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| CASE REPORT |
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| Year : 2021 | Volume
: 8
| Issue : 3 | Page : 308-311 |
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Antenatal diagnosis and management of cystic hygroma with hydrops fetalis
Saloni Pugalia1, Shruti Shetty1, Sabrina Mhapankar2
1 MGM Hospital, Sector-4E, Kalamboli, Navi Mumbai, Maharashtra, India
2 Department of Obstetrics and Gynecology, MGM Medical College and Hospital, MGM Institute of Health Sciences (Deemed to be University), Sector-1, Kamothe, Navi Mumbai, Maharashtra, India
| Date of Submission | 20-May-2021 |
| Date of Acceptance | 27-May-2021 |
| Date of Web Publication | 03-Sep-2021 |
Correspondence Address:
Dr. Saloni Pugalia
MGM Hospital, Sector-4E, Kalamboli, Navi Mumbai 410218, Maharashtra.
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/mgmj.mgmj_28_21
Cystic hygroma is a congenital thin-walled cyst that contains lymphatic fluids. Ultrasonography has helped in detecting more and more cases of cystic hygroma with fetal hydrops. Fetal hydrops and cystic hygroma can be diagnosed in the first and second trimesters of gestation and are associated with a higher incidence of aneuploidy and have higher mortality rate. The mortality rate of cystic hygroma diagnosed before 30 weeks is 93%, and 84% of those are associated with progressive non-immune hydrops. We present three case series of cystic hygroma progressing to hydrops fetalis. Keywords: Aneuploidy, chromosomal abnormalities, cystic hygroma, non-immune hydrops
How to cite this article:
Pugalia S, Shetty S, Mhapankar S. Antenatal diagnosis and management of cystic hygroma with hydrops fetalis. MGM J Med Sci 2021;8:308-11 |
How to cite this URL:
Pugalia S, Shetty S, Mhapankar S. Antenatal diagnosis and management of cystic hygroma with hydrops fetalis. MGM J Med Sci [serial online] 2021 [cited 2022 Jan 25];8:308-11. Available from: http://www.mgmjms.com/text.asp?2021/8/3/308/325537 |
| Introduction | |  |
Cystic hygroma is a congenital abnormality of the vascular lymphatic system. It can be associated with aneuploidy(50%) and structural anomalies (33.8%). Some of these fetuses develop hydrops fetalis leading to intrauterine fetal demise.[1]
Their birth prevalence is 1 in 5000. Cystic hygroma is of two types based on septations: septated and non-septated. Small cystic hygroma usually regresses spontaneously, whereas large cystic hygroma is associated with hydrops fetalis. When diagnosed in utero, the survival rate of fetuses affected with cystic hygroma is only 2–6%. When hydrops is present with cystic hygroma, the mortality rate is near 100%.
| CASE REPORT 1 | | |
Patient Mrs X, 24-year-old, primigravida with 18 weeks and 2 days gestation presented to us for a normal antenatal checkup. There was no history of hypertension and diabetes. She had an Rh-positive blood group and had a non-consanguineous marriage. The gestational age of the fetus was determined by fetal biometry, and it was corresponding with the menstrual history of the patient.
The ultrasound examination done at 12 weeks had not picked up any abnormality. An anomaly scan was done to find congenital anomalies, and it was suggestive of a large anechoic cystic collection at the back of the neck with multiple septations, suggestive of cystic hygroma, presence of skin edema, bilateral pleural effusion, ascites, and mild placentomegaly, suggestive of hydrops [Figure 1] and [Figure 2].
Prognosis of the fetus and possibility of chromosomal disorder, structural disorder, and risk of infection were explained to the patient. The patient was advised for amniocentesis for fetal karyotyping. However, the patient declined amniocentesis and opted for termination of pregnancy.
The patient was induced with Tab. Mifepristone 200 mg oral followed by Tab. Misoprostol 200 mcg oral after 48 h.
Patient aborted [Figure 3] (the time interval between the last dose of Tab. Misoprostol and abortion is 4 h).
| CASE REPORT 2 | | |
Patient Mrs Y, 28-year-old, gravida 2 para 1 live 1 with 19 weeks and 4 days of gestation, came to MGM Hospital for Women at Kalamboli, Navi Mumbai. She was Rh-positive and had a non-consanguineous marriage.
The first trimester scan at 12 weeks 2 days detected nuchal translucency of 10 mm (increased). The gestational age of the fetus was determined by fetal biometry parameters, and it was corresponding with the menstrual history of the patient. The patient was kept under regular surveillance. A fetal anomaly scan done at 18 weeks revealed a bilateral cystic lesion behind the neck suggestive of cystic hygroma. The fetus also had bilateral pleural effusion, pericardial effusion, ascites, subcutaneous edema in limbs, and the features suggestive of hydrops fetalis [Figure 4] and [Figure 5]. | Figure 4: USG showing cystic swelling at the back of neck with multiple septations
Click here to view |
Prognosis of the fetus and possibility of chromosomal disorder, structural disorder, and risk of infection were explained to the patient. The patient was advised for fetal karyotyping. However, she opted for termination of pregnancy rather than fetal karyotyping.
The patient was induced with the following:
Tab. Mifepristone 200 mg oral followed by
Tab. Misoprostol 200 mcg oral after 48 h
followed by Tab. Misoprostol 200 mcg oral after 6 h.
