Ex utero intrapartum treatment in the management of giant cervical teratomas

PurposeThe purpose of this study is to present the outcome and technical details of the Ex Utero Intrapartum Treatment (EXIT) procedure performed in the management of the fetus with a giant cervical teratoma.MethodsA retrospective review of the medical records of patients undergoing the EXIT procedure between September 1995 and September 2010 was performed.ResultsEighty-seven EXIT procedures were performed. In 20% of cases (17/87), the indication was giant cervical teratoma. There were 10 females and 7 males. Polyhydramnios was present in 82%. Median gestational age at EXIT was 35 weeks (range, 30-39 weeks). Median birth weight was 2.5 kg (range, 1.7-3.7 kg). Access to the airway under placental support was established in all cases via direct laryngoscopy/bronchoscopy in 8 patients (47%) and via surgical exploration (tracheostomy or retrograde intubation) in 9 patients (53%). The mortality rate under placental support was zero. Seven patients had the tumors resected immediately after the EXIT, 6 patients had the resection later, and 4 patients died before resection. The neonatal mortality rate was 23% (4/17 patients). Patients who died had severe pulmonary hypoplasia that resulted from the upward traction by the giant cervical mass on the airway and compression of the lungs against the thoracic apex.ConclusionsWe conclude that the EXIT procedure continues to be the optimal delivery strategy for patients with prenatally diagnosed giant cervical teratomas and potential airway obstruction at birth. A thorough evaluation of the prenatal images and an experienced multidisciplinary team are key factors for an effective approach to the obstructed fetal airway.     

Spectrum of intrapartum management strategies for giant fetal cervical teratoma

Background/Purpose: The management of the fetus with a large neck mass that obstructs the airway remains a clinical challenge. The authors review their experience with giant fetal cervical teratoma and discuss options for management. Methods: A retrospective review of all patients referred since 1994 for prenatal management of a fetal neck mass was performed. Variables examined included gestational age at diagnosis and delivery, size and location of the neck mass, presence of fetal hydrops, associated anomalies, management methods, operating time, and outcome. Results: Seven patients were identified with a prenatal diagnosis of giant cervical teratoma. Four patients had fetal hydrops; of these, 2 died in utero of hydrops, and a third fetus underwent elective termination. The remaining hydropic and previable fetus underwent fetal surgery for resection of the mass. The 3 nonhydropic patients underwent ex utero intrapartum treatment (EXIT) procedures for airway control. Endotracheal intubation was possible in one patient, and one received a tracheostomy. In the third fetus, neither intubation nor tracheostomy were possible, and resection of the neck mass was performed on placental support. There were no deaths in the surgical group. Conclusions: The management of fetal giant cervical teratoma includes a spectrum of options. For the rare previable fetus with hydrops, fetal resection may be indicated. In patients with airway obstruction, EXIT procedure provides the luxury of time to obtain airway control either by intubation, tracheostomy, or, if necessary, tumor resection on placental support. J Pediatr Surg 38:446-450.     

Two cases of congenital airway obstruction managed with ex utero intrapartum treatment procedures: anesthetic implications

An ex utero intrapartum treatment (EXIT) procedure provides sufficient time to gain control of the potentially obstructed fetal upper airway while uterine placental circulation is maintained during cesarean section. We report 2 cases in which fetal congenital upper airway obstruction was managed without complications during EXIT procedures. We also discuss general considerations concerning the obstetric patient and the performance of intramuscular fetal anesthesia. Before the hysterotomy, sevoflurane at 1.5 minimum alveolar concentration was administered to assure sufficient uterine relaxation during EXIT. The 2 parturients remained hemodynamically stable during the procedure and uterine and placental perfusion was adequate. Nasotracheal intubation was possible in 1 fetus after a cervical mass was dissected. In the other, a tracheostomy was created. After the umbilical cord was clamped, the concentration of sevoflurane anesthetic gas was reduced and oxytocin and methylergometrine were administered to induce adequate uterine contractions within a few minutes. Both neonates survived the EXIT procedure with no complications.     

Perinatal management of congenital oropharyngeal tumors: The ex utero intrapartum treatment (EXIT) approach

Purpose

To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT).

Methods

We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012.

Results

Four patients were included in the series, three females and one male. The diagnoses were epignathus (n = 2) and congenital epulis (n = 2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185 min (range: 166–281) and median maternal operative time was 99 min (range: 85–153). Median maternal blood loss was 550 ml (range: 350–2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18–66). Mean fetal birth weight was 2.7 kg (range: 2.4–3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities.

Conclusions

We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities.     

