Dr. Bhavana GirishDr. Manisha Madhai Beck Dr. Manisha Madhai Beck is working as Associate Professor and Head of Unit at Christian Medical College, Vellore. Having completed her MBBS and MD from Delhi University, she trained in Fetal Medicine at the Wellington Regional Fetal Medicine Centre at Wellington, New Zealand, between 2012 and 2014. She also trained in fetal imaging at the Bangalore Fetal Medicine Centre and has been accredited by the Fetal Medicine Foundation, UK, for anatomy scans. Her areas of interest include prenatal diagnosis, genetics, fetal imaging, perinatal medicine and high risk obstetrics. She has several publications to her name, both in international and national journals. She is currently the Secretary of Vellore Obstetric and Gynaecology Society.
A 23-year-old primigravida was referred to our hospital at 36 ? 5 weeks gestation with scan showing gross polyhydramnios and fetus having a large neck mass. She had no antenatal risk factors. An anomaly scan done at 22-week gestation was told to be normal.
Repeat scan done here showed gross polyhydramnios
with the amniotic fluid index (AFI) being 44 cm. The fetus
was growth restricted with an estimated fetal weight of
1.8 kg. There was a 8 9 6 cm left anterolateral mass on
the fetal neck, which was solid, well defined with minimal
vascularity (Fig. 1). There seemed to be no retrosternal
extension of the mass. There was no other structural
abnormality to account for the fetal growth restriction
(FGR) with polyhydramnios. Her glucose tolerance test at
fifth month was normal.
Based on the above scan findings, a probable diagnosis
of cervical teratoma or lymphangioma was made.
Compression of the esophagus due to the neck mass was thought to be the cause of gross polyhydramnios. Fine needle aspiration biopsy of mass was not carried out since, being an invasive procedure, it might initiate labor. Magnetic resonance imaging (MRI) was not performed since the neck mass appeared benign on scan. The presence of polyhydramnios resulted in excessive fetal movements which would have made MRI technically challenging.
Since there was a high probability of hypoxia of the newborn after birth due to huge mass obliterating the airway, the decision to deliver the fetus using EXIT procedure was made.
A multidisciplinary team, comprising of senior obstetricians, neonatologists, pediatric anesthetists and pediatric otolaryngologists, was set up. Following several meetings, a detailed algorithm of steps to secure the fetal airway was formulated. Plan was made to attempt endotracheal intubation followed by rigid bronchoscopy, and tracheostomy was kept as a rescue plan. The time limit for each procedure was set at 5, 10 and 15 min after the partial delivery of the fetus.
A detailed consent was obtained from the parents regarding the risks involved, including risks of perinatal death (in case airway could not be secured) and/or fetal brain damage. The maternal complications such as postpartum hemorrhage, need for massive blood transfusion and ICU (intensive care unit) care were also explained. Four units of whole blood were arranged preoperatively. A mock drill was carried out by the team a day prior to surgery, during which details such as position of the personnel and equipments, patient’s position and movement of doctors according to planned sequence were discussed. Severe fetal bradycardia (fetal heart rate B80 beats per minute) and/or evidence of placental separation were accepted as thresholds for abandoning the procedure.
General anesthesia (GA) was planned for the mother
along with invasive arterial blood pressure monitoring and
adequate intravenous access. Uterus was kept relaxed with
two minimal anesthetic concentration (MAC) of isoflurane.
Additionally, terbutaline (250 mcg subcutaneously) and
magnesium sulfate were required to maintain uterine
relaxation.
Laparotomy was done using Pfannenstiel incision. Once
the desired relaxation was achieved, the uterus was opened
by low transverse incision, well away from the placenta.
The uterus was opened cautiously such that the bag of
membranes was left intact. Controlled artificial rupture of
membranes (ARM) was done using spinal needle to avoid
sudden decompression of the uterus which would accelerate
placental separation.
The fetal head and the upper torso of the fetus, including the right upper arm, were carefully delivered by the obstetricians. The lower torso, cord and the placenta were left inside the uterine cavity to keep the uteroplacental circulation going. Amnioinfusion, using 1 l of warm ringer lactate, was carried out to maintain the uterine volume. A pulse oximeter was connected to the right hand of the fetus to check the oxygen saturation levels. A neonatologist monitored the fetal heart by continuous auscultation during the entire procedure.
The fetal airway was secured by the coordinated efforts of the pediatric anesthetist, otolaryngologists and neonatologists. Since the mass impinged on the field, the mass was lifted laterally by one of the assistants, after which the airway could be visualized (Fig. 2). An endotracheal tube size 3 mm was negotiated through the airway, successfully in the second attempt by direct laryngoscopy. The position of the ET tube was checked using rigid bronchoscope and measuring the ET CO2 (end-tidal carbon dioxide levels). It took 7 min to secure the fetal airway.
Once the airway was secured, the rest of the fetal body
was delivered. Cord was clamped and cut, and baby was
handed over to the neonatologists. The isoflurane was
immediately stopped, and intravenous oxytocin infusion
was started, using 20 units of oxytocin in 500 ml of normal
saline. Placenta was allowed to separate spontaneously,
and uterotonics were given prophylactically to help contract
the uterus. Fortunately, our patient did not have much
blood loss. The total blood loss during the procedure was
700 ml.
The girl baby, weighing 1.8 kg at birth was immediately
shifted to level 3 nursery. The cord pH was 7.25 with a
base deficit of -8.7. Baby had an MRI which confirmed
the prenatal findings, and a differential diagnosis of soft
tissue sarcoma, undifferentiated teratoma or neuroblastoma
was suggested. Biopsy, however, showed malignant rhabdoid
tumor. Due to poor prognosis associated with the condition, parents opted for palliative care. Baby died on
Day 5 of life.
The EXIT procedure is a life-saving procedure for delivery of fetuses with airway obstruction. When such fetuses are delivered by traditional lower segment Caesarean section (LSCS) perinatal death and/or hypoxia are likely to occur, due to lack of airway. In the EXIT procedure, the partially delivered fetus is maintained on placental circulation till its airway is established [1]. The benefits of the EXIT procedure to the fetus have to be balanced against the maternal risks involved [2].
EXIT procedure, in contrast to the conventional LSCS, requires the use of general anesthesia for uterine relaxation. This is of paramount importance in preserving the uteroplacental gas exchange, while attempts are being made to secure the fetal airway. This, in turn, may lead to uterine atony and may lead to massive obstetrical hemorrhage [3]. Despite these theoretical concerns, our patient had blood loss of only 700 ml. A review of the EXIT procedure has also found the risks of PPH to be more theoretical than real [1].
The neonatal outcome following EXIT procedure depends mainly on size, location and underlying etiology of neck mass [4]. In some cases, early neonatal death has been reported in spite of successful establishment of the airway due to associated pulmonary hypoplasia [5]. In our case, although the EXIT procedure was successful in securing the complicated airway, we could not salvage the baby due the presence of malignant neck tumor.
Conflict of interest: Manisha Beck, Ekta Rai, Reeta Vijayaselvi, Mary John, Naina Picardo, Sridhar Santhanam, Maneesh Kumar and Bejamin Ross declare they have no conflict of interest.
Ethical Statements: All procedures performed on our patient were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendment or comparable ethical standards.
Informed Consent: Informed consent was obtained from the patient whose details are included in the case report. Additional consent was obtained for publication of scan images and the photograph of the baby.
