Lymphangioma

Marlene A. Soma and Jay Paul Willging

History

The otolaryngology team is consulted by the obstetric team about a 28-year-old P2G1A0 woman in her 34th week of pregnancy. She had developed polyhydramnios, and a screening ultrasound revealed a large cervicofacial mass of the fetus occupying mostly the anterior triangle of the neck with extension into the upper mediastinum. Magnetic resonance imaging (MRI) confirmed the presence of a multiloculated cystic mass with poorly defined borders that infiltrated the tongue base and floor of mouth. The lesion appeared to be displacing the fetal trachea and was causing compression of the fetal esophagus. Plans for an elective cesarean section delivery are made for the future with an otolaryngology team present in the delivery room to assist with management of the fetal airway.

The patient unfortunately enters spontaneous preterm labor before the scheduled delivery date. The otolaryngology team is called emergently to the operating room.

Differential Diagnosis—Key Points

Several key points in this history point to potential problems in the care of this child:

1. Airway obstruction at birth is a life-threatening condition and is associated with a high mortality rate, especially if there is delay in securing an airway or if there is inability to ventilate the neonate. Hypoxia, acidosis, and ischemic brain injury will result in significant morbidity, even death, in a child who may otherwise be normal. In the situation of antenatal diagnosis of fetal airway obstruction as in this case, a planned multidisciplinary approach to delivery is advocated to maximize the outcome for both the mother and child. An EXIT (ex utero intrapartum technique) procedure allows the fetus to be delivered with maintenance of the uteroplacental circulation until a fetal airway has been established. The obstetric team, two anesthesiology teams, otolaryngology, neonatology, pediatric surgery, maternal-fetal medicine specialists, an echocardiographer, and two full surgical scrub teams should be briefed and prepared for every contingency. Reliable maternal fetal circulation can be maintained for longer than 1 hour. If an experienced team of clinicians and surgeons is not available, a child might be delivered by cesarean section, maintaining the child at the level of the maternal heart, on placental support (operation on placental support [OOPS]). Reliable maternal fetal circulation can be maintained for only 5 minutes.

3. The EXIT procedure differs from a routine cesarean section in that the goal is to achieve a state of uterine hypotonia with preservation of uterine volume to maintain the uteroplacental circulation. A level of fetal anesthesia without cardiac depression is also desired. In contrast, during a cesarean section, the goal is to maximize uterine tone to prevent postpartum hemorrhage and to avoid fetal anesthesia and potential respiratory depression.

4. The differential diagnosis of a congenital cystic cervicofacial mass causing extrinsic airway obstruction such as described in this case includes teratoma, lymphangioma, and arteriovenous malformation. Less common lesions causing extrinsic obstruction include branchial cleft cyst, fetal goiter, sarcoma, and neuroblastoma. The term CHAOS (congenital high airway obstruction syndrome) has been used to describe fetal upper airway obstruction of an intrinsic nature. Intrinsic airway lesions include laryngeal atresia, stenosis or web, tracheal atresia or stenosis. If there is no tracheoesophageal connection, the secretions produced by the fetal lungs cannot be excreted causing the lungs to expand and the diaphragm to flatten or invert. If the esophagus is compressed or obstructed as well, fetal swallowing is impaired and polyhydramnios may result. Regardless of the precise diagnosis of the obstructing lesion, the initial approach to the airway will be the same.

5. The approach to the compromised newborn airway must be orderly. If a neonate is spontaneously breathing, the degree of airway obstruction must be assessed to determine the need for elective intubation in the delivery room. If the airway is compromised, endotracheal intubation with an armored endotracheal tube should be attempted. If the endotracheal tube cannot be passed because of a mass compressing the lumen of the trachea (most of these can be overcome with gentle pressure on the endotracheal tube), a rigid broncho-scope can be passed into the airway to offer a method of ventilation. In some situations, traction on the mass by an assistant may help to elevate it from the airway structures. With a bronchoscope in place, a tracheotomy can then be undertaken in a controlled fashion.

6. If a bronchoscope cannot be passed, a tracheotomy must be immediately performed. This may require reflection or partial re-section of a neck mass. If the cervical component of the mass precludes access to the anterior base of the neck, a thoracotomy should be performed to access the mediastinal trachea. Proper positioning of the tracheotomy between the second and third tracheal rings is important because the fetal trachea may be pulled out of the chest owing to hyperextension of the mass and neck.

7.