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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Rev. chil. pediatr. vol.91 no.3 Santiago jun. 2020

http://dx.doi.org/10.32641/rchped.vi91i3.1385 

CLINICAL CASE

Ex-Utero Intrapartum Treatment for airway management in congenital giant neck masses

Jaime Orrego G.1 

Juan Camilo Mosquera-Hernández2 

Santiago Ardila-Giraldo2 

Laura Torres-Canchala3 

Edgar Alzate4 

Juan Pablo Benavidez5 

1 Newborn Unit, Fundación Valle del Lili, Cali, Colombia.

2 Facultad de Ciencias de la Salud, Universidad Icesi, Cali, Colombia.

3 Centro de Investigaciones Clínicas, Fundación Valle del Lili, Cali, Colombia.

4 Pediatric Surgery Service, Fundación Valle del Lili, Cali, Colombia.

5 High Complexity Obstetric Unit, Fundación Valle del Lili, Cali, Colombia.

Abstract:

Introduction:

Congenital head and neck masses are associated with perinatal asphyxia and brain injury, increasing the risk of death. The EXIT (Ex Utero Intrapartum Treatment) technique con sists of ensuring the newborn’s airway while is still receiving placental support. This technique has not been standardized in developing countries.

Objective:

To describe the clinical outcomes of two infants who underwent the EXIT technique.

Clinical Case:

We present two cases, one with lymphatic malformation diagnosed at 20 weeks of gestational age (WGE) and the second one, a preterm newborn with thyromegaly and polyhydramnios, diagnosed at 35 WGE. In both cases, during the C-section, the EXIT technique was performed with a team of a neonatologist, a gyne cologist, an anesthesiologist, a pediatric surgeon, an otolaryngologist, a nurse, and a respiratory therapist. In both patients, the neonatologist achieved to secure the airway through orotracheal intubation at the first attempt. In the first case, lymphatic malformation was confirmed and re ceived sclerotherapy, and the second one was diagnosed with congenital hypothyroidism which was managed with levothyroxine. The patients needed invasive mechanical ventilation for 7 and 9 days, respectively, and were discharged without respiratory complications.

Conclusions:

In these patients, the EXIT technique was a safe procedure, carried out without inconvenience. A multi disciplinary approach and the availability of a neonatal intensive care unit are needed to reduce potential complications and ensure postnatal management. Timely prenatal diagnosis is essential to perform this technique.

Keywords: Orotracheal Intubation; Newborn; Head and Neck Neoplasms; Ex Utero Intrapartum Treatment; EXIT

What do we know about the subject matter of this study?

The EXIT technique has shown to be a safe and effective approach in congenital conditions with risk of perinatal asphyxia due to com pression of the airway that decreases morbidity and mortality. It requires a timely diagnosis and a multidisciplinary team to carry it out.

What does this study contribute to what is already known?

This research contributes to the standardization of this technique in middle-income countries.

Introduction

As part of the congenital high airway obstruction syndrome (CHAOS), congenital head and neck masses are associated with high mortality and hypoxic- ischemic brain injury in newborns (NBs)1,2. The most frequent causes are lymphatic malformations, which occur in 1:1,775 live births3.

Congenital head and neck masses can cause severe compression and distortion of the airway, leading to high difficulty in ensuring ventilatory support at birth, increasing the risk of death or complications due to pe rinatal hypoxia. In order to prevent these outcomes, the EXIT technique (ex utero intrapartum treatment) has been proved as a safe procedure for intervening in the patient’s airway to avoid altering the tissue perfusion and oxygenation of the NB. This technique requires a multidisciplinary approach including neonatologists, otolaryngologists, anesthesiologists, obstetrician-gynecologist, pediatric surgeons, nurses, and respiratory therapists trained in maternal-fetal medical care. The refore, it is essential prenatal diagnosis through MRI4.

The EXIT technique, described by Norris et al in 19805, consists of securing the airway through orotra cheal intubation (OTI), rigid bronchoscopy or tra cheostomy (according to the severity of the obstruc tion), with no interruption of maternal-fetal circula tion through the placental support which controls the respiratory and cardiac functions6,7,8. Once the airway is secure, the umbilical cord is cut and the mother and NB are treated separately according to their require ments.

