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47 Cards in this Set
- Front
- Back
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What does Tracheoesophageal Anomalies cause? |
This condition is frequently complicated by aspiration of secretions into the lungs causing:
Pneumonia
Choking
Hypoxemia
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What is Tracheoesophageal fistula (TEF)? |
Tracheoesophageal fistula (TEF) is a connection or hole between the lower esophagus and the trachea.
(So feeding can enter trachea and cause baby to choke/desat) |
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What are some Tracheoesophageal Anomalies? |
Esophageal atresia is a narrowing or obstruction of the esophagus.
There are several types of esophageal atresia.
In most cases, the upper esophagus ends blindly and does not connect with the lower esophagus and stomach.
***Over 30% will have other abnormalities such as heart disorders or other gastrointestinal tract disorders.
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What are the clinical signs for esophageal atresia? |
Accumulation of secretions in the mouth
Respiratory distress, especially during feedings
Repeated regurgitation of feedings
Polyhydranimos in utero |
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What is the diagnosis of TEF? |
Polyhydramnios will arouse suspicion of esophageal atresia or other obstruction of the gastrointestinal tract. If suspected, an attempt to pass a nasogastric tube through the mouth or nose into the stomach should be made. |
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What test will provide a definite diagnosis of TEF? What will it show? |
A CXR or esophagram will provide the definitive diagnosis. It will show an air filled pouch, and air in the stomach and intestine. |
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What is Choanal Atresia? What are the two types? |
Is a congenital anomaly of the anterior skull base characterized by closure of one or both posterior nasal cavities. (More common- nasal doesn't separate properly) Approximately 60% of reported cases are unilateral with a right-sided predominance.
Bilateral choanal atresia is commonly associated with other congenital anomalies.
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Bilateral choanal atresia causes what? When is it suspected? What do newborns require with this condition? |
respiratory distress at birth. Neonatologists suspect the diagnosis when they can not pass a 6 French catheter through the nasal cavities into the nasopharynx. Also suspected as respiratory distress improves when the neonate cries Most newborns with the condition require early intubation. |
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Unilateral lesions diagnosis |
Unilateral lesions may go undiagnosed until the child presents with persistent unilateral nasal drainage. (This is because they can still breathe through one nostril) |
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What is the preferred diagnosis for choanal atresia? |
The CT scan with 3 mm transverse sections is the preferred method to confirm a diagnosis of unilateral or bilateral choanal atresia |
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Treatment for choanal atresia |
Surgery -In cases associated with severe craniofacial anomalies, a tracheotomy may be needed until definitive repair can be performed safely. In cases where the skull base has a relatively normal shape bilateral choanal atresia should be repaired shortly after birth. |
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Congenital diaphragmatic hernia (CDH) is |
is the absence of the diaphragm, or a hole in the diaphragm. |
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Where does CDH occur? Occurs in how many births? |
This can occur on either the left or right side, but is most common on the left (90%) It occurs in approximately 1 in 2200 births The contents of the abdomen including the stomach, intestines, liver and spleen may go through the hole and into the chest preventing the normal development of the lung (pulmonary hypoplasia) on that side. This prevents the growth of the other lung |
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There are two major types of diaphragmatic hernia: |
Bochdalek hernia Morgagni hernia |
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Bochdalek hernia |
This type is common (95%) and involves an opening on the back side of the diaphragm. The stomach, intestines and liver or spleen usually move up into the chest cavity. |
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Morgagni hernia: |
