Gastrointestinal: Cleft Lip and Palate

Gastrointestinal Disorders: Structural Disorders

• Cleft lip and/or Cleft palate are distinct facial defects that can occur together or separately
• Both defects result from malformations during embryonic development

Cleft Lip Facts

• Merging of the upper lip is usually completed between 7-8 weeks AOG (Age of Gestation)
• Results from the failure of the maxillary and median nasal processes to fuse
• Occurs more often in Asians
• Least likely in Blacks
• Unilateral clefts are generally below the center of one nostril
• Bilateral clefts are beneath both nostrils
• Complete clefts extend into the base of the nose and gums, deforming upper teet
• Incomplete clefts are small notches, or a little more than a notching of the vermillion border

Cleft Palate Facts

• Fusion of the palate is completed between 7-12 weeks AOG (Age of Gestation)
• It is an Incomplete fusion of the palate which creates communication between the mouth and the nose
• This is more common with Cleft lip
• Occurs alone in the midline and may involve the soft and hard palate
• If occurring with a cleft lip, the cleft palate will be in midline of the soft palate and on one or both sides of the hard palate
• Occurs less often in Asians and more common in Caucasians
• Incidence is 1:800 live births among males and 1:2000 live births among females

Cleft Lip/Palate Cause

• Genetics-chromosomal abnormalities, inherited increase incidence in relatives
• Teratogens like maternal smoking or viral infections cause Folic acid deficiency/avitaminosis

Cleft Lip/Palate Diagnosis

• Cleft lip is apparent at birth and can be detected by sonogram while in utero
• Cleft palate may not be detected without assessment of the mouth by palpating with a finger

Cleft Lip/Palate Management

• Surgery can be performed and depends on the issue; Cleft lip is repaired with cheiloplasty, and cleft palate uranoplasty
• Cleft lip repair is done at 6-12 weeks to avoid oral deprivation and trauma and difficulty for the parents and deformed face
• Cleft palate repair is done at 12-18 months to take advantage of normal palatal changes, before child develops faulty speech habits

Nursing Diagnosis: Preoperative

• High risk for aspiration (breastmilk, formula or mucus) related to anatomic defect
• Ineffective coping related to birth of child with visible and/or structural defect
• Altered nutrition: less than body requirements related to infant’s inability to form an adequate seal

Nursing Diagnosis: Postoperative

• High risk for infection related to surgical procedure and accumulation of formula and secretions in pre-oral cavity
• Ineffective breathing pattern related to anesthetic and increased secretions
• Impaired tissue integrity related to surgical correction of cleft
• Knowledge deficit related to diagnosis, treatment, prognosis, and home care needs
• Altered nutrition: less than body requirements related to surgery and feeding difficulties

Nursing Care

• Parent is helped to cope with the impact of the defect by pointing out positive attributes and viewing the infant as a whole, as well as explaining surgical procedure and showing pictures before and after surgery
• Adequate nutrition using ESSR is provided, using interventions like enlarging the nipple, stimulating the suck reflex, and allowing the child to finish what has been placed in the mouth

CL and CP Postoperative Nursing Care

• Maintain direct open upward position for CL and lie on Abdomen for CP
• Elbow Restraints are needed for CL and maintained until palate is healed (4-6 weeks) for CP
• Infection prevention involves assessing vital signs, cleansing suture line with normal saline or sterile water, cleaning cleft areas with feedings, use cotton swab with a half-strength peroxide for removals of crust or order use of antibiotic cream and hand-washing and sterile technique
• Check for bleeding like frequent swallowing and restlessness
• No sucking or pacifiers should occur postoperatively
• Nutrition with clear liquid with rubber tipped syringe to mouth side can be done with CL and large hole nipple to side can be done with CP

Esophageal Atresia (EA) and Tracheoesophageal Fistula (TEF)

• Esophageal atresia (EA) features failure of the esophagus to develop a continuous passage
• Tracheoesophageal fistula (TEF) is an abnormal connection between the esophagus and the trachea
• Incidence is rare; 1:3000-5,000 live births, equal sex incidence, associated with low birth weight, prematurity and congenital disorders (VACTERL)

Types of Esophageal Atresia

• Simple esophageal atresia with no fistula (Isolated): Upper and lower ends of esophagus are blind, no connection to the trachea
• Esophageal Atresia with proximal TEF: Upper end of the esophagus ends into the trachea
• Esophageal Atresia with distal TEF: Upper end of esophagus is blind, lower end connects into the trachea = most common
• Esophageal Atresia with double TEF: Both upper and lower ends of esophagus open into the trachea by a fistula
• Isolated TEF: No Esophageal atresia but with fistula between normal esophagus and trachea

Clinical Manifestations of TEF

• Three C's (3 C’s of TEF) include choking, coughing, cyanosis resulting from aspiration from overflow of secretions from the blind pouch,
• Other indications includes copious, fine white frothy bubbles of mucus in the mouth and nose which signifies EA
• Increased respiratory distress may also be an indicator
• Distended abdomen occurs when air from the trachea passes through the fistula into the stomach

TEF Diagnosis

• A delay in diagnosis can be fatal because of aspiration
• History (polyhydramnios=EA) and clinical manifestations
• Inability to pass NGT (to confirm placement, aspirate stomach contents) and radiography to determine exact type of anomaly by radiopaque catheter and chest films
• (-) air in the GIT=EA without TEF

TEF Management

• Prevent aspiration pneumonia and give broad-spectrum antibiotics
• Perform surgery, closing the fistula and anastomosing esophageal segments, and make sure there is a NGT for feeding until esophagus heals as well as a chest tube
• Gastrostomy is for gastric decompression, provision of constant drainage of the esophageal pouch

Omphalocele and Gastroschisis

• Omphalocele involves protrusion of intraabdominal contents into the base of umbilicus covered by a translucent sac, containing herniated organs (intestines)
• Gastroschisis involves protrusion of intraabdominal contents through defect in abdominal wall lateral to umbilical ring which is never with a peritoneal sac.

