Hygroma of the fetal neck: causes of development and probable outcome

Causes of fetal neck hygroma

Polluted air can greatly affect the health of the unborn child.

Neck hygroma in the fetus (also known as ganglion or lymphangioma) is a consequence of impaired development of the lymphatic system in the first months after conception. This disease may be accompanied by other pathologies.

The causes of cystic hygroma of the fetal neck are presumably the following:

• unfavorable environment;
• abnormalities of the chromosomal system;
• infectious diseases during pregnancy;
• harmful addictions of the mother (drug addiction, alcoholism, smoking);
• burdened heredity;
• genetic mutations.

Poor ecology is one of the most important factors. The fact is that children born in industrial regions are more likely to encounter genetic mutations. In general, with cystic hygroma of the fetal neck, it is very difficult to identify the cause; doctors tend to assume that the pathology develops under the influence of several factors at once, leading to disruption of the development of the lymphatic system in the prenatal period.

Causes such as abnormalities of the chromosomal system predispose to the appearance of cystic hygroma of the fetal neck. Neck hygroma is common in children with Down syndrome. Pathology may accompany Shereshevsky-Turner syndrome and Roberts syndrome - chromosomal pathologies. In all cases, there is a pronounced delay in mental (Down syndrome, Roberts syndrome) or physical (Shereshevsky-Turner syndrome) development.

Compounded heredity as a cause of fetal neck hygroma includes the presence of congenital pathologies of the lymphatic system in close relatives.

Pathology develops in the first two months of fetal growth. Hygroma of the neck can occur against the background of an infectious disease in the mother in the first trimester of pregnancy. When this pathology occurs, it is extremely important to promptly identify the disorder and related problems, which may include dangerous genetic mutations. Hygroma of the fetal neck is very dangerous and can be fatal. The prognosis depends on concomitant diseases.

Description of congenital pathology

Cystic hygroma of the fetal neck is a benign tumor, which has the shape of a bag, where there are cysts of various sizes (from one millimeter to five centimeters), inside of which there is an accumulation of serous fluid and mucus. Children with this pathology experience paresis of the facial nerve, curvature of the spine and lower jaw, as well as bones at the back of the head, impaired swallowing reflex and difficulty breathing.

Hygroma is an isolated neoplasm, but sometimes fluid from the cysts seeps into the surrounding soft tissue, under the skin, and fiber. The tumor can be located on one or both sides of the neck, and cystic hygroma can also appear in another place, occupying a large area.

Quite often, hygroma of the fetal neck is accompanied by diseases such as Turner, Down or Kleitenfelter syndrome, Edwards or Patau disease, mosaicism.

Clinical picture

The pathology has no characteristic symptoms and manifestations, and is detected exclusively during routine screening. Disruption of the development of the lymphatic system and the formation of hygroma in the fetus do not affect the well-being of the mother.

In most cases, hygroma can be diagnosed using a routine ultrasound at 12 weeks of pregnancy.

Hygroma of the fetal neck is of three types:

• cavernous;
• cystic;
• simple.

Cavernous lymphangioma is a cavity in the cervical spine filled with lymph. These cavities are formed from muscle fibers, intertwining lymphatic and blood vessels. The basis is a dense frame of connective tissue.

Cystic lymphangioma of the fetal neck is one or more cavities with liquid contents, which can either communicate with each other or be separated by septa. It is formed from connective tissue and has a dense structure. This form of pathology is characterized by the absence of communication with the lymphatic system. The size of cysts can vary from a few millimeters to 3-4 centimeters; there are known cases of tumors the size of a child’s head.

Simple lymphangioma or hygroma of the fetal neck is a pathological proliferation of lymphatic vessels, the walls of which form the tumor cavity. Such hygromas are localized strictly in one area of ​​the skin and can grow into the subcutaneous tissue.

In a simplified classification, hygromas are designated by size. A small tumor less than 50 mm in diameter is called microcystic lymphangioma. In case of significant proliferation of blood vessels or connective tissue with the formation of tumors larger than 50 mm, macrocystic hygroma is diagnosed.

Fetal chest tumors

Tumors in the fetal chest are very rare, but a variety of tumors can develop in the fetal chest and heart. Lymphangioma and hemangioma can be found in the axilla and mediastinum.