Patient aborted [Figure 6] (the time interval between the last dose of Tab. Misoprostol and abortion is 6 h). | Figure 6: Aborted fetus showing cystic hygroma with hydrops fetalis along with placenta
Click here to view |
| CASE REPORT 3 | | |
Patient Mrs Z, 35-year-old, gravida 2 para 1 live 1 with 29 weeks and 4 days of gestation, unregistered and unbooked, came with complaints of pain in abdomen for 1 day. She had an Rh-positive blood group and non-consanguineous marriage. She had no previous history of congenital anomaly. On evaluation, she had a scan of 28 weeks 1 day gestation showing bilateral cystic swelling behind the neck suggestive of cystic hygroma, presence of subcutaneous edema involving the skull, neck, and abdomen, suggestive of hydrops fetalis. She had no history of hypertension and diabetes.
On examination
General condition fair
Pulse rate: 84/min
Blood pressure: 110/70 mmHg
No pallor, no icterus, no edema
Systemic examination: normal
Per abdomen examination:
Uterus 28 weeks size
Longitudinal lie
Cephalic presentation
Fetal heart sound present/100 beats/min
Uterine contractions: 2–3 in 10 min lasting for 20 s
Per speculum examination, cervix and vagina, healthy
Per vagina examination: cervix was 5 cm dilated, 40% effaced, station-1, bulging membranes
Patient and relatives were explained regarding the poor prognosis of baby and were observed for spontaneous progression of labor
The patient delivered vaginally but the baby died after few hours [Figure 7]. | Figure 7: Aborted fetus showing mild cystic hygroma at the back of neck with airway abnormalities
Click here to view |
| Discussion | | |
Cystic hygroma is one of the most common abnormalities seen in sonography. They arise from the failure of the primitive lymphatic tree to connect to the venous system. Blind lymphatic pouch results in dilation of the lymphatic sac.[2]
It is mainly located at the cervicofacial and axillary regions.[3]
Its incidence is 1 in 100 pregnancies in the first trimester.[4]
The prevalence of the potential causes of nonimmune hydrops fetalis (NIHF) depends on gestational age at the time of presentation.[5]
They have highly associated with chromosomal abnormalities, especially aneuploidy, cardiac anomalies, skeletal abnormalities, fetal hydrops, and intrauterine fetal demise.[1]
In the first trimester, it is most commonly associated with trisomy 21 followed by Turner syndrome (45X) and trisomy 18, whereas in the second trimester, most commonly it is associated with Turner syndrome (45X).
Small cystic hygroma usually regresses spontaneously, whereas large cystic hygroma is associated with hydrops fetalis.
Hydrops fetalis is defined as the abnormal accumulation of fluid in at least two different fetal compartments.[6] Hydrops fetalis is of two types: immune and non-immune based on the presence or absence of rhesus isoimmunization and histologic evidence of erythroblastosis.
Hydrops is detected sonographically as two effusions or one effusion plus anasarca.
Edema may be prominent around the scalp, or trunk, and extremities. As hydrops progresses in severity, they can be associated with placentomegaly and hydramnios.
The incidence of NIHF is estimated at 1 in 3000 pregnancies.[7] Overall, the prognosis is poor with a perinatal mortality rate of 52–98%.[8]
Cystic hygroma and hydrops fetalis in the second trimester have been associated with poor prognosis.
| Conclusion | | |
The association of cystic hygroma and hydrops fetalis in the second trimester have been associated with adverse perinatal outcome. Care must be taken in the fetus for early diagnosis of cystic hygroma so that early termination of pregnancy can be performed if the patient desires.
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.
Financial support and sponsorship
MGMIHS.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Malone FD, Ball RH, Nyberg DA, Comstock CH, Saade GR, Berkowitz RL, et al; FASTER Trial Research Consortium. First-trimester septated cystic hygroma: Prevalence, natural history, and pediatric outcome. Obstet Gynecol 2005;106:288-94.  |
| 2. | Bekker MN, van den Akker NMS, de Mooij YM, Bartelings MM, vanVugt JMG, Gittenberger-deGroot AC. Jugular lymphatic maldevelopment in Turner syndrome and trisomy 21: Different anomalies leading to nuchal edema. Reprod Sci 2008;15:295-304. https://doi.org/10.1177/1933719107314062. |
| 3. | Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al; ISSVA Board and Scientific Committee. Vascular anomalies classification: Recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015;136:e203-14. |
| 4. | Podobnik M, Singer Z, Podobnik-Sarkanji S, Bulić M. First trimester diagnosis of cystic hygromata using transvaginal ultrasound and cytogenetic evaluation. J Perinat Med 1995;23:283-91. |
| 5. | Ota S, Sahara J, Mabuchi A, Yamamoto R, Ishii K, Mitsuda N. Perinatal and one-year outcomes of non-immune hydrops fetalis by etiology and age at diagnosis. J Obstet Gynaecol Res 2016;42:385-91. |
| 6. | Bellini C, Hennekam RC. Non-immune hydrops fetalis: A short review of etiology and pathophysiology. Am J Med Genet A 2012;158A:597-605. |
| 7. | Bellini C, Donarini G, Paladini D, Calevo MG, Bellini T, Ramenghi LA, et al. Etiology of non-immune hydrops fetalis: An update. Am J Med Genet A 2015;167A:1082-8. |
| 8. | Ismail KM, Martin WL, Ghosh S, Whittle MJ, Kilby MD. Etiology and outcome of hydrops fetalis. J Matern Fetal Med 2001;10:175-81. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
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