This technique was initially recommended for the management of tracheal occlusion in NB with severe congenital diaphragmatic hernias9,10 but was quickly extrapolated to patients with CHAOS or congenital lung lesions11.

Although Cruz-Martinez et al described the EXIT technique in Mexico in 201512, this procedure has not been completely standardized in middle-income cou ntries. The objective of this paper is to describe the clinical outcomes of two patients who were managed with the EXIT technique and to review the develop ment of this procedure in a tertiary referral hospital in southwestern Colombia.

Clinical Case

Case 1

Female NB, first child of a 21-year-old mother, with prenatal care started at 9 weeks of gestation. At week 20, the ultrasound scan detected a cyst-like cer vical mass. Subsequently, an MRI showed a large mass that affects the neck and the right side of the chest, extending between the ear and the right thoracic area, of 12 x 15 cm approximately (Figure 1a). In the 36th week of pregnancy, a C-section was performed and the EXIT technique was used during that procedure. The neonatologist could secure the airway through OTI on the first attempt (Figure 1b), and the patient was then transferred to the neonatal ICU.

Figure 1 a) Fetal magnetic resonance imaging (MRI) shows a large multicystic mass affecting the right neck, the supraclavicular fossa, the axillary fossa and the posterolateral wall of the right chest compatible with veno-lymphatic vascular malformation (white arrow), without evidence of airway compres sion; b) Photograph of a newborn with a veno-lymphatic malformation in the neck and left anterior chest; c) Postnatal MRI shows a large multicystic mass, affecting the posterior cervical, carotid and right retropharyngeal space, extending to the mediastinum and to the anterior and posterior right chest wall (white arrows). 

Postnatal MRI showed findings consistent with lymphatic malformation, therefore, we initiated scle rotherapy with adequate clinical response (Figure 1c). On day 7 of life, the OTI was withdrawn and on day 16, ventilatory support was also discontinued and the patient was discharged on day 21 of life without respi ratory complications.

Case 2

Male NB, second child of a 17-year-old mother, with prenatal care that started at 30 weeks of gestation. At week 33, an ultrasound scan detected mild polyhydramnios. At week 35, a neck mass with mar ked vascularization of around 4x3 cm was observed in the anterior region of the neck, from the sternum to the maxilla on the left side, causing compression of the trachea and esophagus. A C-section was indicated at 36 weeks of gestation and the EXIT technique was performed during this procedure. The neonatologist achieved OTI on the first attempt, and then the patient was transferred to the neonatal ICU for postnatal care.

MRI showed a 5x3 cm lesion causing a lobe-shaped mass effect in the pharyngeal mucosal space of the neck in the anterior region surrounding the trachea, the esophagus, and displaced bilaterally the jugular vein and carotid artery in a lateral and posterior direction without compressing the airway (Figure 2). The TSH levels presented increased T3 and decrease T4, con firming the diagnosis of congenital hypothyroidism which, associated with the significant increase in gland volume, defines the probable diagnosis of thyroid dys- hormonogenesis. Levothyroxine was indicated which shows adequate clinical response.

Figure 2 Postnatal MRI sagittal section shows important thyromegaly/goiter. 

On day 9 of life, the orotracheal tube was removed and the patient continued with non-invasive mechani cal ventilation until the 12th day. He was discharged at 14 days of life without respiratory complications and with outpatient care.

Discussion

In our patients, airway obstruction puts them at high risk of severe hypoxia or death. The implementation of the EXIT technique was the safest and most efficient option to maintain tissue perfusion to redu ce the risk of complications underlying neonatal as phyxia.

The EXIT technique was initially described in pa tients with tracheal occlusion but its use has rapidly spread including growing pulmonary malformations as congenital cystic adenomatoid malformations that can compress the remaining normal lung tissue13, laryngeal membrane, tracheal atresia or stenosis, tho racic abnormalities, among others. (Table 1) shows such malformations14,15.

Table 1 Indications to implement the EXIT technique. 

