J Gynecol Obstet Hum Reprod 51 (2022) 102252

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Journal of Gynecology Obstetrics

and Human Reproduction
journal homepage: www.elsevier.com

Review

Ex utero intrapartum technique (EXIT): Indications, procedure methods

and materno-fetal complications − A literature review
Andrew Spiers, Guillaume Legendre, Florence Biquard, Philippe Descamps,
Romain Corroenne*
Department of Obstetrics and Gynecology, Angers University Hospital, Angers, France

A R T I C L E I N F O A B S T R A C T

Article History: A congenital malformation of the head, neck or thorax can lead to upper airway compression with a risk of
Received 9 June 2021 asphyxia or neonatal death. To secure and protect the upper airway, the Ex Utero Intrapartum Therapy
Revised 24 September 2021 (EXIT) procedure has been developed. The procedure allows delivery of the fetus via a hysterotomy while
Accepted 6 October 2021
relying on the placenta as the organ of respiration for the fetus prior to clamping of the umbilical cord.
Available online 9 October 2021
A high level of expertise is necessary for successful completion of the EXIT procedure, which is not void of
maternal and fetal risks. In this literature review, we present the indications, procedure methods and
Keywords:
materno-fetal complications associated with the EXIT procedure.
EXIT procedure
FETO
© 2021 Elsevier Masson SAS. All rights reserved.
Airway obstruction
Tracheal occlusion

Introduction Indications

At birth, airway obstruction is associated with a high risk of neo-
natal morbidity and mortality. The Ex Utero Intrapartum Therapy
(EXIT) procedure was developed in the 1990s to reduce the risk of
airway obstruction at birth [1−3]. During a scheduled caesarean
delivery, the EXIT procedure consists of delivering the fetal head and
shoulders so that the respiratory tract can be safely liberated before
the umbilical cord is clamped, thereby utilizing the placental oxygen
perfusion [4]. The EXIT procedure may be considered in the presence
of a fetal condition that hampers the newborns ability to breathe and
to successfully adapt to extra uterine life [4]. Due to the limited num-
ber of published case reports, currently, not a single recommendation
or practice guideline is available from an international maternal-fetal
society [5].
Initially developed to remove a tracheal occlusion device, placed
in utero for the treatment of congenital diaphragmatic hernia, the
indications for EXIT procedures have been extended to include other
congenital anomalies such as Congenital High Airway Obstruction
Syndrome (CHAOS) or compressive intra- or extra-thoracic lesions
[3,6−8].
In this up to date literature review, we present the indications,
procedure methods and the maternofetal risks incurred during an
EXIT procedure.
The EXIT procedure may be considered in the presence of an
upper airway obstruction, detected in utero, which could potentially
be fatal in case of traditional delivery, where the maternal oxygen
placental support is cut off after clamping of the umbilical cord.
The procedure has three recognized indications: 1) the manage-
ment of an obstructed upper airway, 2) the resection of a compres-
sive cervical or thoracic mass, 3) miscellaneous indications where the
fetus is not expected to successfully transition to extrauterine life [9].
Although indications for EXIT procedures reported in literature per-
tain primarily to airway clearance with little mention of other indica-
tions [10].
1- Management of airway obstruction
Obstruction of the fetal airways is most often secondary to an
extrinsic compression by a cervical, pharyngeal or thoracic mass
[6,11,12]. More rarely, it may be secondary to an intrinsic stenosis
(Congenital High Airway Obstruction Syndrome [CHAOS]) or an iatro-
genic obstruction following in utero tracheal obstruction in the treat-
ment of congenital diaphragmatic hernia [10].
Extrinsic causes

Lesions are most often isolated and securing the airway at birth
* Corresponding author at: 4 rue Larrey, 49100. improves the long-term prognosis of postnatal surgical treatment
E-mail address: romain.corroenne@chu-angers.fr (R. Corroenne). which can later be performed under optimal conditions [3,13,14].

