Liechty Kenneth W, Hedrick Holly L, Hubbard Ann M, Johnson Mark P, Wilson R Douglas, Ruchelli Eduardo D, Howell Lori J, Crombleholme Timothy M, Flake Alan W, Adzick N Scott
Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
J Pediatr Surg. 2006 Jan;41(1):230-3. doi: 10.1016/j.jpedsurg.2005.10.081.
The use of the ex utero intrapartum treatment (EXIT) procedure has salvaged many fetuses with giant neck masses. Despite an adequate airway, a subset of these patients die from an inability to achieve adequate gas exchange.
We reviewed our experience with the EXIT procedure from 1996 to 2004. The EXIT was used to deliver 23 fetuses with giant neck masses.
Three fetuses with giant cervical teratomas died of severe pulmonary hypoplasia. On postmortem, these patients had severe airway distortion by the mass. The carina was retracted superiorly to the first or second rib resulting in compression of the lungs in the apices of the chest and pulmonary hypoplasia. Hypoplasia was reflected in the lung weights of 24 vs 38 g and 17 vs 34 g for age-matched normal lung.
Unsuspected obstructive fetal neck masses can be fatal because of an inability to secure an airway. Prenatal ultrasonography can identify fetuses at risk, allowing the fetus to be salvaged using the EXIT procedure. Despite obtaining airway control, a subset of these patients will die because of pulmonary hypoplasia. When counseling patients with large cervical masses it is important to discuss potential pulmonary hypoplasia in these patients.
子宫外产时处理（EXIT）手术挽救了许多患有巨大颈部肿物的胎儿。尽管气道通畅，但这些患者中有一部分死于无法实现充分的气体交换。
我们回顾了1996年至2004年期间我们使用EXIT手术的经验。EXIT手术用于分娩23例患有巨大颈部肿物的胎儿。
3例患有巨大颈部畸胎瘤的胎儿死于严重肺发育不全。尸检时，这些患者的肿物导致严重气道变形。隆突向上回缩至第一或第二肋骨水平，导致肺尖部受压及肺发育不全。肺发育不全表现为与年龄匹配的正常肺相比，肺重量分别为24克对38克以及17克对34克。
未被怀疑的梗阻性胎儿颈部肿物可能因无法确保气道通畅而致命。产前超声检查可识别有风险的胎儿，从而通过EXIT手术挽救胎儿。尽管实现了气道控制，但这些患者中有一部分仍会因肺发育不全而死亡。在为患有巨大颈部肿物的患者提供咨询时，重要的是要讨论这些患者潜在的肺发育不全情况。
