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.     

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.     

Case series: Combined spinal epidural anesthesia for Cesarean delivery andex utero intrapartum treatment procedure

PURPOSE: To report the use of regional anesthesia and iv nitroglycerin to provide anesthesia and uterine relaxation for three Cesarean deliveries (CD) involving ex utero intrapartum treatment (EXIT) of potentially life-threatening airway obstruction in the newborn. CLINICAL FEATURES: Case 1--a 36-yr-old woman at 38 weeks' gestation was scheduled for an elective CD for fetal skeletal dysplasia and micrognathia. Case 2--a 34-yr-old woman at 35 weeks gestation had a fetal ultrasound revealing fixed neck flexion and micrognathia consistent with fetal arthrogryposis. Case 3--a 27-yr-old woman presented at 38 weeks gestation for CD for severe fetal micrognathia, with mandibular growth below the fifth percentile. For each case, a combined spinal epidural anesthetic was performed with 0.75% bupivacaine, fentanyl and morphine intrathecally followed by placement of a multiorifice epidural catheter. Prior to uterine incision patients received a loading dose followed by an iv infusion of nitroglycerin. Uterine relaxation was sufficient in all cases for delivery of the fetus, and allowed for evaluation by direct laryngoscopy and intubation while maintaining fetal-placental circulation. The surgical procedures were completed without incident. CONCLUSIONS: Anesthesia and uterine relaxation for CD and EXIT procedures can be safely provided with regional anesthesia and iv nitroglycerin.     

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.     

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。     

Prenatal percutaneous needle drainage of cystic sacrococcygeal teratomas.

Prenatal ultrasound (US) permits in utero diagnosis of sacrococcygeal teratoma (SCT), follow-up of tumor size, and the early identification of complications, allowing for a more timely and appropriate delivery. The recommended management of large SCTs is delivery by cesarean section (CS) to prevent dystocia, tumor rupture, hemorrhage, and death. However, even delivery by CS can be difficult, necessitating a large hysterotomy that adds to maternal morbidity. The authors report two cases of cystic SCTs in which prenatal percutaneous drainage allowed for an uncomplicated vaginal delivery. In the first case, a large unilocular cystic SCT was diagnosed at 31 weeks' gestation on prenatal US. The fetal presentation was breech, and the mass was steadily increasing in size, preventing spontaneous version. At 37 5/7 weeks, the cyst was percutaneously drained under US guidance allowing for successful external version. Repeat drainage just before induction of labor permitted a successful vaginal delivery. In the second case, the cystic SCT was percutaneously drained just before induction of labor at full term, again allowing for an uncomplicated vaginal delivery. Prenatal percutaneous needle drainage of cystic SCTs offers an alternative to CS that results in decreased risks for both mother and fetus.     

Management of fetal mediastinal teratoma

Background/Purpose

Mediastinal teratomas are rare congenital germ cell tumors that prenatally can compress mediastinal structures and cause hydrops. Two possible presentations of massive fetal mediastinal teratoma include hydrops leading to fetal demise, or fetal esophageal and airway compression causing late-gestation polyhydramnios and preterm labor. The authors present 2 cases of fetal mediastinal teratoma that illustrate successful strategies for either of these presentations.

Methods

A 37-year-old woman carrying a fetus with a mediastinal mass and secondary hydrops at 23 weeks of gestation underwent in utero resection of the mass. Delivery was by cesarean delivery at 25 weeks because of preterm labor. A 24-year-old mother carrying a fetus with a mediastinal mass and severe polyhydramnios at 36 weeks of gestation underwent an ex utero intrapartum therapy procedure for establishment of an airway and tumor resection on uteroplacental support.

Results

These strategies resulted in physiologic improvement in the first case and controlled resection and resuscitation in the second. The first patient had significant sequelae of prematurity including bronchopulmonary dysplasia but is currently well at 9 months of age. The second patient is well at 1 year of age.

Conclusions

Massive fetal mediastinal teratoma can result in fetal or neonatal mortality by a variety of mechanisms. Optimal prenatal and perinatal management is required to salvage fetuses compromised by this lesion.     

Ultrasonographic guidance for location of the trachea during the EXIT procedure for cervical teratoma

Cervical teratomas are rare tumors of infancy that are associated with a high mortality rate caused by compression and distortion of the infant's airway. Recent attempts at managing the fetal airway before delivery have focused on the EXIT (ex-utero intrapartum treatment) procedure, in which time to secure an airway is provided while preserving uteroplacental gas exchange. The authors report the use of intraoperative ultrasound guidance during an EXIT procedure for an infant with massive cervical teratoma to aid in identification of the trachea.     

