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Cystic Hygroma and Fetal Hydrops

 

This section is in memory of

    Kristen Marie
    Aug. 19,1995

    Courtney Paige
    March 6, 1996

    Brandon Lee
    Nov. 3, 1997

My sister Charleen's little Angels

Definition: the term "hygroma" means moist tumor. Cystic hygromas are anomalies of the lymphatic system characterized by single or multiple cysts within the soft tissues, usually involving the neck.

Incidence: Cystic hygroma's were found in 1 in 200 spontaneously aborted fetuses with crown-rump length greater than 3 cm.

Explanation: A congenital malformation resulting from the so called jugular lymphatic obstruction sequence, and is due to a lack of communication between the cervical lymphatic vessels and the jugular venous system. This is a lethal anomaly that has long been recognized in aborted fetuses, often associated with generalized hydrops. However, if a communication between the two systems occurs before fetal death, the hygroma may regress leaving redundant skin folds in the neck(pterygium colli).

The majority of female fetuses with cystic hygroma probably have Turner's syndrome. This, the ultrasound detection of a cystic pouch at the back of the neck is highly suggested of monosmy x. However, nuchal hygromas are also part of other malformation syndromes, such trisomy 21, Noona's syndrome and Robert's syndrome. In addition, a few familiar observations of cystic hygromas suggest that this condition may also be inherited as an autosomal recessive trait.

Associated Anomalies: Cystic hygromas are very frequently associated with chromosomal aberrations and, consequently, with a wide variety of anatomic defects.

Hydrops: edema. Retaining of fluids in the body. Hydrops and generalized edema are more frequently associated with cystic hygromas.

Marked edema or fetal hydrops, results from vascular damage and high output heart failure.

Prognosis: The prognosis is different depending on the presence or absence of associated hydrops. In our experience, the mortality rate of cystic hygromas with fetal hydrops is 100%. Prognosis data about fetal cystic hygromas without associated hydrops are scanty.

The natural history of fetal cystic hygromas has not been clearly established. Some infants diagnosed in the second trimester with isolated hygromas may develop nonimmune hydrops as gestation progresses. Others may undergo canalization of the lymphatic channels and resolution of the hygromas. Regression results in redundant skin in the cervical region and webbed neck.

Hydrops fetalis

Hydrops fetalis (fetal hydrops) is a serious condition caused by Rh sensitization and other conditions. A fetus affected by hydrops becomes extremely swollen because of buildup of fluid in the body tissues. Organs such as the liver and spleen become enlarged, the fetus develops anemia, and the ability of the fetus's blood to carry oxygen is decreased.

Even with treatment, fetal hydrops can cause death of the fetus.

About Hydrops Fetalis

 

Links associated with Cystic Hygroma
Please submit any websites that address the topics of Cystic Hygroma,
Fetal Hydrops or associated disorders.

Contact A Family - Contact Group started in 1983. It offers contact with other parents where possible. It publishes a periodic newsletter. The group represents around 200 families in the UK, mainly with affected children. Additionally the group has contact with families in Australia and the USA.

BabyCenter.com Forum regarding Cystic Hygroma

 

>>Unfortunately, I have no further information regarding Cystic Hygroma or Fetal Hydrops. If you have questions, please contact your physician or visit the provided links.

 
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