Basic information for parents of children with biliary atresia (N.B.: This is NOT medical advice. Consult your doctor for advice.[edit source]
Biliary Atresia
Biliary atresia is a condition, in which a baby’s bile ducts- tubes that drain bile from the liver to the bowel- are missing or are blocked because of scarring.
Introduction
Bile is a green, viscous liquid made by the baby’s liver. It helps the baby’s intestines to digest fats. Normally, bile flows through tiny bile ducts within the liver into larger ducts that lead to the gallbladder and intestine. The gallbladder, which has the shape of a small balloon, sits under the liver. It stores bile until the body needs it to digest food. Food triggers the gallbladder to contract and squeeze a small amount of bile into the intestine.
In babies with biliary atresia, the abnormal or scarred bile ducts prevent bile from flowing out of the liver into the intestine. Instead, bile backs up in the liver and spills into the blood giving rise to jaundice, that is, a yellowish colour to the eyes. This is because bile contains a yellow pigment, called bilirubin. The baby passes pale or white stools.
The exact cause of biliary atresia is not known. In most babies with biliary atresia, the ducts form but get fibrosed due to some inflammation. In some babies, the bile ducts may be abnormal at birth (congenital). Such babies may have abnormalities in other structures namely the intestine, spleen, and portal vein, a big vein that goes to the liver.
To get rid of the jaundice, babies with biliary atresia need surgery. The goal is to remove the blocked ducts outside the liver and create a way for bile to drain straight from the liver into the intestines. This surgery is called the Kasai procedure, named after Professor Kasai from Japan, who developed it in the 1960s.
Without surgery, biliary atresia leads to liver failure. A baby with liver failure may be able to have a liver transplant. Biliary atresia is the most common reason for liver transplants in children.
Symptoms
(1) Worsening jaundice: All babies get jaundice in the first week of life. Normally this clears up within the first two weeks. If your baby develops jaundice after that period and it lasts longer than two weeks, immediately consult your baby’s doctor.
(2) Pale or white stools: Babies with biliary atresia also have very pale, gray, or white stools. Bile gives stools their usual yellow, green, or brown color. If bile cannot flow out of the liver into the intestine, your child’s stool will not have these colors. Instead, bile becomes backed up in their liver. This increases the amount of the pigment bilirubin in their blood. It gets passed out in their urine and makes the urine darker.
(3) Backed-up bile in the liver causes scarring or cirrhosis of liver, which is irreversible and progressive condition. Cirrhosis can cause other symptoms, like fluid collecting in the tummy (ascites) and an enlarged spleen.
Diagnosis
History and clinical examination
Blood tests to find out about baby’s liver function
Ultrasound scan to find more about gall bladder and bile ducts
If these tests suggest bile flow is blocked, then surgery is advised. During surgery, doctors can check how well the bile ducts are developed by doing a special x-ray investigation called “operative cholangiography” and can rule out or diagnose atresia. During the same surgery, the surgeon will most likely do the Kasai procedure to improve bile drainage.
Treatment
The surgery is called Kasai procedure (porto-enterostomy). “Porto” is related to liver, “enter” means intestine and “ostomy” means joint. It literally means “to attach a piece of intestine to drain bile from the liver”.
Kasai Procedure
The surgeon removes the scarred, abnormal bile ducts outside and below the baby’s liver. A piece of the small intestine that can reach up to the liver is selected and it is connected to the under surface of the liver to drain the bile. It is a difficult operation that is done under general anaesthesia, takes about three hours and may need blood transfusion. The baby will be in the intensive care unit (ICU) for a couple of days and in the ward for further seven to ten days.
The Kasai procedure tends to be more successful if it is done earlier. It is ideal to do the surgery in the first two months after birth. The later it is done, the less are the chances of success.
Follow up
Frequent follow up to check whether there is bile flow and the liver function
· Few children recover with good liver function and bile flow
· Some will do well for a while, even a few years before their liver damage gets worse and their liver fails
· For some children, a Kasai procedure will not help because their liver is already damaged. These children with liver failure may be considered for a liver transplant
Important message:
Any infant who is otherwise well, has deepening jaundice, and is passing pale or white stools, should be suspected of having biliary atresia; and a Paediatric Surgeon should be consulted as soon as possible. Mrchitnis (talk) 08:46, 20 January 2024 (UTC)
Basic information for parents of children with biliary atresia[edit source]
This is very basic information for parents of children with biliary atresia. It explains when to suspect this problem, and how it is diagnosed and treated. This is NOT medical advice. Consult your doctor for advice. Mrchitnis (talk) 08:48, 20 January 2024 (UTC)
Basic info to the parents of children with choledochal cyst[edit source]
Choledochal Cyst
A choledochal cyst is a problem with the tubes (ducts) that carry bile from the liver to the gallbladder and intestine. The tubes get bigger than normal (dilated), or pouches form on the tubes. The bile does not flow well, which can cause jaundice and liver problems.
