Matthew 25:40

"And the King will answer and say to them, ‘Assuredly, I say to you, inasmuch as you did it to one of the least of these My brethren, you did it to Me.’ "

Monday, July 6, 2009

Shone's Complex

Shone’s complex is a congenital heart disease, consisting of
multiple levels of left sided obstructive lesions including
supravalvar mitral ring, parachute mitral valve, subaortic
stenosis, and coarctation of aorta. This is a very rare malformation
and a very few cases have been reported in literature.

Shone’s complex is a rare congenital heart disease described
by Shone et al initially in 1963. It typically consists of four
obstructive lesions of the left side of the heart and circulation
namely parachute like mitral valve, supravalvar mitral ring,
subaortic stenosis , and coarctation of aorta. There is a complete
form of Shone’s complex wherein all the four lesions are present;
however incomplete forms with two or three lesions are also
described. Other coexisting mitral valve anomalies have been
reported such as fused chordae, single papillary muscle and
“typical” (Ruckman & Van Praagh) congenital mitral stenosis.
The LVOT obstruction features may include subaortic stenosis,
valvar aortic stenosis, bicuspid aortic valve, and coarctation of

Supravalvar mitral ring is a circumferential ridge or
membrane, which arises from the left atrial wall overlying
the mitral valve and is frequently attached to the mitral valve.
The ring may range from a thin membrane to a thick discrete
fibrous ridge. It may vary in its extent. Adhesion to the valve
may impair opening of the leaflets causing mitral-valve inflow
obstruction in some patients. In other patients, the ring may
be large and protrude into the mitral-valve inflow thus causing

Parachute mitral valve is defined as a unifocal attachment
of mitral valve chordae independent of the number of papillary
muscles. A true parachute mitral valve (PMV) is characterized by
attachment of the chordae to a single or fused papillary muscle;
however PMV also includes asymmetrical mitral valves having
two papillary muscles, one of which is dominant and elongated,
with its tip reaching to the valve leaflets. The unifocal attachment
of the chordae results in a restricted valve opening and subvalvar
obstruction and, rarely, valvar regurgitation. Oosthoek et
al suggested that these morphological features distinguish a
parachute-like mitral valve from a true PMV.

Shone’s complex is a rare congenital anomaly. Fewer than 100
patients have been reported in the literature. It is mostly detected
in childhood as the patient becomes symptomatic by the age of
2 years. The usual symptoms are dyspnea, nocturnal cough,
tachypnea, poor feeding, failure to thrive, fatigue, and signs
and symptoms of heart failure and reduced cardiac output. The
child usually has recurrent episodes of wheezing and respiratory
tract infections due to pulmonary congestion and exudation of
fluid into the lungs. The patient may occasionally present with
acute pulmonary edema.

It is extremely unusual for a patient to remain largely
asymptomatic throughout childhood and get incidentally
detected during adulthood while evaluating for some unrelated
illness. The present case therefore assumes significance. This
patient had been largely asymptomatic (except for NYHA class I
dyspnoea which he had ignored) in childhood. He had presented
to us for meningitis during which his clinical examination
revealed evidence of mitral stenosis along with left ventricular
outflow tract obstruction and aortic coarctation. This prompted
us to investigate the patient in detail. The echocardiographic
findings revealed the features of complete form of Shone’s

A literature search revealed a few articles mostly case reports.
Goswami et al reported Shone’s anomaly in a young gravid
female mimicking preeclampsia at 25 weeks gestation. Most of
the other reports are in children. Most of these reports are from
foreign literature. To the best of our knowledge the present case
report is the first report of Shone’s anomaly from India.
A good outcome is possible in patients with Shone’s complex,
provided the surgical intervention is undertaken early before
the onset of pulmonary hypertension. Mitral valve repair
along with resection of supramitral ring is preferable over valve
replacement. Other surgical procedures depend upon existence
of associated cardiac anomalies, which ultimately define late
surgical outcome.

Click here for Case Report referenced.

Saturday, July 4, 2009

Ventricular Septal Defect (VSD)

What is a ventricular septal defect?
A ventricular septal defect is an opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles. VSD is a congenital (present at birth) heart defect. As the fetus is growing, something occurs to affect heart development during the first 8 weeks of pregnancy, resulting in a VSD.

Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta.

A ventricular septal defect allows oxygen-rich (red) blood to pass from the left ventricle, through the opening in the septum, and then mix with oxygen-poor (blue) blood in the right ventricle.
What are the different types of VSD?
Two basic types of VSD include the following:
1. perimembranous VSD - an opening in the upper section of the ventricular septum, near the valves, occurs in 75 percent of all VSD cases.
2. muscular VSD - an opening in the lower section of the ventricular septum occurs in up to 20 percent of all VSD cases.

Ventricular septal defects are the most commonly occurring type of congenital heart defect, occurring in 14 to 17 percent of babies born each year.

What causes ventricular septal defect?
The heart is forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Ventricular septal defects occur when the partitioning process does not occur completely, leaving an opening in the ventricular septum.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most ventricular septal defects occur sporadically (by chance), with no clear reason for their development.

Why is ventricular septal defect a concern?
If not treated, this heart defect can cause lung disease. When blood passes through the VSD from the left ventricle to the right ventricle, a larger volume of blood than normal must be handled by the right side of the heart. Extra blood then passes through the pulmonary artery into the lungs, causing higher pressure than normal in the blood vessels in the lungs.

A small opening in the ventricular septum allows a small amount of blood to pass through from the left ventricle to the right ventricle. A large opening allows more blood to pass through and mix with the normal blood flow in the right heart. Extra blood causes higher pressure in the blood vessels in the lungs. The larger the volume of blood that goes to the lungs, the higher the pressure.

The lungs are able to cope with this extra pressure for while, depending on exactly how high the pressure is. After a while, however, the blood vessels in the lungs become diseased by the extra pressure.

As pressure builds up in the lungs, the flow of blood from the left ventricle, through the VSD, into the right ventricle, and on to the lungs will diminish. This helps preserve the function of the lungs, but causes yet another problem. Blood flow within the heart goes from areas where the pressure is high to areas where the pressure is low. If a ventricular septal defect is not repaired, and lung disease begins to occur, pressure in the right side of the heart will eventually exceed pressure in the left. In this instance, it will be easier for oxygen-poor (blue) blood to flow from the right ventricle, through the VSD, into the left ventricle, and on to the body. When this happens, the body does not receive enough oxygen in the bloodstream to meet its needs.
Because blood is pumped at high pressure by the left ventricle through the VSD, tissue damage may eventually occur in the right ventricle. Bacteria in the bloodstream can easily infect this injured area, causing a serious illness known as bacterial endocarditis.

Some ventricular septal defects are found in combination with other heart defects (such as in transposition of the great arteries).

What are the symptoms of a ventricular septal defect?
The size of the ventricular septal opening will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. A VSD permits extra blood to pass from the left ventricle through to the right side of the heart, and the right ventricle and lungs become overworked as a result. The larger the opening, the greater the amount of blood that passes through and overloads the right ventricle and lungs.

Symptoms often occur in infancy. The following are the most common symptoms of VSD. However, each child may experience symptoms differently.

Symptoms may include:

  • fatigue
  • sweating
  • rapid breathing
  • heavy breathing
  • congested breathing
  • disinterest in feeding, or tiring while feeding
  • poor weight gain
The symptoms of VSD may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is a ventricular septal defect diagnosed?
Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the opening from the left side of the heart to the right.

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest where the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have.
However, other tests are needed to help with the diagnosis, and may include the following:
  • chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. With a VSD, the heart may be enlarged because the right ventricle handles larger amounts of blood flow than normal. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an x-ray.
  • electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.
  • echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the septal opening, and determine how large the opening is, as well as much blood is passing through it.
  • cardiac catheterization - a cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.

Treatment for ventricular septal defect:

Specific treatment for VSD will be determined by your child's physician based on:
your child's age, overall health, and medical history, extent of the disease,
your child's tolerance for specific medications, procedures, or therapies, expectations for the course of the disease , your opinion or preference.

