The most common diagnostic tools used to investigate the effect is lymphangioma and ultrasound, Doppler ultrasound, computed tomography (CT scan) and magnetic resonance imaging. Ultrasound shows hypoechogenic superficial multilocular cystic mass, but failed to show expansion retropharyngeal, axillary or mediastinum. With a CT scan, it may be difficult to distinguish the mass of soft tissue structures. More recently, MRI has been shown to be better in describing the boundaries of tumors and can more accurately describe the network.
There is a lot of literature and controversy regarding prenatal ultrasound diagnosis and outcome of lymphangioma. Results are basically dependent on the discovery of a genetic associations and lesions. Between 30% and 70% of fetuses with cystic hygroma reported to have chromosomal aberration, especially Turner syndrome or Down syndrome. The presence of lymphatic abnormality in the fetus during the second trimester has been associated with the results. Gallagher et al, report, like many others, that the fetal hydrops develops progressively up to 75% of cases and usually cause fetal death. If the karyotype is normal and there is no second malformations, mortality associated approaching 100%. Resolution of nuchal edema or cystic hygroma have been reported in the first trimester fetus with a good final result. In a review of 100 consecutive fetuses with nuchal thickening, or cystic hygroma detected at 10-15 weeks gestation, Nadel et a1.21, concluded that, if the karyotype is normal and there is no septations the masses and hydrops fetalis, then the prognosis is good.
Lymphangioma axillary seems to be more frequently associated with chromosomal anomalies. In a review of the literature, Thomas concluded that the fetus has a lymphangioma detected on ultrasound in the first trimester to undergo karyotyping. With normal karyotype have a good prognosis, while those with an abnormal karyotype have a poor prognosis. Detection of the mid to late third trimester was associated with good results (Algorithm 1).
Postnatally, the majority of lymphangioma appear before the age of 2 years, but they can appear at any time, and, in fact, have even been reported in the geriatric population. The main disorders of the lymphatic system illustrates the spectrum of conditions ranging from developmental defects in the peripheral lymphatic malformations to the thoracic duct, cysterna chyli and mesenteric nodes. Various manifestations can be patient. The common denominator in these patients is an abnormality in the lymphatic channels that lead to dilated proximal channel, limfostasis and lymphatic leakage.
The yield on postnatal lymphangioma is directly associated with the engagement area and treatment modalities used. In their review of 20 years of 52 children with cervical lymphangioma, cervicomediastinal and intrathoracic, Glasson and Taylor observed that two-thirds of patients are asymptomatic. Enlargement, inflammation, infection, difficulty eating, and respiratory symptoms can occur. Acute airway obstruction associated with the involvement of the pharynx and larynx, can be seen through the infiltration of large lesions. They also observed that the extension to the mediastinum causing respiratory symptoms are not too severe. Approximately 75% of patients require only one surgical procedure. The aim of the operation is a perfect excision as far as possible without compromising the structure or function vital.
The most common complication of surgical treatment is excessive accumulation of fluid. 263 lymphangioma in operation for over ten years, the average rate of complications was 31.3% with local complication rate was 50%, largely composed of seroma or hematoma. The second most frequent complication is damage to the nerves is important, in particular, the cervical branch of the facial nerve, accessory nerve, phrenic, vagus and sympathetic chain (resulting in Homer's syndrome), particularly after surgery for lesions cervicomediastinum. Neurological complications accounted for 17% of total complications. In order to reduce the incidence of certain complications, the use of intraoperative nerve stimulator is highly recommended. Lesions requiring thoracotomy may experience complications cylothorax. Complications in the thoracic responsible for 13.4% of all complications in our study.
Very large cervical lesions can be life threatening and may require tracheostomy early to secure the airway, tracheostomy should be performed until the lesions have completely cut. Laser carbon dioxide (CO2) is given by mycrolaryngoscopy facilitate excision of the lesion. Neodymium: YAG laser treatment for lesions of the oral cavity have also been reported to be successful. Both modalities require repeated treatments to control.
