|Year : 2019 | Volume
| Issue : 1 | Page : 47-49
Hydrops fetalis with septated cystic hygroma
Reddy Ravikanth1, Rajalakshmy Prasannan2
1 Department of Radiology, Malabar Medical College, Kozhikode, Kerala, India
2 Department of Obstetrics and Gynecology, Malabar Medical College, Kozhikode, Kerala, India
|Date of Web Publication||11-Mar-2019|
Department of Radiology, Malabar Medical College, Kozhikode - 673 315, Kerala
Source of Support: None, Conflict of Interest: None
Hydrops fetalis is excess accumulation of fluid in the fetus, and fetal cystic hygroma is a manifestation of early lymphatic obstruction. Depending on the severity and cause of hydrops, there may be anasarca of fetus, placentomegaly, ascites, pleural effusions, and/or pericardial effusions. Fetal hydrops and cystic hygroma can be diagnosed in the second trimester of gestation and is associated with a higher incidence of aneuploidy and high mortality. Previously, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to Rh isoimmunization. At present, hydrops fetalis is caused by other conditions and is known as nonimmune hydrops. Here, we present a case of an 18-week pregnancy with fetal cystic hygroma and nonimmune hydrops fetalis.
Keywords: Amniocentesis, anomaly scan, chorionic villous sampling, genetic counseling, hydrops fetalis, second trimester, septated cystic hygroma
|How to cite this article:|
Ravikanth R, Prasannan R. Hydrops fetalis with septated cystic hygroma. Apollo Med 2019;16:47-9
| Introduction|| |
Ballantyne first described hydrops fetalis in 1892, although this condition had been recognized for almost 200 years. The hallmark of the disease is the abnormal accumulation of fluid in body cavities such as pleural, pericardial, and peritoneal and anasarca with a wall thickness of >5 mm and is due to an imbalance in fluid homeostasis, with fluid accumulation exceeding resorption. A thin-walled, multiseptate cystic structure posterior to the fetal head and neck, situated eccentrically with respect to the long axis of the fetus without evidence of a vertebral column defect is diagnostic of a cystic hygroma.
| Case Report|| |
A 22-year-old multiparous pregnant woman with 18 weeks of gestation, without prior surveillance, was diagnosed with an extensive and septated cystic hygroma [Figure 1]a and [Figure 1]b, associated with edematous scalp [Figure 2], generalized anasarca [Figure 3], bilateral pleural effusions [Figure 3], ascites, and oligohydramnios during sonography evaluation of anomalies. There was no history of consanguineous marriage, Rh isoimmunization, toxoplasmosis, rubella or syphilis infection, or family history of birth defects. She underwent amniocentesis, which revealed Turner's syndrome (45, X). After explaining the disease and associated fetal morbidity, the couple accepted the medical termination of pregnancy. Pregnancy was terminated at 19 weeks of gestation and was induced with 400 μg of vaginal misoprostol.
|Figure 1: Sagittal (a) and axial (b) antenatal sonography images of the fetus at 18 weeks of gestational age showing an extensive septated cystic hygroma (arrows)|
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|Figure 2: Axial sonography image of the fetal head showing diffusely edematous scalp (arrows)|
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|Figure 3: Sagittal sonography image of the fetus demonstrating generalized anasarca (red arrows) and bilateral pleural effusions (blue arrows)|
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| Discussion|| |
Hydrops fetalis refers to fluid accumulation in serous cavities and/or edema of soft tissues in the fetus. It is labeled as nonimmune if there is no indication of fetal and maternal blood group incompatibility. The incidence is approximately 1 in 2500 to 1 in 3500 neonates. One possible cause of hydrops include cystic hygromas. Cystic hygroma is one of the most common abnormalities seen sonographically in the first and second trimesters which are characterized by single or multiple congenital cysts of the lymphatic system most commonly found within the soft tissues of the neck. Cystic hygromas are malformations of the lymphatic system that appear as fluid-filled, membranous cysts, lined by true epithelium in the anterolateral or occipitocervical area. They result from the jugular lymphatic obstruction sequence, in which the normal communication between the jugular veins and the jugular lymphatic sacs fail to develop by 40th day of gestation. They are highly associated with chromosomal abnormalities. In fetuses with cystic hygroma in the first trimester, trisomy 21, trisomy 18, and trisomy 13 are most prevalent. In the second trimester, Turner's syndrome is most prevalent. Almost all fetuses with cystic hygroma and hydrops die antenatally. Cystic hygroma with a normal chromosomal configuration with no other associated anomalies has a spontaneous resolution before 20 weeks of gestation while those associated with chromosomal anomalies have a poor prognosis. Cystic hygromas without hydrops usually regress completely.
