Pediatric Gastrointestinal Disorders

Common Pediatric Gastrointestinal Disorders

• Common pediatric G.I. disorders affect the mouth, esophagus, stomach, and intestines.
• Conditions include cleft lip/palate, tracheo-esophageal fistula, pyloric stenosis, hernias, intussusception, Hirschsprung's disease, and Crohn's disease.

Cleft Lip and Palate (Disorders of the Mouth)

• Cleft lip is a failure of fusion of the maxillary process with nose elevation on frontal prominence.
• The extent of a cleft lip can be a small notch, or a large cleft reaching the floor of the nose.
• Cleft lip may occur on one side (unilateral) or both sides (bilateral).
• Cleft palate results from the failure of the hard palate to fuse with each other and the soft palate.
• Cleft lip commonly occurs with cleft palate.
• Cleft palate can be complete or incomplete.
• The incidence of cleft lip is approximately 1 in 750 births.
• Cleft lip is seen predominantly in males, while cleft palate is more common in females.
• The incidence of cleft palate is 1 in 2500 births.
• Approximately 15% of infants with cleft lip/palate have associated defects.
• Cleft lip and cleft palate are facial malformations during early fetal development.
• The exact cause is unknown, but genetics and environmental factors may have a role.
• Potential causes: medications during pregnancy (anticonvulsants, acne treatments), exposure to viruses or chemicals, exposure to X-rays, maternal alcohol intake, and maternal smoking.
• The fusion of maxillary and premaxillary processes typically occurs during the 5th and 8th week of gestation.
• The palatal processes fuse about a month later.
• A failure of fusion leads to cleft lip and cleft palate.
• Clinical manifestations: appearance of a notch or gap in the lip, feeding difficulties, speech delays or abnormalities, ear infections, hearing loss, and crooked, poorly shaped, or missing teeth.
• Prenatal diagnosis is sometimes possible via maternal ultrasonography.
• Physical examination after birth confirms cleft lip or palate.
• A gloved finger placed in the mouth or visual examination confirms the diagnosis.
• Cleft lip and palate can be identified at birth and detected via ultrasound at about 16 weeks into pregnancy.
• Cleft lip management may require one or two surgeries based on the severity.
• Initial cleft lip surgery happens at around 3 months of age.
• Common surgical corrections: Tennison Randall Triangular Flap (Z-plasty), and Millard's Rotational Advancement technique.
• Cleft palate correction requires many surgeries over 18 years.
• First surgical repair happens between 6-12 months to promote a functional palate, reduce fluid entering the middle ears, and aid teeth development.
• At 8 years old a bone graft may be needed to fill in the upper gum line.
• Braces are needed to straighten the teeth once the permanent teeth grow.
• Nursing management includes care of the baby at birth, before surgery, and after surgery.
• Cleft lip can make it difficult for the baby to make a good seal around the nipple.
• Babies with cleft palate usually need special bottles and techniques to feed properly.
• Special bottles for feeding babies with clefts: Mead-Johnson Cleft Palate Nurser, Haberman Feeder, and Pigeon Nipple.
• Pre-operative nursing care includes consent, investigative report checking and NPO for 6 hours before surgery.
• Post-operative nursing duties: close observation and monitoring, observe for bleeding, turn baby's face to one side for draining secretions and protect surgical site from injury.
• An arched device called a Logan bow might be used to prevent tension on the suture site.
• Administer prescribed medications and ensure that the baby does not put any object in the mouth.
• The nursing care process for newborns with cleft lip and cleft palate includes nursing assessment, interviewing the family and physical exams
• During the interview the nurse must explore the family’s acceptance of the newborn and inquire about effective feeding methods.
• Physical examination includes monitoring temperature, apical pulse, respirations, breath sounds, skin turgor/color, neurologic status, alertness and responsiveness

Nursing Care Planning and Goals

• Goal setting and planning adapt to surgical plans
• Major goals: maintaining adequate nutrition, increasing family coping, reducing the parents’ anxiety and guilt and preparing them for the future repair.

Nursing Management

• Maintain adequate nutrition
• Breastfeeding is successful because breast tissue may mold to close the gap
• Expressed breast milk used instead of formula if newborn cannot be breastfed
• A soft nipple with a cross-cut made to promote easy flow of milk may work well
• Positioning is unilateral, the nipple should be aimed at the unaffected side.
• Maintain the infant in an upright position during feeding.
• Tools for feeding include Lamb’s nipples (extra long nipples) and special cleft palate nipples molded to fit into the open palate area
• One of the simplest and most effective methods may be the use of an eyedropper or an Asepto syringe with a short piece of rubber tubing on the tip (Breck feeder).
• Encourage verbalization about their feelings.
• Serve as a model for the family caregiver's attitudes toward the child.
• Providing information about cleft repairs encourages questions and reassurance.