https://doi.org/10.1016/j.jogoh.2021.102252
2468-7847/© 2021 Elsevier Masson SAS. All rights reserved.
A. Spiers, G. Legendre, F. Biquard et al.
Extrinsic causes include: cystic lymphatic malformations, teratomas,
congenital salivary glands cysts, severe micrognathia, fetal goiter and
congenital epulis [15]. These malformations are often accompanied
by a hydramnios, secondary to the external compression of the upper
digestive tract which directly impairs the fetus' ability to swallow.
[16].
- Lymphatic malformations
Cystic lymphatic malformations are the second most common
neonatal soft tissue tumors after hemangiomas [17] and represent
the most frequent indication of EXIT procedures [18]. They result
from an anomaly in the lining between lymphatic vessels and the
venous circulation [18]. The morphology and severity of these lesions
varies widely [19]. In the postnatal period, macrocystic lymphatic
malformations appear as soft and depressible lesions, unlike micro-
cystic lymphatic malformations which infiltrate tissue, causing their
expansion. A risk of neonatal asphyxia exists when an increase in tis-
sue volume causes compression of the upper aero-digestive tract.
Cystic lymphatic malformations can also be accompanied by swal-
lowing, speech and dental articulation disorders in case of expansion
into the oral or pharyngolaryngeal cavities [20].
A cystic lymphatic malformation diagnosed during the second tri-
mester of pregnancy is often of latero-cervical origin and is associated
with a chromosomal abnormality in 60% of cases as well as with other
congenital malformations. These lesions are significantly associated
with hydrops fetalis and intrauterine fetal demise [21]. In contrast,
when diagnosed at a later term, during the third trimester of preg-
nancy or at birth, the lymphatic malformation is more often of ante-
rior cervical origin. This type of lesion is rarely associated with a
chromosomal abnormality and the associated mortality rate is low.
[21]
In the event of a normal fetal karyotype and in the absence of
other associated malformations, the prognosis of the EXIT procedure
appears favorable [22].
- Teratomas
The head and neck are the 4th most common location of congeni-
tal teratomas [5]. These germ cell-derived tumors are rarely malig-
nant in nature [21,23]. They appear as a very heterogeneous cystic
tumor with calcifications. Teratomas can be appear anywhere from
the mandible to the collarbone and can spread to the tongue and pal-
ate, to the mediastinum or wrap around the neck, making a tracheot-
omy at birth particularly difficult [21]. In addition, during the
postnatal period, cervical teratomas can bleed spontaneously into the
upper airways. Intubation to protect the upper airway can therefore
be necessary, even in the absence of airway compression [5]. The
complete postnatal resection of teratomas is often possible with a
good long-term prognosis, even in the presence of metastases [16].
- Congenital cysts of the salivary glands [24]
The incidence of congenital salivary gland cysts is rare, estimated
at 0.7% of newborns [24]. These collections of fluid appear secondary
to atresia of the salivary ducts of the sublinqual or submandibular sal-
ivary glands. In some cases, the cysts can be very large and occupy the
entire oral cavity, thereby obstructing the upper airway at birth
[25,26].
- Severe micrognathia
In case of severe micrognathia, secondary to hypoplasia of the
mandible, glossoptosis can obstruct the upper aero-digestive tract
making endotracheal intubation difficult at birth [27,28]. Severe
micrognathia can be isolated but is more frequently associated with
2
Journal of Gynecology Obstetrics and Human Reproduction 51 (2022) 102252
other abnormalities such as otocephaly, dysgnathia complex and
pathologies such as Pierre Robin, Treacher-Collins, Nager or velocar-
diofacial syndromes [29]. Most newborns with micrognathia do not
have an airway obstruction. In contrast, severe micrognathia with air-
way obstruction can be complicated with hypoxic/ischemic encepha-
lopathies or neonatal death [30]. The EXIT procedure is reserved for
the most severe cases of mandibular hypoplasia.
There are no radiological signs that can predict the risk of upper
airway obstruction in severe micrognathia. An EXIT procedure may
be considered in the event of a Jaw index (anteroposterior diameter
of the mandible / biparietal diameter x 100) below the 5th percentile
associated with signs of obstruction of the aero-digestive tract, such
as the absence of a visible stomach, hydramnios or glossoptosis [29].
However, management of micrognathia is evolving and in many
cases, these mothers may have a normal cesarean delivery as long as
there is a surgical, neonatal, and anesthesia team present at the deliv-
ery to manage the airway of the neonate at birth.
Intrinsic stenosis - CHAOS
CHAOS is a rare malformation, caused by the absence of the cana-
lization of the upper airway at around 10 weeks of gestation, which
can lead to intrauterine or neonatal death [31]. Upper airway