de Serres et al. have proposed the following system for staging of cystic hygroma of the head and neck:
- Stage I – Unilateral infrahyoid (17% complication rate)
- Stage II – Unilateral suprahyoid (41% complication rate)
- Stage III – Unilateral and both infrahyoid and suprahyoid (67% complication rate)
- Stage IV – Bilateral suprahyoid (80% complication rate)
- Stage V – Bilateral infrahyoid and suprahyoid (100% complication rate).
Cystic hygroma can be visualized using prenatal ultrasonography by 10 weeks' gestation. Sonography of cystic hygroma shows a thin-walled cystic mass on the posterolateral aspect of the neck. Identification of the nuchal ligament within the cyst constitutes the most specific sign for the diagnosis of cystic hygroma. Differential diagnoses include occipital encephalocoele, occipitocervical meningomyocoele, nuchal edema, and posterior teratoma. A detailed ultrasound should be performed, including fetal echocardiogram, to rule out other anomalies that may indicate the cause for cystic hygroma. Cystic hygromas are also sometimes associated with cardiac malformations, most commonly coarctation of aorta and a hypoplastic left heart. They may also be associated with abdominal, pleural, and pericardial effusion. Further imaging modalities include fast-spin MRI which can be used to determine the extent of fetal cystic hygroma and amniocentesis or chorionic villous sampling to diagnose chromosome abnormalities or specific genetic syndromes.
| Conclusion|| |
Sonography is essential for prenatal diagnosis of hydrops fetalis and fetal cystic hygroma. Genetic evaluation of the fetus and parents is required to establish or exclude aneuploidy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Abrams ME, Meredith KS, Kinnard P, Clark RH. Hydrops fetalis: A retrospective review of cases reported to a large national database and identification of risk factors associated with death. Pediatrics 2007;120:84-9.
Has R. Non-immune hydrops fetalis in the first trimester: A review of 30 cases. Clin Exp Obstet Gynecol 2001;28:187-90.
Merz E, Bahlmann F. Cystic hygroma. In: Merz E, Bahlmann F, editors. Ultrasound in Obstetrics and Gynecology. 2nd
ed. New York: Thieme Medical Publishers; 2005. p. 23-9.
Papp C, Beke A, Mezei G, Szigeti Z, Bán Z, Papp Z, et al.
Prenatal diagnosis of turner syndrome: Report on 69 cases. J Ultrasound Med 2006;25:711-7.
Cohen MM, Schwartz S, Schwartz MF, Blitzer MG, Raffel LJ, Mullins-Keene CL, et al.
Antenatal detection of cystic hygroma. Obstet Gynecol Surv 1989;44:481-90.
Brun JL, Taine L, Horovitz J, Maugey-Laulom B, Carles D, Saura R, et al.
Prenatal diagnosis of nuchal edemas and cystic hygromas of the neck 49 cases. J Gynecol Obstet Biol Reprod (Paris) 1995;24:162-9.
Beke A, Joó JG, Csaba A, Lázár L, Bán Z, Papp C, et al.
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;25:83-92.
de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg 1995;121:577-82.
Malone FD, Ball RH, Nyberg DA, Comstock CH, Saade GR, Berkowitz RL, et al.
First-trimester septated cystic hygroma: Prevalence, natural history, and pediatric outcome. Obstet Gynecol 2005;106:288-94.
Rotmensch S, Celentano C, Sadan O, Liberati M, Lev D, Glezerman M, et al.
Familial occurrence of isolated nonseptated nuchal cystic hygromata in midtrimester of pregnancy. Prenat Diagn 2004;24:260-4.
Tanriverdi HA, Hendrik HJ, Ertan AK, Axt R, Schmidt W. Hygroma colli cysticum: Prenatal diagnosis and prognosis. Am J Perinatol 2001;18:415-20.
[Figure 1], [Figure 2], [Figure 3]