FETAL CERVICAL TERATOMA
CASE PRESENTATION & LITERATURE REVIEW
Bhat R, Stone PR, Pease P, Kuschel C, Hallam L.
National Women's Hospital, Claude Road, Epsom, Private Bag 92-189, Auckland, New Zealand
RenukaB@ahsl.co.nz
Background:
Fetal cervical teratomas are rare tumours (occurring with an incidence of about 1 in 40,000 live
births) that are almost always benign. Their detection in the prenatal period allows for the
anticipation of difficulties and consultation with and establishment of a multidisciplinary team.
Common obstetric complications include polyhydramnios leading to delivery, dystocia, or stillbirth.
The main immediate neonatal difficulty is in being able to establish an airway due to the effect of
the tumour mass resulting in distortion and obstruction of the larynx and trachea. Once the airway
is secured, the tumour can be assessed for its suitability for excision, often with a good outcome.
Antenatal MRI scans have been used to further define the anatomy of these tumours. Establishing
the extent of involvement of various anatomical structures may be critical to management and is
helpful in predicting the outcome.
Various strategies are available for the establishment of an airway, including the EXIT [extrauterine intrapartum treatment] procedure.
We present a case report and discussed various issues in management and a review of literature
based on a MEDLINE search from 1966 to November 2000, using the keywords cervical teratoma,
neck mass, MRI, prenatal diagnosis, EXIT procedure, fetal therapy, perinatal management.
Case:
A 26-year-old primigravida presented at 29 weeks with polyhydramnios due to a large cervical
mass causing neck extension. A small fetal stomach was visible, and normal swallowing was seen.
The polyhydramnios needed repeated amnio-reductions. This was performed after corticosteroid
administration for lung maturity. The karyotype was 46XY. Fetal echo was normal. Intermittent
fetal bradycardia was postulated to be as a result of vagal compression by the tumour. The extent
of the mass was confirmed by magnetic resonance imaging, as was the tracheal compression and
deviation. Elective delivery was planned by conventional caesarean section, as there was
hyperextension of the neck. Several discussions were held with the parents and the
multidisciplinary team regarding the method of securing the airway at delivery. The parents
declined extraordinary measures except laryngotracheal intubation or rigid bronchoscopy. An EXIT
procedure was discussed and both the parents and the paediatric surgeons and anaesthetists
considered it unnecessary. Caesarean section was performed at 36 weeks after spontaneous
rupture of membranes. Intubation was unsuccessful and the baby could not be resuscitated. Postmortem confirmed an immature teratoma. There was significant laryngotracheomalacia, which may
have contributed to the difficulty in intubation. An unusual finding in our case was the association of
pulmonary hypoplasia; the combined weight of the lungs was 27 grams (normal range 46 +/- 16).
Discussion:
Cervical teratomas are complex anomalies that require a multidisciplinary team approach for the
best chance of successful management. 167 cases of fetal cervical teratomas have been reported
till 1990. Polyhydramnios is associated with 20% of tumours. 30% of fetuses die of airway
obstruction at birth. Only 2 cases of pulmonary hypoplasia associated with cervical teratomas
have been reported. The cause for this is unclear. There have been no comparative trials
between the EXIT procedure and laryngo tracheal intubation at birth. Our case draws attention to
the presence of pulmonary hypoplasia and its impact on the immediate outcome. This can be
crucial to the success of the EXIT procedure.