obstruction blocks the flow of fetal pulmonary fluid through the air-
ways and lungs, increasing intrathoracic pressure [32]. This increase
in pressure leads to pulmonary congestion and cardiac compression
responsible for heart failure and hydrops fetalis [33,34]. CHAOS is
defined by the triad of radiologic signs: large hyperechogenic lungs,
flattening or inversion of diaphragmatic domes and dilation of the
distal airways [35,36]. The syndrome may also be associated with
other birth defects such as esophageal atresia, anal imperforation,
renal agenesis, sexual ambiguity, hydrocephalus, anophthalmia, spi-
nal abnormalities or syndactylia or can be present in genetic syn-
dromes (Fraser, X-fragile). The presence of associated pathologies
explains the higher risk of stillborn or intrauterine fetal demise
among cases of CHAOS [34,37,35].
Morbidity and mortality have been reduced since the develop-
ment of the EXIT procedure [7,11,38−41]. However, most studies
present the pediatric outcome at an average age of 24 months and
the long-term outcome, in particular the functional prognosis
(speech, etc.), remains unknown [40].
Iatrogenic stenosis - removal of a tracheal obstruction
The EXIT procedure was originally developed for the purpose of
removing clips placed, in utero, on the trachea of fetuses with a dia-
phragmatic cupola hernia [42]. The placement of clips has been
replaced by the placement of a balloon in the trachea via fetoscopy.
This recent treatment approach is currently being evaluated in a
multi-center randomized clinical trial [43]. This treatment strategy
includes a second fetoscopy, performed at around 36 weeks of gesta-
tion, in order to deflate the balloon before birth. The EXIT procedure
remains a recourse that can be performed in emergencies, or in case
of spontaneous labor before the second fetoscopy has been per-
formed. Teams are currently working on the development of a tra-
cheal occlusion system, by balloon, that is reversible under the effect
of a magnetic field. This development would make it possible to avoid
a second fetoscopy or an emergency EXIT procedure [44].
2- Management of hemodynamically significant compressive tho-
racic tumors
The EXIT procedure can also be considered in the presence of a
large thoracic mass such as a cystic adenomatoid malformation or a
mediastinal teratoma which compresses the trachea. In this case, the
aim of the EXIT procedure is to remove the mass, taking advantage of
A. Spiers, G. Legendre, F. Biquard et al.
the placental oxygen infusion [31]. Removal of thoracic mass can
facilitate ventilation and improve venous cardiac return, two essen-
tial parameters for the success of ECMO in severe pulmonary hypo-
plasia, often present in this context [45].
3- Miscellaneous indications where the fetus is not expected to suc-
cessfully transition to extrauterine life
In certain cases of severe cardiothoracic malformations, the secur-
ing of airways by an EXIT procedure is not possible and the successful
establishment of ECMO during the procedure, allows for a graduated
and adapted postnatal treatment strategy [46−51]. Although the suc-
cess of this procedure has already been described [51], due to the rar-
ity and complexity of cases, the EXIT procedure for the purpose of
establishing ECMO in cases of large cervical mass has not been exten-
sively reported. However, it has been noted that this procedure
increases neonatal morbidity due to anticoagulation, sternotomy and
ECMO. Carrying out an EXIT procedure with the implementation of
ECMO should only be done as a last option after all other measures
have failed [51].
In cases of severe congenital diaphragmatic hernia, ECMO is
sometimes necessary and the EXIT procedure for the implementation
of ECMO has been studied in this indication. Shieh et al. compared
the neonatal outcome of children with severe congenital diaphrag-
matic hernia who received ECMO via an EXIT procedure to those who
received ECMO after birth. No significant difference in survival rate,
or pulmonary, cardiac and psychomotor development scores in the
first 6 years of life was found [49].
In exceptional cases, the EXIT procedure may be considered for
the separation of conjoined twins. Ossowski et al. presented a case of
thoracopagus twins with heart disease (single ventricle) on one of
the conjoined twins [52]. It was possible to intubate and separate the
two twins without complications during the procedure. However, in
most cases, conjoined twins are better served by delivery, stabiliza-
tion, detailed imaging studies and controlled tissue expansion proce-
dures.
Method of performing an exit procedure
Prenatal assessment
The success of the EXIT procedure is strongly correlated to the
accuracy of the diagnosis, prenatal evaluation is therefore essen-
tial. After the discovery of, or upon suspicion of, a malformation
involving the upper airways, the patient should quickly be
referred to a referral center for an ultrasound, fetal MRI and
genetic investigations [4].
Various ultrasound prognostic factors which allow for the indica-