Congenital childhood fibrosarcoma is a very rare mesenchymal tumor that primarily develops in the soft tissue of the distal extremities and occasionally in unusual locations such as the lungs and heart. Prenatal diagnosis is possible after 26 weeks of pregnancy using ultrasound and MRI, but congenital infantile fibrosarcoma is usually diagnosed as a hemangioma.

Congenital cardiac tumors are rare, with a prevalence of 0.01%-0.05%. Rhabdomyoma is the most common cardiac tumor in infants and children, accounting for 60 to 86%, followed by fibroma, teratoma, myxoma, and hemangioma. However, differential diagnosis between these tumors is difficult. Rhabdomyoma can occur as single or multiple lesions in the ventricles and can be detected in utero. On prenatal ultrasound, rhabdomyoma appears as a homogeneous, echogenic intracardiac mass and is most often located in the ventricle, especially the interventricular septum (Fig. 5). Cardiac rhabdomyomas are often multiple, occurring in more than one chamber of the heart, and can cause arrhythmias, obstruct blood flow to the heart, or reduce contractility.

Figure 5:
Ultrasound images of cardiac rhabdomyoma in a 34-week fetus with tuberous sclerosis. A. _ Four-chamber image of the fetal heart shows echogenic masses in the interventricular septum (arrow) and in the left and right ventricles (arrowheads). B. _ Ultrasound of the fetal brain shows inferior echogenic hamartomas in the frontal subependymal regions of the lateral ventricles (arrows).

Diagnostics

An ultrasound examination will immediately “see” the problem (if there is one), but not in the very early stages of pregnancy

Pathology can only be detected by routine ultrasound. Moreover, it is not advisable to do it for a period of less than 12 weeks, due to the low information content.

In the early stages, pathology can be suspected by the characteristic thickening of the cervical-collar area of ​​the fetus. An ultrasound shows thickening of this area, but this does not in all cases indicate a hygroma. You can consider the suspected pathology in more detail in the second trimester. An ultrasound examination can detect cystic growths in the neck area, as well as suggest their nature by the nature of the formation of walls and partitions in the tumor cavity.

Neck hygroma in the fetus often has chromosomal causes of development, so additional examinations are carried out to identify chromosomal disorders, such as Down or Robertson syndrome. Additional examinations that may be prescribed:

• chorionic villus biopsy;
• placentocentesis;
• amnion membrane puncture;
• puncture of umbilical cord vessels.

Based on the results of a comprehensive examination, a diagnosis is made and a decision is made on further treatment.

It should be noted that the presence of hygroma with an accompanying chromosomal disorder is an indication for termination of pregnancy. This is due to the fact that children with such disorders have severe impairments in physical and mental development, mental retardation and developmental disorders of the reproductive system are diagnosed. In addition, hygroma with chromosomal abnormalities in itself is a serious disease, which often ends in intrauterine fetal death in the later stages.

A favorable outcome for fetal neck hygroma is only possible if the benign neoplasm is not associated with abnormalities of the chromosomal system, but is caused, for example, by trauma.

No one can influence the decision to continue the pregnancy except the woman herself, so you should understand the specifics of treatment for this pathology.

Symptoms of pathology

The first signs of the disease can be observed immediately after birth or in the first few years of life. Most often, cystic hygroma does not manifest itself in any way during the intrauterine development of the fetus. The expectant mother does not experience any discomfort, so pathology is often detected after the birth of the baby. Often, signs of the disease do not appear for the first two years of his life.

As the child grows, the cervical hygroma increases in size, causing pain, and increases during physical activity. Hyperemia begins to develop in the affected area, breathing and swallowing reflex are impaired, and apnea may occur.

A rounded neoplasm up to five centimeters in size appears in the neck area, which can be soft or hard, and cause pain when palpating and moving the head. If there is an inflammatory reaction, redness occurs on the skin. Often, pathology contributes to the appearance of dystrophy due to the inability to eat normally.

The neoplasm can put pressure on blood and lymphatic vessels and disrupt the activity of many body systems. The tumor cannot resolve on its own, so it requires immediate treatment.

Treatment methods

If pregnancy continues, treatment of hygroma is carried out in the first days of the newborn’s life. Congenital disorder is treated in two ways - conservative and surgical. If conservative treatment can be carried out from the first days of life, surgical treatment is postponed for several months or even years, depending on the form of hygroma and the severity of associated symptoms.

