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Cystic hygroma
Dr M Osama Yonso and Dr Donna D'Souza et al.
A cystic hygroma (CH) refers to a type of congenital lymphangioma (lymphovascular
malformation).
Epidemiology
They usually occur in the fetal - infantile and paediatric populations with most lesions presenting
by the age of two. The estimated prevalence in the fetal population is at ~ 0.2 - 3 %.
Clinical presentation
Patients in the infantile or paediatric populations can present with pain, dyspnoea, infection,
haemorrhage or respiratory compromise.
Pathology
They are thought to arise from delayed development / maldevelopment - failure to communicate
with the venous system of the lymphatic system of the neck. Like other lymphangiomas, there
are endothelial lined cavernous lymphatic spaces. They are microscopically comprised of
endothelium lined cystic spaces with scanty stroma. They can signicantly vary in size. Lymphatic
vascular malformations may be mixed with other forms of vascular malformation, including
capillary or venous.
Location

They occur most commonly in the neck - which is then also termed nuchal cystic hygroma (
occurs in ~ 80% of cases)
mediastinum

2,3

and axilla, with only 10% of cases extending to the

and only 1% conned to the chest 1.

Associations

Associated anomalies can be common


aneuploidic anomalies : 50 - 80 % of cystic hygromas can be associated with an

aneuploidic abnormality
Turner syndrome : most frequent aneuploidic association
Down syndrome : second most frequent aneuploidic association
trisomy 13
trisomy 18
triploidy 11
non aneuploidic
congenital cardiac anomalies :
aortic coarctation : commonest cardiovascular anomaly
hypoplastic left heart syndrome
8

pentalogy of Cantrell
Apert syndrome
Cornelia de Lange syndrome
fetal alcohol syndrome
11

Fryns syndrome
lethal multiple pterygium syndrome
limb hypertrophy
Noonan syndrome
Pena Shokeir syndrome 11
Radiographic features
They are usually well circumscribed and are of uid density. Cystic hygromas may also have an
inltrative appearance, and may be uni or multilocular. The density can also be variable with a
combination of uid, soft-tissue density and fat.
Antenatal ultrasound

On antenatal ultrasound they may present as a nuchal cyst and may show septations + / evidence of fetal anasarca / hydrops fetalis. The presence of septations may indicate a poorer
outcome. Greater volumes ( > 75 mm3 according to one study 9) are thought to correlate with
increased karyotypic abnormality and poorer fetal outcome

9-10

CT

Commonly seen as a hypo-attenuating ill dened cystic mass.


MRI

Reported signal characteristics include


T1 : predominantly low signal - unless there are haemorrhagic components
T2 : predominantly high signal
T1 C+ (Gd) : no enhancement on any component except occasional faint enhancement of
rim
Complications
development of non-immune hydrops fetalis : which often indicates a poorer prognosis
respiratory obstruction from pharyngeal oedema 11
Treatment and prognosis
Management may be by surgical excision or by injection with OK-432, a preparation containing
Streptococcus pyogenes antigens, which induces an inammatory response and subsequent
obliteration of the abnormal cavities. Most fetuses with cystic hygromas have a poor prognosis
although it may improve in utero on its own in a very small proportion of cases. Spontaneous
remission does not necessarily exclude an abnormal karyotype.

Dierential diagnosis
Considerations on antenatal ultrasound include
cervical teratoma
occipital encephalocoele
cervical meningocoele

References
1. Brown LR, Reiman HM, Rosenow EC et-al. Intrathoracic lymphangioma. Mayo Clin. Proc.
1986;61 (11): 882-92. Pubmed citation
2. Faul JL, Berry GJ, Colby TV et-al. Thoracic lymphangiomas, lymphangiectasis,
lymphangiomatosis, and lymphatic dysplasia syndrome. Am. J. Respir. Crit. Care Med.
2000;161 (3 Pt 1): 1037-46. Am. J. Respir. Crit. Care Med. (link) - Pubmed citation
3. Miyake H, Shiga M, Takaki H et-al. Mediastinal lymphangiomas in adults: CT ndings. J
Thorac Imaging. 1996;11 (1): 83-5. Pubmed citation
4. Shaer K, Rosado-de-christenson ML, Patz EF et-al. Thoracic lymphangioma in adults: CT
and MR imaging features. AJR Am J Roentgenol. 1994;162 (2): 283-9. AJR Am J Roentgenol
(abstract) - Pubmed citation
5. Webb WR, Higgins CB. Thoracic imaging, pulmonary and cardiovascular radiology.
Lippincott Williams & Wilkins. (2005) ISBN:078174119X. Read it at Google Books - Find it at
Amazon
6. Mernagh JR, Mohide PT, Lappalainen RE et-al. US assessment of the fetal head and neck:
a state-of-the-art pictorial review. Radiographics. 1999;19 Spec No : S229-41. Pubmed
citation
7. Merz E, Bahlmann F. Ultrasound in obstetrics and gynecology. Thieme Medical Publishers.
(2005) ISBN:1588901475. Read it at Google Books - Find it at Amazon
8. Hsieh YY, Lee CC, Chang CC et-al. Prenatal sonographic diagnosis of Cantrell's pentalogy
with cystic hygroma in the rst trimester. J Clin Ultrasound. 1998;26 (8): 409-12. J Clin
Ultrasound (link) - Pubmed citation
9. Rosati P, Guariglia L. Prognostic value of ultrasound ndings of fetal cystic hygroma
detected in early pregnancy by transvaginal sonography. Ultrasound Obstet Gynecol.
2000;16 (3): 245-50. doi:10.1046/j.1469-0705.2000.00223.x - Pubmed citation
10. Rosati P, Guariglia L. Transvaginal ultrasound detection of septated and non-septated
cystic hygroma in early pregnancy. Fetal. Diagn. Ther. 12 (3): 132-5. - Pubmed citation
11. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme.
(2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon

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Systems: Chest, Paediatrics, Vascular, Obstetrics, Head & Neck

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case7A well dened cystic lesion is seen in neck extending on both sides of midline.
Consequent raised nuchal translucency is seen 5,8 mm.
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