Lymphatic malformations adjacent to the airway in neonates: Risk factors for outcome

Abstract

To evaluate imaging, treatment, and outcomes in neonates with a lymphatic malformation (LM) adjacent to the airway and to evaluate risk factors that can predict outcome. Methods: A retrospective case series was conducted of ten patients treated between January 2011 and July 2019. The main outcome measures included airway compromise and clinical response to sclerotherapy ± surgery. Categorical data were compared using the Fisher's exact test. Results: Ex-utero intrapartum therapy was performed in four cases, among whom one died due to sepsis. All patients underwent schlerotherapy, with surgical debulking in two. Four patients showed a good clinical response, and five started experimental systemic treatment. Patients with bilateral disease and patients with an LM with > 180 °tracheal surrounding were significantly at risk for airway compromise (bilateral: n = 6/6 versus n = 0/4, p = 0.005; > 180 °: n = 5/5 versus n = 1/5, p = 0.048). The need for LM treatment in the neonatal period was indicative of a poor clinical response ('non-responders' 5/6, 'responders' 0/4; p = 0.048). Conclusions: This study indicates that bilateral disease and > 180 °tracheal surrounding are risk factors for airway compromise in neonates with an LM adjacent to the airway. In addition, the need for early treatment seems to be indicative of a poor clinical response.

Anesthesia for fetal operative procedures: A systematic review

“…After full-text screening against inclusion criteria, 168 articles, accounting for 6,761 procedures, were selected ( Figure 1 ) ( 11 – 178 ). Fourteen of the included studies, not providing information on the procedure outcome, were only considered for the initial description of anesthesia modality and in the summary of anesthetic drugs used ( 13 , 25 , 31 , 42 , 53 , 60 , 66 , 109 , 110 , 123 , 142 , 147 , 148 , 165 ). Characteristics of the included studies can be found in Supplementary Material .…”

Lymphatic malformations in children: retrospective review of surgical and interventional management

“…Life-threatening complications such as airway compromise may require either an orotracheal intubation or a tracheostomy before treatment, including ex utero intrapartum treatment (EXIT) procedure when prenatal diagnosis is made [ 2 , 4 , 27 ]. Recent studies showed that sclerotherapy was a safe and effective procedure to rapidly reduce the size of the lesion [ 28 , 29 ]. Surgery can be also considered, as well as systemic treatment with Rapamycin (Sirolimus ® ) in spontaneously breathing neonates [ 30 ].…”

Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era—A Comprehensive Review

“…MRI gives important additional information regarding tissue differentiation, which aids in differentiating between benign and malignant tissue and between a venous and lymphatic malformation. In addition, MRI is very helpful in the assessment of the extent of the CVM and its relation with adjacent structures [29]. For AVMs, digital subtraction angiography (DSA) is the gold standard diagnostic method, owing to its capacity to confirm AV-shunting, assess the angioarchitecture of the nidus, As in VMs, the majority of LMs can be classified as common LMs within the ISSVA classification; however, sometimes LMs appear as complex lymphatic anomalies.…”