Anesthetic management for newborn pharyngeal teratoma]

Epignathus (pharyngeal teratoma) is a rare disease of newborns associated with a high mortality secondary to airway obstruction in the neonatal period. We report anesthetic management of a newborn with epignathus who underwent tumor resection. He was delivered vaginally at 39 weeks of gestation and Apgar scores were 9 at 1 and 5 min. The tumor originated from the palate, almost filled the oral cavity and protruded through the mouth with its external part 6 x 7 cm in size. He could breathe with the head and mass turned to the left. The excision of the tumor was scheduled on the fifth day of life. Mask ventilation and laryngoscopy were considered impossible. Fiberoptic nasal intubation was successfully performed with topical anesthesia without sedation. Tumor was resected with blood loss of 103 gm. The trachea was extubated on the third postoperative day and the postoperative course was uneventful. For safe management of cases of pharyngeal teratoma, careful preoperative assessment of the airway is most important and sufficient preparation and careful intubation are mandatory to keep airway patent. The perioperative bleeding from the tumor and the airway obstruction by the tumor or its remnant after the excision could also be hazardous to the airway.     

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 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.     

Anesthesia for Ex Utero Intrapartum Treatment (EXIT procedure) in Fetus with Prenatal Diagnosis of Oral and Cervical Malformations: Case Reports

Background and objectivesFetus prenatally diagnosed with neck tumors, or with any other disease that obstructs the airways, should not be treated conventionally, as the assistant physician has to face two challenges right after the infant's delivery: the limited time to establish the access to the potentially difficult airways and the lack of anesthesia of the neonate in case of instrumentation of the airways. The ex utero intrapartum treatment, i.e., the EXIT procedure consists of maintaining the fetoplacental circulation during the cesarean section, until the airways of the fetus be secured.Case ReportsFemale patient, 37 years old, G3P2, 38 weeks pregnant, having polyhydramnios and fetus diagnosed with large cervical masses by prenatal ultrasound. A cesarean section was performed using the EXIT procedure to enable safe access to the infant's airways. After hysterotomy, the fetus was intubated by direct laryngoscopy. The neonate was immediately transferred to another operating room, where cervical tumor resection of the neck tumor and tracheostomy were successfully performed. Female patient, 27 years old, G3P1A1, 32 weeks pregnant, whose fetus was prenatally diagnosed with a large oral tumor. As the tumor obstructed the fetus’ airways, a tracheostomy was performed when the fetus underwent EXIT procedure. It was then possible to use direct laryngoscopy for neonate intubation. The fetus underwent tumor resection and was sent to the Neonatal Intensive Care Unit.ConclusionsReports describe the successful use of general anesthesia with isoflurane for cesarean delivery followed by the EXIT procedure in fetus diagnosed with tumors obstructing the airways.     

Management of a prenatally diagnosed huge teratoma arising from the soft palate

Teratomas arising from the oral cavity are relatively rare and can cause life-threatening airway obstruction immediately after birth. We report a case of a huge teratoma arising from the soft palate detected prenatally. To save the patient, a caesarean section and ex utero intrapartum treatment (EXIT) were planned at 29 weeks of gestation. However, during EXIT, the patient's cardiopulmonary status deteriorated due to unstable cord blood flow secondary to uterine contractions. EXIT was abandoned, the patient was delivered and an emergency tracheotomy performed. The tumour was successfully excised 4 hours after tracheotomy. The tumour weighed 1,591 g and was 20 x 22 x 12 cm. The patient, a female, weighed 715 g. Histopathology showed Grade II teratoma. The postoperative course was uneventful and she is now 5 years old with no neurological sequelae. EXIT is indicated in patients who have a high risk for airway obstruction immediately after birth. However, if EXIT must be abandoned, as in this case, urgent tracheotomy is mandatory. Planned prevention of airway obstruction at delivery is indispensable for successful outcome and requires excellent coordination among obstetricians, neonatologists, anaesthesiologists and paediatric surgeons.     

