Cleft Lip and Palate: Structural Disorders

Structural Disorders

• Structural disorders are a category of gastrointestinal disorders
• These are distinct facial defects that occur alone or in combination
• These malformations occur in embryonic development

Cleft Lip and Cleft Palate

• Cleft lip and cleft palate are distinct facial defects that can occur separately or together
• Both conditions arise during embryonic development
• Merging of the upper lip is typically completed by the 7th to 8th week of AOG (amenorrhea duration)
• Fusion of the palate occurs between the 7th and 12th week of AOG

Cleft Lip

• Cleft lip’s definition is the failure of the maxillary and median nasal processes to fuse
• This results in an incomplete fusion of the lip
• It occurs more often in Asians and less commonly in Caucasians, with the lowest incidence in Blacks
• It is more common in males, about 1 in 800 live births
• Can involve deformation of dental structures
• Classification includes complete, incomplete, unilateral, and bilateral types

Cleft Palate

• Cleft palate is defined as the incomplete fusion of the palate
• It creates a communication between the mouth and nose
• Incidence is about 1 in 2000 live births
• It is more common in females
• Although often associated with cleft lip, it can occur independently
• If with a cleft lip, occurs midline of the soft palate and one or both sides of the hard palate
• Cleft palate alone occurs in the midline

Characteristics of Cleft Lip and Cleft Palate

• Infants with cleft palate often have difficulty feeding
• The infant is unable to generate a negative pressure and create suction in the oral cavity
• Recurrent otitis media is common due to fluid entering the middle ear, potentially causing tympanic membrane scarring and hearing loss

Causes of Cleft Lip and Palate

• These abnormalities are multifactorial, but are commonly due to genetics
• Inherited chromosomal abnormalities are associated with other defects
• Environmental teratogens like maternal smoking (11-12% of cases), viral infections and folic acid deficiency are also causative

Diagnosis and Management of Cleft Lip and Palate

• Cleft lip can be detected through sonogram while in utero
• Cleft palate may only be detected with a thorough oral assessment
• Surgery is the primary intervention
• Cleft lip repair is managed with cheiloplasty
• Cleft palate repair is managed with uranoplasty

Timeline for Surgical Repair

• Cleft lip repair is typically done earlier at 6-12 weeks to avoid oral deprivation
• This also prevents trauma and difficulty for the parents and encourages bonding
• Cleft palate repair occurs later at 12-18 months, before the child develops faulty speech habits
• A general rule of 10 is often followed (10 gms Hgb, 10lbs, 10 weeks)

Nursing Diagnosis: Preoperative

• High risk for aspiration due to anatomical defect
• Ineffective coping related to the birth of a child with a visible and/or structural defect
• Altered nutrition related to the infant's inability to form an adequate seal

Nursing Diagnosis: Postoperative

• High risk for infection related to surgery and accumulation of fluids
• Ineffective breathing pattern related to anesthetic and increased secretions
• Impaired tissue integrity related to surgical correction
• Knowledge deficit related to diagnosis, treatment and home care
• Altered nutrition related to surgery and feeding difficulties

Nursing care for Cleft Lip and Palate

• Focus on positive attributes of the infant to help parents cope
• Encourage parents to hold and cuddle the infant
• Explain the surgical procedure and expected outcome, providing before and after pictures

Provide Adequate Nutrition (ESSR)

• Enlarge the nipple during feeding, using a rubber-tipped medicine dropper or aseptosyringe as an alternative
• Stimulate the suck reflex, allow the child to swallow fluid appropriately, and provide rest periods

Positioning during Feeding

• Use an upright position to facilitate swallowing and discourage aspiration
• Burp frequently to prevent regurgitation or vomiting

Postoperative Care

• Gentle aspiration of mouth and nasopharyngeal secretions if necessary
• Be aware of no suctioning due to potential trauma to the site
• Position the infant upright or in an infant seat after CL repair
• Lay the infant on the abdomen after CP repair

Postoperative Monitoring

• Check for bleeding, indicated by frequent swallowing, increased pulse rate, and restlessness
• Logan's bow/butterfly adhesive may be used to approximate the suture line

Protecting the Operative site

• Use restraints (elbow pinned to the infant's clothing) to prevent rubbing of the incision by the hands

Restraints

• Use elbow restraints or jacket restraints for patients, and monitor restrained limbs frequently
• Minimize crying and anticipate needs to reduce tension on the suture line
• Avoid pacifiers, tongue depressors, thermometers, spoons, straws and hard food items