Bile is a green, viscous liquid that helps the body to digest fats. Bile is formed in the liver, and it flows through tiny bile ducts within the liver into two larger ducts. Outside the liver, these ducts join to form the common bile duct.
The common bile duct connects the liver to the small bowel. The gallbladder, a small balloon-like pouch, stores bile until the body needs it to digest food. Food triggers the gallbladder to contract and squeeze a small amount of bile out into the intestine.
Cysts can slow or block the flow of bile. This results in bile ducts getting swollen and irritated (inflamed) or infected. This is called cholangitis. Over time, it causes scars in the liver (cirrhosis). A scarred liver cannot work well. Children with choledochal cysts may also get an inflamed (swollen) pancreas (pancreatitis).
Introduction
Choledochal cyst is a congenital abnormality, as it is present at birth. It happens while a baby is being formed, inside the mother’s womb. The exact reason why cysts form is not known. It may be due to the damaged and weak wall of the bile ducts. There are five types of choledochal cysts, based on where the cyst is in the ducts and what shape the cyst has. They are three to four times more common in girls than in boys. Choledochal cysts are common in babies of Eastern Asian descent. People who have had choledochal cysts for many years have a higher chance of getting cancer of their bile ducts, in the third or fourth decade of life. The chance of getting cancer is much lower if the cyst is removed early in life.
Symptoms
It can present in infancy, a few years later, or even as an adult
Common symptoms:
- Jaundice
- Lump in the upper right side of the tummy
- Fluctuating jaundice with pale or white stools and fever when they have an infection (cholangitis)
- Severe tummy pain going to the back if they have an inflamed pancreas (pancreatitis)
Diagnosis
(1) May be seen on an antenatal ultrasound scan
(2) If presents after birth:
(a) Blood tests to check about jaundice and whether the liver function
(b) Ultrasound scan of your child’s abdomen helps the doctor to know
(i) whether there is a cyst
(ii) where it is
(iii) Its shape
Occasionally the following specialised imaging studies are done:
- Computerized tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
Treatment
Medicines
If your child has infected bile ducts (cholangitis), your child will be given antibiotics to fight the infection. This may need to be done in the hospital.
Surgery
The treatment for a choledochal cyst is surgery. The purpose is to remove the cyst and create good bile flow from the liver to the small intestine.
· The surgery is called “excision of choledochal cyst and hepaticojejunostomy”. “Excision” is to remove. “Hepatic” has to do with the liver. “Jejunum” is the middle segment of the small intestine. “Ostomy” is to connect.
· The surgery is done under general anesthesia. It will take approximately three hours. The child may need to have a blood transfusion.
- Operative cholangiography: During the operation, the Surgeon injects dye (contrast) into your child’s bile ducts and then takes an X-ray on the operation table. This helps the surgeon to understand the nature of the cyst and its relations to the surrounding organs.
The surgeon cuts the common bile duct above and below the cyst and removes the cyst
· A piece of small intestine is taken and attached to the bile duct under the liver so that bile can flow from the liver into the intestine.
After surgery
· After surgery, your child will be in the recovery room for about an hour. Then he/she will stay in the intensive care unit (ICU) for a few days and the children’s ward for a further seven to ten days.
· Follow-up visit with the surgeon two to three weeks after the surgery to make sure the incision is healing well and check whether the jaundice is coming down.
· After healing from surgery, your child will need to visit the doctor from time to time for regular check-ups and if they have any symptoms. It is important to make sure that bile keeps flowing well. Your child may need some medicines to help the flow of bile. Mrchitnis (talk) 08:56, 20 January 2024 (UTC)
Basic info for the parents of a child with congenital diaphragmatic hernia[edit source]
Congenital Diaphragmatic Hernia
A congenital diaphragmatic hernia (CDH) occurs when the thin muscle that separates the chest from the tummy, called the diaphragm, does not form completely. This leaves a hole or a defect between the tummy (abdomen) and chest.
