Use of an ex utero intrapartum treatment procedure in a fetus with prenatally diagnosed neck teratoma
Background. The neck is the second most common site for fetal teratomas after the sacrococcygeal region. In cases of airway obstruction, neck teratomas have a poor prognosis for life. Ex utero intrapartum treatment (EXIT) can lower neonatal mortality with a multidisciplinary approach and an oxygenation procedure for the newborn with persistent utero-fetal circulation.Pribushenya O.V., Tarletskaya O.A., Pankratova O.A., Drozdovsky D.B., Medvedeva N.N., Petrovskaya N.V.
Case report. The paper describes a clinical case of fetal neck teratoma diagnosed at 32 weeks’ gestation and complicated by bilateral hydrothorax and polyhydramnios. Fetal magnetic resonance imaging (MRI) was performed after repeated therapeutic amniodrainage, which established changes in the anatomy of the airway with tracheal displacement and compression. The trachea was laterally displaced and only partially tracked. EXIT was recommended. An EXIT procedure with bronchoscopy was done at 35 weeks’ gestation. A live boy was born, who weighed 2660 g and had an Apgar score of 6/mechanical ventilation. Surgical removal of the teratoma was successfully performed in the baby at the age of 9 days.
Conclusion. When a neck tumor is prenatally detected, additional MRI is needed to determine the degree of airway displacement and/or obstruction and to schedule delivery, by attracting specialists rendering newborn care.
Keywords
teratoma
fetal tumor
magnetic resonance imaging (MRI)
ex utero intrapartum treatment (EXIT)
prognosis
medical genetic counseling
References
- Mota R., Ramalho C., Monteiro J., Correia-Pinto J.., Rodrigues M., Guimarães H. et al. Evolving indications for the EXIT procedure: the usefulness of combining ultrasound and fetal MRI. Fetal Diagn. Ther. 2007; 22: 107-11. https://dx.doi.org/10.1159/000097106.
- Caldeira A., Pacheco J., Fernandes S., Lanca F. The multidisciplinary challenge of anesthesia for ex utero intrapartum treatment: a case report. Rev. Bras. Anestesiol. 2020; 70: 59-62. https://dx.doi.org/10.1016/j.bjan.2019.12.010.
- Lazar D.A., Olutoye O.O., Moise K.J.Jr., Ivey R.T., Johnson A., Ayres N. et al. Ex-utero intrapartum treatment procedure for giant neck masses--fetal and maternal outcomes. J. Pediatr. Surg. 2011; 46(5): 817-22. https://dx.doi.org/10.1016/j.jpedsurg.2011.02.006.
- Laje P., Peranteau W.H., Hedrick H.L., Flake A.W., Johnson M.P., Moldenhauer J.S., Adzick N.S. Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation. J. Pediatr. Surg. 2015; 50(2): 311-4. https://dx.doi.org/10.1016/j.jpedsurg.2014.11.024.
- Novoa R.H., Quintana W., Castillo-Urquiaga W., Ventura W. EXIT (ex utero intrapartum treatment) surgery for the management of fetal airway obstruction: A systematic review of the literature. J. Pediatr. Surg. 2020; 55(7):1188-95. https://dx.doi.org/10.1016/j.jpedsurg.2020.02.011.
- Masahata K., Soh H., Tachibana K., Sasahara J., Hirose M., Yamanishi T. et al. Clinical outcomes of ex utero intrapartum treatment for fetal airway obstruction. Pediatr. Surg. Int. 2019; 35(8): 835-43. https://dx.doi.org/10.1007/s00383-019-04494-1.
- Shamshirsaz A.A., Aalipour S., Erfani H., Nassr A.A., Stewart K.A., Kravitz E.S. et al. Obstetric outcomes of ex-utero intrapartum treatment (EXIT). Prenat Diagn 2019; 39(8): 643-6. https://dx.doi.org/10.1002/pd.5477.
- Stefini S., Bazzana T., Smussi C., Piccioni M., Frusca T., Taddei F. et al. EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list. Int. J. Pediatr. Otorhinolaryngol. 2012; 76(1): 20-7. https://dx.doi.org/10.1016/j.ijporl.2011.09.015.
- Bouchard S., Johnson M.P., Flake A.W., Howell L.J., Myers L.B., Adzick N.S., Crombleholme T.M. The EXIT procedure: experience and outcome in 31 cases. J. Pediatr. Surg. 2002; 37(3): 418-26. https://dx.doi.org/10.1053/jpsu.2002.30839.
Received 06.04.2020
Accepted 21.05.2020
About the Authors
Oksana V. Pribushenya, MD, PhD, head of the Scientific Laboratory for Medical Genetics, Republic Scientific and Practical Center „Mather and child”, professor of the department of obstetrics, gynecology and reproductive health “BelMAPE”. Tel.: (37517)254-62-53; (37529)623-12-55. Fax: (37517)233-85-13. E-mail: pribushenya@yandex.ru. ORCID: 0000-0001-8186-2849. eLibrary SPIN: 2707-1880. 220053, Orlovskaya str., 66, Minsk, Belarus.Olga A. Tarletskaya, doctor of ultrasound diagnostics of the department of prenatal diagnostics of the Republican Scientific and
Practical Center “Mother and Child” MH RB. Tel.: (37517)254-62-53. 220053, Orlovskaya str., 66, Minsk, Belarus.
Olga A. Pankratova, MD, PhD, Head of the Department of Pregnancy Pathology No. 2, Republican Scientific and Practical Center “Mother and Child” MH RB.
220053, Orlovskaya str., 66, Minsk, Belarus.
Dmitry B. Drozdovsky, intensive care physician of the neonatal intensive care unit of the Republican Scientific and Practical Center “Mother and Child” MH RB. Tel.: (37517)254-62-53. 220053, Orlovskaya str., 66, Minsk, Belarus.
Natalia N. Medvedeva, obstetrician-gynecologist of the admission department, Clinical Maternity Hospital in Minsk Region. Tel. (37529)663-62-62.
220000, Francis Skariny str., 16, Minsk, Belarus.
Nadezhda V. Petrovskaya, obstetrician-gynecologist of the Department of Medical Genetics, UZ “Mogilev Regional Medical and Diagnostic Center”. Tel.: (37522)222-41-65.
212030, Pervomaiskaya str., 59A, Mogilev, Belarus.
For сitation: Pribushenya O.V., Tarletskaya O.A., Pankratova O.A., Drozdovsky D.B., Medvedeva N.N., Petrovskaya N.V. Use of an ex utero intrapartum treatment procedure in a fetus with prenatally diagnosed neck teratoma.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2020; 9: 262-267 (in Russian)
https://dx.doi.org/10.18565/aig.2020.9.262-267