Seminars in Pediatric Surgery 22 (2013) 44–49

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Ex Utero Intrapartum Therapy

Julie S. Moldenhauer, MDn
The Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania

ar ticle inf o abs t rac t

The Ex Utero Intrapartum Therapy (EXIT) procedure was initially developed to secure the airway in
Keywords: fetuses at delivery after they had undergone in utero tracheal occlusion for congenital diaphragmatic
Congenital cystic adenomatoid hernia. Indications for the EXIT procedure have been expanded to include any delivery in which
malformation prenatal diagnosis is concerned for neonatal airway compromise, such as large neck masses and
Congenital diaphragmatic hernia Congenital High Airway Obstruction Syndrome, or when a difficult resuscitation is anticipated such as
Congenital High Airway Obstruction with large lung lesions. Uteroplacental blood flow and gas exchange are maintained through the use of
Syndrome inhalational anesthetics to allow optimal uterine relaxation with partial delivery of the fetus and
Ex Utero Intrapartum Therapy (EXIT)
amnioinfusion to sustain uterine distension. Using the EXIT procedure, sufficient time is provided on
Fetal neck mass
placental bypass to perform life-saving procedures such as bronchoscopy, laryngoscopy, endotracheal
Placental bypass
intubation, tracheostomy, cannulation for extracorporeal membrane oxygenation, and resection of lung
masses or resection of neck masses in a controlled setting, thus avoiding a potential catastrophe.
& 2013 Elsevier Inc. All rights reserved.

Introduction Technical aspects of the EXIT procedure

Prenatal imaging has allowed accurate diagnosis of fetal Prenatal evaluation

anomalies that can impact the neonatal airway at delivery. The
early recognition of potential airway compromise permits altera-
tions in delivery management, such as ensuring the presence of
neonatal resuscitation teams that can be life-saving. Cesarean
delivery to facilitate fetal/neonatal resuscitation on placental
support was described in early reports to manage airway obstruc-
tion.1,2 The utility of halothane to promote uterine relaxation for
fetal tracheal intubation on uteroplacental circulation has also
been described and highlighted the need for prolonged uterine
relaxation.3 The EXIT procedure was initially designed to reverse
tracheal occlusion performed on fetuses with severe congenital
diaphragmatic hernia with additional indications described shortly
thereafter.4–6 With the EXIT procedure, uteroplacental blood flow
and gas exchange are maintained through the use of inhalational
anesthetics to allow optimal uterine relaxation with partial delivery
of the fetus and amnioinfusion to sustain uterine distension. This
additional time on placental bypass is used to perform life-saving
procedures such as bronchoscopy, laryngoscopy, tracheostomy,
cannulation for extracorporeal membrane oxygenation, resection
of lung masses, or resection of neck masses in a controlled setting,
thus converting a potential catastrophic emergency into a planned
procedure. This review is based on our clinical experience with more
than 90 EXIT procedures at the Center for Fetal Diagnosis and
Treatment of the Children’s Hospital of Philadelphia.
Accurate prenatal diagnosis is essential to identify any mater-
nal/fetal pair that are potential candidates for the EXIT procedure.
Making the diagnosis early in gestation is also important so that
the mother can be referred to a tertiary care center that is not
only capable of performing an EXIT, but also has in place the
detailed imaging studies that are crucial for perioperative plan-
ning. Optimal imaging includes high resolution fetal sonography,
three dimensional fetal sonography, ultrafast fetal magnetic
resonance imaging, and fetal echocardiography. Amniocentesis for
fetal karyotype is recommended. Additional genetic studies may be
warranted depending on the presence of associated findings. Care
must be taken to assess the placenta, as abnormal placentation such
as placenta previa or evidence of subchorionic hemorrhage might
increase the risk of intraoperative complications. Proper planning
and execution of an EXIT procedure involves a very large multi-
disciplinary team as outlined in Table 1. The team is also responsible
for providing patient education and ensuring parental understand-
ing of the risks, benefits, and potential outcomes associated with the
EXIT procedure.
Procedure

The EXIT procedure is not simply a ‘‘fancy’’ cesarean delivery.