Small ventricular septal defects may close spontaneously as your child grows. A larger VSD usually requires surgical repair. Regardless of the type, once a ventricular septal defect is diagnosed, your child's cardiologist will evaluate your child periodically to see whether it is closing on its own. A VSD will be repaired if it has not closed on its own - to prevent lung problems that will develop from long-time exposure to extra blood flow. Treatment may include:
medical managementSome children have no symptoms, and require no medication. However, most children may need to take medications to help the heart work better, since the right side is under strain from the extra blood passing through the VSD.

Medications that may be prescribed include the following:
1. digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently.
2. diuretics - the body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body.

Adequate Nutrition Infants with a larger VSD may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include the following:
1. high-calorie formula or breast milkSpecial nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow properly.
2. supplemental tube feedingsFeedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle feed may receive their formula or breast milk through the feeding tube alone.

Infection Control Children with certain heart defects are at risk for developing an infection of the inner surfaces of the heart known as bacterial endocarditis. A common procedure that puts your child at risk for this infection is a routine dental check-up and teeth cleaning. Other procedures may also increase the risk of the heart infection occurring. However, giving children with heart defects an antibiotic by mouth before these procedures can help prevent bacterial endocarditis. It is important that you inform all medical personnel that your child has a VSD so they may determine if the antibiotics are necessary before a procedure.

Surgical Repair The goal is to repair the septal opening before the lungs become diseased from too much blood flow and pressure. Repair is indicated for defects that are causing symptoms, such as poor weight gain and rapid breathing. Your child's cardiologist will recommend when the repair should be performed based on echocardiogram and cardiac catheterization results.Your child's VSD may be repaired surgically in the operating room or by a cardiac catheterization procedure. One method currently being used to close some VSDs is the use of a device called a septal occluder. During this procedure, the child is sedated and a small, thin flexible tube is inserted into a blood vessel in the groin and guided into the heart. Once the catheter is in the heart, the cardiologist will pass the septal occluder into the VSD. The septal occluder closes the ventricular septal defect providing a permanent seal.The operation is performed under general anesthesia. Depending on the size of the heart defect and your physician's recommendations, the ventricular septal defect will be closed with stitches or a special patch. Consult your child's cardiologist for more information.

Postoperative care for your child:
In most cases, children will spend time in the intensive care unit (ICU) after an VSD repair. During the first several hours after surgery, your child will most likely be drowsy from the anesthesia that was used during the operation, and from medications given to relax him/her and to help with pain. As time goes by, your child will become more alert.

While your child is in the ICU...which is where I come in...special equipment will be used to help him/her recover, and may include the following:
1. ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he/she is too sleepy to breathe effectively on his/her own. Many children have the ventilator tube removed right after surgery, but some other children will benefit from remaining on the ventilator for a few hours afterwards so they can rest.
intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medications that help your child recover from the operation.
arterial line - a specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.
2. nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
3. urinary catheter - a small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluids from the body.
chest tube - a drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
4. heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values.

Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.
Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff may also ask for your input as to how best to soothe and comfort your child.

After discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

Care for your child at home following VSD surgical repair:
Most infants and older children feel fairly comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's physician will discuss pain control before your child is discharged from the hospital.
Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster.

After surgery, older children usually have a fair tolerance for activity. Your child may become tired quicker than before surgery, but usually will be allowed to play with supervision, while avoiding blows to the chest that might cause injury to the incision or breastbone. Within a few weeks, your child should be fully recovered and able to participate in normal activity.
You may receive additional instructions from your child's physicians and the hospital staff.
Long-term outlook after VSD surgical repair:Most children who have had a ventricular septal defect repair will live healthy lives. Activity levels, appetite, and growth will return to normal in most children. Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a specific time period after discharge from the hospital.
Consult your child's physician regarding the specific outlook for your child.

Information obtained from RUMC

Friday, July 3, 2009

Duodenal Atresia

Duodenal atresia represents complete obliteration of the duodenal lumen. A duodenal diaphragm (or duodenal web) is thought to represent a mild form of atresia. Duodenal stenosis (incomplete obstruction of the duodenal lumen) is discussed with duodenal atresia because the 2 disorders together represent a spectrum of similar intrauterine events.

