Prenatal management of cystic hygroma and long term outcomes

Cystic hygroma is a benign congenital malformation of the lymphatic system. The rate of incidence is 1-4/1000 and it can be inherited as an autosomal recessive. There is a 35-50 % chance of chromosomal abnormalities with cystic hygroma. Also euploid fetuses have 50 % structural malformation risk. The most common malformations are cardiac malformations. There’s a 15 % chance of live births for affected fetuses. If there are neither chromosomal abnormalities nor structural malformations, a 95 % normal short-term pediatric outcome can be estimated. However, the overall prognosis in fetuses with cystic hygroma is poor, and a healthy survival rate is less than 5 %. There are prenatal, natal and postnatal treatment methods

PDF

___

1. Chervenak FA, Isaacson G, Blakemore KJ, Breg RW, Hobbins JC, Berkowitz RL, Tortora M, Mayden K, Mahoney MJ. Fetal cystic hygroma: cause and natural history. N Engl J Med. 1983;309(14):822–5.

2. Azar G, Snijders RJM, Gosden CM, Nicolaides KH. Fetal nuchal cystic hygroma: associated malformations and chromosomal defects. Fetal Diagn Ther. 1991;6(1-2):46–57.

3. Christion-Lagay ER, Fishman SJ. Vascular anomalies. Surg Clin North Am. 2006;86(2):393-425.

4. Kharrat R, Yamamoto M, Roume J, Couderc S, Vialard F, Hillion Y, Ville Y. Karyotype and outcome of fetuses diagnosed with cystic hygroma in the first trimester in realation to nuchal translucency thickness. Prenat Diagn. 2006;26(4):369-72.

5. Malone FD, Ball RH, Nyberg DA, Comstock CH, Saade GR, Berkowitz RL, Gross SJ, Dugoff L, Craigo SD, Timor-Tritsch IE, Carr SR, Wolfe HM, Dukes K, Canick JA, Bianchi DW, D'Alton ME; FASTER Trial Research Consortium. First-trimester septated cystic hygroma: prevalence, natural history, and pediatric outcome. Obstet Gynecol. 2005;106(2):288–94.

6. Fisher R, Partington A, Dykes E. Cystic hygroma: comparison betwen prenatal and postnatal diagnosis. J Pediatr Surg. 1996;31(4):473-6.

7. Vile Y. Nuchal translucency in the first trimester of pregnancy: ten years on and still pain in the neck? Ultrasound Obstet Gynecol. 2001;18(1):5-8.

8. Palladini D, Volpe P. Ultrasound of fetal congenital anomalies. 1st edition. Informa, London, 2007.

9. Hubbard A, Crombleholme TM, Adzick NS. Prenatal MRI evaluation of giant neck masses in preparation fort he fetal EXIT procedure. Am J Perinatol. 1998;15(4):253-7.

10. Malone FD, Canick JA, Ball RH, Nyberg DA, Comstock CH, Bukowski R, Berkowitz RL, Gross SJ, Dugoff L, Craigo SD, TimorTritsch IE, Carr SR, Wolfe HM, Dukes K, Bianchi DW, Rudnicka AR, Hackshaw AK, Lambert-Messerlian G, Wald NJ, D'Alton ME; First- and Second-Trimester Evaluation of Risk (FASTER) Research Consortium. A Comparison of first trimester screening, second trimester screening, and the combination of both for evaluation of risk for Down syndrome. N Eng J Med. 2005;353(19):2001–11.

11. Comstock CH, Malone FD, Ball RH, Nyberg DA, Saade GR, Berkowitz RL, Ferreira J, Dugoff L, Craigo SD, Timor-Tritsch IE, Carr SR, Wolfe HM, Bianchi DW, D'Alton ME; FASTER Research Consortium. Is there a nuchal translucency millimeter measurement above which there is no added benefit from first trimester serum screening? Am J Obstet Gynecol. 2006;195(3):843-7.

