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The term “univentricular heart” encompasses a wide range of cardiac alterations characterized by the fact that just one ventricle supports systemic and pulmonary circulation.
Clinical symptoms and subsequent treatment are determined by the amount of flow that reaches the lungs. Depending on this, defects can be separated into two groups.
Echocardiogram is the most important tool to define the anatomy of the heart and the large vessels in patients with single ventricle.
Total caval-pulmonary deviation, or Fontan circulation, is achieved through a series of procedures in several stages:
Evolution following this treatment is very good. The survival rate is around 90% after 10 years and 85% after 15 years.
In some cases, a heart transplant may be required in the long term due to improper functioning.
It is a cancer that develops in muscle and soft tissue. It can therefore be found in any part of the body, although most commonly in the head and neck, including the eye sockets. Despite being a rare cancer, as are all tumours in children, it is the most common cancer of the soft tissue found in childhood. This disease is more common in boys than in girls.
Although mainly found in the head and neck, it may also occur in the genitourinary system such as the bladder and prostate in boys and the vagina or uterus in girls. It may also appear in other places such as the limbs (arms and legs) and, less commonly, in the abdomen and around the genitals and anus. Symptoms vary depending on the location of the tumour.
More than half of all soft tissue sarcoma found in children are rhabdomyosarcoma. Most children are diagnosed under nine years old, but this type of cancer can appear at any age.
Different symptoms are produced depending on where tumours are located.
Malignant neoplasms are rare, but they are one of the most important causes of morbidity and mortality in this age group. Around 1,000 patients under 14 years of age are diagnosed with cancer every year in Spain. Rhabdomyosarcoma represents 6% of cancers in children meaning there are 60 new cases every year in Spain.
The child’s doctor will perform a very careful examination and to reach a diagnosis the doctor will request several tests, which may include:
These tests will help to determine the size and location of the tumour and whether it has spread to any other part of the body.
Rhabdomyosarcoma is a highly malignant type of tumour and must therefore be treated with a combination of therapies including surgery, chemotherapy and radiotherapy.
Each of these treatments is administered depending on the condition of the tumour and the age of the child.
There are currently no known measures to help prevent this type of tumour.
Retinoblastoma is a malignant intraocular tumour that occurs in babies aged 12-24 months. In 95% of cases the baby survives, but early detection is important to combat the disease. It is essential to detect the disease in time to save the child’s life and to preserve the eyeball whenever possible.
This is tumour that occurs as a result of a mutation of chromosome 13 and which originates in the retina, the light-sensitive layer of tissue that allows the eye to see.
In 60% of cases the mutation that causes retinoblastoma only affects the eye (somatic retinoblastoma) but in some children the mutation may affect all the cells in the body. This is known as “germinal retinoblastoma”. 90% of children with this disorder have no family history of the disease. In Spain, the survival rate is over 95%, but it is important to detect it as soon as possible.
There are two types of retinoblastoma:
The main symptoms of the disease are:
The disease affects 15,000 to 25,000 infants.
To detect the disease, a thorough examination of the eye using an ophthalmoscope after dilating the pupils is carried out. Apart from this, an ocular ultrasound or a brain scan can be carried out, as well as genetic testing of the patient and sometimes their family.
Retinoblastoma requires personalized treatment determined by the characteristics of the tumour and the age of the patient. Methods used to combat it include:
Treatment will vary according to the characteristics of the tumour (size, location, laterality, and extraocular extension).
A white reflection in a child’s pupil is symptomatic of the disease and must therefore be urgently treated.
Truncus arteriosus is a congenital heart defect. At birth, the heart has only one blood vessel and one valve, instead of the usual two arteries and two valves. It is always associated with a ventricular septal defect, which is a hole in the partition that separates the two ventricles.
The single output vessel from the heart means that oxygenated blood is mixed with unoxygenated blood and then enters the lungs and the rest of the body. The arteries that supply the whole body and the lungs originate from this single vessel. The subsequent increased volume of blood that enters the lungs may result in congestive heart failure and lung damage.
The most common symptoms of truncus arteriosus are:
It is a rare heart disorder that affects fewer than 1 in 10,000 children and both genders are affected equally. It may be related to chromosome abnormalities such as DiGeorge syndrome, also known as 22q11 deletion.
Truncus arteriosus is diagnosed via echocardiogram, either prenatally or during the first few hours of life. This is usually sufficient to mean that further diagnosis and tests are not required.
It is repaired during prenatal surgery, during which the two circulations are separated and a conduit inserted into the pulmonary artery. The ventricular septal defect will also be closed.
Most children who undergo surgery recover and go on to develop normally.
In some cases further procedures may be necessary once the child reaches adulthood.
Complete transposition of the great arteries (TGA) is a congenital heart defect in which the aorta fully, or almost fully, exits the right ventricle (RV) and the pulmonary artery fully, or almost fully, exists the left ventricle (LV).