There are two common types of CDH:
- Defect on the side of the chest (Bochdalek): Common and causes more severe problems
- Defect in the front (Morgagni)
We will discuss Bochdalek hernias.
Introduction
Diaphragmatic hernia is a congenital abnormality as it is present at birth. It occurs early in pregnancy while the diaphragm is forming. We do not know the exact cause why it happens. It occurs in 1 in every 5000 babies.
In babies with CDH, organs that should be in the tummy, slip through the hole, into the chest. The intestines slip through most commonly. Other organs namely the stomach, liver, spleen, and, rarely, the kidneys can also herniate through the defect. These organs take up space that should be available for a baby's lungs to grow. As a result, babies born with CDH have smaller lungs than normal.
Blood pressure in the lungs of some of these babies is high (pulmonary hypertension). This prevents blood from getting to the lungs easily, to collect oxygen.
Some babies with CDH have birth defects in the heart which may also require treatment.
Symptoms
(1) Severe trouble in breathing (distress) soon after the birth. This is because:
· The lungs may be smaller than normal.
· The heart may not be able to pump blood easily to the lungs because of the thick walls and high pressure in the blood vessels in the lungs
· The lungs may be pushed to one side by organs that slip through the hole between the belly and the chest
(1) Bluish color of the skin due to lack of oxygen (cyanosis)
(2) Rapid breathing
(3) Fast heart rate
Diagnosis
· On a routine antenatal ultrasound scan. In this case, it will be best to arrange the delivery in a hospital where Neonatologist and Paediatric Surgeon are available.
· In the majority of cases, it is suspected after noticing that the baby has trouble in breathing.
· On examination, there may be:
o Irregular chest movements
o No breath sounds on the affected side
o Bowel sounds, usually heard in the tummy, are heard in the chest instead
o The lower tummy feels less full than in other babies
Investigations
· Chest X-ray:
o Organs from the belly in the chest
o Lungs may look smaller than normal
o The heart pushed to one side
· Ultrasound scan of the baby's heart (echocardiography):
o This can help them determine the severity of the high blood pressure in the baby's lungs.
o Any birth defects in the heart
The seriousness of CDH depends on:
o Restriction on the growth of baby's lungs due to the lack of space in the chest
o The severity of the high blood pressure is in the baby's lungs
Treatment
Treatment for congenital diaphragmatic hernia is surgery to gently bring the child's abdominal organs down from the chest and close the hole in the diaphragm. This is expected to help the lung to grow slowly.
Before surgery
Surgery is not an emergency, and it is essential to optimize the baby’s condition before surgery. The timing of the operation depends on:
o The baby's condition
o The degree of high blood pressure in the lungs
o Response to treatment
Most babies with CDH will be on a machine (ventilator) to support breathing before and after surgery, until their lungs are mature enough to take over.
Surgery
The surgery will be performed under general anesthesia. It is best to make a small incision either in the abdomen or the chest to perform the surgery.
After bringing the organs from the chest to the tummy, the surgeon will close the hole in the diaphragm. If the hole is too large to close, the surgeon will use an artificial patch to close it.
The surgery usually takes about two hours. Many children need a chest tube for a few days. This allows any fluid that collects in the chest to come out and helps the lungs to expand.
After surgery
After surgery, the baby will stay in our neonatal intensive care unit (NICU), and will most likely need machine (ventilator) support for breathing. If your baby goes home soon, the surgeon will schedule a follow-up visit two to three weeks after the operation to make sure the wound is healing well.
With specialized care, including surgery to fix the hole, many babies with CDH survive, but some may not. After surgery to close the defect, some children have no long-term problems from CDH. Others have ongoing health issues, such as frequent chest infections, gastroesophageal reflux, developmental delay, and curvature of the spine (scoliosis). Mrchitnis (talk) 09:01, 20 January 2024 (UTC)
Basic info for the parents of a child with a dermoid cyst[edit source]
Dermoid Cyst
Dermoid cysts are closed sacs that form within the body. A cyst may contain fluid, pus, some other tissue from the body, or foreign matter.
Dermoid cysts form in or on the skin, usually on the face near the eyebrow, on the scalp, or on the chest over the collarbone. Rarely, a dermoid cyst near the surface of the body extends below the skin and connects to deeper structures. Very rarely, dermoid cysts develop in other areas of the body instead of the skin.
