RESULTS:
Hemangiomas, formed by arteriovenous connections grouped within spaces
of flow voids, are the most frequent benign tumors of childhood and
within these, at the vascular level, the most common in the head and
neck, generating alterations in children’s development, both
physiological as psychosocial (4,8). Its prevalence reaches up to 4-10%
in the child population, without being able to have data regarding its
prevalence in relation to its prenatal diagnosis (4,8) Anatomically,
when these lesions are located in Superficial dermis are called
superficial hemangiomas, manifesting as raised, red lesions; when they
are located in the reticular dermis or in the subcutaneous cellular
tissue, they appear as raised masses covered with normal or
bluish-colored skin, known as deep hemangiomas. When the same
superficial and mixed components coexist in the same lesion, hemangiomas
are called mixed hemangiomas (4).
Hemangiomas can also be divided into two genetically distinct subtypes,
infantile and congenital, and can also present as an isolated event or
in a syndromic association (4). Infantile hemangiomas are red-blue
protruding lesions that can appear up to 4 weeks after birth and
continue to develop 5 to 6 months after it, in rapid growth that can
even lead to doubling in size, all this to later gradually involute from
the very beginning from 6 to 12 months of life and up to 4 years (2,4).
Its proliferation will also depend on the phase in which the lesion is
located, which can be proliferative in which 80% of hemangiomas reach
their maximum volume at 5 months (early proliferative phase) or up to 9
months (early proliferative phase). late proliferative), this is
followed by the phase of partial regression and total regression (8).
This type of hemangiomas can be divided into localized (single, focal)
and segmental (plaque-shaped and multifocal) lesions (1,9).
On the other hand, congenital hemangiomas are present at birth and can
be rapidly involuting, partially involuting, or non-involuting. The
appearance of these lesions occurs in the twelfth week of gestation,
which is why its prenatal diagnosis is possible if the ultrasound
evaluation is carried out by trained personnel (1). Commonly, the skin
manifestations of neonatal hemangiomas are related to liver
manifestations, which is why it is always necessary to actively search
for similar lesions at the liver in ultrasound assessments (4).
A retrospective study carried out at the University of Chile with 174
patients diagnosed with infantile hemangioma found that 61% of the
lesions were located on the head and neck, 21% on the trunk, 10% on
the upper limbs, 4% on the lower limbs and 3% in the anogenital
region. In these patients, the presence of arteriovenous shunts was also
identified in 20% of the lesions and direct afferent branches of the
main regional arteries in 15%, which were associated with delayed
involution of the lesions in some patients and hindered therapeutic
response (8).
The use of ultrasound, especially in the prenatal diagnosis of this type
of lesions, seeks to establish the presence of secondary obstructive
compromise, especially of the airway that could configure a problem at
the time of birth, establishing even the need to perform a cesarean
section with the EXIT procedure. (1). Likewise, the evaluation of
lesions through the application of Doppler flow helps to classify
lesions into low-flow and high-flow lesions, thus predicting their
behavior and the response to drug treatment or the appropriate moment
for postnatal surgical management (4, 9).
The introduction of fetal echocardiography with its basic cuts and
especially the cut of 3 vessels and trachea (3VT) in routine prenatal
ultrasound has also made it possible to detect the presence of venous
anomalies such as persistent left superior vena cava, this especially
when it is related to other congenital heart diseases (CHD),
aneuploidies, heterotaxy and extracardiac anomalies (6,10)
PLSVC has a prevalence in the general population of 0.3-0.5%, which
increases to 4-8% in patients with congenital heart disease (5,10,11).
It has its anatomical origin at the junction of the left jugular and
left subclavian veins, running anterior to the aortic arch and the left
pulmonary artery to the lateral border of the left atrium to finally
drain into the coronary sinus in 90% of cases and in 10% in the left
atrium. Dilation of the coronary sinus then becomes one of the main
ultrasound signs of PLSVC in the 4-chamber view, as well as the presence
of a fourth vessel to the left of the pulmonary trunk and the ductus
arteriosus or the appearance of abnormally large vessels ordered (from
right to left: aorta, pulmonary artery, PLSVC) in the 3VT section
(5,6,12).
A prospective cohort study sought to assess the outcomes of patients
with isolated prenatally diagnosed PLSVC compared with those associated
with other abnormalities. A total of 256 fetuses were included in this,
of which 113 fetuses entered the group with isolated PLSVC; none of
these fetuses had adverse neonatal outcomes, however 10 cardiac
abnormalities (8.8%) and five extracardiac abnormalities (4.4%) were
subsequently diagnosed in the postnatal period. In the group of fetuses
with extracardiac anomalies and cardiac anomalies of the 143 analyzed
fetuses, 27 of them with septal defects, 23 with conotruncal anomalies,
and 17 cases of obstructive disease of the left ventricular outflow
tract were detected, setting heart disease as the most common
abnormalities. In the study, 11 postnatal deaths were identified, 7 in
the neonatal period and 4 after postnatal cardiac surgery; all deaths
were attributed to complications from associated pathologies, since the
presence of PLSVC could not be related as a cause of perinatal death
(7,10)
In relation to aneuploidies, a retrospective study in which 95 cases of
patients with PLSVC were analyzed and found a prevalence of 13.5% of
aneuploidies in their population. Similarly, it was identified that
isolated cases of PLSVC had better obstetric outcomes than non-isolated
cases, a group in which the presence of cardiac and extracardiac
anomalies worsened neonatal outcomes (11). One of the anomalies that has
also been related to PLSVC is the VACTERL anomaly, which has been
analyzed in several studies, finding an incidence of association ranging
from 3.2% to 25% (13).
Only one of the articles reviewed reported a 57-year-old male who
attended the emergency department for chest pain and in whom an
irregular mass in the pericardial chamber was documented during
echocardiography. During the surgical procedure, it was established that
this mass was a cardiac hemangioma measuring 110x65x45 in the wall of
the right atrium that seemed to involve part of the interatrial groove;
additionally, the lesion presented a communication towards a 3 mm
diameter IPVC between the coronary sinus and hemangioma (14).