Abstract
Hydrops fetalis refers to the excessive accumulation of fluid in the extracellular compartment of the fetus. Cystic hygroma is a congenital malformation of the cervical lymphatic system responsible for an accumulation of lymphatic fluid commonly in the retrocervical region. Cystic hygromas are most often associated with chromosomal abnormalities. When a cystic hygroma is diagnosed in utero, the fetal survival rate is only 2-6%. When hydrops fetalis is present with cystic hygroma, the mortality rate is close to 100%. We present here the case of a 23-year-old primigravida in whom an ultrasound performed at 17 weeks of amenorrhea revealed a posterior cervical cystic hygroma associated with hydrops fetalis, the evolution of which was marked by intrauterine fetal death two weeks later.
Keywords: Hydrops fetalis, Cystic hygroma, Chromosomal abnormalities, Prenatal diagnosis.
References
- Has R. Non-immune hydrops fetalis in the first trimester: a review of 30 cases. Clin Exp Obstet Gynecol. 2001;28(3):187-90. Erratum in: Clin Exp Obstet Gynecol 2002;29(1):following table of contents. Recep H [corrected to Has R]. PMID: 11530871.
- Cunningham, Kenneth Leveno, Steven Bloom, John Hauth, Dwight Rouse, Catherine Spong. Cystic hygroma. In: F. Cunningham, Kenneth Leveno, Steven Bloom, John Hauth, Dwight Rouse, Catherine Spong, eds. William’s Obstetrics. 23rd ed. New York: McGraw Hill Professional; 2009: 356-357.
- Damaskos C., Garmpis N., Manousi M. et al.: Cystic hygroma of the neck: single center experience and literature review. Eur Rev Med Pharmacol Scie, 2017; 21: 4918–4923.
- Merz E, Bahlmann F. Cystic hygroma. In: Merz E, Bahlmann F, eds. Ultrasound in Obstetrics and Gynecology. 2nd ed. New York, NY: Thieme Medical Publishers; 2005: 23-29.
- Papp C, Beke A, Mezei G, Szigeti Z, Bán Z, Papp Z. Prenatal diagnosis of Turner syndrome: report on 69 cases. J Ultrasound Med. 2006 Jun;25(6):711-7; quiz 718-20. doi: 10.7863/jum.2006.25.6.711. PMID: 16731887.
- Beke A, Joó JG, Csaba A, Lázár L, Bán Z, Papp C, Tóth-Pál E, Papp Z. Incidence of chromosomal abnormalities in the presence of fetal subcutaneous oedema, such as nuchal oedema, cystic hygroma and non-immune hydrops. Fetal Diagn Ther. 2009;25(1):83-92. doi: 10.1159/000201946. Epub 2009 Feb 13. PMID: 19218808.
- Gedikbasi A, Oztarhan K, Aslan G, Demirali O, Akyol A, Sargin A, Ceylan Y. Multidisciplinary approach in cystic hygroma: prenatal diagnosis, outcome, and postnatal follow up. Pediatr Int. 2009 Oct;51(5):670-7. doi: 10.1111/j.1442-200X.2009.02846.x.Epub 2009 Mar 13. PMID: 19419502.
- Veerabathini BC, Manthani K, Hussain A. Congenital Central Nervous System Malformations: A Rare Case of an Encephalocele and Literature Review of Its Associations, Imaging Modalities, Radiological Findings, and Treatments. Cureus. 2021 Jun 27;13(6):e15959. doi: 10.7759/cureus.15959. PMID: 34336454; PMCID: PMC8315298.
- Benacerraf BR, Frigoletto FD Jr. Soft tissue nuchal fold in the second-trimester fetus: standards for normal measurements compared with those in Down syndrome. Am J Obstet Gynecol. 1987 Nov;157(5):1146-9. doi: 10.1016/s0002-9378(87)80279-x. PMID: 2961264.
- Pugalia, Saloni; Shetty, Shruti; Mhapankar, Sabrina1. Antenatal diagnosis and management of cystic hygroma with hydrops fetalis. MGM Journal of Medical Sciences 8(3):p 308-311, Jul–Sep 2021. | DOI: 10.4103/mgmj.mgmj_28_21.
Corresponding Author
Ayoub KHTIRA
Department of Gynecology and Obstetrics, Military Hospital of Instruction Mohamed V, Rabat, Morocco