n
It involves the use of general anesthesia, prolonged partial
Corresponding author at: Julie S. Moldenhauer, MD, The Center for Fetal
Diagnosis and Treatment, Children’s Hospital of Philadelphia, 34th St & Civic
delivery of the fetus, and a large multidisciplinary skilled team.
Center Boulevard, Philadelphia, PA 19104-4399. In contrast, cesarean delivery is most commonly performed using
E-mail address: moldenhauerj@email.chop.edu regional anesthetic with complete and rapid delivery of the

1055-8586/$ - see front matter & 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1053/j.sempedsurg.2012.10.008
 J.S. Moldenhauer / Seminars in Pediatric Surgery 22 (2013) 44–49 45

Table 1 Preoperative ultrasound is performed to confirm fetal position

The EXIT team. and placental margins. This allows for planning the approach to
laparotomy and hysterotomy. Maternal monitoring consists of
Preoperative evaluation:
High risk obstetricians continuous electrocardiography, pulse oximetry, invasive arterial
Pediatric surgeons blood pressure monitoring, and end-tidal CO2 monitoring.
Social workers Anesthesia is induced through rapid sequence technique using
Anesthesiologists an intravenous combination of thiopental, succinylcholine and
Radiologists
Fetal cardiologists
fentanyl. This is immediately followed by endotracheal intuba-
Neonatologists tion. Anesthesia is maintained using desflurane, halothane, or
isoflurane, alone or in combination, titrated to maintain uterine
Intraoperative team:
Maternal anesthesiologist relaxation. The laparotomy is performed via a low transverse
Fetal anesthesiologist abdominal skin incision. Placental location and planned approach
Scrub nurses2 to hysterotomy dictate whether the laparotomy will include
Circulating nurses2 muscle dividing or midline fascial entry. Once the uterus is
Airway cart nurse
Maternal-fetal medicine specialists
exposed, sterile intraoperative ultrasound is performed to con-
Fetal cardiologist for intraoperative echocardiography firm fetal position and map the margins of the placenta. Special
Pediatric surgeons considerations to investigate prior to the hysterotomy include
Obstetrician breech presentation requiring cephalic version, anterior placenta
ECMO team
without an adequate placenta-free window necessitating poster-
Neonatal team: ior or fundal uterine entry, or amnioreduction in the presence of
Neonatologist massive polyhydramnios to prevent rapid decompression of the
Surgical advanced practice nurses
Neonatal nurses
uterus and more accurately delineate placental margins. Needle
Second operating room team on standby in adjacent OR aspiration of large cystic lesions or fetal ascites can also be
performed to assist in the manipulation of the fetus. The hyster-
otomy is typically made in the lower uterine segment in a
neonate, and a much smaller surgical team. The goal of the EXIT transverse fashion. However, this is commonly extended into
procedure is to preserve uteroplacental blood flow for a pro- the more muscular, active portion of the uterus. If this occurs, the
longed period of time so that a surgical procedure can be safely mother should be counseled to avoid labor in any subsequent
performed on the fetus. This is achieved through uterine relaxa- pregnancy and anticipate scheduled cesarean delivery. Two full
tion and maintenance of uterine volume. High concentrations of thickness stay sutures are placed under continuous ultrasound
inhalational anesthetics in conjunction with additional tocolytics guidance in a placenta free portion of the uterus devoid of fetal
are used to provide uterine relaxation. In order to maintain parts or umbilical cord, and the amniotic cavity is entered using
uterine volume, only the head and upper torso are delivered onto the Bovie. A uterine stapling device is used to create a bloodless
the maternal abdomen (Figure 1). The lost amniotic fluid is hysterotomy as illustrated in Figure 2.9 The hysterotomy is
replaced with a continuous warmed amnioinfusion of physiolo- extended to adequately and easily deliver the fetal head, neck
gical solution to maintain uterine volume and prevent placental and upper torso without tension on the hysterotomy incision that
separation (Table 2). would cause bleeding. In cases of large neck or oral masses, the
Details of the EXIT procedure have been described else- hysterotomy might be quite large to accommodate delivery
where.5–8 In brief, the patient is positioned on the operating without interrupting the hemostatic stapled hysterotomy edges.
table in the supine position with a leftward tilt to displace her The fetus is then partially delivered up to the level just above
uterus off of her underlying vena cava, improving preload and the umbilicus and appropriately positioned for further proce-
avoiding hypotension. Pneumatic compression boots are placed. dures. An amnioinfusion catheter is placed in the uterus and
warmed physiologic solution is continuously infused to maintain
uterine volume. Intramuscular vecuronium, fentanyl and atropine
are given to the fetus to ensure adequate anesthesia. Peripheral

