Figure 1
Antenatal sonography fetal head and neck longitudinal section
Thickening of subcutaneous tissue in fetal head and neck regions-Nuchal fold thickening.
Nonimmune hydrops fetalis with cystic hygroma Colli: The sonographic features
SectionPaediatric radiology
Case TypeClinical Cases
AuthorsGian S.Shergill1, Arvinder Singh2, Harbhajan Kaur3, Ramesh Chander2
Connected authors
19 years, female
Clinical History
A 19-year-old female patient, primigravida, was referred for routine antenatal examination. No H/o blood transfusion incompatibility, diabetes mellitus, spontaneous abortions or any blood transfusion reactions.
She was mildly anaemic with haemoglobin 9.8gm%, B.P 140/90mm Hg, fasting blood sugar 82mg%, blood urea 34mg% , S.Bilirubin 0.6, SGOT and SGPT within normal range.
She was mildly anaemic with haemoglobin 9.8gm%, B.P 140/90mm Hg, fasting blood sugar 82mg%, blood urea 34mg% , S.Bilirubin 0.6, SGOT and SGPT within normal range.
Imaging Findings
Sonographic features of fetus on antenatal examination were as follow:
Fetal gestational age was 15 weeks 6 days. Amniotic fluid and AFI were within normal range. Placenta was fundal, anterior in location grade 1 maturity with placental thickness of about 4.2cm. There was evidence of thickening of subcutaneous tissue of the fetal scalp and nuchal fold [Fig. 1].
A well defined bilobed cystic mass with a thick central septa was seen in the fetal nape region suggestive of cystic hygroma Colli [Fig. 2].Significant amount of bilateral fetal pleural effusion was seen with partial relaxation atelectasis of both lungs. Marked abdominal subcutaeous oedema measuring about 9-10mm thick[Fig. 3]. Mild degree of pericardial effusion and significant amount of fetal ascites noted [Fig. 4]. Fetal heart rate show some variability in rhythm over the course of examination.No gross cardiac defect was detected.Fetal abortion was finally done due to marked fetal respiratory distress.
Fetal gestational age was 15 weeks 6 days. Amniotic fluid and AFI were within normal range. Placenta was fundal, anterior in location grade 1 maturity with placental thickness of about 4.2cm. There was evidence of thickening of subcutaneous tissue of the fetal scalp and nuchal fold [Fig. 1].
A well defined bilobed cystic mass with a thick central septa was seen in the fetal nape region suggestive of cystic hygroma Colli [Fig. 2].Significant amount of bilateral fetal pleural effusion was seen with partial relaxation atelectasis of both lungs. Marked abdominal subcutaeous oedema measuring about 9-10mm thick[Fig. 3]. Mild degree of pericardial effusion and significant amount of fetal ascites noted [Fig. 4]. Fetal heart rate show some variability in rhythm over the course of examination.No gross cardiac defect was detected.Fetal abortion was finally done due to marked fetal respiratory distress.
Discussion
Hydrops is defined as an abnormal accumulation of serous fluid in at least two body cavities or tissues. Hydrops may be immune or nonimmune in origin. Non immune is defined as absence of a detectable circulating antibodies against red blood cells in the mother.
This condition was first documented by Ballantyne in 1892 and incorrectly described as hereditary in origin. Levine and coworkers in 1939 reported a link between maternal sensitization of fetal blood antigen Rh factor and hydrops fetalis-erythroblastosis fetalis later known as Immune Hydrops fetalis (IHF). In 1943, Potter reported cases where HF was not due to the antigen immunologic response as previously described by Landsteiner and Weiner, the term Nonimmune Hydrops Fetalis (NHF) was given. The various causes other than maternal blood incompatible were aneuploidy (20%), abnormal fetal cardiac structure/function (35%), haematological disease (15%), infections (10%), lymphatic or venous obstruction due to cystic hygroma and idiopathic causes (5%) [1, 2].