Dermoid cysts are common in children. They are usually painless and benign, which means they don't threaten your child's health. But they may become infected. If the cyst is in a spot that is easy to see, you or your child may not like the way it looks.
Surgeons remove dermoid cysts to prevent infection. They also remove dermoid cysts if you or your child don't like the way it looks i.e. for cosmetic reasons. Dermoid cysts will not go away without an operation.
Dermoid Cysts in Children
Dermoid cysts can affect any child. They form during pregnancy while a baby develops in the mother's tummy.
Dermoid cysts occur when skin cells and other structures related to skin — like hair, sweat glands, oil glands or fatty tissue — get trapped in a pocket as the baby grows. The cysts are present at birth (congenital). But because they grow slowly, sometimes you may not see them until later childhood or early adulthood.
Symptoms
In most cases, the only symptom of a dermoid cyst is a small, painless lump under the skin that you can see or feel. The lump may be the same color as your child's skin, or it may have a pale yellow color. Dermoid cysts usually show up in certain parts of the body:
- On the face near the eyebrow
- On the scalp
- On the chest
- Over the collarbone
If a dermoid cyst gets infected, it may hurt. It may become swollen and red. The infection may cause a fever. An infected cyst may burst.
Diagnosis
The doctor will examine your child, feel the lump, and ask questions about it. The doctor will want to know when you first found the lump and how fast it's growing. Usually, a physical examination is all the doctor needs to diagnose a dermoid cyst.
If the doctor thinks your child's cyst is connected to other tissues, your child may need to have other tests that will help the doctor see how far the cyst extends:
- X-ray
- Ultrasound scan
- CT (computed tomography) scan
- MRI (magnetic resonance imaging)
The only treatment for a dermoid cyst is to remove it through surgery. Your child can go home on the same day as the surgery.
Before surgery, we will give your child medicine to make them sleep without pain (general anesthesia). The surgeon will:
- Make a small cut, or incision, in the skin over the cyst.
- Remove the cyst and close the incision.
The surgery takes about an hour or less, and your child will be in the recovery room for another hour.
Surgery
After surgery, we will give your child regular pain medicines. You'll need to keep the incision clean and dry until it heals. The surgery team will teach you how to care for the incision and what to give your child at home for pain. They will tell you whether you need to limit your child's activity for a while.
About two to three weeks after surgery, your child will need to see the surgeon for a follow-up visit. The surgeon will make sure the incision is healing and your child is recovering well.
Mrchitnis (talk) 09:03, 20 January 2024 (UTC)
Baisc info for the parents of babies with esophageal atresia[edit source]
Esophageal atresia & tracheoesophageal fistula
Introduction:
Normally the food pipe (esophagus) and the windpipe (trachea) lie next to each other, but there is no connection between the two. During the development stage, in the fetus, they form by division of one common tube. If this separation doesn’t happen as expected, it results in this group of abnormalities. These abnormalities are congenital, meaning they are present at birth.
A gap in the esophagus is called “esophageal atresia (EA)”. An abnormal communication between the esophagus and trachea is called “tracheoesophageal fistula (TEF)”. People often use the terms TEF and EA interchangeably. But each name refers to a specific problem. TEF and EA usually occur together, but sometimes a child has just one and not the other.
There are five types of tracheoesophageal fistula and esophageal atresia. In the most common form, the upper part of the esophagus has a blind end, and the lower part of the esophagus connects to the trachea. About 85% of children with TEF/EA have this type of problem.
Presentation of the commonest type:
Maybe suspected and even diagnosed by an antenatal ultrasound scan
After birth:
· Excessive salivation soon after birth.
· Baby doesn’t tolerate feeds; coughs and chokes every time baby is fed
Babies with EA/TEF, commonly have abnormalities of other organ systems, called the VACTERL association. Each letter stands for a possible problem:
- V = vertebral: problems with the bones in the spine
- A = anus: problems with the anus and rectum
- C = cardiac: problems with the heart
- TE = tracheoesophageal fistula and esophageal atresia
- R = renal: kidney problems
- L = limb: problems with arms and legs
Diagnosis
When TEF/EA is suspected, a nasogastric tube (NG tube) is passed via the baby’s mouth. In a normal baby, the tube goes down the esophagus and into the stomach.
In children who have EA, the NG tube hits the blind end, usually about 10cm from the baby’s gums. This tube can be seen on an X-ray curled up on itself.