Table 2
Essential elements of the EXIT procedure.

Uterine relaxation
Inhalational agents
Tocolytic agents

Maintenance of uterine volume

Amnioinfusion
Partial delivery

Fetal
Anesthetic
Blood product availability
Echocardiography
Intravenous access
Pulse oximetry

Maternal
Fig. 1. Overview of the EXIT procedure. The head and upper torso of the fetus are Arterial line
delivered while the abdomen and legs remain within the uterus. Amnioinfusion Blood product availability
catheter is in place. Team members are simultaneously positioning the fetus and ECG
placing an IV in the right arm. Pulse oximetry covered by aluminum foil has been End-tidal CO2
placed on the left hand. An airway has been established and the endotracheal tube Pulse oximetry
is in place, prior to initiating resection of the fetal lung lesion.
46 J.S. Moldenhauer / Seminars in Pediatric Surgery 22 (2013) 44–49
Fig. 2. Use of the uterine stapling device to create a bloodless hysterotomy. Note
the cut edge of the uterus is hemostatic.
intravenous access is obtained while a pulse oximeter is placed
on the fetal hand and covered with aluminum foil. Fetal heart rate
and hemoglobin saturation are then continuously monitored by
pulse oximetry. Fetal cardiac function is continuously monitored
by sterile intraoperative fetal echocardiography. The need for
fetal medications, intravenous volume and blood transfusion is
determined by alterations in cardiac function and the intraopera-
tive findings.
The fetal procedure is initiated after appropriate positioning.
Provided that uteroplacental circulation remains intact, the range
of procedures that can be performed during an EXIT procedure
includes direct laryngoscopy, rigid or flexible bronchoscopy,
tracheostomy, resection of neck, oropharyngeal, or thoracic
masses, and cannulation for ECMO. Regardless of the indication
for EXIT, the fetal airway should always be secured as an initial
step, in case the procedure is abandoned early due to a complica-
tion such as placental abruption. Once the airway is established,
surfactant is administered through the endotracheal tube or
tracheostomy if clinically indicated or gestational age is appro-
priate. Hand ventilation is then initiated using a Mapleson bag
and circuit guided by pulse oximetry readings. Finally, if possible,
umbilical arterial followed by venous lines are inserted using an
‘‘intracath’’ technique prior to cord clamping. In anticipation of
cord clamping and delivery of the baby, coordination between the
surgical, obstetric, anesthetic and neonatal teams is crucial to
optimize neonatal resuscitation and minimize maternal bleeding.
This involves a decrease in the amount of inhalational agents,
oxytocin bolus infusion, and uterotonic agents to the ready. The
newborn is taken to an adjacent resuscitation room and stabi-
lized. The need for additional neonatal surgery is assessed at that
time, and an adjacent operating room and team is set up. Delivery
of the placenta and assessment of uterine tone dictate further use
of uterotonic agents and prolonged continuous oxytocin infusion.
After closure of hysterotomy and laparotomy, the maternal
inhalational agent is discontinued, then glycopyrrolate and neos-
tigmine are given. Once the mother resumes spontaneous
respirations, adequate ventilation, and return of protective air-
way reflexes, she is extubated. Maternal postoperative care is
similar to that after cesarean delivery.
Special considerations
There are multiple points throughout the EXIT procedure for
potential complications that must be anticipated and contingency
plans made. Because of the normal physiologic adaptations in
pregnancy, the potential for maternal anesthetic complications
must always be considered. An edematous airway might make
intubation difficult. Increase in gastric reflux and the gravid
uterus place the mother at increased risk for aspiration pneumo-
nitis. The thrombophilic state of pregnancy combined with a
prolonged surgical procedure places the mother at risk for venous