In preliminary studies, less than 20% of all hydrops fetalis cases fell into the NHF category. After the introduction and initiation of immunoglobulin prophylaxis for IHF, the NHF category has increased to approximately 90% of all hydrops cases. The incidence of NHF is about 1 to 1500 to 1 to 4000 deliveries [2]. Although there is no specific test to diagnose NHF, careful sonographic screening for fetal abnormalities is key to early detection and appropriate treatment.
Sonography is the investigation of choice for the detection of hydrops fetalis as it is
mutliplanar, easy operability, low cost and non-invasive. The various sonographic features in HF are: polyhydramnios, placenta oedema (>5cm), dilated umbilical vein, pleural/pericardial effusions, fetal ascites and generalised anasarca with subcutaneous thickness > 5mm [3, 4]. Lymphatic or venous maldevelopment may be the causative factor in NHF either from compression or high cardiac output failure [5].
Earlier hydrops fetalis cases were treated in utero by injecting albumin into the fetal abdominal cavity and by removal of accumulated fluid from the serous cavities [6]. Cordocentesis provides access for direct fetal therapy and diagnostic purposes - blood group and Rhesus typing, serology and PCR for congenital infections and chromosomal studies [7].
Cystic hygroma colli is frequently associated with Turner's syndrome. Karyotypes other than monosomy X are common, and perinatal survival is unlikely in the presence of hydrops fetalis [8].
Hydrops fetalis is a challenging entity for clinicians. Early diagnosis and treatment greatly improves perinatal outcome, though prognosis depends on the underlying etiology.
This condition was first documented by Ballantyne in 1892 and incorrectly described as hereditary in origin. Levine and coworkers in 1939 reported a link between maternal sensitization of fetal blood antigen Rh factor and hydrops fetalis-erythroblastosis fetalis later known as Immune Hydrops fetalis (IHF). In 1943, Potter reported cases where HF was not due to the antigen immunologic response as previously described by Landsteiner and Weiner, the term Nonimmune Hydrops Fetalis (NHF) was given. The various causes other than maternal blood incompatible were aneuploidy (20%), abnormal fetal cardiac structure/function (35%), haematological disease (15%), infections (10%), lymphatic or venous obstruction due to cystic hygroma and idiopathic causes (5%) [1, 2].
In preliminary studies, less than 20% of all hydrops fetalis cases fell into the NHF category. After the introduction and initiation of immunoglobulin prophylaxis for IHF, the NHF category has increased to approximately 90% of all hydrops cases. The incidence of NHF is about 1 to 1500 to 1 to 4000 deliveries [2]. Although there is no specific test to diagnose NHF, careful sonographic screening for fetal abnormalities is key to early detection and appropriate treatment.
Sonography is the investigation of choice for the detection of hydrops fetalis as it is
mutliplanar, easy operability, low cost and non-invasive. The various sonographic features in HF are: polyhydramnios, placenta oedema (>5cm), dilated umbilical vein, pleural/pericardial effusions, fetal ascites and generalised anasarca with subcutaneous thickness > 5mm [3, 4]. Lymphatic or venous maldevelopment may be the causative factor in NHF either from compression or high cardiac output failure [5].
Earlier hydrops fetalis cases were treated in utero by injecting albumin into the fetal abdominal cavity and by removal of accumulated fluid from the serous cavities [6]. Cordocentesis provides access for direct fetal therapy and diagnostic purposes - blood group and Rhesus typing, serology and PCR for congenital infections and chromosomal studies [7].
Cystic hygroma colli is frequently associated with Turner's syndrome. Karyotypes other than monosomy X are common, and perinatal survival is unlikely in the presence of hydrops fetalis [8].
Hydrops fetalis is a challenging entity for clinicians. Early diagnosis and treatment greatly improves perinatal outcome, though prognosis depends on the underlying etiology.
