Bilateral renal lymphangiectasia: Literature review of a rare entity.

Renal lymphangiectasia (RL) is a rare condition in which lymphatic vessels are dilated giving rise to cyst formation in peripelvic, perirenal and intrarenal locations. Knowledge about RL is limited and based upon individual case reports. This can be genetic or acquired. There is no significant association with any gender or age. It can be manifested as focal or diffuse forms and can be unilateral or bilateral. Most of the cases present with abdominal or flank pain. The diagnosis is based on radiological imaging. Due to rarity of diseases, it has potential to be misdiagnosed as other cystic disease of kidneys. The treatment is mainly conservative but prolonged follow up for associated complications like hypertension and renal vein thrombosis is required. We have presented a case of bilateral renal lymphangiectasia with the review of available literature.

Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.

Perirenal lymphatics: anatomy, pathophysiology, and imaging spectrum of diseases.

Despite being rarely discussed, perinephric lymphatics are involved in many pathological and benign processes. The lymphatic system in the kidneys has a harmonious dynamic with ureteral and venous outflow, which can result in pathology when this dynamic is disturbed. Although limited by the small size of lymphatics, multiple established and emerging imaging techniques are available to visualize perinephric lymphatics. Manifestations of perirenal pathology may be in the form of dilation of perirenal lymphatics, as with peripelvic cysts and lymphangiectasia. Lymphatic collections may also occur, either congenital or as a sequela of renal surgery or transplantation. The perirenal lymphatics are also intimately involved in lymphoproliferative disorders, such as lymphoma as well as the malignant spread of disease. Although these pathologic entities often have overlapping imaging features, some have distinguishing characteristics that can suggest the diagnosis when paired with the clinical history.

Hamartomatous polyp of the palatine tonsil: histological considerations and review of the literature.

The hamartomatous polyp is a rare benign hamartoma of the palatine tonsil, usually encountered during the second decade of life. It may be reported under various terms in the literature, like lymphangioma of the tonsil, angiofibrolipoma, lymphangiomatous tonsillar polyp and lymphangiectatic fibrous polyp. Macroscopically, it appears as a large, pale, pedunculated mass. Typically, a hamartomatous polyp is asymptomatic or manifests mild symptoms, like foreign body sensation. It is not related to a generalised lymphatic malformation process. Despite its typical appearance, an excisional biopsy is necessary to rule out a malignancy. Histological findings are consistent with a squamous epithelial covering, a core of loose fibrous and adipose tissue with sparse lymphoid aggregations and dilated lymphatic channels filled with lymph and lymphocytes. Several embryologically based theories suggested its pathogenesis; however, recurrent tonsillitis does not play an established role. A typical tonsillectomy is suggested as a sufficient therapeutical approach with no tendency for recurrence.

Long-term efficacy of lymphovenous anastomosis for the treatment of retroperitoneal lymphangiectasia: A case report and a review of literature.

Lymphovenous anastomosis (LVA) represents an alternative treatment for retroperitoneal lymphangiectasia. In contrast to sclerotherapy or excision, which may risk lymphatic obstruction and subsequent lymphedema, LVA preserves existing lymphatic architecture and transit. This report shows long-term efficacy of LVA for functional decompression of a symptomatic pathologically dilatated retroperitoneal lymphatics. A 47-year-old female with retroperitoneal lymphangiectasia refractory to multiple percutaneous drainages and treatments with sclerosing agents underwent LVA with anastomosis of a dominant segment of retroperitoneal lymphangiectasia to the deep inferior epigastric vein. Postoperative serial magnetic resonance imaging with 3-dimensional volume calculation over the 27 months follow-up showed evidence of decompression of the lesion with patent bypass. There were no known immediate complications nor requirement of further interventions. The patient's subjective pain also decreased substantially. This report confirms long-term efficacy of LVA for retroperitoneal lymphangiectasia as an alternative to sclerotherapy and surgical excision in the setting of previously failed treatments.

Paediatric acute respiratory distress syndrome: consider the role of lymphatics.

We present a case of a 7-day-old male infant with severe respiratory disease requiring venoarterial extracorporeal membrane oxygenation therapy with evidence of lymphangiectasia on lung biopsy. Differentiating primary versus secondary lymphangiectasis in this patient remains a riddle despite extensive investigations including an infective screen, lung biopsy and whole-genome sequencing. In addition to the standard therapies used in paediatric acute respiratory distress syndrome, such as lung-protective ventilation, permissive hypoxaemia and hypercarbia, nursing in the prone position, early use of muscle relaxants, rescue intravenous corticosteroids and broad-spectrum antibiotics, the patient was also given octreotide despite the absence of a chylothorax based on the theoretical benefit of altering the lymphatic flow. His case raises an interesting discussion around the role of lymphatics in the pathophysiology of paediatric and adult respiratory distress syndrome and prompts the exploration of novel agents which may affect lymphatic vessels used as an adjunctive therapy.

Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue.

Diffuse pulmonary lymphangiomatosis (DPL) is rare in adults. It is characterized by abnormal proliferation, dilatation, and thickening of the lymphatic channels in the lungs, pleura, and mediastinal soft tissue. Here, we report a case of DPL in a young adult man with recurrent productive cough. Chest computed tomography (CT) showed bilateral interlobular septal and peribronchovascular thickening and mediastinal soft tissue infiltration. Lung biopsy through video-assisted thoracic surgery demonstrated proliferation and dilatation of irregular lymphatic spaces, lined by flattened endothelial cells that were positive for CD31, D2-40, and factor VIII-related antigen on immunohistochemical staining. After treatment with propranolol for six months, the chest CT showed improved interlobular septal and peribronchovascular thickening and a unilateral pleural effusion, which turned bloody. Radiologic features can suggest the diagnosis of DPL. Surgical biopsy with adequate section size is critical in the diagnosis. Propranolol might be an effective and safe therapeutic option for patients with DPL.

Congenital interstitial lung diseases: What the anesthesiologist needs to know.

Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Some diseases have a very poor prognosis, whereas others have a benign course with appropriate treatment. The current manuscript reviews congenital interstitial lung diseases that typically affect neonates and young children and may be encountered by the pediatric anesthesiologist.

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.

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.

Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. We present here a case of a young man with DPL in whom treatment with sirolimus and propranolol rapidly achieved positive radiological and clinical effects.

Bilateral Renal Lymphangiectasia with No Significant Morbidity for Over 25 Years: A Case Report.

BACKGROUND Renal lymphangiectasia is a rare benign disorder with unknown pathophysiology. Renal lymphangiectasia can affect both males and females with no known age predilection. Its diagnosis can be accomplished with radiological images and confirmed by aspiration of lymphatic fluid in certain cases. However, there is no clear presentation to be relied on, apart from incidental findings or presentation of complications, such as hypertension due to mass effect on the kidney or renal failure from chronic compression and obstruction or renal vein thrombosis. Management is directed toward symptomatic relief and protection of the kidneys from failure or obstruction. The timing of possible complications and the duration of conservative therapy are undetermined. CASE REPORT Here, we present a case of a healthy 39-year-old woman with bilateral renal lymphangiectasia. It was initially discovered and confirmed to be lymphangiectasia when she was 13 years old and underwent bilateral renal aspiration. She recently presented to the Emergency Department with abdominal symptoms that were found to be caused by diverticulitis. The radiological images showed the persistence of her previous diagnosis of bilateral renal lymphangiectasia. She has had the same condition for more than 25 years, with no related complications or further intervention beyond conservative management. CONCLUSIONS Renal lymphangiectasia has a benign long-term course with insignificant and minor effects in certain patients regardless of the considerable size of cysts and bilateral involvement of the kidneys. The findings of our case could reassure patients with a diagnosis of a similar condition.

Conjunctival lymphangiectasia in a pediatric patient with neurofibromatosis type 1.

Conjunctival lymphangiectasia, a rare condition characterized by dilated lymphatic channels in the bulbar conjunctiva, generally develops as a consequence of a local lymphatic scarring or distal mechanical outflow obstruction following surgery, radiotherapy, neoplastic disease, or other disease processes. The actual cause often remains unknown. We report a case of a unilateral conjunctival lymphangiectasia in a 7-year-old girl with neurofibromatosis type 1 (NF-1) and discuss a possible association between the two entities.

Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]. / Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico.

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood. A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/ day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization. Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente. Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5- 4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalidad.

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature.

BACKGROUND: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. METHOD: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. RESULTS: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3-4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. CONCLUSION: Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

Transthoracic ultrasound: an essential diagnostic tool in a very rare case of thoracic lymphangiomatosis.

Thoracic lymphangiomatosis (proliferation of anastomosing lymphatic vessels, of different sizes, in pulmonary, pleural and mediastinal regions) is an extremely rare disorder occurring mostly in childhood. We present a diffuse pulmonary lymphangiomatosis (DPL) case in a young adult female patient in which repeated surgical biopsies were inconclusive and transthoracic ultrasound-guided (TUS) biopsy led to the diagnosis. Even histologically, DPL is very difficult to differentiate from other lymphatic diseases such as lymphangioma and lymphangiomyomatosis, requiring an experienced pathologist and proper immunohistochemistry staining. This case highlights the importance of TUS-guided biopsies in the armamentarium of imagistic techniques in this very rare case.