This type is rare (only 5%) and involves an opening in the front of the diaphragm, just behind the breast bone. The liver or intestines usually may move up into the chest cavity. |
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Diagnosis CDH? |
CDH can be detected on prenatal ultrasound at around 18 weeks when the bowel is seen in the chest of the fetus. Sometimes the CDH is not diagnosed until after the baby is born. Physical examination Chest x-ray Blood gases |
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Clinical sign CDH |
Often presents with cyanosis and respiratory distress soon after birth Prognosis is related to the time of onset and degree of respiratory impairment Examination shows the abdomen to be flat Air entry is reduced on the affected side Heart sounds are often displaced |
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CDH Treatment |
Respiratory support with intubation and ventilation is usually require Do not resuscitate with bag and mask A nasogastric tube should be passed Gas exchange and acid-base status should be assessed Acidosis may need correction with bicarbonate infusion Inhaled nitric oxide (NO) or ECMO may reduce pulmonary hypertension |
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CDH Treatment what should be considered early after resuscitation ? |
Surgery |
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Micrognathia: AKA ________ Is? |
Micrognathia (commonly known as Pierre-Robin Syndrome) is: a group of abnormalities marked primarily by a very small lower jaw with a tongue that falls back and downward. The syndrome may include a high arched palate or cleft palate. |
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Micrognathia The tongue appears ______ and is placed ______? Occurs in how many births? |
Tongue appears large (is actually normal size but big relative to jaw) and is placed unusually far back in the oropharynx It occurs in 1 out of 2000 births 50 – 70% have a cleft palate |
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Clinical Signs of Micrognathia |
Very small jaw with marked receding chin High arched palate Cleft soft palate Choking on tongue |
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Diagnosis and Treatment of Micrognathia |
Physical examination is usually sufficient to diagnose this condition. Treatment is to maintain the patient’s airway patency until the mandible grows to its appropriate size – usually 6 months to a yearIn moderate cases, the patient requires placement of a nasopharyngeal airway to avoid airway blockage. In severe cases, surgery is indicated for recurrent upper airway obstruction. Mandibular lengthening is now the treatment of choice Tracheostomy is sometimes required. Feeding must be done very carefully to avoid choking and aspiration of liquids into the airways. |
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Congenital Heart Disease How many babies in US are born with this ? When is the fetal heart completely formed? When do these heart defects occur and why? |
One out of every 100 babies born in the United States has a congenital heart defect. Congenital heart defects are the most common birth defects. The fetal heart is completely formed by eight weeks into the pregnancy. Congenital heart defects happen during this crucial first eight weeks of the baby's development. The vast majority of congenital heart defects have no known cause. |
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Acyanotic Heart Defects |
Problems that cause too much blood to pass through the lungs.
Problems that cause too little blood to travel to the body. Acyanotic (non-cyanotic) heart defects allow oxygen-rich blood (that should be traveling to the body) to re-circulate through the lungs, causing increased pressure and stress in the lungs. |
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Cyanotic Heart Defects |
Problems that cause too little blood to pass through the lungs |
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Patent Ductus Arteriosus (PDA) |
This defect allows blood to mix between the pulmonary artery and the aorta. Prior to birth, there is an open passageway between the two blood vessels, which closes soon after birth. When it does not close, some blood returns to the lungs. Patent ductus arteriosus is often seen in premature infants. |
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Treatment of PDA |
In premature infants, an attempt is made to close the patent ductus by fluid restriction and prostaglandin inhibitors (e.g., indomethacin).
Surgical ligation (tying) of the patent ductus is undertaken if these steps do not close the ductus.