In general, hygroma itself most often does not pose a danger, since it is a benign neoplasm. The decision to undergo urgent surgery immediately after birth can only be made if the tumor is pressing on important nerve endings or impeding breathing.

Conservative therapy

Ural irradiation warms up well and promotes regeneration processes

Conservative therapy is based on three methods:

• puncture of the tumor followed by sclerosis;
• use of glucocorticosteroids;
• physiotherapeutic methods.

First, a puncture (puncture of the tumor) is performed, with the help of which the accumulated fluid is removed from the cystic neoplasm. The procedure is painless, since before the puncture an anesthetic injection is given into the area of ​​further manipulation. The doctor uses a syringe to make a puncture in the cavity, then uses the same syringe to remove fluid from the cyst.

After the cavity is cleared of liquid contents, a sclerotherapy drug - bleomycin or hemoblock - is injected directly into it. This procedure glues the walls of the vessels from which the tumor is formed, and also promotes further resorption of the hygroma.

After the sclerotherapy procedure, glucocorticosteroids can be used. They are also injected directly into the cavity of the tumor. These medications prevent inflammatory processes, reduce existing swelling and prevent the re-formation of the cyst.

To restore the function of the lymphatic system in the affected area, a course of physiotherapy is prescribed. Electrophoresis of the cervical-collar area is most often recommended. Can be used alone or with medications, for example, hormonal drugs Hydrocortisone. The choice of physiotherapy technique largely depends on the size of the tumor.

The second popular and effective treatment method is ultraviolet irradiation of the affected area. This method stimulates local metabolic processes, eliminates congestion and swelling, and accelerates tissue restoration.

As a rule, a conservative treatment method provides a good therapeutic effect, after which the risk of relapse is minimized. However, after a course of therapy, the child must undergo regular examinations, since no one is immune from the re-formation of hygroma in the same area.

Surgery

The surgical method is inferior in effectiveness to conservative therapy. This is associated with a high risk of complications during surgery, as well as re-formation of hygromas. The fact is that conservative treatment is aimed at restoring the functioning of the lymphatic vessels, as a result of which the re-formation of hygroma is unlikely. Surgical removal of the tumor allows you to quickly and effectively get rid of a cosmetic defect, but does not significantly affect the functioning of the lymphatic system, which is why relapse is possible in the future.

Surgery is most often resorted to in two cases - a visible physical defect caused by a hygroma, and negative consequences associated with breathing problems or compression of nerve endings by a tumor.

The classic method of treatment is removal with a scalpel. The operation is performed under general anesthesia. The doctor removes the tumor along with the tissue that forms the cyst capsule, then applies sutures. The disadvantages of the operation are that a scar remains and the wound may become infected.

The second method, more gentle and effective, is laser burning of the hygroma. The hygroma is destroyed from the inside, as laser radiation causes the liquid in the tumor tissue to boil. The advantages of the method are fast rehabilitation, absence of scars. With laser removal there is a minimal risk of bleeding and relapse.

Fetal brain tumors

Fetal intracranial tumors are rare, accounting for 0.5% to 1.9% of all tumors in children. The most common brain tumor is teratoma, followed by astrocystoma, craniopharyngioma, and primary neuroectodermal tumor. Intracranial tumors are usually detected in the third trimester. Most of them have a poor prognosis. Such tumors may cause spontaneous intracranial hemorrhage or dystocia during labor.

Teratoma is the most common congenital neoplasm, consisting of tissue derived from all three germ layers, and can occur in a variety of locations. Fetal brain teratoma typically appears as a large, solid and/or cystic tumor, often replacing normal brain tissue (Figure 1) and sometimes eroding the skull. Most fetuses diagnosed in utero with intracranial teratomas die before or shortly after birth. The prognosis worsens with increasing tumor size and decreasing gestational age at diagnosis.