The EXIT technique should be performed under general anesthesia to ensure uterine hypotonia and thus allow adequate placental circulation, in order to reduce the risk of umbilical cord prolapse, and placen tal abruption16. This procedure requires a trained mul tidisciplinary team and the availability of a neonatal ICU8. Also, it has to be planned based on the needs of each patient and therefore early diagnosis is essential for its implementation.

In our cases, one of them had late prenatal care which increases the risk of complications due to its implementation without prior planning. (Table 2) des cribes the functions of each participating team member.

Table 2 Interdisciplinary team for the EXIT technique. 

(Figure 3) describes the algorithm of the EXIT tech nique. The surgical incision can be either midline or lower-transverse (Pfannenstiel incision) or, following hysterotomy, lower-transverse (Keer incision) where the fetus is partially delivered (head, upper chest, and at least one upper limb)4. As it is necessary to avoid work of breathing and body movements to achieve orotracheal intubation, it may be necessary the use of fetal anesthesia immediately after pulling out an upper limb, intramuscularly in the deltoid17,18. The rest of the body remains within the uterine cavity.

Figure 3 Algorithm to implement the EXIT technique. 

It is recommended to infuse Hartmann solution at 37°C continuously to preserve the volume and tempe rature of the uterus, and the use of an oximeter in the upper extremity to monitor heart rate and O2 saturation12.

Under optimal conditions, fetal oxygenation from the placenta can be extended up to 150 minutes19. OTI is the main measure of management. In the event of OTI failure, bronchoscopy and tracheotomy are the se cond and third management options, respectively20,21.

In some cases, partial or complete resection of the mass will be necessary to free the trachea from external compression to allow intubation or tracheotomy and therefore access to the airway22. In our patients, the neonatologist was able to secure the airway with OTI on the first attempt.

Once the airway is secure, the anesthesia is discon tinued, the umbilical cord is clamped and cut, and the rest of the body is delivered. Finally, the afterbirth is carried out by administering uterotonic drugs to close the uterine and abdominal walls12.

It should be noted that the EXIT technique is a transitional measure to ensure patient ventilation, the refore, the cervical mass that generates or can poten tially cause airway obstruction must be timely addres sed. MRI is the best tool to identify more clearly the patient’s condition since it allows differentiation of the altered tissue from normal one23. In the cases of this study, antenatal and postnatal MRI was performed, allowing the team to make targeted decisions (inclu ding the EXIT technique).

The EXIT technique, although safe and with a high success rate, can generate maternal-perinatal compli cations. The mother requires general anesthesia and prolonged intraoperative periods since uterine relaxa tion is necessary to maintain placental perfusion and gas exchange, which entails an increased risk of blood volume loss and hysterectomy24. In the fetus, compli cations are related to the inability to maintain utero placental gas exchange due to umbilical cord compres sion, placental abruption, or uterine relaxation17,25. Laje et al reported that 26% of patients with teratoma managed with the technique died7 and Macarthur et al reported that mortality related to this technique increases by 36% if the patients have any chromosomal abnormality26. There were no complications in the pa tients in this study.

Conclusions

The EXIT technique is an option when facing the imminent risk of perinatal asphyxia due to airway compression in the fetus. In order to implement it, a multidisciplinary team must be trained and have ac cess to technological tools that guarantee the survival of the newborn. In cases where the EXIT technique is indicated, early diagnosis of fetal conditions is essential for timely planning.

Ethical Responsibilities

Human Beings and animals protection: Disclosure the authors state that the procedures were followed ac cording to the Declaration of Helsinki and the World Medical Association regarding human experimenta tion developed for the medical community.

Data confidentiality: The authors state that they have followed the protocols of their Center and Local regu lations on the publication of patient data. exempted from obtaining an informed consent, which is recorded in the respective form.

Rights to privacy and informed consent: The authors have obtained the informed consent of the parents (tu tors) of the patients and/or subjects referred to in the article. This document is in the possession of the co rrespondence author.

Conflicts of Interest: Authors declare no conflict of interest regarding the present study.

Financial Disclosure: Authors state that no economic support has been asso ciated with the present study.

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Received: August 19, 2019; Accepted: November 17, 2019

Correspondence: Laura Torres-Canchala. E-mail: laura.torres@fvl.org.co.

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