tion of an EXIT procedure have been described: A cervical mass with
a solid component, a mass >5 cm in volume, polyhydramnios, flatten-
ing or inversion of the diaphragmatic copula and tracheal deviation
or compression [2,7,9,13−15,53−55]. Doppler ultrasound and fetal
echocardiography may also be useful in studying the vascularization
of large compressive masses and in assessing vascular repercussions
on the fetus [4,56].
Fetal MRI complements the ultrasound examination and makes it
possible to ascertain the exact location of a compressive mass, to
assess the degree of airway obstruction and to provide details in the
event of associated malformations [57−60].
The EXIT procedure should ideally be carried out between 37 and
39 weeks of gestation [53], although Novoa et al. in a literature
review of 235 procedures presented an optimal term of 35.1 weeks
of gestation [10]. The selection of potential candidates and planning
must in any case be done early in order to prepare for the procedure
before spontaneous labor.
3
Journal of Gynecology Obstetrics and Human Reproduction 51 (2022) 102252
A multidisciplinary team (2 teams)
The success of an EXIT procedure requires the presence of a large
multidisciplinary team [4]. Around twenty people are expected to be
present during the procedure: obstetricians, pediatric surgeons,
anesthesiologists, neonatologists, pediatricians, otolaryngologists
and nurses in the operating room. During the procedure, two pedi-
atric teams should be present to manage the newborn after cord
clamping.
The prior preparation of the team through simulation exercises is
essential [61].
EXIT procedure [4]
- Concerning the mother
General anesthetic by rapid sequence induction and oro-tracheal
intubation is indicated [62]. Anesthesia is maintained by volatile
halogenated anesthetic agents (desflurane, halothane or isoflurane
alone or in combination) which achieve stable uterine relaxation
throughout the procedure while maintaining optimal placental per-
fusion compared to locoregional anesthesia. Additional tocolytic
agents may be needed (indomethacin, terbutaline, nitroglycerin) [5].
Due to a high risk of maternal hemorrhage, blood supplies should be
available during the procedure [4]. Uterotonic (oxytocin) are contra-
indicated before delivery to prevent placental disruption before the
baby is intubated.
Maternal monitoring is carried out using an electrocardiogram,
pulse oximetry and invasive blood pressure monitoring, in order to
detect early hypotension secondary to concentrations of anesthetic
products, venous dysfunction or possible bleeding. If necessary
maternal blood pressure can be controlled with a vasopressor accord-
ing to institutional preferences [62].
In the operating room, the mother is placed on a gynecology oper-
ating table, allowing the operator who will perform the fetal intuba-
tion to be in place between the mother’s legs. The mother is placed in
dorsal decubitus position, slightly rolled to the left to mitigate pre-
load and avoid hypotension [4]. After performing a low transverse
laparotomy and exposing the uterus, an intraoperative ultrasound is
performed to verify the fetal position and the placental margins. In
case of hydramnios, needle drainage may be done before the hyster-
otomy, to prevent placental abruption in the event of the too rapid
emptying of amniotic fluid. The hysterotomy is facilitated by the
ultrasound-guided placement of two absorbable knots at the extrem-
ities of the intended hysterotomy site in an area away from the pla-
centa and umbilical cord. First, a small scalpel uterine incision (1
−2 cm) is made. Then a uterine stapling device is introduced into the
uterine cavity and fired to perform a wall incision uterine incision
(including the membranes) to limit bleeding. A transverse segment
hysterotomy is preferable, although the incision can be vertical on
the uterine body depending on the placental position [63]. However,
an incision on the uterine body contraindicates future vaginal deliv-
ery. After delivery of the baby, the edges of the uterine incision have
to be cut to remove the staples.
- Concerning the fetus
After the hysterotomy, the head, neck, upper torso and one arm of
the fetus are exteriorized and the head is placed in an occiput poste-
rior position. In order to maintain uterine volume, a Ringer lactate
solution at 37 °C is administered into the uterus at a free flow via an
amnio infusion catheter [4].
In addition to the anesthetics administered to the mother mater-
nal, anesthetics including Fentanyl (10 mg/kg) and verocuronium
(0.1 mg/kg) are injected into the fetal deltoid muscle.
A. Spiers, G. Legendre, F. Biquard et al.
Continuous fetal monitoring includes a sterile pulse oximetry sen-
sor placed in the hand for continuous oxygen saturation and heart
monitoring. A venous line is inserted into the exteriorized fetal arm
to obtain hemoglobin and fetal blood gas. Also, fetal echocardiogra-
phy can identify fetal heart rate, myocardial contractility, atrioven-
tricular valve competency and ductal constriction.