thromboembolic events, thus pneumatic compression boots are
indicated until the patient is fully ambulatory. Maternal hypoten-
sion related to general anesthetic commonly requires titration of
phenylephrine to maintain adequate blood pressure for uteropla-
cental perfusion and avoidance of fetal hypoxia, and thus a
maternal arterial line is helpful for monitoring purposes.
The use of deep general anesthesia to maintain uterine
relaxation is a set up for potential postpartum hemorrhage. It is
imperative that at the time of cord clamping, the inhalational
anesthetic is decreased and any additional tocolytics are discon-
tinued to allow restoration of normal uterine tone and minimize
maternal blood loss. The empiric use of an oxytocin bolus
followed by continuous infusion helps to restore uterine tone.
Uterine massage, additional uteronics such as methylergonovine
and carboprost or misoprostol and placement of a B-Lynch suture
may be required to decrease maternal hemorrhage due to uterine
atony. Hysterectomy to control bleeding would be reserved as a
life-saving maneuver. Because of the potential for postpartum
hemorrhage, blood products should be readily available if needed.
Maternal outcomes associated with the EXIT procedure compared
to cesarean delivery showed higher estimated blood loss at the
time of procedure (1104 mL vs 883 mL, p o 0.001), longer surgical
operating time (110 min vs 57 min, p o 0.0001) and higher rate of
wound complications (15% vs 2%, p ¼ 0.02).10 However, there
were no differences in hematocrit level change, total postoperative
infection rate, endometritis rate, or length of stay.
Fetal bradycardia or cardiac dysfunction can occur at any time
during the EXIT procedure. This can be directly related to the
procedure being performed and transient, such as with the
mechanical compression associated with excision of a large lung
lesion. The deep general anesthetic that the mother receives
crosses the placenta and can cause fetal cardiac dysfunction,
requiring alterations in dosing of anesthetic, maternal intrave-
nous fluid boluses, or direct administration of medication/fluid to
the fetus. Acute changes in fetal heart rate prompt immediate
investigation, as cord compression can be quickly relieved by
fetal repositioning or increased amnioinfusion. Poor fetal cardiac
function as a result of hypovolemia can be ameloriated with a
fetal transfusion, therefore warm fetal blood products should be
readily available.
Patients should always be counseled about the possibility of
an unsuccessful EXIT procedure and fetal/neonatal demise.
Maternal anesthetic complications, placental abruption, or fetal
distress might result in abandonment of the procedure. Inability
to establish an airway is also a possibility. In these scenarios an
emergent situation is created. Therefore, a second operative team
is available for neonatal resuscitation in an adjacent operating
room while the first team remains with the mother.
Indications for the EXIT procedure
Although initially developed to establish an airway in fetuses
that had undergone in utero tracheal occlusion for the treatment
of severe congenital diaphragmatic hernia, the indications for the
EXIT procedure have expanded. Any fetus with a prenatal
diagnosis that is consistent with potential airway compromise
or cardiorespiratory instability at birth are potential candidates
for the EXIT procedure. This would include large neck masses
 J.S. Moldenhauer / Seminars in Pediatric Surgery 22 (2013) 44–49
such as cervical teratomas or lymphangiomas, oropharyngeal
masses, severe micrognathia, Congenital High Airway Obstruction
Syndrome, large lung lesions, large mediastinal masses, special
circumstances with thoracoomphalopagus conjoined twins, and
EXIT-to-ECMO for severe congenital diaphragmatic hernia, among
others.
Lung lesions
The antenatal natural history of lung lesions such as congeni-
tal cystic adenomatoid malformation (CCAM), bronchopulmonary
sequestration (BPS), or hybrid of the two, can be quite variable,