Pain Management and Nutrition

• Minimize pain using opioids, then acetaminophen PRN
• Provide clear liquid nutrition initially

Preventing Infection

• Assess vital signs
• Cleanse the suture line with normal saline/sterile water and cleanse cleft areas with water after feeding
• Use cotton swabs to apply half-strength peroxide to loosen crusts without causing trauma
• Apply antibiotic cream if prescribed

Prognosis

• Speech therapy may be needed even with good anatomic closure
• Speech impairment may be caused by insufficient functioning of the soft palate and nasopharynx
• Improper tooth alignment and hearing loss may need to be addressed

Esophageal Atresia with Tracheoesophageal Fistula

• Esophageal atresia (EA) is defined as the failure of the esophagus to develop a continuous passage
• The esophagus ends in a blind pouch with no connection to the stomach
• Tracheoesophageal fistula (TEF) is an abnormal connection between the esophagus and trachea

Incidence

• Rare, found in approximately 1 in 3000 to 5000 live births
• Equal sex incidents
• Associated with low birth weight and prematurity
• Can occur with other congenital disorders such as vertebral, anorectal, cardiovascular, tracheoesophageal, renal or limb abnormalities (VACTERL)

Types of Esophageal Atresia with Tracheoesophageal Fistula

• Simple esophageal atresia with no fistula (isolated EA): upper and lower ends are blind
• EA with proximal TEF: the upper end of the esophagus connects to the trachea
• EA with distal TEF: the upper end is blind, the lower end connects to the trachea
• EA with double TEF: both upper and lower ends connect to the trachea through a fistula
• Isolated TEF: no esophageal atresia, but with fistula between normal esophagus and trachea

Clinical Manifestations

• The 3 C's of TEF are choking, coughing, and cyanosis because of aspiration from pouch overflow
• Copious, fine white, frothy mucus in mouth and nose
• Increased respiratory distress after feeding
• Distended abdomen due to air passing from the trachea into the stomach

Diagnostic Tests

• Diagnosis should be prompt so that it does not cause aspiration
• A history of polyhydramnios and clinical manifestations may be indicative
• Inability to pass NGT and aspirate stomach contents suggests EA
• Radiography confirms the exact anomaly

Management

• Broad-spectrum antibiotics are given
• Surgery to repair the anomaly involves closing the fistula and anastomosing esophageal segments
• A NGT tube is used for feeding until the esophagus heals, with a chest tube placed

Palliative Measures

• Multiple stages are indicated for premature infants or those with severe conditions
• Ligation of TEF includes gastrostomy for gastric decompression, cervical esophagostomy as an alternative and esophageal replacement

Complications of surgery

• Anastomotic leak can cause purulent chest tube drainage, and increased WBC counts
• Esophageal motility disorders include dysphagia, and GER

Maintain Open Airway (Pre-op)

• Use continuous or low intermittent suction after birth
• Elevate the infant in a supine position on an inclined plane at least 30 degrees to minimize reflux

Post Op care

• Assess respiratory function and observe respiratory difficulties
• Suction PRN
• Provide chest tube care
• Keep chest tube lower than chest, and check for clots, amount, color and drainage

Optimum Nutrition (Post op)

• Elevate and secure the gastrostomy tube 4" above the level of the stomach
• This is to prevent reflux to the healing esophagus allowing any swallowed air to escape
• Observe carefully to make sure that the infant is able to swallow without choking

Omphalocele and Gastroschisis

• This section defines and outlines the nursing care for omphalocele and gastroschisis conditions

Omphalocele

• Omphalocele is defined as the protrusion of intraabdominal contents into the base of the umbilicus
• It is covered by a translucent sac (peritoneum) leading to rupture and possible evisceration
• Herniated organs include the intestines, and may include the stomach and liver

Gastroschisis

• Gastroschisis is a protrusion of intraabdominal contents through a defect in the abdominal wall lateral to the umbilical ring
• It is characterized a lack of a peritoneal sac
• The exposed intestines are covered by membranes

Incidence and Cause

• Incidence of omphalocele and gastroschisis is often associated with other congenital anomalies, such as heart defects and imperforate anus
• Both of these are caused by abdominal contents not returning in time
• Abdominal contents fail to return into abdominal wall as it closes by the 10th week

Management and Nursing Care

• Immediate surgical repair is implemented
• This entails replacing the abdominal contents and closing the abdominal wall
• Main goal is to protect the sac from trauma and infection

