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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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Diffuse lymphangiomatosis is a rare disorder characterized by abnormal proliferation of lymphatic channels. It can involve just one organ or multiple organs, such as liver, spleen, lungs, and bone. This disorder generally presents in children and young adults, but in rare cases, patients first present with symptoms in adulthood. Here, we describe a 48-year-old HIV-positive man who presented with shortness of breath. Computed tomography scan revealed a large right-sided pleural effusion and a heterogeneously enhancing liver. Thoracentesis demonstrated a chylous effusion and subsequent liver biopsy revealed a proliferation of dilated lymphatics to establish a diagnosis of lymphangiomatosis., (Published by Elsevier Inc.)

Lymphangiomatosis is a rare congenital disorder that results in multiorgan system lymphatic invasion. Symptoms due to axial skeletal involvement can range from chronic bone pain to severe deformity resulting in radiculopathy, myelopathy, and even paralysis. The authors present a case of lymphangiomatosis of the clivus, C-1, and C-2, resulting in chronic pain. The patient was successfully treated with percutaneous transoral clivoplasty and vertebroplasty, without disease progression or return of symptoms at 2 years.

The thoracic lymphatic vessels are pulsating channels which drain actively the fluid of lung parenchyma interstitium and pleural cavities. Their unidirectional valves that avoid reflux of contents, direct the current of fluid to the connection of thoracic duct to subclavian vein or to the thoracic duct itself by these pulsations. The ascending parietal and visceral currents have anastomoses between them. The parietal currents (internal thoracic anteriorly, external axillaries in lateral and paravertebral in posterior) drain the lymph of thoracic wall. Pleural cavities and the visceral currents, drain that of lungs and mediastinal organs. The thoracic duct goes upward in the posterior mediastinum and usually connects to the venous confluent of the left subclavian vein. It receives a part of thoracic lymph and also drains the lymph of trunk and inferior limbs. About a half or two thirds of thoracic duct lymph is originated from liver and intestines. The intestines have the lymph of digestion with the fatty elements, i.e., the chyle., (Copyright © 2013. Published by Elsevier Masson SAS.)

Lymphedema is a chronic disease associated with a congenital or acquired disorder of the lymphatic vessels or lymph nodes. Untreated lymphedema can lead to complications and disability. Clinical Lymphology deals not only with lymphedema of the extremities but also of the head, the genitals and the internal organs (lymphostatic enteropathy, chylaskos, chylothorax, chylopericard etc). Symptoms of this disorder are often misdiagnosed or not recognized. Ignorance and trivialization of lymphedema causes insufficient treatment, which then is not carried out to the extent as it is possible today by scientific findings. Even today delayed or not optimal treatment causes a long ordeal for many patients.The fact that lymphedema for those affected is a major psychological and social burden, which is limiting the quality of life, has also often been unregarded. The knowledge of anatomy, physiology and pathophysiology as well as the knowledge of causes are necessary for diagnosis, so that early treatment can be initiated.

A pregnant woman presented to a local hospital with abdominal pain and hemorrhagic shock. Emergency caesarean section ruled out an obstetric cause and revealed a large mass, interpreted as a hematoma, with active bleeding of unknown origin. Because of her poor clinical condition, the patient was admitted to our hospital. Computed tomographic findings were suspicious for bleeding originating from the splenic artery. Laparotomy confirmed the presence of a ruptured splenic artery. A splenic artery aneurysm-a relatively well known entity during pregnancy-was absent. Hemostasis was achieved by clipping the artery. A large pancreatic cystic mass, which was misinterpreted earlier as a hematoma, was surgically removed. The pathologic examination revealed a pancreatic lymphangioma, an uncommon benign tumor. The ruptured splenic artery was presumably related to the pancreatic lymphangioma and vascular changes caused by pregnancy. A splenic artery rupture in co-occurrence of a pancreatic lymphangioma is a unique presentation which has not been reported previously., (Copyright © 2013 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.)

The Mulliken and Glowacki classification (1982) differentiated vascular anomalies into two groups based on their endothelial characteristics: hemangiomas and vascular malformations. Vascular anomalies are localized defects of the vasculature that affect a limited number of vessels in a restricted area of the body. These defects are secondary to errors in vascular morphogenesis. Depending on the type of vessel involved, the vascular malformation group was subdivided into high-flow (such as arteriovenous malformation and arteriovenous fistula) and low-flow lesions (such as venous and lymphatic malformations). Depending on the type of lesion, the location and degree of involvement and the clinical effect, different types of treatment would be required. For the purpose of this review, we concentrate solely on vascular malformations: the clinical features, genetics, diagnosis, and current treatment options.

