What is Abdominal Cystic Lymphangioma?
It is defined as a condition in which there is a blockage in the lymphatic system. It is rare and congenital condition. It is benign in nature. It leads to the aggregation of fluid and forms bubble below the skin. It is associated with the karyotype problems like turner and Down syndrome. The lymph vessels divide anywhere but mostly they occur in the head and neck. They are mostly present at the birth or may arise after couple of years. They have no chance to become malignant and they cause only esthetic problems.
What is the patho physiology of abdominal cystic lymphangioma?
The pathogenesis of abdominal cystic lymphangioma was studied by Whimster in the year 1976. The lymphatic structures are found in the deep subcutaneous tissues which are separated from the normal lymph vessels. They have a communication with the superficial lymph vesicle by vertical and dilated lymph channels. Whimster also stated that the cisterns might arise from the primitive lymph sac that does not form a connection with the remaining lymphatic system during the developmental stages. The muscle fibers form a thick coat that plays a role in the rhythmic contractions along the primitive sac. These increase the intra mural pressure. It leads to the dilation of channels that comes from the cisterns to the skin. These dilated projecting vessels form pouches constitute the vesicles of lymphangioma circumscriptum.
The lymph and radiographic studies support the Whimster observations. It is said that the big cisterns extend into the skin and beyond the clinical lesions. These lymphomas which are deep in the skin do not have any communication with the regular lymph system. The etiology for this disconnection is not known. Under the microscope the vesicles look like a dilated lymph channels that allow the papillary dermis to expand. They are associated with the acanthosis and hyperkeratosis. The upper dermis consists of multiple channels which extend deep into the dermis which is rich in the fat and connective tissue. The deeper vessels have thick walls which contain smooth muscle. The lumen has lymphatic fluid along with the red blood cells, macrophages, lymphocytes and neutrophils. The channels consists of endothelial cells and the interstitium which has many lymphoid cells and show fibroplasia. The nodules in the lymphangioma are large and irregular in shape. The reticular dermis and subcutaneous tissue have irregular channels which have a single layer of endothelial cells. It also contains the incomplete layer of smooth muscle cells .The stroma is rich in the connective tissue and has many inflammatory cells. These tumors go deeply inside the muscle. The cystic and cavernous lymphangiomas can be distinguished from the histological features. There are multiple vesicles seen in the history of lymphangioma circumscriptum which are present on the skin. They may also be present at birth or after the birth. They increase in the number as the age increases and the area of skin also increases. There are vesicles and abnormalities in skin which may not be noticed. The lesion is asymptomatic and does not show the occurrence of disease but the patients bleed and show the drainage of fluid from the ruptured vesicles. The cavernous lymphangioma occurs in the infancy. It is a rubber like nodule which shows no skin change. It is present in the face, trunk and extremity. The lesions grow at faster rate and form raised hemangiomas. There is no familial history. The cystic hygroma is present in the deep subcutaneous tissue. The cystic swelling is present in the axilla, neck and groin. It is noticed after the birth. The fluid is filled again when the lesion is drained. The lesions increase in the size and must be removed by the surgery.
What is the diagnosis of abdominal cystic lymphangioma?
This disorder is mainly diagnosed with the help of histopathology.
In pre natal cases the ultrasound is very effective method of diagnosis.
The amniocentesis can be done later on. It helps to identify the genetic disorders.