In children who have both EA and TEF, the X-ray will also show gas in the baby's stomach. Because the esophagus has a gap, the only way gas can get into the stomach is through an abnormal channel. So, finding gas in the baby's stomach means the baby has a fistula.
Children who have only EA (without a fistula) do not show gas in the stomach.
Fig 3: X-ray of a baby with EA without TEF, no gas in the stomach
Babies with EA +/- TEF need the following tests to rule out VACTERAL associations:
· Ultrasound of the kidneys, spine, and the heart (ECHO)
· X-rays of the arms and legs
Surgery is the only treatment for TEF and EA. Most babies with the conditions will have surgery soon after birth. Some babies who have other, associated serious problems may need to wait a short time before having surgery. The type of surgery depends on the type of abnormality. In many cases, surgeons correct the problem with one operation. Sometimes it needs to be done in stages. All these operations are done under general anaesthesia.
Surgery
Surgery for TEF + EA combined
At the start of the operation, the surgeon will have a look inside the trachea with a thin camera (telescope) to identify the abnormal connection, the fistula. This is called bronchoscopy. The surgeon will make a small cut in your child’s chest, usually on the right side, just below the scapula. Then, the abnormal connection between the trachea and the esophagus, the fistula, is closed. Next, if the two ends of the esophagus are close to each other, the surgeon will join them together.
If the two ends of the esophagus are too far apart to join them safely, the surgeon will close the abnormal channel, but not repair the esophagus. The surgeon will place a feeding tube in your child’s stomach, called a gastrostomy.
Surgery for TEF/EA usually takes about 2 to 3 hours and babies may need blood transfusion.
When your baby leaves the operating theatre, they may need to use a machine (ventilator) to help the baby breathe. We will care for your baby in our Neonatal Intensive Care Unit (NICU) both before and after the surgery.
Surgery for EA without TEF
The Surgeon will do an operation to insert a tube in the baby’s stomach for feeding, and gastrostomy. Depending upon the available resources, the Surgeon may decide to wait for a few weeks for the esophagus to grow before attempting to bring the two ends together. This way, the baby’s esophagus is saved. It is ideal to do so but may not always be possible.
The Surgeon may decide to bring the blind end of the esophagus out in the neck, esophagostomy, to drain saliva out. This baby will need a “new” esophagus at about 10 months of age. Part of the baby’s colon or stomach is used to replace the esophagus. This is a major operation that needs expertise and care.
Surgery for TEF without EA
The repair can usually be done through a small incision in the baby’s neck. The surgeon will find the abnormal connection, divide it, and close the trachea and the esophagus.
Gastrostomy
A tube is placed in the baby’s stomach, by a small cut on the baby’s tummy. Mummy will be taught to feed the baby via this tube and to look after this tube. After about three weeks, the tube will be replaced by a plastic, button-like device that is used for feeding. The gastrostomy will stay till the baby swallows well either with its natural esophagus or a replaced one.
Esophagostomy
It is the opening of the blind upper end of the esophagus in the neck. Mummy will be taught to give “sham feeds” by allowing the baby to suck for a few minutes each day. This feed comes out of the esophagostomy, but it allows the baby to learn how to swallow.
After Surgery
If the two ends of the esophagus are joined together, the Surgeon usually passes a feeding tube across the joint into the stomach. The baby will be fed gradually through this tube, starting from the day after the operation. The feeds will be increased to full volume over the next 2-3 days. Oral feeding can be started after about a week, if necessary, after doing a special x-ray to rule out any leak at the joint.
If your baby is healthy and the repair was simple, he/she will need to stay for two to three weeks. If your baby has other health problems, esophageal atresia with no fistula, or if the repair was delayed or got complicated, your baby may need to be in the hospital for weeks to months.
About three weeks after discharge from the hospital, your child will need to see the surgeon for a follow-up visit. The surgeon will make sure the incision is healing and your child is recovering, gaining weight, and growing properly. Your baby may need further follow-up appointments to make sure everything is working as expected.
The most common long-term problem for babies with TEF/EA is a condition called gastroesophageal reflux. We can initially treat this with medicine, but it is quite likely that the baby will need an operation, fundoplication, to prevent reflux.