Diffuse lymphangiomatosis is a rare idiopathic condition that occurs mostly in children, is characterized by a non-neoplastic proliferation of lymphatic vessels, leading to organ dysfunction, chylous effusions, and death. A closely related condition-the Gorham-Stout syndrome-is also characterized by lymphangiomatosis and chylous effusions, but also with massive osteolytic changes ("vanishing bone disease"). A 33-year-old woman presented with a 5-year history of worsening chylous effusions and organomegaly. An extensive evaluation has ruled out most diagnoses. A complete radiographic skeletal study did not disclose any osteolytic changes. However, a Tc99 bone scan has demonstrated an absence of osteoblastic activity in some bones. An autopsy confirmed the diagnosis of diffuse lymphangiomatosis, but with histologically normal bone. If this unusual imaging pattern will be reproduced in future cases, a much needed diagnostic aid may help decrease the frequent diagnostic delays in diffuse lymphangiomatosis.

Based on various hypotheses concerning lymphangiogenesis published in the literature, different putative mechanisms of lymphangioma development are discussed including failure of the lymphatic system to connect with or separate from the venous system, abnormal budding of the lymphatic system from the cardinal vein, or acquired processes such as traumata, infections, chronic inflammations, and obstructions. Increasingly, the possible influence of lymphangiogenic growth factors on the development of lymphangiomas is discussed. The proved expression of different growth factors in the endothelium of lymphangiomas leads to new hypotheses regarding the pathogenesis of lymphangiomas. Thus, further studies on the lymphangiogenesis and the development of lymphangiomas will have to clarify as to whether lymphangiomas are true malformations or neoplastic in nature.

Lymphatic disease is quite prevalent, and often not well clinically characterized. Beyond lymphedema, there is a broad array of human disease that directly or indirectly alters lymphatic structure and function. The symptomatic and objective presentation of these patients can be quite diverse. In this review, we have attempted to provide a systematic overview of the subjective and objective spectrum of lymphatic disease, with consideration of all of the categories of disease that primarily or secondarily impair the functional integrity of the lymphatic system. Lymphedema is discussed, along with chromosomal disorders, lymphangioma, infectious diseases, lymphangioleiomyomatosis, lipedema, heritable genetic disorders, complex vascular malformations, protein-losing enteropathy, and intestinal lymphangiectasia.

Diffuse lymphangiomatosis is a very rare congenital disease, characterized by diffuse or multifocal lymphangioma in the skeletal tissue, spleen, liver, mediastinum, and/or lung. The prognosis is usually poor, especially for children with thoracic lesion, and treatments for the disease are controversial. The authors report a 9-year-old boy with diffuse lymphangiomatosis involving the thorax with pleural effusions, the spleen, and systemic bone. The patient was treated with pegylated interferon alfa-2b, and achieved good clinical and radiological improvement.

Hemangioma is a distinct category of benign vascular tumors characterized by presentation within the first weeks of life, rapid growth during the first year and variable degree of spontaneous involution over a period of several years. Recent research reported that CD8+ T cells in hemangiomas, and the endothelia of hemangioma uniquely expressed leukocyte marker FCgammaRII and myeloid cell marker. Presence of high levels of indoleamine 2,3-dioxygenase in proliferating hemangiomas and significantly decreasing during involution was also confirmed. Topical application of imiquimod cream, an immune regulator, to proliferating hemangiomas apparently accelerated regression of the lesions. These findings suggest immune response may be involved in the pathogenesis of hemangioma. The endothelia of hemangioma may express various markers to escape the immune surveillance. An immune response may be one of the mechanisms for hemangioma regression. Strategies with systemically or locally applying immune regulator into the tumor may be an applicable way in accelerating the involution of hemangioma.

Purpose: To evaluate the role of ultrasonography (US) and color Doppler imaging (CDI) in the diagnosis of orbital tumors in children., Design: Retrospective nonrandomized interventional case series., Methods: This study included 42 children with intraorbital and periorbital tumors who were evaluated in our clinic. All children underwent a complete clinical evaluation as well as orbital US and CDI. The children then underwent operation or were followed up, based on the clinical diagnosis and the findings on the imaging modalities., Results: The mean age of the patients at diagnosis was 22.5 months (range 2 weeks-14 years old). Eighteen patients (12 female and 6 male) were diagnosed with hemangioma based on the findings in US and CDI; 16 patients (9 female and 7 male) were diagnosed with dermoid, 5 patients (4 female and 1 male) with lymphangioma, 2 patients with rhabdomyosarcoma, and 1 patient with a subperiostal abscess. Twenty-two patients underwent operation, and the diagnosis was confirmed on pathology. Twenty patients did not undergo operation and continued to be followed up in the clinic (mean follow-up period 38.2 months). The disease course in all patients who did not undergo operation was consistent with the working diagnosis., Conclusion: Both US and CDI are useful modalities in the diagnosis of intraorbital and periorbital tumors in the pediatric age group.