Protecting the Sac

• Monitor the sac and ensure that it is covered with sterile gauze soaked in normal saline
• This prevents drying
• Place dried sterile gauze over sterile moist gauze and cover with plastic wrap
• This provides additional protection from heat and moisture loss
• Also monitor for S/S of infection

Prepare for surgery by:

• Preop: NPO, IVF, NGT for decompression
• Postop: measures to control pain, prevent infection, fluid and electrolyte balance, adequate nutritional intake

Disorders of Motility

• A gastroesophageal reflux is characterized by the return of gastric contents into the esophagus

Definition

• Gastroesophageal reflux (GER) is the backward movement of gastric content due to neuromuscular disturbance with lower esophageal sphincter being lax

ER vs. GERD

• GER becomes GERD when complications such as failure to thrive, bleeding, or dysphagia occur
• GERD represents symptoms and tissue damage resulting from GER
• Patients may have reflux due to transient LES relaxation, delayed gastric emptying because of overfeeding
• Can be associated symptoms such as apnea, bronchospasm, laryngospasm, pneumonia

Risk Factors

• Prematurity (hypotonia)
• Infants with bronchopulmonary dysplasia
• Asthma
• TEF/EA repair
• Gastrostomy placement
• Neurologic disorders (cerebral palsy)
• Cystic fibrosis
• Scoliosis and Hernia

Clinical Manifestations

• Effortless and non-projectile vomiting or spitting of gastric contents
• Poor weight gain
• Irritability and excessive crying
• Symptoms in older children such as heartburn
• Respiratory problems - recurrent pneumonia, chronic cough, wheezes, stridor or gagging
• Trouble swallowing

Diagnosis

• History and child's feeding pattern and clinical manifestations
• Barium swallow
• 24 hour pH probe study to determine reflux episodes, scintigraphy( radionuclide scanning)

Management and Nursing Care

• The management involves diet-thickened feedings, medications(H2 antagonists, PPI, protinetic agents), and may involve surgery
• For children that face severe complications, a Nissen fundoplication valve is employed
• For patients that undergo operations related to abdominal surgery, the same procedure with care must be in place
• Skin care on the gastrostomy site
• Maintaining adequate nutrition
• Positioning-Prone after feeding, medications and side effects
• Skin care on the gastrostomy site

Hirschprung's Disease

• Also know as Congenital Aganglionic Megacolon
• This is due to mechanical obstruction due to inadequate motility of the intestines

Cause and S/S

• Babies with this birth defect are born without intestinal nerve cells called ganglion cells
• These nerve cells allow the intestine to relax so stool can pass through the intestine and out of the body
• Without these special nerve cells, the intestine can't relax and it becomes very narrow

Clinical Manifestations: Newborn

• Failure to pass meconium within 24-48 hours after birth
• Abdominal distention, bile-stained emesis, refusal to suck, and irritability

Clinical Manifestations: Infancy

• Failure to thrive and abdominal distention
• Episodes of vomiting, and constipation with diarrhea

Childhood S/S

• Chronic constipation hallmark of megacolon
• Ribbon like foul smelling stools, visible peristalsis, fecal masses are easily palpable

Complications and Nursing care:

• Obstruction occurs if abdominal area increases
• Bowel perforations, s/s: Peritonitis increased
• Monitor v/s including abdominal circumference and ominous signs related to enterocolitis)
• Repeated saline enemas to decrease bacterial flora

Intussusception

• This is an invagination of the cecum in the colon that produces obstruction of the intestines.

Nursing Care

• Monitoring for s/s complications and stools
• If its caused by obstruction this leads to detection of perforation

Hypertrophic pyloric stenosis:

• Hypertrophic obstruction caused by circular muscle of the pyloric canal

Cause and Nursing Care

• Child is "good eater"/"avid nurser" who vomits occasionally or shortly after feeding and eagerly accepts a second feeding after vomiting
• Electrolyte depletion-metabolic alkalosis, readily palpable olive shaped mass in the upper abdomen
• Diets and nutrition
• Blood tests

Celiac Disease

• Disease of the proximal intestine characterized by abnormal mucosa and permanent intolerance of gluten
• Genetic disposition

Nursing care and Definition

• Failure to thrive, diarrhea noted after introduction of gluten -containing grains in the diet, typically between 1-5 y/o
• Educate gluten diet, and not to discontinue