As they grow older, children with TEF/EA usually can eat normally and have a good quality of life. They need to eat slowly and chew thoroughly and avoid eating big chunks of especially dry food. Mrchitnis (talk) 09:08, 20 January 2024 (UTC)
Basic info for the parents of children with gastroesophageal reflux. This is NOT medical advice. Contact your doctor for the correct advice.[edit source]
Gastroesophageal reflux disease
Introduction:
Normally, the food goes from the food pipe (esophagus) into the stomach. Once food is in the stomach, a ring of muscle fibers, at its junction with the esophagus, prevents it from going back. If this muscle does not work well, food can leak back into the esophagus. This is called gastroesophageal reflux. “Gastro” is related to the stomach, “esophageal” means related to the food pipe, and “reflux” is to go back.
The stomach secretes hydrochloric acid, which is necessary for the digestion of the food. The inner lining of the stomach is used to the presence of this acid, but the inner lining of the esophagus is not. The problems that arise due to food and acid going back into the esophagus are known as GER disease (GERD).
Gastroesophageal reflux is normal in infants. This is due to a lack of maturation of the mechanism that prevents reflux. Majority of infants reflux from time to time, during the first six months of their lives. But it does not affect their general health and they outgrow this condition. In some cases, reflux may persist into childhood and can cause varying degrees of esophageal damage.
If the reflux persists beyond infancy or if it is associated with weight loss or breathing difficulty, it is considered abnormal. Neurologically impaired children are more prone to GERD.
Symptoms
· Excessive vomiting, worse after eating
· Not feeding well
· Excessive crying due to pain
· Weight loss or not growing well
· Cough, especially after eating
· Wheezing or other breathing problems
Diagnosis
The child's symptoms and physical examination
Investigations
· Special x-rays of the esophagus and stomach called “contrast swallow and meal”: The child is given a special liquid (usually barium) that is seen on the x-ray monitor. Its progress is followed as the child swallows it.
· 24-hour esophageal pH monitoring: A fine “wire” or electrode passed via the child’s nose, and it rests at the lower end of the esophagus. It is attached to a “minicomputer” that records how often and for how long stomach acid enters the esophagus.
· Esophagogastroscopy and biopsy: Under anaesthesia, a fine tube with a camera is passed via the child’s mouth into the esophagus and the stomach to check whether there is any redness, ulceration, or narrowing of the esophagus. A small piece of the lining of the esophagus can be taken for examination under a microscope.
Treatment
Medical treatment
· Burp your baby after drinking about 30mls from the bottle, or after feeding on each side if breastfeeding
· Hold the baby upright for 20-30 minutes after feeding
· Raise the head of the crib and let the baby/child sleep in upright/reclining position
· Add one tablespoon of cereal to 60mls of formula or expressed breast milk
· Give smaller feeds more often: feed every two hours rather than every three hours
· Medications to reduce acid: Proton pump inhibitors (PPI)- e.g., Omeprazole
Complications
· Aspiration pneumonia caused by stomach contents going into the lungs
· Esophagitis: Redness, irritation and swelling of the esophagus
· Anaemia: due to bleeding from the inflamed esophagus
· Stricture: Scarring and narrowing of the esophagus
Surgery
· Necessary if medical treatment under supervision fails
· For complications like stricture of the esophagus
· In neurologically impaired children- low threshold for surgery
· If associated with mechanical problems like herniation of the stomach in the chest
Operation
Fundoplication: To wrap the upper part of the stomach around the lower esophagus
Purpose: to help the natural mechanism to prevent reflux
Commonest operation: Nissen fundoplication
Approach: Open v/s laparoscopic (key-hole surgery)
Fig.2: Nissen fundoplication
In neurologically impaired children it is combined with feeding gastrostomy- insertion of a tube in the stomach to feed.
Precaution: to avoid making the wrap too tight
After surgery: Soft diet for three weeks
Follow up: after three weeks. Wound check and start solid food slowly
Results:
· Good if done for (1) Herniation of stomach
(2) Poor feeding
· Poor if done for (1) Chest symptoms
(2) In neurologically impaired children
Complications: (1) The Child cannot swallow or burp: if the wrap is too tight
(2) Failure: Refluxrecurs when the wrap gives way or becomes loose
Complications more common in neurologically impaired children Mrchitnis (talk) 09:13, 20 January 2024 (UTC)
Basic info for the parents of children with Hirschsprung's disease. This is NOT medical advice. Contact your doctor for the correct advice.[edit source]
Hirschsprung’s disease
Introduction
Herald Hirschsprung was a Danish Paediatrician, who described children suffering from chronic intractable constipation, for the first time, in the 1930s. This condition is known by his name.
