Table 2 treatments and outcome
From: Pulmonary lymphangiomatosis: insights into an ultra-rare disease
Case | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | Patient 9 | Patient 10 | Patient 11 | Patient 12 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Therapy | Sirolimus | NIV, lung-transplantation | Surgery with mediastinal cyst removal and thoracic duct ligation | Thoracoscopic pericardial fenestration, removal of the mediastinal tumor, Ductus thoracic ligature and talcum pleurodesis | Thoracoscopic pericardial fenestration, followed by sirolimus one months later which was paused after the patient became pregnant | Thoracoscopic talcum pleurodesis, thoracic duct ligation without success, followed by thoracic radiation due to persistent chylothorax and sirolimus | Sirolimus | Thoracoscopic talcum pleurodesis, thoracic radiation, sirolimus, oxygen therapy | Sirolimus | Sirolimus recommended | Sirolimus + propranolol only for 9 months Followed by pericardiectomy, adhesiolysis and talcum pleurodesis due to deterioration, continuation with sirolimus | Sirolimus |
Outcome | Clinical and radiological improvement after 3 months of therapy with sirolimus, stable disease in the following 25 months, afterwards loss of follow up | Death 8 years after diagnosis due to a complication after lung transplantation which was performed due to a progressive disease | Loss of follow-up | 1 month after surgery no symptoms anymore, afterwards loss of follow up | Improvement of pericardial effusion 12 months after sirolimus therapy, stable disease during pregnancy and time afterwards until now (another 26 months) | Clinical and radiological improvement and stabilization 6 months of sirolimus, treatment for 45 months, afterwards loss of follow up | Clinical and radiological improvement for 11 months after initiating sirolimus, loss of follow up after a treated infection | Regressive chylothorax after 3 months of sirolimus treatment, discontinuation due to phlebitis for one months, followed by reuptake, afterwards loss of follow up | Clinical improvement, discreet progression in CT scan (initiation of sirolimus was delayed to concerns of the patient in terms of adverse event and started after progression of symptoms) | Stable situation for 3 months, then loss of follow-up | Clinical and radiological improvement for approx. 3 months; after combining pharmacological and surgical treatments improvement of symptoms, lung function, X-ray findings and 6MWT | Stable clinical and radiological situation after 6 months |