Differential Diagnosis List
Nonimmune hydrops fetalis with cystic hygroma Colli
Immune hydrops - erythroblastosis fetalis
Turner Syndrome associated with Hydrops
Congenital infectious diseases
Final Diagnosis
Nonimmune hydrops fetalis with cystic hygroma Colli
References
[1] Karen Having , Stephanie Bullock (2011) Fetal Anasarca. Journal of Diagnostic Medical Sonography 27(1) 19 –25
[2] Zlatun Fatusic (Jan-) Non Hydrops Fetalis. Donald School Journal of Ultrasound in Obstetrics and Gynaecology 1(1): 105-110
[3] I. M. Shirley, A. Richards and R. H. T. Ward (1981) Ultrasound diagnosis of hydrops fetalis due to fetal tachycardia. British Journal of Radiology 54, 815-817
[4] Arthur C.Fleischer ,Allen P.Killam,Frank H Boehm,Alastiar A Huchison,Thomas B Jones,Max I Shaff,Jeffery M Barret,Anne M Lindsey,A Everette JamesB Jones,Max I Shaff,Jeffery M Barret,Anne M Lindsey,A Everette James (Oct) Hydrops Fetalis: Sonographic Evaluation and Clinical Implications. Radiology 141:163-68
[5] Carl M Rumack, Stephanie R Wilson, J , William Charboneau, Jo-Ann M Johnson,Associate editor (2005) Hydrops Fetalis. Diagnostic Ultrasound 3rd Edition Vol.2, page 1459-92
[6] Hiroshi Shimokawa , Kenji Hara , Hirotaka Maeda , Shingo Miyamoto , Takashi Koyanagi , and Hitoo Nakano (1988) Intrauterine treatment of idiopathic hydrops fetalis. J. Perinat. Med 16 ; 133
[7] Timo R. de Haan, Dick Oepkes, Matthias F.C. Beersma, Frans J. Walther. (2005) Aetiology, Diagnosis and Treatment of Hydrops Foetalis. Current Pediatric Reviews 1, 63-72
[8] Droste S,Hendricks SK,Von Alfrey H,Mack LA (1991) Cystic hygroma colli: perinatal outcome after prenatal diagnosis. J Perinat Med 19(6):449-54
Case information
| URL: | https://www.eurorad.org/case/10140 |
| DOI: | 10.1594/EURORAD/CASE.10140 |
| ISSN: | 1563-4086 |
Figure 2
Antenatal sonography fetal neck region cross sectional view
Well defined bilobed cystic mass in the fetal nape region posteriorly with a central thick septa - Cystic hygromata Colli.
Figure 3
Antenatal sonography fetal chest and abdomen
Fetal pleural effusion with partial relaxation atelectasis of lung, marked thickening of subcutaneous tissue in chest and abdominal regions- fetal anasarca.
Figure 4
Antenatal sonography fetal chest and abdomen
Fetal pericardial effusion and fetal ascites.
Antenatal sonography fetal head and neck longitudinal section
Thickening of subcutaneous tissue in fetal head and neck regions-Nuchal fold thickening.
Gian S Shergill Inder Scan Centre Amritsar
Gian S Shergill Inder Scan Centre Amritsar
Antenatal sonography fetal neck region cross sectional view
Well defined bilobed cystic mass in the fetal nape region posteriorly with a central thick septa - Cystic hygromata Colli.
Gian S.Shergill Inder Scan Centre Amritsar
Gian S.Shergill Inder Scan Centre Amritsar
Antenatal sonography fetal chest and abdomen
Fetal pleural effusion with partial relaxation atelectasis of lung, marked thickening of subcutaneous tissue in chest and abdominal regions- fetal anasarca.
Gian S.Shergill Inder Scan Centre Amritsar
Gian S.Shergill Inder Scan Centre Amritsar
Antenatal sonography fetal chest and abdomen
Fetal pericardial effusion and fetal ascites.
Gian S.Shergill Inder Scan Centre Amritsar
Gian S.Shergill Inder Scan Centre Amritsar