and are discussed elsewhere in this issue of Seminars in Pediatric
Surgery. In some instances there is rapid growth in the midtri-
mester that can result in fetal hydrops and intrauterine fetal
demise, and in other cases the lesion appears isoechoic on third
trimester ultrasound and cannot be identified.11–13 The mass
effect from these large lesions can result in polyhydramnios from
altered fetal swallowing, hypoplasia of the normal lung tissue,
and vena caval compression and cardiac compression leading to
hydrops.14,15 The CCAM volume ratio (CVR) was developed to
stratify those fetuses that would be at high risk to develop
hydrops or neonatal respiratory compromise.16
At CHOP, the use of EXIT for delivery is determined by the
continued presence of a large lesion with mass effect such as
polyhydramnios, mediastinal shift, or impending hydrops at a
late gestational age. In these situations, difficult resuscitation is
anticipated and the use of the EXIT procedure can facilitate
stabilization and resection of the lung lesion on placental bypass.
In a review of 9 patients undergoing resection of lung lesions
during the EXIT procedure at CHOP, overall survival was 89%.17
The mean gestational age was 35.4 weeks and the mean CVR was
2.2 at EXIT. Average time on placental bypass was 65 min.
Continuous intraoperative fetal echocardiography was used to
facilitate fetal intraoperative management and 6 fetuses received
fluid supplementation, blood transfusion or additional medica-
tions. Four neonates required ECMO. The mean number of
ventilator days was 34 and the average length of stay was 60
days. Infants with high risk lung lesions have age-appropriate
neurodevelopmental scores on longer term follow-up.18 Our
current experience with the EXIT procedure for lung masses is
22 cases with 20 survivors.
Congenital High Airway Obstruction Syndrome (CHAOS)
The prenatal diagnosis of CHAOS is made when the combina-
tion of large echogenic lungs, flattened or inverted diaphragms,
dilated airways distal to the obstruction, and fetal ascites and/or
hydrops is seen.19 Complete or nearly complete intrinsic airway
obstruction in the fetus prevents egress of lung fluid from the
tracheobronchial tree resulting in this constellation of findings.
The etiology for the obstruction includes laryngeal atresia, lar-
yngeal web, tracheal atresia, and laryngeal cyst.
CHAOS is a rare condition, although the true incidence is
unknown. A thorough fetal examination looking for any asso-
ciated anomalies is warranted in a case of suspected CHAOS.
Presumed to be a sporadic malformation with unknown recur-
rence risk, additional findings increase the likelihood of an
underlying syndromic etiology. Additional findings raise the
suspicion for Fraser syndrome, an autosomal recessive disorder
characterized by cryptophthalmos, cutaneous syndactyly, mal-
formations of the larynx and genitourinary tract, craniofacial
dysmorphism, orofacial clefting, mental retardation, and muscu-
loskeletal anomalies.19–21
47
With complete fetal airway obstruction, hydrops with massive
ascites can develop due to cardiac compression from the hyper-
plastic lungs and from lymphatic obstruction. Outcome asso-
ciated with CHAOS was previously thought to be lethal.19
However, review of the antenatal natural history of CHAOS
suggests that hydrops can be tolerated in utero for a prolonged
period of time and delivery via EXIT can salvage these neonates.22
The EXIT procedure allows for adequate time on placental bypass
to perform bronchoscopy and secure the airway, typically
through the use of tracheostomy.
Resolution of hydrops from decompression through a tracheo-
laryngeal or a tracheoesophageal fistula can occur in the third
trimester. Close sonographic surveillance is warranted through-
out gestation monitoring for worsening or improvement of fetal
status. In a series of 12 fetuses diagnosed with CHAOS at CHOP,
8 had accompanying hydrops at initial imaging.23 Three fetuses