In full-term infants, the surgery is postponed until 6 months to 3 years of age, unless problems develop. |
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Atrial Septal Defect (ASD) |
Is an abnormal opening between the right and left atria causing an abnormal blood flow through the heart. Some children may have no symptoms and appear healthy. However, if the ASD is large, permitting a large amount of blood to pass through the right side, symptoms will be noted. |
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Treatment ASD |
ASD may not require treatment if there are few or no symptoms, or if the defect is small. Surgical closure of the defect is recommended if the ASD is large or if symptoms occur. Recently, a new procedure has been developed to close the defect without surgery. This involves the introduction of a closure device into the heart through wires or catheters. |
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Coarctation of the Aorta |
The aorta is narrowed or constricted, obstructing blood flow to the lower part of the body and increasing blood pressure above the constriction. Usually there are no symptoms at birth, but they can develop as early as the first week after birth. If severe, symptoms of high blood pressure and congestive heart failure develop, and surgery may be considered. |
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Treatment of Coarctation of the Aorta |
Surgery is usually advised. Occasionally, balloon angioplasty (using a technique similar to that used to open the coronary arteries, but performed on the aorta) may be an alternative to surgical repair. |
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Cyanotic Heart Defects |
Cyanotic heart defects cause too little blood to pass through the lungs. These defects allow blood that has not been to the lungs to pick up oxygen (and, therefore, is oxygen-poor) to travel to the body. The body does not receive enough oxygen with these heart problems, and the baby will be cyanotic |
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Tricuspid Atresia |
In this condition, there is no tricuspid valve, therefore, no blood flows from the right atrium to the right ventricle. |
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Tricuspid Atresia is characterized by |
a small right ventricle a large left ventricle diminished pulmonary circulation Cyanosis A surgical shunting procedure is often necessary to increase the blood flow to the lungs. |
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Treatment of tricuspid atresia |
Surgery is required to repair the effects of tricuspid atresia. Operative strategies depend on whether the lungs are receiving too little or, occasionally, too much blood. In the most common situation, a series of operations is required. |
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Transposition of the Great Vessels |
With this congenital heart defect, the positions of the pulmonary artery and the aorta are reversed. The aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs. The pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again |
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Treatment of Transposition of the Great Vessels |
Immediately after diagnosis, a prostaglandin drip is started to maintain the ductus arteriosus and the foramen ovale between the pulmonary and systemic circulations. Palliative measures include enlarging or creating a hole in the atrium to allow oxygenated blood into the general circulation Reconstructive open heart surgery will redirect the blood flow appropriately. A newer surgical technique known as an arterial switch procedure may allow permanent correction within the first month of life. |
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Tetralogy of Fallot |
This condition is characterized by the following four defects: VSD A ventricular septal defect that allows blood to pass from the right ventricle to the left ventricle without going through the lungs Pulmonic Stenosis a narrowing at or just beneath the pulmonary valve that partially blocks the flow of blood from the right side of the heart to the lungs Right Ventricular Hypertrophy the right ventricle is more muscular than normal Overriding Aorta the aorta lies directly over the ventricular septal defect |
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Treatment of Tetralogy of Fallot |
Surgery to repair the defects in the heart is always performed relatively early in life. Sometimes a preliminary surgery to create increased blood flow to the lungs is done before definitive corrective surgery. Corrective surgery widens the narrowed pulmonary valve, and the ventricular septal defect is closed. |
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Truncus Arteriosus |
Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery. |
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Truncus Arteriosus treatment |
Surgery is needed to treat this condition. Two procedures are available: 1. banding of the pulmonary arteries coming off the truncus the other is complete repair. 2. Complete repair appears to be the preferred option but as the child grows, repeat surgical procedures may be necessary. |
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Hypoplastic Left Heart Syndrome (HLHS) |
A combination of several abnormalities of the heart and the great blood vessels. The degree of underdevelopment differs from child to child. The functional ability of the left ventricle can be reduced to the extent of not being able to pump an adequate blood volume to the body. Hypoplastic left heart syndrome can be fatal without treatment. |
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In hypoplastic left heart syndrome, most of the structures on what side are affected and how? |
left side of the heart (including the left ventricle, mitral valve, aorta, and aortic valve) are small and underdeveloped. |
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Treatment of Hypoplastic Left Heart Syndrome (HLHS) |
Once the diagnosis of hypoplastic left heart is made, a number of things are done. First, the patient is put on a ventilator to assist with breathing if necessary. Also prostaglandin E1 is started to prevent the ductus arteriosus from closing. Intravenous fluids are started and medicine to help the heart beat stronger is often begun. |
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There are two surgical approaches to hypoplastic left heart syndrome. |
1. One is to do a heart transplant early in life. It also means that the patient will have to take a number of medicines to prevent rejection of the new heart. 2. The other option is a series of operations done over the first 3 years of life; this option is called the Norwood procedure. The Norwood procedure is relatively new, but outcomes have been good so far. |
