Case report
Perinatal airway management of neonatal cervical teratomas

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Abstract

Cervical teratomas are rare but life-threatening neonatal tumors and management of the fetus with a cervical teratoma that threatens the airway remains a clinical challenge. This has been revolutionized by advances in fetal imaging and management of the airway at delivery including the use of Ex-utero Intrapartum Treatments (EXIT procedures). We present a retrospective case series of three neonates managed over a 12-month period. Following pre-natal fetal MRI and a multi-disciplinary management approach, two newborns were managed by prompt post-natal endotracheal intubation while an EXIT procedure was required in one. All three underwent surgical resection in the first few days of life. A decision regarding the best means by which to manage the airway in fetal cervical teratoma requires fetal MRI and a multi-disciplinary team approach to determine whether EXIT, or a safer approach from a maternal perspective can be employed. We also recommend routine endotracheal intubation at birth, due to the risk of spontaneous intra-tumoral hemorrhage. The need for surgery should be planned early, as rapid growth of the tumor can threaten the viability of the overlying skin and surrounding structures.

Introduction

Teratomas, etymologically ‘monstrous tumors’, are rare congenital tumors derived from pluripotent cells from all three primordial germ-cell layers. As such, they involve at least one-type of tissue originating from these three embryonic layers, each with varying degrees of differentiation. They also can include non-germ totipotential embryonic cells [1]. They are usually benign; however there have been very rare cases of malignancy or malignant transformation [2], [3], [4]. These can be from areas of germ cell or non-germinal malignant tumor patterns [5].

Teratomas can occur in almost any part of the body [6]. However, they are most commonly located in the sacrococcygeal region, followed by the ovaries, testis, anterior mediastinum, retroperitoneum, and finally the head and neck which account for less than 5% of the total [7]. Cervical teratomas have a nearly 100% mortality due to airway complications if not pro-actively managed, due in part to enlargement of the lesion in the first few days of life [8], [9], [10]. Poor prognostic indicators on pre-natal sonography include: large size (>5 cm), polyhydramnios and hydrops fetalis [6], [9], [11], [12]. Management starts at the time of fetal diagnosis and involves a multi-disciplinary team including: obstetricians, sonologists, pediatric radiologists, neonatologists and pediatric surgery and otolaryngology teams. Ex-utero Intrapartum Treatments (EXIT procedures) are employed in the delivery of fetuses with large head and neck masses to obtain a stable airway while the newborn is maintained on utero-placental circulation [13]. The availability of fetal MRI allows for a more accurate assessment of the airway prior to birth, and therefore more fastidious use of intrapartum treatments.

In light of our recent experience with establishment of a multi-disciplinary fetal airway management team, we performed a retrospective review of newborns with cervical teratomas at our institution.

Section snippets

Methods

After gaining ethical approval from The Royal Children's Hospital Human Research Ethics Committee a retrospective analysis was performed by searching the theatre audit database for keyword “teratoma”. We present the three cases of cervical teratoma that were identified over the preceding twelve-month period.

Case 1

A baby girl from a 22-year-old primigravida mother who was heterozygous for hemochromatosis (carrier phenotype) was found to have a complex cervical mass that was initially detected at the 20-week antenatal ultrasound scan, measuring 45 mm × 30 mm × 34 mm. At 25 weeks the mass measured 52 mm × 43 mm × 45 mm with no evidence of obstructive polyhydramnios. Fetal MRI confirmed a displaced but non-obstructed airway. At 36 weeks the child was delivered via elective cesarean section with neonatal, anesthetic and

Discussion

Our experience with three cases of cervical teratoma underscores the importance of a multi-disciplinary approach to the management of these complex tumors. This encompasses clinical expertise in antenatal imaging, obstetric care, airway management and tumor resection. Close collaboration between radiologists, obstetricians, neonatologists, anesthetists, otolaryngologists, pediatric and plastic surgeons is essential to achieve an optimal outcome for both the mother and child. With careful

Consent

Informed consent was obtained from the patient's guardians before publication of this case series and accompanying images.

Competing interests

The authors declare that they have no competing interests’.

Acknowledgements

The authors would like to acknowledge the work of all the team members involved in the care of these children at the Royal Children's Hospital. Particularly members of the Fetal Medicine Team headed by Associate Professor Ricardo Palma Dias, Neonatal Team headed by Dr. Michael Stewart and Mr. Joe Crameri of the Department of Surgery.

References (19)

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