Tracheo-esophageal Fistula (Disorder of Esophagus)

• Tracheo-esophageal fistula and esophageal Atresia malform the digestive system, causing the esophagus to not develop properly.
• The esophagus is a tube that normally carries food from the mouth to the stomach.
• Esophageal Atresia is the failure of the esophagus to form a continuous passage from the pharynx to the stomach.
• Incidence of Tracheo-esophageal fistula is 1 in 3500 births.
• Males have predominance.
• Esophageal Atresia with or without fistula is most common among premature births at %50.
• EA is often accompanied by other anomalies e.g. vertebral column diseases, anorectal malformations, cardiac defects, renal anomalies and limb abnormalities
• The presence of maternal polyhydramnios and a single umbilical artery should alert pediatricians.
• Clinical features: violent response on feeding, infant coughs and chokes, fluid returns through nose and mouth, cyanosis occur, infant struggles and excessive secretions coming out of the nose and constant drooling of saliva.
• Frothy saliva is indicating of a key symptom that a baby has a birth defect, causing them to be unable to swallow properly, resulting in excessive drooling and appearance of foamy saliva in the mouth.
• Babies develop frothy white bubbles in the mouth accompanied by choking and difficulty feeding.

Diagnostic Evaluation

• Ultrasound can detect atresia in the pregnant woman.
• soon after birth vigorous coughing, choking in response to feeding is an important manifestation.
• During suspicion, attempt is made to pass a feeding tube through nose or mouth into stomach, tube does not pass into the stomach being indicative of atresia.
• X-ray of chest shows air filled esophageal pouch and air in stomach, or if the feeding tube has been inserted, it appears coiled up in the upper esophageal pouch.
• An ultrasound enables identification of the type of tracheo-esophageal pouch.
• Polyhydramnios is the excessive amount of amniotic fluid during pregnancy
• It is a common prenatal sign of esophageal atresia (EA)
• A fetus with EA cannot swallow amniotic fluid due to the blockage in their esophagus causing accumulation of fluid in the uterus.
• It is a key indicator for suspecting EA on an ultrasound scan.
• A normal fetus swallows amniotic fluid as part of their development, but with esophageal atresia, this swallowing is impaired, resulting in increased amniotic fluid levels (polyhydramnios).
• During prenatal ultrasound, a healthcare provider may see polyhydramnios alongside a small or absent stomach bubble

Management

• The management of tracheo esophageal fistula is mainly surgical.
• Surgical intervention depends upon the distance between the proximal and distal pouch of esophagus, type of defect, condition of neonate and his weight.
• If the distance between proximal and distal tube is less than 2.5 cm, and the infant condition is good, primary repair is done by division and ligation of the fistula along with end to end anastomosis.
• Pre-operative nursing management includes stopping any feed after diagnosis as well as NG tube aspiration frequently to aspirate pooled secretions.
• Nurses should maintain patency of NG tube by irrigating it with NS, while observing for respiratory distress and administer prescribed medications.
• Administer IV fluids as prescribed whilst, maintaining I/O chart and hydration status of child.
• Administer oxygen, keep child in semi-upright position and monitor vitals of child.

Post-Operative TEF care

• Respiratory management includes monitoring oxygen saturation and respiratory status
• Potential need for mechanical ventilation, with humidified oxygen delivery to prevent airway dryness
• The feeding management includes no oral intake initially, and reliance on nasogastric or gastrostomy tube feeding.
• Use of contrast swallow study (esophagram) to assess for anastomotic leaks before initiating oral feeding, and gradual introduction.
• Anti-reflux medications: Proton pump inhibitors (PPIs) or H2 blockers prevent gastroesophageal reflux (GER).
• Appropriate pain medication administration is needed with monitoring, along with management of chest-tube if placed.
• Regular monitoring of vital signs is needed with early mobilization as tolerated, follow-up appointments with a pediatric surgeon for esophageal dilatations.

Pyloric Stenosis (Disorder of Stomach)

• Pyloric stenosis is defined as narrowing and obstruction of the lower portion of the stomach (pylorus).
• The narrowing prevents food from moving from the stomach into the intestine.
• Cause: hypertrophy and hyperplasia of circular muscles of stomach.
• It occurs typically in infants between 2-8 weeks of age in 1 in 500-1000 live births.
• Pyloric Stenosis is 5times more in more boys than girls, with no particular cause.
• Genetic predisposition and environmental factors are risk factors
• Clinical features appear between 2-4 weeks of age.
• Regurgitation and non-bilious vomiting that may occur during and after feeding is initial presentation.
• Vomiting becomes progressively forceful, until it is projectile in nature with contents that contain gastric and mucous contents, streaks of blood but does not contain bile..
• Infants present as hungry with weight loss, poor weight gain, dehydration, less bowel movement
• Diagnostic evaluations involves careful physical exams that reveals a "firm olive" sized mass in epigastrium, epigastric distension may be seen with visible peristalsis waves from left to right.
• Diagnostic evaluations also include barium meals that reveal narrowed pylorus.