Anesthetic management of the ex-utero intrapartum treatment (EXIT) procedure for giant epignathus and of the tumor excision

We report an anesthetic management of the ex-utero intrapartum treatment (EXIT) procedure performed in a fetus with giant epignathus due to laryngeal atresia at 28 weeks' gestation. Anesthesia of the mother was induced with thiamylal and vecuronium, and maintained with 4% sevoflurane in 100% oxygen before delivery. Sevoflurane provided excellent uterine relaxation. To maintain the arterial pressure, the patient received acetate Ringer and ephedrine 4mg. After hysterotomy, a pulse oxymeter and an ultrasound transducer were applied to monitor fetal Sp(O2) and heart rate. No anesthetic agents were injected into the fetus in addition to transplacental sevoflurane. Tracheostomy was performed on the fetus by pediatric surgeons on placental support. The uterine tone improved soon after discontinuing sevoflurane, intramyometrial injection of oxytocin and ergometrine infusion after delivery. Excision of the tumor was performed on day 2 of life. Pediatric surgeons tried to excise it totally, but it was hard to differentiate the tumor from the normal tissue, and partial excision was performed. After the excision, the neonate weighed 944 g and excised specimen weighed 253 g. Though the neonate was immature and the tumor was very large, no perioperative complications were associated with EXIT and the tumor excision.     

A case of laryngeal atresia (congenital high airway obstruction syndrome) with chromosome 5p deletion syndrome rescued by ex utero intrapartum treatment

The authors report a case of laryngeal atresia (congenital high airway obstruction syndrome [CHAOS]) that was diagnosed prenatally. The patient underwent successfully tracheostomy by ex utero intrapartum treatment (EXIT). The fetal ultrasonography and magnetic resonance imaging MRI showed a typical CHAOS pattern with expanded hyperechogenic lungs, inverted diaphragms, and a dilated trachea. Recently, 3 cases of prenatally diagnosed CHAOS were reported to be treated successfully by EXIT. The clinical manifestation and course of this case was not similar to these 3 cases. The 3 previous patients did not fare as well during gestation and were delivered earlier than that in our case. In our case, fetal hydrops was seen at 23 gestational weeks, but it gradually subsided and disappeared at 30 gestational weeks. The fetus was stable and well. After delivery at 39 weeks, the baby received respiratory assistance by ventilator assistance. After 3 days, she could breath well on her own. The patient also had chromosome 5p deletion syndrome and perineal groove. More experience in treating CHAOS cases with EXIT to fully estimate its clinical course and prognosis is needed.     

Anesthetic management of the exit (ex utero intrapartum treatment) procedure

The EXIT (ex utero intrapartum treatment) procedure is used to maintain fetal-placental circulation during partial delivery of a fetus with a potentially life-threatening upper airway obstruction. We performed the EXIT procedure on a fetus with a large intra-oral cyst. Sevoflurane was used as the anesthetic because of its rapid titratability. Sevoflurane provided excellent maternal and fetal anesthesia. Modifications to previously described monitoring techniques for the EXIT procedure were also used.     

Anesthetic management for EXIT (ex-utero intrapartum treatment) of a twin gestation: one normal and one with a large epignathus

We experienced cesarean section of a twin gestation of which one was normal and the other had a large epignathus. Anesthesia was induced with rapid administration of propofol and suxamethonium, then her trachea was intubated and operation was started. Anesthesia was maintained with 3% sevoflurane in 100% oxygen. Five minutes from the start of the surgery, the first baby was born with Apgar score of 3/7. One minute later, the head of the second with large epignathus was out of the uterus. But we judged that her trachea was difficult to intubate, but she showed spontaneous respiration. Then we inserted a tracheal tube (ID 2 mm) to her nose for the airway, and she was carried into the infant warmer after amputating the umbilical cord. After the delivery, sevoflurane was discontinued, and propofol, fentanyl and ketamine were started. Cesarean section was finished with total blood loss of 1,900 g including amniotic fluid. In the infant warmer, tracheostomy was performed on the second baby, and the baby was carried to NICU. Thirteen days later, the epignathus was extracted without complication. General anesthesia with inhalational agent is usually chosen for cesarean section with EXIT. For EXIT, uterine relaxation and fetal akinesia are necessary, but deep inhalational anesthesia causes massive bleeding, hypotension and loss of spontaneous respiration of the fetus. Twin gestation with one normal and the other with airway trouble has many problems. We have to consider mother, normal baby and troubled baby during EXIT. We should treat them appropriately with prudence.     

Remifentanil-nitroglycerin combination as an anesthetic support for ex utero intrapartum treatment (EXIT) procedure

A 21 year old healthy parturient was diagnosed as having a fetus with micrognathia. She was scheduled for an ex-utero intrapartum treatment (EXIT) procedure. General anesthesia consisted of remifentanil, nitrous oxide, and midazolam. Intravenous nitroglycerin was used for uterine relaxation. This technique offered hemodynamic stability and provided uterine relaxation that may be rapidly terminated. For the fetus, it provided excellent transplacental anesthesia. Supplemental direct fetal anesthesia is recommended to prevent the rapid decline of analgesia/anesthesia that occurs after placental separation.     