Evacuation of stool and gas is one of the most sophisticated functions, we human beings have acquired through evolution. For this to happen, areas of the bowel, especially the large bowel, must work in perfect coordination. An unbelievably large network of fine nerves (like fine electrical cables) and tiny ganglia (electrical plugs) transmit the necessary instructions that originate in the brain and the spinal cord (a bundle of big nerves in the backbone).
The absence of ganglia in a variable length of the large bowel, starting from the rectum, results in a child not being able to pass stool and gas effectively. It results in a dilated or distended large bowel (colon). This is known as “congenital megacolon”- congenital as it is present since birth, “mega” is large; “congenital aganglionosis”- “aganglionosis”- the absence of ganglions (in the large bowel) and “Hirschsprung’s disease.”
Presentation
(1) Newborn baby:
(i) Delay in passage of meconium (first dark stool) beyond 2 days
(ii) Does not pass meconium, tummy gets bloated, and baby starts vomiting green stuff- bowel obstruction
(iii) Infrequent stooling and failure to gain weight
(iv) Loose foul-smelling stool and temperature (enterocolitis)
(2) Older child:
(i) Chronic (long-standing) constipation- irregular bowel habits
(ii) Unable to pass stools without enemas
(iii) Bloated tummy
(iv) Fails to grow
(v) Frequent attacks of loose foul-smelling stool
Diagnosis
History and clinical examination
Investigations
(1) X-ray abdomen: Dilated loops of bowel especially large bowel sometimes with air-fluid levels
(2) Contrast enema: A special x-ray is done while injecting x-ray medicine into the baby’s bottom and viewing the progress under the screening machine. This can substantiate the diagnosis, indicate how far the problem extends in the large bowel and show if there are any complications like enterocolitis.
Typical findings: Normal calibre rectum
Cone-shaped transition zone
Dilated colon
(3) Rectal biopsy: A small piece is sent to the Pathologist to check under a microscope for the presence or absence of ganglion cells
(i) Suction biopsy: This can be done in the ward. Three tiny pieces are taken from the inner lining of the rectum with the help of a syringe-like apparatus. Can only be done in young infants.
(ii) Full-thickness biopsy: Needs to be done in operation theatre under anaesthesia.
(4) Multiple colonic biopsies: to decide the extent of the problem
(i) Laparoscopic- through keyhole surgery
(ii) Open- after cutting the tummy
Treatment
Initial medical treatment:
(i) Intravenous fluids (saline) if the child is not feeding well
(ii) Intravenous antibiotics if enterocolitis is suspected
(iii) Rectal washouts with warm saline
Surgical treatment:
Principle: To remove the bowel that does not have ganglion cells and bring the bowel with normal ganglion cells down to join the lowermost end of the rectum. This is called the “pull through” operation, as the normal bowel is pulled through the anus.
There are various operations described and done by different surgeons. All of them have comparable results. It is ideal to do the operation in the first month of life or early infancy as a single-stage procedure. This avoids the colostomy- opening the bowel on the skin.
They can be done as a combination of the following three approaches:
(a) An open operation- with a cut on the tummy
(b) Assisted with laparoscopy- key-hole surgery
(c) Through the anus- transanal
All of them are major operations, done under general anaesthesia and may need blood transfusion. The baby will stay in the hospital for about a week and need to go back to the operation theatre in about three weeks for the Surgeon to check the baby’s bottom under anaesthesia. Some babies may need their bottom to be dilated regularly for a few weeks. This will be taught to you in the ward.
Baby/child may need a temporary colostomy,an operation in which the large bowel is opened on the skin for the child to pass stool in a (colostomy) bag, if:
(i) Rectal washes do not help
(ii) Sick infant with enterocolitis
(iii) Late presentation with distended bowel
· Nursing sisters will teach you how to look after a colostomy.
· A colostomy DOES NOT transmit infection to anyone else.
Follow up
Initially after three weeks, then every month x 2-3 months, and later at least once a year
Prognosis
· Even after a correctly performed operation, some children will continue to have symptoms related to passing stool, as the rest of their large bowel, even though it looks normal, does not function 100% well. This can be treated with simple measures.
· Most children will have a decent quality of life after a successful pull-through operation. Mrchitnis (talk) 09:22, 20 January 2024 (UTC)