Figure 1:
Ultrasound findings (cerebral teratomas) in a 21-week fetus. A. _ A huge solid and cystic mass (arrows) in the posterior fossa replaces and compresses the cerebral hemispheres (asterisk). IN .
Color Doppler sonography shows noticeable blood flow in the tumor. Most fetal astrocytomas occur supratentorially in the posterior fossa. Fetal astrocytomas range from benign to malignant tumors. Glioblastoma accounts for 2%-9% of congenital brain tumors and 60% of fetal brain astrocytomas. On antenatal ultrasound, glioblastoma appears as a large, homogeneous, hyperechoic mass including supratentorial brain parenchyma. They are usually accompanied by hydrocephalus. The prevalence of hemorrhage in congenital glioblastoma is 18%, which is significantly higher than in children and adults. The rapid growth of congenital glioblastoma may be associated with a higher incidence of bleeding, which explains its poor prognosis. In several reported cases, the cause of immediate death was heart failure due to anemia caused by the sequestration of large amounts of blood by the tumor.

Fetal intracranial hemorrhage usually mimics brain tumors (Figure 2). In many cases, a differential diagnosis between an intracranial tumor and a hematoma is not possible, but some findings may be helpful in the differential diagnosis. The hematoma is usually well demarcated and demonstrates a decrease in size and a change in echo pattern on subsequent ultrasound examinations.

Figure 2:
Ultrasound of an intracranial hematoma in a 29-week fetus. A. _ Axial image shows a large mixed echogenic mass (arrow) on the left side of the cerebral hemisphere. The cranial vault (arrowhead) is shifted to the right. These findings are very similar to a brain tumor, but the boundaries of the mass are much better defined . B. _ Doppler imaging demonstrates a lack of blood flow within the mass.

Consequences and complications

In most cases, the fetus dies before the due date

Successful delivery and fetal survival with hygroma on the neck is rare. In more than half of the cases of development of this pathology, intrauterine fetal death occurs in the second half of pregnancy.

In another third of cases, the pathology is associated with severe chromosomal abnormalities, so even in the case of successful pregnancy and uncomplicated childbirth with subsequent treatment of hygroma, the child has a delay in mental or physical development.

In other cases, when there are no chromosomal abnormalities, parents may encounter the following problems:

• birth injuries;
• asphyxia when passing through the birth canal;
• severe disturbances of swallowing and respiratory function due to large hygroma on the neck;
• paresis and paralysis of the facial nerves due to compression by the tumor;
• jaw deformation;
• spinal deformity.

Some complications cannot be treated and will remain with the child for life.

In most cases, doctors recommend termination of pregnancy with this diagnosis, so the woman should consult with a competent specialist.

Forecast

With the development of cystic hygroma, there is a risk of fetal death before birth. It is also possible to develop asphyxia during childbirth. If there were no problems with childbirth, a sick child may have many problems in the future. During the operation, paresis of the facial nerve may develop.

Sometimes in the prenatal period spontaneous resorption of hygroma or its significant reduction was observed.

If the operation is successfully performed and there are no concomitant pathologies, the child fully recovers and lives a full life. Relapses of the disease do not occur.

Prevention

Various reasons lead to the development of fetal neck hygroma, and a woman can influence some of them. Of course, no one is immune from chromosomal abnormalities, and even geneticists often cannot predict their occurrence, but you can protect yourself from injuries and infections during pregnancy. To do this, you need to approach the issue of conception wisely and plan for a new addition to the family, and not rely on chance. Strong immunity, timely vaccinations against infectious diseases and careful attention to one’s own health are what can prevent the development of hygroma in the fetus.

In addition, when planning a pregnancy and subsequent bearing a child, a woman should eat properly and not drink alcohol or drugs. Smoking and other bad habits and addictions should be stopped at least six months before the expected conception.

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Fetal liver tumors

Liver tumors account for approximately 5% of all congenital tumors. A wide variety of both benign and malignant neoplasms can develop in the fetal liver, including hemangioendothelioma, mesenchymal hamartoma, hepatoblastoma, hepatocellular adenoma, and other metastases. Imaging findings for fetal liver tumors may overlap widely, and early detection and antenatal surveillance of these tumors is important for fetal, maternal and postnatal care.

The histogenesis of benign vascular tumors of the liver is not entirely clear, which has led to some confusion in terminology. In the literature, vascular tumors are variously described as hemangioma, infantile hemangioma, hepatic arteriovenous malformation, and infantile hemangioendothelioma. Although hemangioma is the most common benign liver tumor, prenatal diagnosis of liver hemagioma is rarely reported. The true incidence of fetal liver hemangioma has certainly been underestimated, since this tumor is often uncomplicated and resolves spontaneously within the first two years of life. On antenatal ultrasound, hepatic hemangioma usually appears as a well-circumscribed mass with a diameter ranging from 1 to 10 cm. It can be single or multiple. Multiple hepatic hemangiomas sometimes appear as part of the syndrome of generalized hemangiomatosis. The internal structure is usually heterogeneous, with hypoechoic areas located centrally, and both hypo- and hyperechogenicity can be observed. CDUS may show vascular circulation with a low resistive index (Fig. 6). The mortality rate for childhood hepatic hemangioma is reported to be 12%-90%. Complications include congestive heart failure, thrombocytopenia, and intra-abdominal hemorrhage due to rupture of the hemangioma.

