fetuses with cardiopathies in the setting of a range of invasive in utero cardiac and vascular interventions and medical treatments and speculate about future directions for this versatile imaging medium.

Renal Lymphangiectasia in the Transplanted Kidney: Case Series and Literature Review.

BACKGROUND: Renal lymphangiectasia is a rare and poorly understood lymphatic disease associated with lymphatic dilation and leakage. To our knowledge, no cases have been described in the context of a transplanted kidney. METHODS: We describe 2 cases of renal lymphangiectasia in transplanted kidneys, both from pediatric donors. RESULTS: The cases of allograft lymphangiectasia are characterized by severe, symptomatic ascites refractory to attempts at medical and surgical management, and ultimately requiring allograft nephrectomy. CONCLUSIONS: While lymphatic complications, particularly lymphoceles, are not uncommon in renal transplantation, lymphangiectasia is a distinct condition which should be considered in renal transplant patients with ascites, after all other sources have been ruled out.

Successful treatment of conjunctival lymphangiectasia accompanied by corneal dellen using a high-frequency radiowave electrosurgical device.

A 62-year-old man presented with a 1-month history of right eye pain unresponsive to artificial tears and topical steroids. At presentation, bullous yellowish fluid collection was noted in the nasal conjunctiva. Corneal thinning and opacity were observed at the 3 o'clock position of the cornea. High-frequency radiowave ablation and biopsy were performed at the affected area. Conjunctival lymphangiectasia was confirmed by excisional biopsy. An improvement in the degree of corneal dellen and chemosis was evident 1 week after ablation. Use of a high-frequency radiowave electrosurgical device may be a simple and effective treatment option for symptomatic conjunctival lymphangiectasia.

Lymphaticovenous anastomosis and resection for genital acquired lymphangiectasia (GAL).

The purpose of this paper is to report on the relationship between lymphoscintigraphic findings and the operative results of lymphaticovenous anastomosis (LVA) as well as the resection of genital acquired lymphangiectasia (GAL). Seventeen patients with GAL who underwent lymphoscintigraphy between April 2012 and June 2016 were included in this retrospective study. LVA and GAL resections were performed for 14 patients. The GALs were resected in a spindle shape to the full thickness of the genital skin, and the wounds were closed primarily. Following the procedure, we investigated the recurrence rate and compared the results with the lymphoscintigraphic findings. The average follow-up period was 19.2 months. Six patients were without recurrence, and eight patients experienced a small recurrence of GAL. The average period from operation to recurrence was 16.8 months (range: 1-41 months). Of the six patients who had genital accumulation in lymphoscintigraphy, two patients experienced recurrence (33.3%), whereas six of the eight patients without genital accumulation experienced recurrence (75.0%). The frequency of cellulitis was observed to be reduced postoperatively in all patients, and recurrence was observed in one patient at 11 months postoperatively. In conclusion, lower limb LVA combined with genital skin tumor resection was effective for preventing the recurrence of GAL in patients who demonstrated communication between the lymphatic vessels in the lower limb and genitals. This procedure also had the benefit of reducing the frequency of cellulitis for an average of 19.2 months of follow-up.

Genital reconstruction with Integra™ artificial dermis after radical resection in a boy with diffuse lymphangiomatosis. / Reconstrucción genital con dermis artificial Integra® tras resección radical en un niño con linfangiomatosis difusa.

OBJECTIVE: Lymphangiomatosis is a rare disease affecting lymphatic vessels that causes a marked increase of them in the affected area. The final objective of treatment of the genital disease is to preserve sexual function and voiding with a satisfactory aesthetic result with the aim to minimize the emotional impact. METHODS: For the first time in children, we report a case of local reconstruction using artificial dermis after the excision of a genital lymphatic malformation in an eight year old patient. RESULTS: We performed surgical excision of the lymphatic malformation genital component and reconstruction of the scrotal and penile area in two steps, using an autologous graft over artificial dermis. No complications were registered. Both aesthetic and functional results were excellent, and spontaneous erections that were not present before, were also evidenced. No local recurrence was seen at two year follow up. CONCLUSION: The use of artificial dermis for genital surface reconstruction enables radical excision of tissues involved by diffuse lymphangiomatosis in this location. Surgical technique is simple, postoperative care is easy, being ambulatory care feasible. Final result is an elastic, fine skin, very satisfactory aesthetically, and functionally normal, which even keeps local sensitivity. In the long term, this will benefit the sexual field, so damaged in this patients.

Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications.

BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed. RESULTS: Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications. CONCLUSION: Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.

Surgical Drainage of Lymphangiectasia Haemorrhagica Conjunctivae.

PURPOSE: To report a case of nonresolving lymphangiectasia haemorrhagica conjunctivae (LHC) successfully treated with surgical drainage. METHODS: Retrospective case report. RESULTS: A 17-year-old white girl presented with a history of a large red lesion affecting her right eye. Approximately 1 year earlier, she had noticed a small painless "red spot" affecting the inferior aspect of the conjunctiva of her right eye. Three months before presentation, she noticed a sudden increase in the size of the lesion. There was no history of trauma, and she was not a contact lens wearer. Her medical history was remarkable for a remote seizure disorder which had been stable on levetiracetam. Magnetic resonance imaging/magnetic resonance angiography of the brain and orbits was unremarkable. Anterior segment fluorescein angiography did not show any flow in the lesion, indicating a lymphatic origin of channels. Surgical drainage of the lesion was performed while sparing the affected conjunctiva resulting in an excellent cosmetic outcome. Incisional biopsy confirmed the diagnosis of LHC. The patient remained free of recurrence 6 months after the procedure. CONCLUSIONS: Nonresolving LHC can be managed successfully with conjunctival sparing surgical drainage with an excellent cosmetic outcome.

Uncommon renal masses: Perirenal extramedullary hematopoiesis and multiple lymphangiomatosis with a perirenal lymphangioma.

OBJECTIVE: To present two cases of infrequent renal masses, trying to achieve the diagnosis before surgery. METHODS: We describe a case referred from the Department of Hematology in which bilateral perirrenal masses were described in the CT scan; after biopsy they where classified as extramedullary hematopoietic tissue. The other case was a patient presenting to the emergency room with dyspnea. CT Scan showed lungs with multiple cysts, chylothorax and a cystic-solid mass in the left perirenal space. In the lung biopsy they reported lung lymphangiomatosis, so we didn't perform renal biopsy. RESULTS: Most renal masses are renal carcinomas (856%). The less common diagnosis are sarcomas, lymphomas, upper urinary tract transitional cell carcinomas, metastases of other primary tumors, the Erdheim-Chester disease, the Castleman disease and benign tumors. All these diseases might show similar images in the CT scan and MRI, being the biopsy and histological study necessary for the diagnosis CONCLUSIONS: Perirenal extramedullary hematopoiesis and perirenal lymphangioma are rare diseases that need a pathologic study for their diagnosis.

Removal of lymphangiectasis using high-frequency radio wave electrosurgery.

PURPOSE: To describe a novel technique using high-frequency radio wave electrosurgery (Ellman Surgitron Dual Frequency RF; Ellman International, Inc) for the management of lymphangiectasis. METHODS: Ablations were performed at the lowest power setting of 1 (of 100) in the cutting mode, producing the least amount of lateral heat. To prevent conjunctival hemorrhage, ablation was initiated immediately before introducing the tip of a needle electrode into the subconjunctival tissue under the target area. After the tip of the electrode reached the target area, ablation was maintained for 1 to 2 seconds on the surrounding area and for a longer time on the dilated lymphatic vessels, until the target conjunctiva blanched. These ablations were performed cautiously with the fine electrode to avoid thermal injury to the adjacent scleral tissue. RESULTS: Persistent lymphangiectasis accompanied by accumulated fluid was successively treated with high-frequency radio wave electrosurgery. Surgical time was less than 5 minutes. There were no intraoperative complications. Fourier domain optical coherence tomography revealed resolution of the accumulated fluid and a decrease of dilated lymphatic vessels. Postoperatively, no notable complications, such as charring, scarring of Tenon capsule, or symblepharon resulting from excessive cauterization, were observed. CONCLUSION: High-frequency radio wave electrosurgery may be a safe, quick, and effective modality for the treatment of symptomatic lymphangiectasis patients.