Antenatal detection of developing nervous system abnormalities and perinatal surgical management

As the use of prenatal ultrasound sonographic study becomes more popular, it is likely that several fetal anomalies of the central nervous system will be diagnosed in utero more frequently and accurately. Obstetric sonogram correlates well with the results of postnatal CT scanning. Now, with improved diagnosis and support facilities for the newborn, neurosurgical intervention is being requested, as more concern is expressed for such fetus. We have experienced seven cases of fetal disorders of the central nervous system-meningocele, meningoencephalocele, myeloschisis, arachnoid cyst and maximal hydrocephalus. When diagnosed late in gestation (after the 2nd trimester), it may be reasonable to consider early delivery at the time of pulmonary maturity, and extrauterine management to decrease secondary damage to the developing central nervous system. The method of delivering a fetus must be tailored to suit each case. We present our program for the management of fetal hydrocephalus-placement of a subcutaneous low profile Ommaya's reservoir for neonate immediately after birth, and then subsequently conversion to a ventriculoperitoneal shunt three or four weeks later. As advances are made in antenatal diagnostic techniques and appropriate therapy, prognostic data on congenital abnormalities of the central nervous system will increase and become more pertinent.     

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.     

Application of a fetal scalp electrode for continuous fetal heart rate monitoring during an ex utero intrapartum treatment

Fetal monitoring is required to avoid hypoxic injury during ex utero intrapartum treatment (EXIT). We performed a tracheostomy under EXIT in a case of suspected airway obstruction caused by a cervical teratoma. The scalp electrode was applied for continuous fetal heart rate monitoring. This device enabled us to promptly deal with fetal bradycardia caused by cord compression. We describe here the usefulness of the fetal scalp electrode for fetal monitoring under EXIT.     

Obstetric hemorrhage during an exit procedure for severe fetal airway obstruction

Purpose

To report a case of massive obstetric hemorrhage occurring during Cesarean delivery for an ex utero intrapartum treatment (EXIT) procedure. Methods to optimize the anesthetic, obstetric, and perinatal management are discussed.

Clinical features

A healthy parturient underwent an urgent EXIT procedure at 32 weeks gestation for a giant fetal neck mass. During the intraoperative period, severe intraoperative hemorrhage occurred from the site of the uterine incision. No evidence of placental bleeding, premature placental separation, or inadequate uterine relaxation was observed during the perioperative period. Placement of a uterine stapling device was unsuccessful in achieving adequate surgical hemostasis. Initial attempts with laryngoscopy and rigid bronchoscopy to secure the fetal airway on placental support were unsuccessful, and early termination of placental support was deemed necessary due to the severity of maternal blood loss. After full delivery of the neonate and termination of placental support, neonatal ventilation with bag-mask ventilation was achieved and successful endotracheal intubation occurred during repeat bronchoscopy.

Conclusions

The risk of obstetric hemorrhage due to uterine relaxation and inadequate surgical hemostasis in patients undergoing EXIT procedures is poorly reported. To reduce adverse maternal and neonatal outcomes, the premature termination of placental support during EXIT procedures may be required in the setting of severe obstetric hemorrhage.     

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.     

The ex utero intrapartum therapy procedure for high-risk fetal lung lesions

Background

Indications for the ex utero intrapartum therapy (EXIT) procedure have expanded to include any fetal anomaly in which resuscitation of the neonate may be compromised.

Methods

We reviewed the medical records of 9 patients after resection of lung lesions during the EXIT procedure.

Results

The mean gestational age at EXIT procedure was 35.4 weeks. All lung masses maintained large sizes late into gestation with mean mass volume/head circumference ratio of 2.5 at presentation and 2.2 at EXIT. Seven of 9 fetuses demonstrated hydropic changes (n = 6) and/or polyhydramnios (n = 5), and underwent prenatal intervention including thoracentesis, thoracoamniotic shunt placement, amnioreduction, and/or betamethasone administration. Overall survival after EXIT for lung mass resection was 89%. The average time on placental bypass was 65 minutes. Postnatal complications included reoperation for air leak (n = 1), reoperation for bleeding (n = 1), and death from sepsis and prematurity (n = 1). Venoarterial extracorporeal membrane oxygenation was used in 4 neonates for persistent pulmonary hypertension. Maternal prenatal complications included polyhydramnios (n = 5), preterm labor (n = 4), and chorioamnionitis (n = 1). One mother required perioperative blood transfusion.

Conclusion

The EXIT procedure allows for controlled resection of large fetal lung lesions at delivery, avoiding acute respiratory decompensation related to mediastinal shift, air trapping, and compression of normal lung.     

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.