What is difference in management of primary lymphedema between adults and children, and how much?

Chylous cyst-venous shunt for the management of central conducting lymphatic anomaly

“…4,5 Recently, a pathogenic variant corresponding to the JAG-1 gene has been reported as the cause of CCLA, thus broadening the spectrum of genes affected by the pathology, which still has a vast field to be studied. 6 Medical treatment such as sirolimus (an mTOR inhibitor) has been shown in several trials to have good results in the management of lymphatic malformations 7,8,9,10 ; however, to-date, there is not enough evidence of its application on central conducting lymphatic anomalies.…”

“…Medical treatment such as sirolimus (an mTOR [mechanistic target of rapamycin] inhibitor) has been shown in several trials to have good results in the management of lymphatic malformations. 7 , 8 , 9 , 10 However, to date, the evidence is not sufficient regarding its application to treat CCLAs. In addition, owing to the complex nature of these lesions, CCLAs have demonstrated a refractory response to some surgical treatments.…”