D. Schindel1,2, D. Twickler2, N. Frost1,2, D. Walsh2, P. S. Munoz2, R. Johnson1,2 1Children’s Medical Center Dallas,Dallas, TX, USA 2UT Southwestern Medical Center,Dallas, TX, USA
Introduction: Fetuses with venolymphatic malformations of the face and neck (VLMFN) are often referred to fetal centers for advanced imaging, consultation, and management. Post natal staging systems have suggested that anatomical involvement of the larynx, tongue and upper airway by the VLMFN correlates with a long-term need for tracheostomy. The purpose of this study is to determine if fetal antenatal MRI images can similarly be applied to predict postnatal airway outcomes in affected children.
Methods: After IRB approval, a retrospective review of all fetuses evaluated for VLMFN at our fetal center was performed. Antenatal MRI images were reviewed and a stage assigned based on anatomical findings in accordance to a published VLMFN staging system. Stage 1: no evidence of polyhydramnios with free egress of amniotic fluid and clear visualization of the aryepiglottal folds and larynx. Stage 2: lesions of the tongue or epiglottis but with normal aryepiglottal folds without polyhydramnios. Stage 3: lesions of the tongue or larynx; non-visualization of the aryepiglottal folds without free egress of amniotic fluid along with polyhydramnios. Postnatal findings on laryngoscopy and bronchoscopy (DLB) and long-term airway outcomes were compared with published outcomes according to the stage
Results: Thirteen fetuses with VLMFN were identified. Six fetuses met stage 1 criteria on antenatal imaging. None had undergone an EXIT. Postnatal evaluation revealed an uninvolved airway. No child subsequently developed airway involvement by the malformation. Two fetal images met stage 2 criteria. Both fetuses had been treated by EXIT and intubated via rigid bronchoscopy. A DLB was performed. One child had minimal involvement of the upper airway by the lesion, was extubated and has had no subsequent airway issues noted. The second child had involvement of the tongue and larynx and a tracheostomy was performed. This child has remained cannulated at follow up. Five fetuses were assigned stage 3. All had been delivered by EXIT and successfully intubated. Postnatal DLB and MRI in all 5 cases showed involvement of the upper airway by the lesion and were managed with tracheostomy. All children requiring a tracheostomy remain cannulated due to persistent symptomatic lesions noted at follow up DLB (RR 4.0 Cl 1.2-13.3). Median follow up was 4 years (range 2-7 years).
Conclusion:While numbers are small, these data suggest that anatomical details obtained by antenatal fetal MRI appear to correlate with previously published staging systems that predict short and long term airway outcomes in children affected by a VLMFN. This information may be useful when counseling expectant families of affected fetuses predicting need for EXIT management and guiding long-term airway expectations.