Annular pancreas occurs when pancreatic tissue surrounds the second portion of the duodenum. If the encirclement is complete, it may be associated with complete or incomplete duodenal obstruction. Since duodenal atresia or duodenal stenosis occurs in all cases of annular pancreas, the anomalous pancreas should be considered a secondary change rather than a primary cause of duodenal obstruction.

The etiology of duodenal atresia and stenosis is unknown. Failure of recanalization of the duodenal lumen remains the favored theory, compared with intrauterine vascular ischemia.
During the third week of embryonic development, the second portion of the duodenum, at the junction of the foregut and midgut, forms biliary and pancreatic buds, which are derived from endoderm. During the next 4 weeks, these buds differentiate into the hepatobiliary system, with the development and subsequent fusion of the 2 pancreatic anlagen. Concurrently, the epithelium of the duodenum undergoes active proliferation, which, at times, completely obliterates the duodenal lumen. Vacuolization, followed by recanalization, reestablishes the hollow viscus.

The second part of the duodenum is the last to recanalize. The early forming biliary system consists of 2 channels arising from the embryonic duodenum. This structure creates a narrow segment of bowel, approximately 0.125 mm in length, that is interposed between the 2 biliary channels. This narrow region is the area most prone to problems, with recanalization and with atresia formation. The ampulla of Vater usually is immediately adjacent to or traverses the medial wall of the diaphragm. The presence of a bifid biliary system, or the insertion of 1 duct above the atresia and 1 duct below it, is rare, occurring when both biliary duct anlagen remain patent. The presence of bile above and below the atresia indicates a bifid biliary system.

United States The incidence of duodenal atresia is 1 per 6000 births. Intrinsic congenital duodenal obstruction constitutes two thirds of all congenital duodenal obstructions (duodenal atresia, 40-60%; duodenal web, 35-45%; annular pancreas, 10-30%; duodenal stenosis, 7-20%).
International The incidence in Finland of congenital obstruction (intrinsic, extrinsic, combined) is 1 per 3400 live births.

If duodenal atresia or significant duodenal stenosis is left untreated, the condition rapidly becomes fatal as a result of electrolyte loss and fluid imbalance.
One half of the neonates with duodenal atresia or stenosis are born prematurely.
Hydramnios occurs in approximately 40% of neonates with duodenal obstruction.
Duodenal atresia or duodenal stenosis is most commonly associated with trisomy 21. About 22-30% of patients with duodenal obstruction have trisomy 21. Other problems associated with trisomy 21 include cardiac defects (most commonly ventricular septal defects and endocardial defects), as well as Hirschsprung disease.

No racial predilection exists.

The incidence of duodenal atresia and duodenal stenosis is approximately equal in males and females.

Infants with duodenal atresia present with vomiting in their first few hours of life, but patients with duodenal stenosis present at various ages. The clinical findings depend on the degree of stenosis. Occasionally, with duodenal web or duodenal stenosis, presentation occurs in adulthood.
In most cases, duodenal atresia occurs below the ampulla of Vater. In a very few cases, the atresia occurs proximal to the ampulla.

Bile-stained vomit in neonates aged 24 hours or younger is the typical presentation of atresia or severe stenosis. Minimal duodenal obstruction in mild stenosis or duodenal membrane may have few symptoms. In a few cases, the atresia is proximal to the ampulla of Vater and the vomit is free of bile.Both duodenal anomalies can be associated with other GI and biliary tract abnormalities (malrotation, esophageal atresia, ectopic anus, annular pancreas, gallbladder or biliary atresia, vertebral anomalies). In addition, duodenal atresia can be associated with a duodenal diaphragm, as well as with congenital abnormalities in other systems. Examples include VATER (vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal anomalies) association and VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) syndrome.Anomalies of the kidneys can occur in VATER association; the most common of these renal abnormalities include aplasia, dysplasia, hydronephrosis, ectopia, persistent urachus, vesicoureteral reflux, and ureteropelvic obstruction.
A few familial cases have been reported.