Advances in obstetric anesthesia: anesthesia for fetal intrapartum operations on placental support

Fetal intrapartum operations on placental support (OOPS), also known as ex-utero intrapartum treatment (EXIT) procedures, are very rare (and often challenging) surgical techniques designed to allow partial delivery (cesarean section) of a fetus with a potentially difficult airway, with subsequent management of the neonatal airway (direct laryngoscopy, fiberoptic bronchoscopy, or tracheostomy) while oxygenation is continuously maintained via the placenta (on placental support). The peripartum management of pregnant women and their fetuses undergoing OOPS is very complex and multidisciplinary, and differs greatly from that of standard cesarean sections. The goal of this article is to review the current recommendations for the peripartum anesthetic management of pregnant women carrying fetuses with fetal congenital malformations undergoing OOPS. Presented in part at the XVIII FIGO World Congress of Gynecology and Obstetrics in Kuala Lumpur, Malaysia, on November 8, 2006, in the session entitled “Labor and Delivery.”     

Neuraxial anesthesia in ex utero intrapartum therapy for parturients with fetal congenital diaphragmatic hernia: a prospective observational study

BackgroundCongenital diaphragmatic hernia (CDH) is characterized by defects in the fetal diaphragm and thoracic herniation of the abdominal viscera. The ex utero intrapartum treatment (EXIT) procedure is used to establish the fetal airway while on placental support. These EXIT procedures are commonly performed under general anesthesia, which increases maternal bleeding and the risk of insufficient placental perfusion subsequently. This study investigated the feasibility of performing neuraxial anesthesia for the EXIT procedure for fetal congenital diaphragmatic hernia to improve outcomes.MethodsParturients with fetal CDH who underwent an EXIT procedure between January 2019 and May 2021 in our institution were recruited. Variables evaluated included gestational age, surgical time, intra-operative blood loss, peri-operative hemoglobin, maternal complications, fetal lung-to-head ratio, time on placental bypass, and postnatal outcome.ResultsTwenty-two cases were included. All procedures were performed under neuraxial anesthesia. The median gestational age at the time of the EXIT procedure was 37 weeks. The median estimated blood loss was 200 mL. There was no report of an adverse maternal event. The placental bypass time was 142.9 ± 72.6 s, and access to the airway was successfully established within the bypass time. Twenty-one neonates reached an Apgar score of 9 at 5 min. In the first two hours after birth, the average pH of neonatal peripheral arterial blood was 7.35 ± 0.07 (n=19), and lactate level 1.85 ± 0.71 mmol/L (n=19).ConclusionsIn the EXIT procedure to establish an airway for fetal CDH, neuraxial anesthesia proved a feasible technique for maternal anesthesia.     

Four cases of the ex utero intrapartum treatment (EXIT) procedure: anesthetic implications

The ex utero intrapartum treatment (EXIT) procedure is a method of maintaining utero-placental circulation during cesarean section to gain time to secure a potentially obstructed fetal airway. Four cases of the EXIT procedure are described with special reference to the maternal anesthetic technique. Deep volatile anesthesia (approximately 2 MAC) with isoflurane or sevoflurane for a prolonged period of time, in three cases in combination with an intravenous nitroglycerin infusion, was used to ensure a fully relaxed uterus during the procedure. All mothers were maintained hemodynamically stable with preserved utero-placentary perfusion. It was possible to intubate the tracheas of two fetuses, whereas in the other two tracheostomies had to be performed. Fetal gas exchange was not negatively affected during the EXIT procedure as evidenced by normal blood gas values in the umbilical artery at the time of delivery. After reducing the concentration of volatile anesthetic, delivery of the neonate and administration of oxytocin, uterine contractility was promptly re-established and there were no signs of uterine atony in the postoperative period. All four neonates survived the procedure without complications.     

The EXIT procedure: experience and outcome in 31 cases

Background: The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. Methods: A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Results: Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 [plusmn] 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 [plusmn] 38.5 beats per minute and 71.2% [plusmn] 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO₂ were, respectively, 7.20 [plusmn] 0.11 and 63.2 [plusmn] 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 [plusmn] 574.1 mL. Conclusion: The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.     

Ex-utero intrapartum treatment procedure for giant neck masses--fetal and maternal outcomes

Background/Purpose

For fetuses with giant neck masses and tracheal obstruction, an ex-utero intrapartum treatment (EXIT) procedure allows for safe nonemergent airway management while on placental support. Our objective was to examine fetal and maternal outcomes after EXIT procedure specifically for giant neck masses.