were terminated, 2 died in utero, and 1 with multiple anomalies
died at birth. Six delivered via EXIT, 5 of 6 survived the neonatal
period, and 4 survived over 1 year. The 3 fetuses that displayed
improvement on serial prenatal ultrasound findings due to a tiny
tracheolaryngeal fistula were discharged home by 2–10 weeks of
age. This study highlights the need for detailed prenatal imaging
to rule out associated anomalies that will likely worsen the in
utero and neonatal course. In addition, serial imaging can be
used to predict neonatal outcomes and aid in determining
which fetuses with CHAOS would benefit from EXIT. Certainly,
fetal imaging studies must demonstrate the presence of a
dilated distal trachea for the EXIT approach to be a therapeutic
consideration.
Neck mass
Airway obstruction due to large neck masses is a life-
threatening situation at delivery. The masses can cause extrinsic
compression and significantly distort the anatomy, resulting in
the inability to establish an airway and associated high mortality.
Prenatal diagnosis of giant neck masses, including cervical ter-
atomas, lymphangiomas, and goiters among others, and subse-
quent EXIT delivery have dramatically improved outcomes in this
group of patients. Particular challenges in this group of patients
come from the mass effect that these masses have. Esophageal
compression alters fetal swallowing, leading to polyhydramnios
and an increased risk for preterm labor and delivery. Prematurity
can be further complicated by accompanying pulmonary hypo-
plasia from abnormal positioning of the fetal airways and
compression of the lungs in the apices of the chest. Amnioreduc-
tion and/or needle aspiration of cystic portions of giant neck
masses can help to facilitate fetal positioning, restore more
typical anatomy, and improve delineation of the placental mar-
gins. When performing an EXIT procedure for a giant neck mass,
it is imperative that thorough prenatal imaging using ultrasound
and fetal magnetic resonance imaging occurs to delineate the
anatomy and predict the course of the airway. Contingency plans
to obtain the airway should be thought out ahead of time, as the
anatomy is commonly distorted and the carina may be displaced
as superiorly as the suprasternal notch. Figure 3 illustrates an
EXIT procedure for a giant cervical teratoma.
In a series of 19 neck masses delivered at CHOP and reported
in 2003, airways were successfully established in 18 of 19
neonates.24 The diagnoses consisted of 10 teratomas, 7 lymphan-
giomas, 1 foregut duplication involving the tongue, and 1 giant
goiter. An airway was established in 68% of patients using direct
laryngoscopy or rigid bronchoscopy. Tracheostomy was per-
formed at EXIT in 3 and 2 underwent retrograde endotracheal
intubation through a temporary tracheostomy after partial mass
48 J.S. Moldenhauer / Seminars in Pediatric Surgery 22 (2013) 44–49
Fig. 3. EXIT for large cervical teratoma. (A) Preoperative view of the large cervical
teratoma. (B) Neck exploration during the EXIT procedure showing the severe
deviation of the trachea. (C) Postoperative view after resection of the teratoma in
an adjacent operating room. (D) Pathology specimen.
resection. Per parental request, attempts to establish an airway
were abandoned after failed orotracheal intubation in the 1 non-
survivor with a gigantic cervical lymphangioma involving the
face, airway, and neck. The long-term survival in this series was
16/18 or 89%.
Our more recently reported experience with the EXIT proce-
dure for 17 cases of giant cervical teratoma in 2012 has also
shown very promising results.25 In this series, polyhydramnios
was present in 82% of cases and 3 patients underwent intrao-
perative amnioreduction. The median gestational age at delivery
was 35 weeks and the median birth weight was 2.5 kg. The
airway was established via direct laryngoscopy/bronchoscopy in
8 patients (47%) and via surgical exploration — either tracheost-
omy or retrograde intubation — in 9 patients (53%). No fetus died