Preferred Examination
Plain radiographs that demonstrate a double-bubble appearance with no distal gas are characteristic of duodenal atresia. Distal bowel gas indicates stenosis, incomplete membrane, or a hepatopancreatic ductal anomaly. Occasionally, a radiograph must be obtained with the patient in the erect or the decubitus position to delineate the duodenal component. If a combination of esophageal atresia and duodenal atresia is present, ultrasonography is preferred.

Limitations of Techniques
No oral contrast materials are necessary in the evaluation of complete duodenal obstruction. Occasionally, a small amount of positive contrast material can be instilled through a feeding tube into the distal stomach and duodenum to differentiate the diaphragm from a long stenosis.
Occasionally, barium enema examination is suggested as an adjunct study in the evaluation of duodenal atresia. Barium enema findings can demonstrate a malpositioned cecum, but this is not always diagnostic of malrotation and volvulus. In addition, if a microcolon is demonstrated, the presence of additional, more distal atresias can be suggested. Succus entericus may be prevented from reaching the colon because of the additional area of bowel obstruction. Multiple atresias are present in approximately 15% of patients. However, most surgeons can determine the presence of malrotation and additional atresias at the time of surgery.

Information Obtained from EMed and Lucina Foundation

Atrial Septal Defect (ASD)

What Is It?
An Atrial Septal Defect (ASD) is a hole in the atrial septum, or muscle wall, that separates the right and left atria (singular = atrium), or upper chambers of the heart. Because of the lower pressure in the right atrium, this hole typically allows oxygenated blood from the lungs to move, or shunt, from the left into the right atrium. This blood proceeds into the right ventricle, which pumps it back to the lungs rather than to the body.ASDs vary in size and in the severity of symptoms they may cause. They account for between 5 and 10 per cent of all cases of congenital heart disease and are twice as prevalent among girls as boys.


What are its effects? Children with significant ASDs are characteristically slender of build and have a heart murmur. The murmur is caused by the extra blood flow across the pulmonary valve. Some children may experience shortness of breath or heart palpitations. However, they are normally active and show no other outward symptoms. There are no exercise restrictions for these children.The larger the defect, the more likely children will have symptoms. Infants with a large ASD may develop congestive heart failure. However, if the defect is small (less than 2 millimeters), there is a very high probability that it will close on its own. Surgery is not usually performed in these cases.Larger ASDs, which are more likely to remain open, cause an excessive flow of blood into the right atrium, right ventricle and pulmonary artery (see animation). This enlarges the right atrium and right ventricle (dilatation) and causes high pressures in the pulmonary artery that will eventually distort its shape and may rarely damage the blood vessels in the lungs.The enlargement of the right atrium can result in abnormal heart rhythms. These effects are not reversed by closing the ASD after the damage has been done. Heart failure is likely when a person with an untreated ASD reaches young adulthood.


Variations of ASD Atrial Septal Defects are divided into three different types on the basis of the position of the hole (or holes) in the atrial septum.The first type of ASD is known as ostium primum defect, or simply, primum (number 1 in the diagram). In this kind of defect, the hole is located in the lower part of the atrial septum, near the tricuspid valve, which opens into the right ventricle.The most common type of ASD (accounting for 50-70% of all cases) is known as ostium secundum defect, or simply, secundum (2). In this case, the hole is located near the center of the atrial septum.The third type of ASD is known as a sinus venosus defect, in which the hole is located near one of the two places where the vena cava (the vein that carries blood from the body to the heart) enters the right atrium. The two kinds of sinus venosus defect are distinguished by whether the hole is near the entry point of the superior vena cava (SVC) (superior vena caval type - 3 in diagram) or of the inferior vena cava (IVC) (inferior vena caval type - 4 in diagram).

How Is It Treated? Babies with congestive heart failure because of volume overload to the lungs may be treated with diuretics such as Lasix (Furosemide) and Aldactone (Spironolactone). These medications can help to reduce the volume of fluid in the lung, which makes it easier for the infant to breathe and eat.Digoxin may also be prescribed. It increases the squeeze (contraction) of the heart muscle and helps it to function more effectively.For those infants whose feeding is affected, nutritional additives may be used to fortify the baby's milk. In more severe cases, nourishment with a naso-gastric tube may be necessary.If slow growth and other symptoms continue despite treatment with medication, surgery may be required to close the ventricular septal defect. The benefits of this surgery are usually dramatic: paleness and rapid breathing are corrected and the rate of growth becomes normal. The mortality risk in this type of surgery is very low.VSDs may be closed by patching (see animation) or suturing during open heart surgery. Small defects may be closed with simple sutures using a monofilament thread made of Prolene or Polypropylene. Larger holes may be covered with patches made of pieces of pericardium (the membrane that covers the heart) or of silk or a synthetic material such as Dacron or Teflon.

