S. R. Gillipelli1, S. E. Peiffer1, K. L. Rialon2, J. R. Bedwell2, D. K. Mehta2, T. C. Lee2, S. G. Keswani2, A. King2 1Baylor College Of Medicine, Department Of Surgery, Houston, TX, USA 2Texas Children’s Hospital, Department Of Surgery, Houston, TX, USA
Introduction: Prenatally diagnosed cervicofacial lymphatic malformations (cf-LM) may be identified on routine prenatal ultrasound, prompting consideration of ex utero intrapartum treatment (EXIT) procedure to mitigate neonatal hypoxia by securing the fetal airway while maintaining placental support. This study aims to characterize the use and neonatal outcomes of EXIT for prenatally diagnosed cervicofacial lymphatic malformations.
Methods: A retrospective single-center study of all patients who underwent EXIT-to-airway for cf-LM (2011-2020) was performed. Demographic data, prenatal findings, intraoperative details, and outcomes were analyzed descriptively (mean [range]).
Results: Six patients with prenatally diagnosed cf-LM underwent EXIT at an average gestational age of 35 [30.3-37] weeks. Prenatally, the average lesion volume on fetal MRI was 192.5 [140-255] milliliters with a mean targeted endotracheal diameter increase (TEDI) of 11.3 [3-18] millimeters. Polyhydramnios was noted in 2/6 (33%) at the time of diagnosis and 4/6 (67%) at the time of delivery. Frequent fetal imaging was used to monitor the lesions with 8 [3-19] prenatal ultrasounds obtained from initial fetal center evaluation to delivery as an adjunct to fetal MRI. Perinatally, successful intubation on placental support was achieved in all cases with an average duration of 49.5 [10-112] days. The average maternal procedure duration was 126 [98-156] minutes with maternal blood loss of 850 [100-1100] milliliters. The time from initial incision to fetal intubation was 26 [20-43] minutes and initial incision to cord clamp/delivery was 46 [31-70] minutes. Tracheostomies were placed in 2/6 (33%) patients, with one at day of life (DOL) 34 and one at DOL 99 to facilitate ongoing respiratory support. Four of 6 (67%) patients received gastrostomy tubes prior to discharge due to swallowing and feeding difficulties with a length of stay of 89 [10-204] days. All patients who survived to discharge were receiving sirolimus therapy of their cf-LM. There was one mortality at DOL 10 due to necrotizing enterocolitis totalis. Another patient experienced a complication of pneumothorax and evidence of barotrauma following EXIT requiring placement of a chest tube (duration of 8 days).
Conclusion: EXIT procedure remains a feasible strategy for mitigating neonatal hypoxia in cases of prenatally diagnosed cervicofacial lymphatic malformations. However, postnatal outcomes are variable with potential long term aerodigestive sequelae.