during the EXIT procedure. The overall neonatal mortality rate
was 23%. Four patients died before resection due to severe
pulmonary hypoplasia that resulted from the upward traction
by the mass on the airway with compression of the lungs on the
thoracic apex. Seven patients had resection of the tumor imme-
diately after EXIT and 6 had delayed resection.
EXIT-to-ECMO
Because of the high likelihood of neonatal cardiorespiratory
compromise associated with severe congenital diaphragmatic
hernia, the EXIT-to-ECMO strategy has been used in this popula-
tion. Prenatal prediction for the severity of diaphragmatic hernia
in our experience has shown that a lung/head circumference ratio
(LHR) o 1 has been associated with a survival of 35% and ECMO
requirement of 75%.26 Those with an LHR 4 1 had a survival rate
of 75% and an ECMO rate of 49%. An intrathoracic liver was
associated with a survival rate of 45% and an ECMO rate of 80%.
The low survival and high ECMO rate among those with severe
CDH defined by LHR o 1 and intrathoracic liver, made this group
a potential cohort for neonatal transition via the EXIT procedure.
An earlier review of the use of EXIT-to-ECMO for severe con-
genital diaphragmatic hernia, defined by liver herniation and LHR
o1.4, percentage of predicted lung volume o15, and/or con-
genital heart disease, showed an overall survival of 64%.27Follow-
up to this study by the same authors in a more recent cohort of
patients with severe CDH, defined as o15% predicted lung
volume, revealed no benefit and therefore the recommendation
for EXIT-to-ECMO in this setting could not be made.28 The
management of CDH remains very challenging and there has
been no clear optimal care defined for this high risk population.
EXIT-to-ECMO has also been described as the intermediate for
stabilization prior to resection of lung lesions.29
Additional Indications for EXIT
Improved prenatal diagnostic techniques are identifying more
potential indications for the EXIT procedure. These include uni-
lateral pulmonary agenesis with airway stenosis, bridge to
separation for thoracoomphalopagus conjoined twins in which
one twin will have cardiovascular deterioration immediately after
delivery which jeopardizes the salvageable twin, severe micro-
gnathia, resection of large mediastinal masses, and oropharyngeal
masses, among others.24,30,31
Conclusion
Delivery via the EXIT procedure should be considered when
prenatal diagnosis raises the concern for neonatal airway com-
promise or cardiorespiratory instability. Careful orchestration of
maternal and fetal care through an experienced, multidisciplinary
team allows adequate time on placental bypass to optimize the
outcome for both. Thus the EXIT procedure changes a potential
catastrophic situation into a controlled, life-saving surgical
approach to delivery and neonatal transition.
 J.S. Moldenhauer / Seminars in Pediatric Surgery 22 (2013) 44–49
References
1. Langer JC, Tabb T, Thompson P, et al. Management of prenatally diagnosed
tracheal obstruction: access to the airway in utero prior to delivery. Fetal
Diagn Ther. 1992;7(1)12–16.
2. Catalano PJ, Urken ML, Alvarez M, et al. New approach to the management of
airway obstruction in ‘‘high risk’’ neonates. Arch Otolaryngol: Head Neck Surg.
1992;118:306–309.
3. Schulman SR, Jones BR, Slotnick N, et al. Fetal tracheal intubation with intact
uteroplacental circulation. Anesth Analg. 1993;76:197–199.
4. Harrison MR, Adzick NS, Flake AW, et al. Correction of congenital diaphrag-
matic hernia in utero VIII: response of the hypoplastic lung to tracheal
occlusion. J Pediatr Surg. 1996;31:1339–1348.
5. Mychaliska GB, Bealer JF, Graf JL, et al. Operating on placental support: the ex
utero intrapartum treatment procedure. J Pediatr Surg. 1997;32:227–231.
6. Liechty KW, Crombleholme TM, Flake AW, et al. Intrapartum airway manage-
ment for giant fetal neck masses: the EXIT (ex utero intrapartum treatment)
procedure. Am J Obstet Gynecol. 1997;177:870–874.
7. Bouchard S, Johnson MP, Flake AW, et al. The EXIT procedure: experiences and