Mesenchymal hamartoma is a benign liver tumor consisting of large, fluid-filled cysts surrounded by loose mesenchymal tissue containing small bile ducts.

Hepatoblastoma is the most common congenital liver disease, but it is less common during fetal life than hemangioma or mesenchymal hamartoma. Hepatoblastoma usually appears as a well-defined echogenic lesion that may have a spoke-wheel appearance. Calcifications and pseudocapsule may be common features of hepatoblastoma.

Figure 6:
Prenatal and postnatal imaging features of hepatic hemangioendothelioma in a 36-week fetus. A. _ Coronal ultrasonography image shows a low-echoic, large mass (arrows) with a lobulated outline in the fetal liver. B. _ Color Doppler image shows prominent blood flow (arrows) at the periphery of the mass. C. _ T2-weighted magnetic resonance image reveals a large mass (arrows) with high signal intensity in the fetal liver. D. _ Postpartum contrast-enhanced computed tomography taken in the arterial phase reveals a large liver mass with peripheral bright enhancement (arrows).

Tumors of the fetal pelvic cavity

Sacrococcygeal teratoma is the most common congenital neoplasm. The prevalence has been reported as 1 in 40,000 live births with a 4:1 female predominance.

It usually appears as a large midaxial exophytic mass in the perineum. It can be almost completely external (type I), internal and external in equal parts (type II), predominantly internal (type III) or completely internal (type IV). On antenatal ultrasound, most of these tumors are seen as a solid or mixed cystic and solid outer caudal mass (Figs. 10, 11). Only a small proportion are completely cystic.

Sacrococcygeal teratoma can cause significant perinatal morbidity and mortality and can lead to various complications such as severe dystonia, fetal edema, polyhydramniosis, or hemorrhage during labor. Because of vascular stealing due to high metabolic demand and secondary high-output heart failure, the prognosis for fetuses with solid tumors is usually poor. Mirror syndrome is a life-threatening condition involving maternal fluid retention and hemodilution, mimicking severe preeclampsia, which manifests as progressive maternal edema “reflecting” the illness of the affected fetus.

Figure 10:
Prenatal ultrasonography findings of a large sacrococcygeal teratoma in a 21-week fetus. A. _ Sagittal image shows a large cystic bulging mass (arrow) in the fetal perineum. IN . Color Doppler sonography revealed thin blood flow in the solid part of the mass.

Figure 11:
Prenatal ultrasonography and magnetic resonance imaging (MRI) findings of an intravertebral sacrococcygeal teratoma in a 24-week fetus. A. _ Multilayered cystic mass with lobular outline (arrow) in the fetal perineum. B. _
Sagittal T2-weighted magnetic resonance image reveals a multilayered cystic mass in the anterior sacrum and coccyx. Rhabdomyosarcoma is the most common soft tissue tumor in children, but is very rare in fetuses. The most common locations are the head and neck (41%), genitourinary tract and abdomen (34%), and trunk or extremities (25%). Prenatal ultrasound may reveal a large echogenic soft tissue mass replacing normal tissue (Figure 12). Differential diagnosis from other soft tissue tumors such as teratoma and fibrosarcoma is usually not possible, and postpartum biopsy is necessary. The prognosis depends on the stage of the disease, age, primary site and tumor subtype.

Figure 12:
Prenatal ultrasonography (US) and magnetic resonance imaging (MRI) findings of pelvic rhabdomyosarcoma in a 36-week fetus. Sagittal image of the fetal pelvis shows a large mass (arrows) in front of the sacrum and coccyx, simulating a solid pelvic teratoma. IN . Color Doppler sonography shows noticeable vascular flow in the mass. C. T2-weighted magnetic resonance image shows a well-demarcated solid mass (arrows) in the posterior inferior pelvic cavity.