Information obtained from Cove Heart Foundation: Congenital Heart Disease

Thursday, July 2, 2009

Scaphocephaly with Craniofacial Surgery

Definition: Scaphocephaly (also know as, dolichocephaly) refers to the condition where the head is disproportionately long and narrow (see cranial index. Scaphocephaly can result from the premature fusion of the sagittal suture (see craniosynostosis) or from external deformation. Scaphocephaly is particularly common among infants who are born prematurely.

Diagnosis: The diagnosis begins with an examination by a pediatrician, pediatric neurosurgeon or craniofacial surgeon. A primary objective of the examination is to rule out craniosynostosis (a condition that requires surgical correction). The initial examination involves questions about gestation, birth, in utero and post-natal positioning (for example, sleeping position). The physical examination includes inspection of the infant's head and may involve palpation (carefully feeling) of the child's skull for suture ridges and soft spots (the fontanelles). The physician may also request x-rays or computerized tomography (a CAT scan, a series of photographic images of the skull). These images provide the most reliable method for diagnosing premature fusion of the sagittal suture (craniosynostosis). In addition, the physician may make (or order) a series of measurements from the child's face and head [more on cranial anthropometry]. These measurements will be used to assess severity and monitor treatment.

Treatment: The treatment of scaphocephaly depends upon the etiology (cause) of the condition: Scaphocephaly resulting from fusion of the sagittal suture (craniosynostosis) must be treated surgically. Parents should consult a pediatric neurosurgeon or a craniofacial surgeon to discuss treatment option. Depending upon severity, scaphocephaly resulting from external/positional deformation can be treated with repositioning and/or head banding. Parents should consult a pediatrician, a pediatric neurosurgeon or a craniofacial surgeon for information on repositioning and/or for referral and a prescription for head banding.

Support Groups:
Plagiocephaly Parents Support
Parents of Premature Babies Inc. (Preemie-L)

Craniofacial Surgery:
The craniofacial area includes the base of the skull, the facial skeleton and underlying soft tissues, the skull vaults and the scalp. Craniofacial surgery involves repairing damage caused by serious injuries as well as congenital deformities and abnormal growths such as tumors.

Congenital deformities include

  • clefts of the lip and palate: In these conditions, all of the parts of the lip and roof of the mouth are present, but they have failed to fuse in a normal fashion. Clefts can appear with varying severity: a cleft lip can be incomplete with a fractional notching of the lip, or complete, extending through the lip and into the nose. ear deformities: In these conditions, the outer ear may be underdeveloped, misshaped, or completely absent.
  • premature fusing of the bones of the head in young children: In the normal infant skull, cracks or “sutures” appear in between bones of the head to allow for brain growth. When one of these sutures closes prematurely, the brain continues to grow, but pushes out toward the area of the skull where the sutures are still open. The result is a malformation of the skull and/or face.
  • misshapen jaws: often caused by misalignment of the teeth and jaws referred to as malocclusion, or mild hypoplasia (inadequate tissue development) which can appear as a recessed upper jaw or other underdeveloped bony area of the face.
  • facial asymmetries: or hemifacial microsomia, a condition wherein one side of the face is smaller than the other, due to underdevelopment of bone and/or cartilage.

After appropriate assessment, surgical treatment may be recommended and this will vary considerably depending on what the particular problem is. Sometimes craniofacial surgery for deformity can be carried out without making visible scars on the face. Craniofacial surgeries carry varying degrees of risk, depending on the particular problem. Sometimes bone or cartilage grafts need to be harvested from other areas of the body such as the ribs.

***See pics below...note: none of these pictures are of any patients that I have cared for; they are all from the internet.