Omphalocele and Gastroschisis Incidence

• Often is associated with other congenital anomalies (heart, genitourinary, TEF and imperforate anus)
• cause : Abdominal contents fail to return when abdominal wall closes by 1oth week
• immediate surgical repair to replace abdominal contents and close abdominal wall

Omphalocele and Gastroschisis Nursing Care

• Protect sac from trauma and infection
• Monitor sac with sterile gauze in to prevent drying as well as placing dry gauze and covering sac
• Monitor vital signs for temperature loss

Gastroesophageal Reflux (GER)

• Return of gastric contents into the esophagus and backward movement, and neuromuscular disturbance in which the cardiac sphincter and lower portion of the esophagus are lax
• The LES (lower esophageal sphincter) has transient relaxation and dysfunctions

Esophageal Reflux vs Esophageal Reflux Disease

• Simple Esophageal Reflux (ER) becomes Esophageal Reflux Disease when complications such as failure to thrive, bleeding or dysphagia develop
• GERD represents symptoms of respiratory issues and tissue damage
• Risk factors include prematurity (hypotonia)bronchopulmonary dysplasia, asthma, TEF/EA repair, gastrostomy placement, neurologic disorders (cerebral palsy), cystic fibrosis, scoliosis and hernia
• Clinical manifestations involve regurgitation, spitting up/vomiting, poor weight gain, hematemesis, melena, irritability, excessive crying, heartburn, anemia, respiratory conditions and dysphagia

Celiac Disease

• A proximal intestine disease characterized by abnormal intestinal mucosa and permanent intolerance to gluten and inherited autoimmune disease.
• 1:300-4,000 live births and appears to be declining and Europeans>Americans; rare in blacks and Asians

Celiac Disease Manifestations

• Steatorrhea ( excessively large, pale, oily, frothy and foul smelling stools)
• Patients cannot absorb nutrients because of malnutrition, therefore patients express Muscle wasting, Anemia, Anorexia, Abdominal distention, irritability and Celiac crisis
• Celiac crisis brings acute severe episodes of profuse, watery diarrhea, vomiting and infections
• Complications include growth retardation, malignant lymphoma of small intestines, esophageal and GI cancers

Esophageal Reflux Diagnoses

• History and child’s feeding pattern and clinical manifestations by 24 hour pH probe study at distal esophagus that requires insertion of small catheter through nose
• Barium swallow (esophagography) establishes presence Scintigraphy-radionuclide scanning can be done

Diagnosing, Managing and Caring for GER

• Diet management involves thickened and diet modifications
• Pharmacology includes H2 blocking agents and Prokinetc agents that Increase resting LES pressure and the rate of gastric emptying
• Surgical intervention is NISSEN FUNDOPLICATION
• Surgery can cause small bowel obstruction, retching, gas-bloat syndrome and Dumping syndrome

Nursing Care for GER

• Identifying children with GERD through Feeding Problems, Respiratory Distress, Episodes of Vomiting
• In order to prepare post-op care, the skin must be clean and medications must be available
• Feeding needs to be small and consistent in specific forms, with specific posturing to reduce effects

Hirschsprung’s Disease

• Hirschsprung’s Disease is also known as Congenital Aganglionic Megacolon
• It is a Mechanical obstruction due to inadequate motility of the intestines because aganglionic means without nerve tissue and megacolon means dilated action in the colon
• accounts for ¼ of all cases of neonatal obstruction, can present as an acute (life-threatening) / chronic disorder, and is more common in males
• Ganglion are not present, and bowel can not relax and stool can not be evacuated

Hirschsprung’s Disease Manifestations and Complications

• Newborn failure to pass meconium within 24-48 hours after birth versus failure to thrive, abdominal distention and possible watery diarrhea may appear in infancy
• Diarrhea or Foul Smelling may appear after
• Obstruction or Enterocolitis (inflammation of small bowel and colon)
• May require management or enemas to properly evacuate fluids from the colon

Intussusception

• Telescoping of one portion of intestines into another, often idiopathic, and common in infants and males with potential Cystic Fibrosis related
• Pain is acute and presents with drawing of the knees onto the chest, followed by intervals of pain and distended abdomen
• Vomiting may be bile (Ampulla of Vatar or red colored
• Masses are often palpable as well
• May require surgery

Pyloric Stenosis

• Hypertrophic condition of the circular muscle of the pyloric canal
• First-born males are most commonly affected
• Unknown genetic cause with possible history of mothers with the same issue
• May present with Projectile vomiting 3ft after feeding, no discomfort and constant hunger
• Diagnostic care involves blood tests, palpication of olive-shaped mass and imaging of elongated muscular region
• Manage by Pyloromyotomy, through a tiny laparoscopic incision