Primary lymphedema complicated by weeping chylous vesicles in the leg and scrotum: report of a case.

We report a case of primary lymphedema complicated by leaking chylous vesicles in the toe and scrotum, caused by lymphangiectasia, which was eventually managed with lymphaticovenular anastomoses after conservative treatments proved ineffective. The patient was a 25-year-old man with a 5-year history of massive swelling with chylous weeping of his right leg and scrotum. Lymphangioscintigraphy (LAS) showed dilated iliac lymph trunks causing lymph reflux. Although he was instructed in standard methods of complex therapy, it did not alleviate his symptoms. Because of the increasing frequency of cellulitis, lymphatic surgery was finally indicated. The operation consisted of lymphaticovenous anastomoses (LVA) in the ankle and groin, using a super-micro-surgical technique. After surgery, his symptoms resolved and have been controlled by self-care. Thus, early LAS to confirm the dilated iliac lymph trunks causing lymph reflux, followed by LVA might be beneficial for the management of this disease.

Conjunctival lymphangiectasia: a report of 11 cases and review of literature.

Conjunctival lymphangiectasia is an uncommon clinical condition in which there is dilatation of lymphatic channels in the bulbar conjunctiva. Conjunctival lymphangiectasia is a rarely appreciated ocular surface disorder that typically occurs as a secondary phenomenon in response to local lymphatic scarring or distal obstruction. Conjunctival lymphangiectasia can either be unilateral or bilateral with focal or diffuse bulbar chemosis. We present 11 cases of biopsy-proven conjunctival lymphangiectasia. Of the 11 cases, 3 presented with bilateral diffuse bulbar chemosis, 1 had diffuse unilateral chemosis, and the remaining 7 presented with focal (<90°) bulbar chemosis. Three of these cases had co-existing pterygium, and one case presented with focal bulbar chemosis and a conjunctival keratin horn. All underwent surgical excision of the involved conjunctiva, either with no graft (n = 6), combined with amniotic membrane transplant (n = 3), or combined with conjunctival autograft (n = 2).

Ocular coherence tomography in lymphangiectasia.

PURPOSE: To report the aid of ocular coherence tomography (OCT) in diagnosing conjunctival lymphangiectasia and correlate clinical and pathological findings. METHODS: Single interventional case report. A 64-year-old man presented with a 2-year history of ocular discomfort, tearing, and a gradually enlarging lesion on the conjunctiva of his left eye. RESULTS: Slit-lamp biomicroscopy revealed localized conjunctival swelling temporally in the left eye, with the lesion protruding between the upper and lower eyelids. Visante (Carl Zeiss-Meditec, Jena, Germany) OCT revealed clear fluid-filled spaces demarcated by septae within the elevated conjunctiva. The lesion was excised, and histopathology of the specimen showed features consistent with lymphangiectasia. CONCLUSIONS: OCT, a valuable tool in imaging of the anterior segment, is also useful in evaluation of conjunctival pathology.

Intestinal lymphangiectasia mimicking primary peritoneal carcinoma.

Intestinal lymphangiectasia is an obstruction of the lymphatic system. We report on a patient with mesenteric adenopathy and an elevated CA125 level, which were suspicious for peritoneal carcinoma. Further evaluation and bowel resection identified intestinal lymphangiectasia. This disease should be considered in patients with mesenteric adenopathy and a small bowel mass.