Evolving Indications for the EXIT Procedure: The Usefulness of Combining Ultrasound and Fetal MRI

Lymphangiomas are benign vascular malformations of the lymphatic system and most commonly present in the neck area. Large lymphangiomas may compress and/or displace the larynx, trachea and esophagus and cause serious respiratory and feeding problems in neonates. Prenatal therapy could eliminate the risks of the mentioned complications. Prenatal therapy may include the EXIT (ex utero intrapartum treatment) procedure. As this procedure has certain risks for both the neonate and mother, the introduction of a safer method is justified. The use of OK-432, as a sclerosing agent, has shown positive results in several published cases of cystic hygroma, but there is no study about the prenatal use of this agent in the treatment of lymphangioma. The aim of this study was to present our experience with intrauterine intralesional injection of OK-432 in the treatment of neck lymphangiomas. Two cases of large multicystic neck lymphangiomas that were closely situated to the fetal airway were treated by single intralesional injection of OK-432. We noticed a progressive decrease in tumor volume throughout gestation. We did not experience any complications and there were no respiratory or feeding problems in the neonates. The esthetical appearance was satisfactory and both children were normal at the age of 2 years and 6 months, respectively. This report suggests that prenatal intralesional injection of OK-432 might be a safe and effective treatment in selected cases with large fetal neck lymphangiomas.

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Intrauterine Treatment of Large Fetal Neck Lymphangioma with OK-432

Mikovic¹,

Simić²,

Egić³

et al. 2009

“…However, direct visualization of the larynx and trachea and the ability to demonstrate airway patency may be challenging. Nevertheless, ultrasound is still quite suitable for visualizing secondary signs of aerodigestive tract obstruction such as polyhydramnios, the absence of a stomach bubble or to diagnose a decrease in fetal swallowing using color Doppler ultrasound [12] . The use of fetal MRI provides a more comprehensive field of view, and with excellent contrast resolution from T 2 -weighted sequences, a more accurate assessment of airway compression can be made.…”

Section: Discussionmentioning

confidence: 99%

Severe Micrognathia: Indications for EXIT-to-Airway

Morris

Lim²,

Elluru³

et al. 2009

The ex utero intrapartum treatment (EXIT) procedure has become an important management option in cases of fetal airway obstruction. Select cases of severe micrognathia may be candidates for EXIT-to-Airway due to high-risk of airway obstruction at birth. Here we present three successful EXIT-to-Airway procedures for the management of congenital micrognathia in its most severe manifestations. Case 1: A 23-year-old G3P1011 with a pregnancy complicated by severe micorgnathia, jaw index <5th percentile, as well as polyhydramnios. At 36 weeks EXIT-to-Airway was performed utilizing a bronchoscopically positioned laryngeal mask airway (LMA) during 23 min of uteroplacental support followed by tracheostomy. Case 2: A 26-year-old G4P0120 with a pregnancy complicated by severe micrognathia, jaw index <5th percentile, and an obstructed oropharynx associated with polyhydramnios. At 37 weeks EXIT-to-Airway was performed with placement of tracheostomy. Case 3: A 36-year-old G6P3023 with fetal magnetic resonance imaging (MRI) revealing esophageal atresia, polyhydramnios, and severe micrognathia with a jaw index <5th percentile. At 35 weeks the patient underwent EXIT-to-Airway with formal tracheostomy during 35 min of uteroplacental bypass. In the most severe cases of fetal micrognathia, EXIT-to-Airway provides time to evaluate and secure the fetal airway prior to delivery. We propose indications for EXIT-to-Airway in micrognathia to include a jaw index <5%, with indirect evidence of aerodigestive tract obstruction such as polyhydramnios, glossoptosis or an absent stomach bubble.

“…Prenatal assessment of fetal lung lesions is optimized by combining ultrasound findings (LHR, CVR) and fetal magnetic resonance imaging (MRI) findings (lesion volume and lung volume) [17] . The CVR is a measure of the CCAM volume adjusted for gestational age [18] , and may be useful in identifying other high-risk fetal lung lesions.…”

Section: Discussionmentioning

confidence: 99%

Giant Pulmonary Sequestration: The Rare Case Requiring the EXIT Procedure with Resection and ECMO

Mychaliska¹,

Bryner²,

Nugent³

et al. 2009

Although most prenatally diagnosed pulmonary sequestrations (PS) are asymptomatic, large lesions are associated with pleural effusions and pulmonary hypoplasia. We present the first reported case of a prenatally diagnosed giant extralobar pulmonary sequestration that required the ex utero intrapartum treatment (EXIT) procedure with resection and extracorporeal membrane oxygenation (ECMO). We discuss the compelling rationale for performing EXIT-resection-ECMO in the setting of a large thoracic mass and anticipated severe respiratory failure at birth.