12. Bronshtein M, Bar-Hava I, Blumenfeld I, Bejar J, Toder V, Blumenfeld Z. The difference betwen septated and non-septated nuchal cystic hygroma in the early second trimester. Obstet Gynecol. 1993;81(5):683-7.

13. Sanhal CY, Mendilcioglu I, Ozekinci M, Yakut S, Merdun Z, Simsek M, Luleci G. Prenatal management, pregnacy and pediatric outcomes in fetuses with septated cystic hygroma. Braz J Med Biol Res. 2014;47(9):799-803.

14. Edwards MJ, Graham JM. Posterior nuchal cystic hygroma. Clin Perinatol. 1990;17(3):611-40.

15. Bianchi DW, Chrombleholme TM, D’Alton ME, Malone FD. Fetology. 2nd edition. Mc Graw Hill, United States, 2010.

16. Liechty K, Chrombleholme TM, Flake AW, Morgan MA, Kurth CD, Hubbard AM, Adzick NS. Intrapartum airway management for giant fetal neck masses: the EXIT procedure. Am J Ostet Gynecol.1997;177(4):870-4.

17. Marwan A, Chrombleholme TM. The EXIT procedure: principles, pitfalls and progress. Semin Pediatr Surg. 2006;15(2):107-15.

18. Chen CP, Jan SW, Liu FF, Huang SH. Echo guided lymphatic drainage by fine-needle aspiration in persistent isolated septated fetal nuchal cystic hygroma. Fetal Diagn Ther. 1996;11(2):150-3.

19. Ogita K, Suita S, Taguchi T, Yamanouchi T, Masumoto K, Tsukimori K, Nakano H. Outcome of fetal cystic hygroma and experience of intrauterine treatment. Fetal Diagn Ther. 2001;16(2):105-10.

20. Ogita K, Taguchi T, Suita S. Experimental study concerning safety dosage of OK-432 for intrauterine treatment. Asisan J Surg. 2006;29(3):202-6.

21. Mikovic Z, Simic R, Egic A, Stosic-Opincal T, Koprivsek K, Stanojevic D, Bogavac M, Popovac M, Mandic V. Intrauterine treatment of large fetal neck lymphangioma with OK-432. Fetal Diagn Ther. 2009;26(2):102-6.

22. Hancock BJ, St-Vil D, Lebs FI, Di Lorenzo M, Blanchard H. Complications of lymphangiomas in children. J Pediatr Surg. 1992;27(2):220-4.

23. Tanigawa N, Shimomatsuya T, Takahashi K, Inomata Y,Tanaka K, Satomura K, Hikasa Y, Hashida M, Muranishi S, Sezaki H. Treatment of cystic hygroma and lymphangioma with use of bleomycine fat emulsion. Cancer. 1987;60(4):741-9.

24. Tanaka K, Inomata Y, Utsunomiya H, Uemoto S, Asonuma K, Katayama T, Ozawa K, Hashida M. Sclerosing therapy with bleomycin for lymphangioma in children. Pediatr Surg Int. 1990;5(4):270-5.

25. Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N. OK432 therapy for lymphangiomas in children: why and how does it work? J Pediatr Surg. 1996;31(4):477-80.

26. Graesslin O, Derniaux E, Alanio E, Gaillard D, Vitry F, Quereux C, Ducarme G. Characteristics and outcome of fetal cystic hygroma diagnosed in the first trimester. Acta Obstet Gynecol Scand .2007;86(12):1442-6.

27. Tricoire J, Sarramon B, Rolland M, Lefort G. Familial cystic hygroma: report of 8 cases in 3 families. Genet Couns. 1993;4(4):265-9.

28. Teaque KE, Eggleston MK, Muffley PE, Gherman RB. Recurrent fetal cystic hygroma with normal chromosomes: case report and review of the literature. J Matern Fetal Med. 2000;9(6):366-9.