Surgical Management

• 2 Stages to the management
• Initially fluids given intravenously to treat dehydration and restore body's normal chemistry.
• Pyloromuotomy or Fredet- Ramstedt surgery is then performed.
• Fredet-Ramstedt operation or Ramstedt pyloromyotomy, treats pyloric stenosis in infants that is performed under general anesthesia.
• During the procedure the pylorus muscle is split lengthwise
• Ramstedt pyloromyotomy remains the gold standard of treatment
• After surgery, infant's stomach needs rest for about 3 hours.
• Place the infant on a strict feeding schedule post-surgery.
• Antibiotics and fluids are continued for 24 hours.
• A nursing assessment includes signs of dehydration such as sunken eyes and monitoring of vitals and palpating of the abdomen.

Nursing Management - Assessment and Monitoring

• Regularly assess for signs of dehydration (sunken eyes, dry mucous membranes, decreased urine output, poor skin turgor)
• Monitor vitals (heart rate, respiratory rate, blood pressure)
• Palpate the abdomen for the “olive” shaped mass of the thickened pyloric muscle.
• Assess projective vomiting after feedings and monitor weight.

Preoperative Management:

• Administer IV fluids with appropriate electrolytes to correct dehydration and electrolyte imbalances based on laboratory results.
• Consider placing an NG tube for gastric decompression and to monitor gastric contents.
• If oral feedings are tolerated, provide small, frequent feedings with thickened formula to minimize vomiting.

Postoperative Management:

• Monitor for pain and administer pain medication as needed.
• wound should remain clean.
• Gradually advance to regular feedings as tolerated, starting with small amounts of breastmilk or formula.
• Administer IV fluids post-operatively and parental support.

Disorders of the Intestine - Hernia

• Hernia is the protrusion of the intestine through a weakness in the abdominal muscles.
• Hernias occurring in newborns are omphalocele, gastroschisis and diaphragmatic hernia
• Inguinal and umbilical hernias are diagnosed hernias during infancy and childhood.
• Two factors must be present
• A defect in the integrity of muscular abdominal wall/ Increased intra-abdominal pressure.
• Some include congenital defects or defects due to trauma
• Hernias can be reducible (when contents can be replaced) or Irreducable (when contents can not be replaced)

Risk Factors

• Young mothers less than 20 years
• Folic acid deficiency
• Hypoxia
• Salicylates, acetaminophen, ibuprofen consumption during pregnancy
• Cocaine and alcohol consumption during pregnancy
• High risk pregnancies complicated by low birth weight babies
• Inguinal Hernia: it is the most common hernia seen in infants and children that occurs in the groin region that may be unilateral or bilateral, more frequently in boys (90%) then girls (10%).
• It is more common in premature than term infants.

Pathophysiology

• In male fetus testicles move to scrotum through the inguinal canal
• Shortly after the baby is born, the inguinal canal close, preventing the testicles from moving back into the abdomen.
• If the closure does not occur completely, a loop of intestine can move into the inguinal canal, through the weakened area of the lower abdominal wall, causing hernia.
• Hernia can be reducible or irreducible.
• Reducible hernia, is contents of the hernia sac can be placed into the abdominal cavity by manipulation, where Irreducible is the hernia sac cannot be reduced or replaced.
• Inguinal hernias in newborns and children result from a weakness in the abdominal wall that's present at birth.
• Symptoms: visible only when an infant cries, coughs or strains during a bowel movement.
• In an older child: the hernia is more apparent when the child coughs, strains during a bowel movement or stands for a long period of time
• Other symptoms vomiting and cramping pain that contains bile with abdominal distension and fever.
• Babies present as irritable and restless, along with strangulation and gangrene.
• Diagnose by physical examination for reducible or irreducible type of hernia.
• Abdominal X-ray or ultrasonography helps in confirming diagnosis.

Management

• Is based on age, health, and type and depending on the hernia.
• The two types of surgeries are: open inguinal hernia repair (herniorrhaphy, hernioplasty) and laparoscopic repair.
• Herniorrhaphy is a procedure that repairs a hernia by pushing bulging tissue back into place and strengthens the muscle wall with an incision in the abdomen above the hernia.
• If the area of muscle to be repaired is large, the surgeon may sew a synthetic mesh over it to reinforce it.