Linfangiectasia renal bilateral / Bilateral renal lymphangiectasia

Introducción: La linfangiectasia renal es una afección infrecuente en la que existe dilatación de los vasos linfáticos renales. Usualmente es bilateral y de buen pronóstico. Objetivos: Presentar un caso de linfangiectasia renal bilateral, sus características clínicas y aspectos del diagnóstico imaginológico. Caso clínico: Paciente femenina de 59 años de edad, hipertensa controlada, que asistió a la consulta externa de urología por dolor lumbar bilateral, de moderada intensidad y coloración rojiza de la orina, ambos de forma intermitente. El examen físico general y regional fue negativo. Se confirmó microhematuria en el examen general de la orina. Los estudios de la analítica sanguínea fueron normales. El ultrasonido renal reportó pielocaliectasia bilateral moderada y quistes parapiélicos, bilaterales, a predominio izquierdo. La tomografía axial computarizada renal contrastada, reveló la presencia de formaciones hipodensas renales bilaterales, de aspecto quístico parapiélicas y en el seno renal, compatibles con linfangiectasia bilateral. La paciente ha evolucionado favorablemente. Conclusiones: La linfangiectasia renal se debe tener en cuenta en el diagnóstico deferencial de la enfermedad quística renal. Para el diagnóstico imaginológico de certeza es esencial la tomografía axial computarizada renal contrastada(AU)

Introduction: The renal linfangiectasia is an uncommon, in which dilatation of the lymphatic renal vessels exists. It is usually bilateral and of good presage. Objectives: To present a case of renal bilateral lymphangiectasia, their clinical characteristics and aspects of the imaginologic diagnosis. Clinical case: Patient feminine of 59 years of age, with controlled hypertension that attended the external consultation of Urology for lumbar bilateral pain, of moderate intensity, and reddish coloration of the urine, both in an intermittent way. The general and regional physical exam was negative. Microhematuria was confirmed in the general exam of the urine. The sanguine studies of the analytic one were all normal ones. The renal ultrasound reported bilateral moderate pielocaliectasia and parapielic cysts, with left prevalence. The renal contrasted computed tomography revealed the presence of bilateral renal hipodenses formations, of aspect cystic parapielic and in the renal sinus, and compatible with bilateral linfangiectasia. The patient has evolved favorably. Conclusions: The renal linfangiectasia it should be kept in mind in the deferential diagnosis of the cystic renal illness. For the imaginologic diagnostic of certainty it is essential the renal contrasted computed tomography(AU)

A 35-Year-Old Man With Recurrent Pneumonia and Whitish Sputum.

CASE PRESENTATION: A 35-year-old man was admitted for recurrent episodes of pneumonia. He complained of a 2-month history of exertional dyspnea and productive cough with whitish and viscous sputum which was poorly responsive to antibiotic therapy. He also reported a weight loss of 5 kg since the first symptoms appeared. There was no dysphagia, fever, or chest pain. He currently did not use medication and did not have a relevant medical history except a current 10 pack-year smoking history. He did not report any history of trauma or respiratory exposure to fats.

Management of visceral vascular anomalies.

Visceral vascular anomalies are common in patients with vascular malformations in other parts of the body and can include lymphatic, venous, and arteriovenous malformations. Depending on the organ or organs involved they may present differently and pose different treatment challenges. Defining the malformation and understanding its extent is paramount in devising management regimens. Medical, interventional, and surgical therapies are often required in combination to treat these complex lesions. There are new and promising advances in the development of therapeutic agents targeting the PI3K/AKT/mTOR pathway. Due to the complex nature of these lesions a coordinated, multi-disciplinary approach is necessary to manage and mitigate symptoms and complications of this diverse group of vascular malformations.

A gene missense mutation in diffuse pulmonary lymphangiomatosis with thrombocytopenia: A case report.

INTRODUCTION: Diffuse pulmonary lymphangiomatosis (DPL) is a rare condition. Most patients with DPL present dyspnea, cough, expectoration, and hemoptysis. There are few reports of DPL accompanied by thrombocytopenia, whose cause remains unknown. PATIENT CONCERNS: An 18-year-old male patient presented with recurrent cough, expectoration, and dyspnea for 5 years, and thrombocytopenia was observed during a 2-month follow-up. DIAGNOSIS: Chest computed tomography showed diffuse patchy shadows in both lungs, and pleural and pericardial effusions. Immunohistochemical lung tissue staining showed lymphatic and vascular endothelial cells positive for D2-40, CD31 and CD34. Routine blood test revealed platelets at 62 × 10 cells/L during follow-up. Bone marrow biopsy was normal. Ultrasound revealed no hepatosplenomegaly. Finally, the patient was diagnosed with DPL accompanied by thrombocytopenia. INTERVENTIONS: He was treated by subtotal pericardial resection, thoracocentesis, and anti-infective therapy. Oral prednisone was administered for 2 months. OUTCOMES: The symptoms of cough and shortness of breath were improved, but thrombocytopenia persisted. We investigated the cause of thrombocytopenia. Whole-exome sequencing identified a mutation in exon 3 of the TNFRSF13B gene in this patient. CONCLUSION: DPL may present with thrombocytopenia and DIC. Patients with thrombocytopenia but not DIC and splenomegaly should be screened for gene mutations.