The key sign is cyanosis, which is when there is a blueish cast to the skin. Cyanosis is more intense in cases of complete transposition of the great arteries where there is no ventricular septal defect (VSD).
It tends to be diagnosed before birth through foetal ultrasound. Around 70% of cases are diagnosed in the first few months of foetal development.
Complete transposition of the great arteries can be associated with other anomalies such as ventricular septal defect, pulmonary stenosis or aortic arch hypoplasia. In these cases it is known as “complex complete transposition of the great arteries”.
The severity of any related defects must be investigated to determine the best type of surgery in each case.
An arterial switch, anatomical correction or the Jatene procedure is the most commonly used technique to treat complete transposition of the great arteries at the neonatal stage. Through this technique, the pulmonary artery is connected to the right ventricle and the aorta to the left ventricle. The coronary arteries are also transferred to the new aorta.
Most patients reach adulthood without needing further procedures and with a quality of life comparable to the normal population.
Tetralogy of Fallot is the most common cyanotic congenital heart defect, causing deoxygenated blood to enter the systemic circulation. Children may show cyanosis, breathing crises, and low oxygen saturation. Diagnosis is made via echocardiography, and treatment is surgical, usually around six months of age.
A congenital cyanotic heart defect is a congenital heart disorder in which deoxygenated blood bypasses the lungs and enters the circulatory system, or where there is a mixture of oxygenated and deoxygenated blood entering the system.
It is caused by structural defects in the heart such as bidirectional shunting, or the incorrect position of the pulmonary artery or the aorta, or any condition that increases pulmonary vascular resistance. The result is the development of collateral circulation.
Children with this heart condition will have the following symptoms:
Tetralogy of Fallot makes up 10% of all congenital heart disorders and is considered the most common cyanotic heart disease. There are around 400 cases for every million births.
Diagnosis is confirmed via 2D echocardiogram.
Repair is carried out around six months old. If the baby suffers a episode before the defect has been corrected, treatment is started in the form of beta blockers to reduce lung spasms.
If very severe cyanotic episodes persist in babies under six months despite this treatment, then palliative surgery needs to be performed to take blood to the lungs. This surgery consists of making a connection between a systemic artery and the pulmonary arteries (a systemic-pulmonary fistula).
The definitive corrective surgery involves closing the ventricular septal defect with a patch and widening the outlet from the right ventricle.
In cases of severe pulmonary insufficiency there is progressive dilatation of the right ventricle in the long term. If this becomes excessive, it is necessary to replace the valve. Risk of lung valve replacement is around 20% after 25 years.
Unfortunately, there are currently no measures that can be taken to prevent this heart condition.
These syndromes are a group of diseases characterised by insufficient blood cell production (anaemia, neutropenia and thrombocytopenia), constitutional malformations and the risk of cancer.
They are usually diagnosed in childhood but there some cases diagnosed in adults. Specifically, these syndromes are: Fanconi anaemia, dyskeratosis congenita or selectively severe congenital neutropenia, Diamond-Blackfan anaemia, Diamond-Shwachman syndrome, and amegakaryocytic thrombocytopenia.
Malformations caused by inherited bone marrow failure syndromes affect the skin, bones, heart and digestive system as well as the urinary system, the central nervous system, and others. They may also affect the area around the bone marrow.
Besides this, they also increase the predisposition to cancers such as acute myeloid leukaemia, myelodysplastic syndrome and squamous carcinoma of the head, neck and reproductive organs.
These syndromes have important biological pathways in common related to cell growth and division such as the activation of the p53 gene that is responsible for halting the cell cycle, cell ageing and cell death. In addition, mutations have been identified in more than 80 genes.
Although this is treated in childhood, once the patient reaches adulthood monitoring is very important. Early diagnosis of these syndromes is essential to ensure the patient receives appropriate treatment. The aim is to minimise toxicity at the same time as allowing genetic counselling, and implementing strategies for cancer prevention and monitoring. This treatment should focus on treating the bone marrow failure, but also any constitutional malformations and extra-haematological manifestations, in addition to cancer treatment.
A multidisciplinary team of specialists with extensive experience of these diseases is necessary to ensure optimum patient care.
The first symptoms are related to a lack of blood cell production:
There are also signs related to malformations such as:
Estimated incidence rates of the different syndromes are:
The usual tests to detect inherited bone marrow failure are:
The following methods are used to treat these pathologies:
To prevent these diseases it is important to avoid smoking, alcohol, sun exposure, and to have a balanced diet. In addition, a cancer prevention programme should be followed with regular visits to the Cancer Prevention Unit and the Ear, Nose and Throat, Maxillofacial and Gynaecology departments.
The disease caused by the Zika virus is contracted by a bite from an infected mosquito, as in the case of dengue fever, chikungunya and yellow fever. It can also be spread through sexual intercourse, pregnant women may transmit it to their children, or through blood transfusions. In Europe there are no cases of infection by mosquito; all cases have been imported.