Algorithms designed to assist with the decision making process
of which management strategy should be considered to secure the
fetal airways have been described [15,64]. Algorithms may vary by
institution, and they have to be adapted on the pathology of each
particular fetus. Firstly, a direct laryngoscopy should be attempted
using a Miller laryngoscope. If unsuccessful, a second direct laryn-
goscopy should be attempted with a Benjamin laryngoscope, then a
rigid bronchoscopy followed by a flexible bronchoscope. If all of
these attempts fail, a tracheostomy may then be considered because.
About 60 min of time is afforded by the placenta under normal con-
ditions [4].
Once the airways have been secured and if no other surgical pro-
cedure (ECMO, resection) is planned, then manual respiration with a
sterile Mapleson breathing circuit mask should be started and the
umbilical cord clamped. Anesthetic gases and tocolytics should be
gradually reduced after cord clamping and an injection of uterotonic
(oxytocin) should be given to the mother to stimulate uterine con-
traction and prevent possible bleeding during and post-delivery.
The newborn is then cared for by the second pediatric team pres-
ent in the operating room.
Result
Although not without risk for the mother and the fetus, the EXIT
procedure is described, in literature, as "safe" when performed in a
referral center by a prepared and experienced multidisciplinary
team.
Novoa et al. recently evaluated the outcome of 235 newborns
after an EXIT procedure [10]. The study found fetal death in utero or
neonatal death in 17% of cases. The mortality rate in literature varies
from 5 to 25%, although there exists a heterogeneity in malforma-
tions, indications and postnatal care across cases [55]. The most fre-
quently reported causes of neonatal death include cardiopulmonary
arrest, pulmonary hypoplasia, and asphyxia following failed intuba-
tion or tracheostomy [53].
Compared to a traditional c-section, the EXIT procedure appears
to increase the risk of bleeding during delivery (1104 mL vs 883 mL, p
<0.01), the duration of the intervention (110 vs 57 min, p <0.0001)
and the occurrence of scar complications (15 vs 2%, p = 0.02) [65]. In
6% of cases, a transfusion is necessary during the procedure [66]. The
risk of uterine rupture in a subsequent pregnancy is not defined in lit-
erature and could be estimated at 11%, similar to that of prenatal
open-hysterotomy spina bifida surgeries [67].
Fetal complications are more frequent at around 13% and are
related to secondary cardiac effects of chest, cervical or umbilical
cord compression during or following the procedure or to the effects
related to anesthetics [10]. Spasms of the umbilical cord exposed to
temperature changes have also been described [64].
Ethics
The practice of this procedure in order to manage these high-risk
newborns raises ethical questions, since it exposes the mother to
risks of bleeding and infection. Parents should therefore be well
informed about possible maternal and fetal complications and the
unpredictable nature of the newborn's status depending on the etiol-
ogy of the airway obstruction. Indeed, the prognosis is strongly
dependent on the etiology but also on the induced prematurity. In
addition, despite successful care at birth, questions still remain about
the long-term prognosis of these children.
4
Journal of Gynecology Obstetrics and Human Reproduction 51 (2022) 102252
Most of these malformations fall into the category of fetal
anomalies that could make a pregnancy eligible for medical ter-
mination depending on local practices. The couple must therefore
be informed about the epidemiology, pathophysiology, available
treatment methods, the prognosis of these lesions, the complica-
tions to be expected and the sequelae frequently observed. If
there is a high probability that the unborn child will have a par-
ticularly serious condition deemed incurable at the time of diag-
nosis, a medical termination of pregnancy may be considered by
the couple regardless of the gestational age, depending on local
practices [68].
Conclusion
The EXIT procedure carries a risk of significant perinatal com-
plications such as neonatal death or maternal hemorrhage and
should only be considered in cases of severe upper airway
obstruction that would significantly impair oxygenation of the
newborn after clamping of the umbilical cord. This procedure
must only be carried out at a referral center by an experienced
multidisciplinary team. Larger studies should be conducted to
explore the long-term benefits of the EXIT procedure and refine
the selection of potential candidates.
Fundings
This research did not receive any specific grant from funding
agencies in the public, commercial, or not-for-profit sectors.
Declaration of Competing Interest
The authors report no conflict of interest and declare that the arti-
cle is original, unpublished and not being considered for publication
elsewhere. All authors fulfill all conditions required for authorship.
Acknowledgement
N/A
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