Methods

The medical records of all patients referred to a comprehensive fetal center for a giant neck mass between 2001 and 2010 were reviewed retrospectively.

Results

Among 24 patients referred, an EXIT procedure was performed in 12 with evidence of tracheal compression. An EXIT procedure was not performed because of minimal tracheal involvement (n = 8), elective abortion (n = 2), fetal demise (n = 1), or obstetric complication (n = 1). In all fetuses, the airway was successfully secured; tracheal intubation was achieved with rigid bronchoscopy (n = 10), direct laryngoscopy (n = 1), and tracheostomy (n = 1). Eleven patients survived to discharge, whereas 1 patient with significant pulmonary hypoplasia died 8 days after emergency EXIT procedure. Of 11 surviving infants, 10 are neurodevelopmentally intact. All mothers who desired future pregnancies have subsequently had uncomplicated deliveries (n = 6).

Conclusions

Ex-utero intrapartum treatment procedure for giant neck mass can be performed safely for both mother and child. Most fetuses can be orotracheally intubated with minimal long-term morbidity. The potential for future pregnancies is preserved.     

The role of the surgeon in the case of a giant neck mass in the EXIT procedure

Large fetal neck masses can present a major challenge to securing an airway at birth, with associated risks of hypoxia, brain injury, and death. The authors report a case of a giant neck mass, diagnosed in a fetus of 28 weeks, treated through ex utero intrapartum treatment procedure to assist in securing an airway followed by excision of the mass on the day after delivery. A multidisciplinary team approach, combined with an accurate prenatal diagnosis obtained through fetal ultrasound magnetic resonance imaging examination, was the key to a successful outcome. The role of the pediatric surgeon was initially to secure the airways through a tracheostomy followed by excision of the mass when the infant's vital parameters had been stabilized.     

O desafio multidisciplinar da anestesia para procedimento intraparto extra‐uterino: relato de caso

The Ex‐ Utero Intrapartum Treatment (EXIT) is a surgical procedure performed in cases of expected postpartum fetal airway obstruction, allowing the establishment of patent airway while maintaining placental circulation. Anesthesia for EXIT procedure has several specific features such as adequate uterine relaxation, maintenance of maternal blood pressure fetal anesthesia and fetal airway establishment. The anesthesiologist should be aware of these particularities in order to contribute to a favorable outcome. This is a case report of an EXIT procedure performed on a fetus with a cervical lymphangioma with prenatal evidence of partial obstruction of the trachea and risk of post‐delivery airway compromise.     

分娩期子宫外产时治疗的麻醉一例

报道一例产前超声诊断胎儿颈部肿块,成功进行剖宫产同时施行子宫外产时治疗（EXIT）的麻醉处理。关注术中麻醉管理,保障EXIT的施行确保母婴安全。     

Verifying correct endotracheal intubation by measurement of end-tidal carbon dioxide during an ex utero intrapartum treatment procedure

The ex utero intrapartum treatment (EXIT) procedure provides time to secure the airway of the fetus while utero-placental circulation supplies the fetus with oxygen. We report the anesthetic management of a fetus with a large neck mass during an EXIT procedure in which the confirmation of correct endotracheal intubation was hampered by parts of the mass, blood, and other fluids. The use of a standard end-tidal carbon dioxide probe provided a reliable signal and proved the endotracheal position of the tube while utero-placental circulation was still intact.     

Die EXIT-Prozedur als anästhesiologische Herausforderung

The ex-utero intra-partum (EXIT) procedure enables the surgeon to perform invasive procedures in an infant during cesarean section before clamping the umbilical cord. Specific anesthesiological concepts are necessary for ensuring sufficient umbilical perfusion. We report the case of a 33-year-old female undergoing cesarean section in the 36th week of pregnancy because of a large fetal cervical tumor. The EXIT procedure was performed in order to secure the infant's airway during delivery. The anesthesiological management and interdisciplinary tasks are discussed in the literature review.     

Airway management for neonates requiring ex utero intrapartum treatment (EXIT)

In utero congenital malformations in the fetus can occasionally lead to an obstructed airway at birth accompanied by hypoxic injury or peripartum demise, without intervention. Ex utero intrapartum treatment (EXIT) may help reduce morbidity and mortality associated with challenging airways by providing extra time on uteroplacental circulation to secure the airway. Meticulous preparation and planning are crucial for this procedure. Many different types of congenital malformations can result in a difficult airway, but there is no correlation between specific malformations and a required type of airway intervention. Based on our experience and literature review, an airway process flow diagram has been created to help assist teams in decision‐making for airway intervention in a neonate during the EXIT procedure. The management of the airway in this scenario involves additional unique considerations that accompany handling a partially delivered newborn in the uterine environment. Extensive preparation and team rehearsal are essential to the success of this procedure.