Lymphangioma has long been thought of as congenital malformations resulting from the failure of lymphatic vessels communicating with the venous system in the fetal period. Alternatively, it is proposed to be true neoplasm originated from the transformation of lymphatic endothelia. To extend the molecular basis of the pathogenesis of lymphangioma, we have characterized the expression of vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFR) in 29 cases of lymphangioma by RNA in situ hybridization. Endothelial cells of lymphangioma co-express transcripts of VEGF-C and its receptors VEGFR-3 (Flt4) and VEGFR-2 (Flk1), which are not detectable in the adjacent connective tissue. In contrast, there is little or no expression of VEGF-C, VEGFR-3, and VEGFR-2 mRNA in endothelial cells of hemangiomas, angiosarcomas, or normal lymphatic vessels of the small or large intestines. The results suggest that VEGF-C and its receptors may take active parts in the formation of lymphangioma by autocrine or paracrine regulation.

Abdominal lymphangiomas are uncommon angiomatous tumor occurring mainly in childhood. This is a retrospective clinicopathologic study of 17 cases of abdominal lymphangioma. The patients included are five children and 12 adults, with a mean age at initial presentation of 30.7 years (age ranges 3-63). The locations of the tumors were mesentery (5), retroperitoneum (4), colon (3), omentum (3), mesocolon (1) and gallbladder (1). Infiltrative growth was more common pattern than entirely circumscribed pattern. Masses were mostly multilocular cysts and contained chyle or serous fluid. On immunohistochemical staining, 16 cases were reactive for either CD31 or factor VIII-related antigen. These fact would suggest that intra-abdominal lymphangiomas simulate the immunohistochemical features of collecting lymphatics. Follow up was possible in 12 cases for 3-50 months (mean 19 months) and only one patient showed local recurrence. Although abdominal lymphangiomas are rare in adulthood and correct preoperative diagnosis is difficult, awareness of such a possibility in adulthood will contribute to make a correct preoperative diagnosis.

Purpose: To highlight the need for referral and prompt intervention when signs of increased intraorbital pressure are present., Clinical Features: A four year old child presented to hospital with orbital swelling of recent onset, nausea and somnolence. Computed tomography and brain scan demonstrated a normal brain with a mass in the left orbit. She was referred for ophthalmic surgery the following day, when she had lost responsiveness of her left pupil to light. Anaesthetic management was highlighted by the presence of preoperative signs and symptoms of increased intraocular pressure--somnolence, nausea and vomiting, and bradycardia. These signs and symptoms were alleviated postoperatively., Conclusion: Rapid onset of orbital swelling with physiological signs of increased orbital pressure necessitate urgent surgical intervention. It is possible that early referral and surgery could have preserved some functional vision in a situation where the light reflex remained in the affected eye. Alleviation of preoperative nausea and vomiting, bradycardia, and somnolence indicates that the preoperative symptomatology was related to vagal stimulation from increased intraocular pressure, usually identified as an oculocardiac reflex.

Renal lymphangioma is a rare benign tumor of the kidney. An example is presented which was initially identified by excretory urography, angiography and biopsy. A 24 year follow up included computed tomography. The radiographic findings, pathogenesis and natural history of this unusual tumor are discussed.

Our purpose was to determine the natural history of unresectable lymphangiomas. From 1986-1992, 11 cases of unresectable lymphangiomas were seen in our institution (age 1 month to 14 years). Locations affected included cervical (8), mediastinal (6), and abdominal (3). Presenting symptoms included abdominal pain, respiratory problems, chylothorax, pleural effusions, and pneumococcal sepsis. Attempts at total excision were performed in all but one patient who had biopsy only. The tumor that could not be removed was then observed for the development of complications. Follow-up of these 11 patients ranged from 2 to 6 years. Two have completely regressed, five have stabilized, and four have required repeat operation. Of the five that have stabilized, none have developed complications such as infection or compression secondary to the mass. In an asymptomatic patient, it appears that the portion of the lymphangiomas remaining will regress or at least not progress, and no further resections were required.

Transillumination of cystic lymphangiomas, MRI of venous vascular malformations, pulsed Doppler and arteriography of arteriovenous malformations, echo-Doppler and radiomeasurement of malformations in the lower limbs are key examinations in the exploration strategy for superficial vascular malformations.