It is disease lasting a short time that can be overcome without complications or the need for admission to hospital. However, there is a relationship between this infection and some neurological disorders. In addition, pregnant women who are infected may give birth to babies with microcephaly.
The incubation period in humans is 3-12 days, up to 15 maximum. Although on many occasions there are no symptoms, when there are the disease is characterised by:
Since 2015, 71 countries have declared transmission of the Zika virus via mosquitoes. In addition, 13 more have stated that the disease has arrived by other means, generally through sexual contact.
In Europe, most cases have been imported from countries where it is endemic, mainly from Latin America but also from South East Asia. In Catalonia in December 2016, there were 150 registered infections, of which 32 were pregnant women.
Between the first seven to ten days of the disease, diagnosis is made using molecular biology techniques (RT-PCR) in blood and urine to detect the virus.
After this period, Zika disappears from the blood and is detected through antibodies in the serum.
There is no specific treatment for this disease. Symptoms generally disappear between three and seven days after infection. They are therefore lessened with analgesics and antipyretics.
There is currently no vaccine for this virus. For this reason, prevention is based on avoiding mosquito bites in countries where it is endemic, as well as using protection during sexual intercourse.
In the case of Catalonia, the risk is associated with the arrival of travellers from countries where it is endemic. Here there is a screening programme for pregnant women and their partners; they are a sensitive group as the virus may be passed to the foetus.
Pulmonary atresia with ventricular septal defect is a rare heart condition characterised by a lack of connection between the right ventricle and the pulmonary arteries.
This is a rare congenital heart defect characterised by no connection between the right ventricle and the pulmonary arteries. It is an extreme type of Tetralogy of Fallot in which blood enters the lungs to be oxygenated by bypassing the heart.
Blood can reach the lungs via the pulmonary arteries themselves, which are not connected to the heart, or via the collateral arteries, which originate from the thoracic aorta and directly supply the lung. There are significant anatomical differences between vessels which must be studied in each individual child.
This condition is very heterogeneous, which creates the variability seen in the pulmonary arteries. Two groups can be distinguished:
The prognosis of this disease depends on the growth of the pulmonary arteries to be able to surgically repair the condition.
It is a rare congenital heart condition which makes up 1-2% of all congenital heart defects.
In most cases, diagnosis is via foetal echocardiogram. This ultrasound will show the lack of connection between the heart and the pulmonary arteries, as well as the presence of VSD. Through this test the size and position of the pulmonary arteries can also be measured.
When a child is born, it has a certain quantity of oxygen, known as “saturation”, in its blood which is around 80-90% of the normal level, although this is enough for the child to develop normally.
Childhood dystonia is progressive and debilitating, but it can be prevented with an early diagnosis and the use of specific therapies that target the genetic defect identified. Currently, several different treatments are used.
Levodopa trial
In children with isolated dystonia, a one to three-month long trial with levodopa must always be carried out. This medication can completely reverse symptoms when the dystonia is caused by a defect in dopamine synthesis. This is called dopa-responsive dystonia.
Such defective dopamine synthesis can be caused by alterations in the genes and proteins that regulate dopamine metabolism. This neurotransmitter is essential in learning, behaviour, and motor function.
Treatment with botulinum toxin
Botulinum toxin is effective at controlling focal dystonia, which affects a muscle group carrying out a specific action, such as, for example, writer’s cramp, or dystonia triggered by walking, treatment for which consists of correcting the abnormal foot or leg position when walking.
In children with generalised dystonia, botulinum toxin can also relieve pain in muscle groups that are particularly affecting the patient’s quality of life.
Treatment with various pharmaceuticals
To treat generalised dystonia in children, different drugs are administered to reduce the tremors, muscle tone, and painful spasms. These include benzodiazepines, GABA antagonists (baclofen), anticholinergics, and α-2 adrenergic receptor agonists. In some patients with paroxysmal dystonia, which is characterised by brief, repetitive involuntary movements at night, anti-epileptics are prescribed.
Intrathecal baclofen pump
When oral medications are not enough, there is a surgical option: the intrathecal baclofen pump. This internal device administers the medication baclofen through a catheter placed in the epidural space (which contains the liquid that surrounds the spinal cord). This helps control generalised dystonia, reducing pain, muscle tone, and spasms and thus improving patients’ quality of life.
This device is used to treat secondary dystonia or dystonia associated with other neurological problems (whether neurometabolic, neurodegenerative, or acquired through brain damage at birth).
Deep brain stimulation or pallidal stimulation
This consists of placing two electrodes in the pallidal nuclei of the brain via a stereotactic procedure, to improve the patient’s motor function and quality of life. Patients who are candidates for pallidal stimulation are those with primary dystonia who have no structural brain lesions in the central nervous system.
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