In comparing lymphangioma cervicofacialis supra and infrahyoid, Ricciardelli and Richardson found that none of 13 children with lesions showing signs tanad infrahyoid ganggauan eating or breathing while eight of the 21 with the complicity suprahyoid experiencing dysphagia or respiratory disorders. The combined use of CO2 laser and surgical approaches, the rate of recurrence of the lesion infrahyoid and suprahyoid is 15% and 81%. There were no postoperative complications in the group infrahyoid, but eight of the 21 patients with lesions suprahyoid suffering from post-operative complications that require repeated surgical procedures. Importance of lesion area in predicting outcomes have been documented further. Ssebagian authors agree that the most common recurrence after excision of the lesion suprahyoid, submandibular and oral. Until now, the conservative surgical management, save all important anatomical structures has become the treatment of choice for this cervicofacialis lymphangioma.
Mandibulofasial oral cystic hygroma and cause defects which require surgical correction. Osborne et al describe their experience with orthognathic surgical correction of mandibular prognathism and malocclusion of teeth associated with recurrence. Based on their results in three cases and review of the literature, the authors concluded that patients with large tumors that have not undergone adequate enlargement does not have to undergo surgery of the mandible at an early age because teradapat possibility of recurrence. Stabilization of tumor size and resolution of mandibular growth before orthognathic surgery led to an increase in the correction of defects generated.
Kilothorax associated with limfangiomatosis mediastinal and bone. Thoracosentesis itself did not succeed in those cases that are reported. Manipulation diet with a low-fat diet, through enteral chain triglycerides, or total parenteral nutrition effective in slowing the flow of lymph in a minority of patients. Thoracotomy with pleurektomi, with or without duct ligation thorasikus, achieve the best results. Thorasikus duct ligation alone is not successful in all cases. Fourteen of 16 patients with intrathoracic limfangiomatosis bone lesions consistent with lymphangioma, showed that the bone lesions may be only a manifestation of a more general process that can include mediastinal lesions. The authors recommend early thoracotomy and pleurektomi on the affected side. Each mediastinal lesions should be cut if possible. The application of fibrin glue on the surface exposed after the procedure can reduce the incidence of postoperative fluid accumulation. Keeping the lungs to expand with no postoperative mechanical ventilation can obliterate the pleural space and support healing.
In an excellent review of the literature recently, Alvarez et al. identify young age and pleural involvement and the involvement of the lung as a poor prognostic factor limfangiomatosis thorax. Although the age distribution in this series is 0 to 76 years, no patients over the age of 11 years died.
Intra-abdominal lymphangioma are rare. They are included in 5-9% of cases are located outside the head, neck, and extremities. In our experience there are two forms: local and diffuse. Local shape easy for total excision but a diffuse form refractory to conventional surgical excision. The importance lies in the abdominal area reports indicate that intra-abdominal lymphangioma is more common in children, with 88% symptomatic, and that the local form is often described as an acute abdomen. Symptoms include abdominal pain, vomiting, and nausea. Ultrasound and CT is the imaging modality that gives rapid diagnosis, thus helping to avoid potentially dangerous complications, such as obstruction, bleeding, rupture and volvulus. Total excision is the treatment of choice. Up to 61% of patients may require bowel resection. Invasion of other organs such as the spleen, pancreas require excision or end on the organ involved. However, not invariable recurrence after complete resection of large lesions. After surgery, ultrasound examination is recommended for monitoring. After the failure of conventional medical and surgical therapy, diffuse abdominal limfangiomatosis life-threatening, treated with argon beam ablation, with acceptable results for 6 months postoperatively.
Lymphangioma esophagus and intestines rarely occurs in children. Most cases are diagnosed in adulthood, usually by endoscopy. The relationship between the cavernous lymphangioma stomach and T-cell lymphoma have been reported. Lymphangioma cause enteropathy that led to the loss of protein in relatively large quantities by intramuscular cysts and subserosal. Mucosa and submucosa small cysts do not cause enteropathy causing protein loss. Electrocautery excision is the preferred treatment. Large cysts require bowel resection.