Thirty cases of orbital lymphangioma were reviewed. Clinical, imaging, and microscopic findings were integrated to develop a pathophysiologic construct and management guidelines. The basic lesion might be considered an abortive vascular system which arborizes among normal structures. Intrinsic hemorrhage expands portions of the small-caliber network into large blood cysts, prompting clinical recognition. While major hemorrhage led to early surgery in 12 cases, long pretreatment intervals could be analyzed in 17 others. Twelve patients had second hemorrhages of varied magnitude, from 2 weeks to 15 years after initial recognition; five patients did not in an average of 6.8 years. Some blood cysts contracted spontaneously. Among 23 operated cases, 12 patients had major new bleeds from 4 days to 12 years after initial surgery; 11 patients did not in an average of 4.2 years. Poor final visual acuity was associated with multiple surgeries. The authors advocate conservatism in surgical case selection and in operative dissection.

Sixteen cases of cervical cystic lymphangioma are reported; all but two were present at birth. Intra-oral extensions were detected in five and three of these caused macroglossia resulting in partial respiratory obstruction. Mal-development of the jaws and consequent dental malocclusion is a possible complication of the condition. In planning the treatment of such patients, maxillo-facial surgeons and orthodontists should be consulted at an early stage. The treatment of choice is complete excision, but where vital structures are involved repeated sub-total excisions are advocated.

Of 215 patients who underwent renal transplantations at the Mayo Clinic, six (2.8 percent) developed perirenal lymphoceles. Diagnosis was made between 5 weeks and 14 months after operation. In all six patients, renal function decreased and eventually a lower quadrant abdominal mass developed. Ipsilateral leg edema, fluid retention, and development of diastolic hypertension should also increase suspicion as to the possibility of lymph collection in the perirenal space. Excretory urography with tomography delineated the lymphocele in each instance; B scan ultrasound may be an additional valuable diagnostic tool. Drainage procedures readily restored normal renal function and, in one instance, drainage also restored the blood pressure to normal. Percutaneous needle aspiration seems to be a temporary measure only, and marsupialization is the preferred definite procedure.

The classification, natural history, and treatment of hemangiomas, arteriovenous fistulas, lymphangiomas, and port-wine stains are in many respects as puzzling now as they were fifty years ago. A review of the literature assesses the progress made and addresses the questions yet to be answered.

We describe a case of spontaneous resolution of a cystic hygroma between 14-16 weeks' gestation in a fetus with trisomy 21 who, at termination at 19 weeks, revealed only mild webbing of the neck. Redundant nuchal skin folds are among the most common features of trisomy 21. Our case supports the hypothesis that this redundant skin of the fetal neck represents early cystic hygromas, which resolve in utero before 16 weeks' gestation.

Cystic hygroma is a benign dysembryoplasia of the lymphatic system of mainly cervical expression. Clinical findings of a stereotyped nature were found in 4 cases of this particular aspect of infantile surgical affections. On operation there was an obvious relation with the internal jugular vein, and an entire segment had to be removed in 2 cases. These data confirm conventional pathogenic theories.

Hepatic lymphangioma is an extremely rare benign tumor, which usually occurs as part of a multiorgan lymphangiomatous involvement. We describe an adult patient with a huge hepatomegaly caused by a diffuse lymphangiomatous infiltration of the liver, which has never been reported before. Extrahepatic localizations could not be demonstrated. On ultrasonography and computed abdominal tomography, the liver lesions were not distinguishable from necrotic metastases. However, the endoscopic and histologic picture as well as the benign clinical evolution during a 2-yr follow-up allowed us to diagnose this rare benign disorder.

Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases.

A unifying concept linking disorders of lymphatic dysplasia, hyperplasia and neoplasia is presented. The central role of disturbed lymphangiogenesis is illustrated in a wide variety of lymphologic syndromes characterized by lymphedema, lymphangiectasia, lymphangioma, and lymphangiosarcoma.

On a routine ultrasound examination, a cystic hygroma and hydrops were noted at 21 weeks' gestation in a fetus with a 45,X karyotype. Serial studies demonstrated a marked reduction in the size of the cystic hygroma and complete resolution of ascites. At birth, the term infant had features characteristic of the Turner syndrome, including a webbed neck. A critical coarctation of the aorta required repair in the neonatal period. Our case provides glimpses of the intrauterine evolution of the Turner phenotype. We suggest that the possibility of survival when such lesions are detected prenatally may be greater than previously thought.

The oncotic pressure of fluid from subdural hematomas and subdural hygromas was compared to that of simultaneously drawn venous blood in 20 patients. There was no significant difference in the oncotic pressure of fluid from subdural hematomas and venous blood; however, the oncotic pressure of fluid from subdural hygromas was significantly less than that of blood. This finding fails to support the Zollinger and Gross modification of Gardner's that chronic subdural hematomas grow and produce symptoms after a latent interval because they attract fluid from the blood via dural vessels.