outcomes in 31 cases. J Pediatr Surg. 2002;37:418–426.
8. Hedrick HL, Flake AW, Crombleholme TM, et al. The ex utero intrapartum
therapy procedure for high-risk fetal lung lesions. J Pediatr Surg. 2005;40:
1038–1044.
9. Bond SJ, Harrison MR, Slotnick RN, et al. Cesarean delivery and hysterotomy
using an absorbable stapling device. Obstet Gynecol. 1989;74:25–28.
10. Scully Noah MM, Norton ME, Sandberg P, et al. Short-term maternal outcomes
that are associated with the EXIT procedure, as compared with cesarean
delivery. Am J Obstet Gynecol. 2002;186:773–777.
11. Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: manage-
ment and outcome. Am J Obstet Gynecol. 1998;179:884–889.
12. MacGillivray TE, Adzick NS, Harrison MR, et al. Disappearing fetal lung lesions.
J Pediatr Surg. 1993;28:1321–1325.
13. Winters WD, Effmann EL, Nghiam HV, et al. Disappearing fetal lung masses:
importance of postnatal imaging studies. Pediatr Radiol. 1997;27:535–539.
14. Thorper-Beeston JG, Nicolaides KH. Cystic adenomatoid malformation of the
lung: prenatal diagnosis and outcome. Prenat Diagn. 1994;14:677–688.
15. Adzick NS. Management of fetal lung lesions. Clin Perinatol. 2003;30:481–492.
16. Crombleholme TM, Coleman B, Hedrick HL, et al. Cystic adenomatoid
malformation volume ratio predicts outcome in prenatally diagnosed
cystic adenomatoid malformations of the lung. J Pediatr Surg. 2002;37:
331–338.
49
17. Hedrick HL, Flake AW, Crombleholme TM, et al. The ex utero intrapartum
therapy for high-risk fetal lung lesions. J Pediatr Surg. 2005;40:1038–1044.
18. Danzer E, Siegle J, D’Agostino JA, et al. Early neurodevelopmental outcome of
infants with high-risk fetal lung lesions. Fetal Diagn Ther. 2012;31:210–215.
19. Hedrick MH, Martinez Ferro M, Filly RA, et al. Congenital high airway
obstruction syndrome (CHAOS): a potential for perinatal intervention.
J Pediatr Surg. 1994;29:271–274.
20. Slavotinek AM, Tifft CJ. Fraser syndrome and cryptophthalmos: review of the
diagnostic criteria and evidence for phenotypic modules in complex mal-
formation syndromes. J Med Genet. 2002;39:623–633.
21. Schauer GM, Dunn LK, Godmilow L, et al. Prenatal diagnosis of Fraser
syndrome at 18.5 weeks’ gestation, with autopsy findings at 19 weeks. Am J
Med Genets. 1990;37:583–591.
22. Lim FY, Crombleholme TM, Hedrick HL, et al. Congenital high airway
obstruction syndrome: natural history and management. J Pediatr Surg.
2003;38:940–945.
23. Roybal JL, Liechty KW, Hedrick HL, et al. Predicting the severity of congenital
high airway obstruction syndrome. J Pediatr Surg. 2010;45:1633–1639.
24. Hedrick HL. Ex utero intrapartum therapy. Semin Pediatr Surg. 2003;10:
190–195.
25. Laje P, Johnson MP, Howell LJ, et al. Ex utero intrapartum treatment in the
management of giant cervical teratomas. J Pediatr Surg. 2012;47:1208–1216.
26. Hedrick HL, Danzer E, Merchant A, et al. Liver position and lung-to-head ratio
for prediction of extracorporeal membrane oxygentation and survival in
isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol.
2007;197(422)e1–e4.
27. Kunisaki SM, Barnewolft CE, Estroff JA, et al. Ex utero intrapartum treatment
with extracorporeal membrane oxygenation for severe congenital diaphrag-
matic hernia. J Pediatr Surg. 2007;42:98–106.
28. Stoffan AP, Wilson JM, Jennings RW, et al. Does the ex utero intrapartum
treatment to extracorporeal membrane oxygenation procedure change out-
comes for high-risk patients with congenital diaphragmatic hernia? J Pediatr
Surg. 2012;47:1053–1057.
29. Kunisaki SM, Fauza DO, Barnewolt CE, et al. Ex utero intrapartum treatment
with placement on extracorporeal membrane oxygenation for fetal thoracic
masses. J Pediatr Surg. 2007;42:420–425.
30. Morris LM, Lim FY, Elluru RG, et al. Severe micrognathia: indications for EXIT-
to-Airway. Fetal Diagn Ther. 2009;26:162–166.
31. Ayres AW, Pugh SK. Ex utero intrapartum treatment for fetal oropharyngeal
cyst. Obstet Gynecol Int. 2010;2010:273410. doi:10.1155/2010/273410 [Epub
2010 Jan 20].