Cleft Lip and Palate

Cleft Lip and Palate

• Fusion of maxillary and median nasal processes normally occurs between weeks 5 and 8 of intrauterine life
• Cleft lip is a failure of this fusion to occur, in varying degrees
• The severity in infants ranges from a small notch in the upper lip to total separation of the lip and facial structure up into the floor of the nose, even with absence of the upper teeth and gingiva
• Deviation can be unilateral or bilateral
• The nose tends to be flattened due to the incomplete fusion of the upper lip
• Cleft lip is more common in boys than girls
• Familial tendency or transmission of multiple genes are common causes
• Teratogenic factors can aid in the formation of cleft lip during weeks 5 to 8 of intrauterine life
• Viral infections or folic acid deficiencies can cause cleft lip and palate

Palatal Process Closure

• The palatal process closes at approximately weeks 9 to 12 of intrauterine life

Cleft Palate

• A cleft palate is an opening in the palate and is usually on the midline
• It may involve the anterior hard palate
• It may be a separate anomaly or occur in conjunction with cleft lip
• Cleft palate tends to occur more frequently in girls than boys

Assessment

• Cleft lip may be detected by sonogram while the infant is in utero
• Cleft palate can be determined by pressing the newborn's tongue with a tongue blade
• Use good lighting to visualize the palate clearly

Cleft Palate Repair

• Usually postponed until a child is 6 to 18 months old
• This allows the anatomic change in the palate contour to occur during the first year of life
• Repairs made before this change may be ineffective and need to be repeated.

Preoperative Period (Cleft Lip)

• Breastfeeding may be possible because the bulk of the mother's breast tends to form a seal against the incomplete upper lip
• The best feeding method involves supporting the baby in an upright position and feeding the infant gently using a commercial cleft lip nipple
• A Breck feeder (similar to a bulb syringe) or a Haberman feeder may be used
• If surgical repair is done immediately, the mother can breastfeed as early as 7 to 10 days postsurgery
• Infants with cleft lip should be bubbled well after feeding due to a tendency to swallow air

Mucous Membrane

• Exposure of a cleft to the nares causes infant to breathe through the mouth
• This causes the oral mucous membranes and lips to dry
• Offer small sips of fluid between feedings to keep the mucous membranes moist, prevent cracks, and prevent fissures that could lead to infection

Preoperative Period (Cleft Palate)

• Infants with cleft palate cannot suck effectively
• Pressing their tongue or a nipple against the roof of their mouth forces milk up into their pharynx, leading to aspiration
• Use a commercial cleft palate nipple with an extra flange of rubber to close the roof of the mouth for feeding
• The nipple can be used with a plastic bottle that can be squeezed gently to compensate for poor sucking

Postoperative Period

• The infant is kept NPO for approximately 4 hours after surgery for cleft lip or palate
• The infant is then introduced to small amounts of liquids (plain water) to prevent vomiting
• No tension is placed on a lip suture line to keep the sutures from falling apart and leaving a large scar
• Infants are fed using a specialized feeder because this causes less suture line tension than bottle or breastfeeding

Postoperative period (Palate)

• Liquids are generally continued for the first 3 to 4 days, and the soft diet is complete when diet heating is complete
• Do not use a spoon when a child begins to eat soft food as it disrupts sutures
• The child clear is put water to rinse the suture line, milk is not included in first fluids offered initially because the milk curd may adhere to the suture line after feeding.

Tracheoesophageal Atresia and Fistula

• Between weeks 4 and 8 of intrauterine life, the laryngotracheal groove develops into the larynx, trachea, and beginning lung tissue
• Anomalies can occur if the trachea and esophagus are affected by a teratogen that prevents the two organs from separating

Esophageal Atresia

• Refers to obstruction of the esophagus
• Often a fistula (opening) occurs between the closed esophagus and the trachea
• A radiopaque catheter can be demonstrated coiled in the blind end of the esophagus on x-ray

Tracheoesophageal Atresia Assessment

• Tracheoesophageal atresia must be ruled out in any infant born to a woman with hydramnios (excessive amniotic fluid)
• Diagnosing a tracheoesophageal fistula before an infant is first fed is important
• The infant will cough, become cyanotic, and have obvious difficulty breathing as fluid is aspirated
• A newborn with excessive mucus in the mouth appears to be blowing bubbles
• Diagnosis can be confirmed if a catheter cannot be passed through the infant’s esophagus to the stomach or the stomach contents cannot be aspirated
• Use a firm catheter since a soft one will curl in a blind end esophagus.

Tracheoesophageal Atresia Management

• Emergency surgery is essential to prevent pneumonia from leakage of stomach secretions into the lungs, dehydration, or electrolyte imbalance
• Antibiotics may be prescribed to help prevent infection
• Gastrostomy may be performed with drainage by gravity to keep the stomach empty of secretions and prevent reflux into the lungs
• Upper right lobe pneumonia from aspiration is a major complication
• Surgery involves closing the fistula and anastomosing the esophageal segments
• Infants must be closely observed post-operatively on days 7 to 10
• This is when the sutures dissolve and leaks can occur at anastomosis sites
• Fluid and air leakage into the chest cavity can cause pneumothorax

Omphalocele

• A protrusion of abdominal contents through the abdominal wall at the point of junction of the umbilical cord and abdomen
• The herniated organs are usually the intestines, but can include stomach and liver
• They are usually covered and contained by a thin transparent layer of peritoneum
• Occurs because, at weeks 6 to 8 of intrauterine life, fetal abdominal contents grow faster than the fetal abdomen and are extruded from the abdomen into the base of the umbilical cord
• At 7 to 10 weeks, the intestine returns to the abdomen when the abdomen has enlarged sufficiently

Omphalocele Manifestation

• Omphalocele occurs when the abdominal contents fail to return in the usual way

Omphalocele Management

• Infants have immediate surgery to replace the bowel before this peritoneal membrane surrounding it ruptures or becomes infected
• If the Omphalocele is large, infants may be prescribed a topical solution, such as silver sulfadiazine
• This prevents infection of the sac with surgical closure, called exomphalos repair

Preoperative Omphalocele

• The peritoneum lining the Omphalocele must not be ruptured or allowed to dry out and crack
• Exposure of intestine to air also causes a rapid loss of body heat
• Immediately place the baby in a warmed incubator
• Do not leave infants under a radiant heat source because the exposed bowel will dry quickly.

Omphalocele: Assessment

• May be revealed by elevated Maternal Serum Alpha-Fetoprotein (MSAFP) examination during pregnancy
• If undetected, the presence of Omphalocele is obvious on inspection at birth
• When identified in utero, Cesarean birth may be performed to protect the exposed intestine
• Document the Omphalocele’s general appearance and its size in centimeters at birth
• Keep the sac moist by covering it with either sterile saline-soaked gauze or a sterile plastic bowel bag until surgery.

Postnatal Omphalocele

• The large amount of exposed intestinal surface means saline has to be at body temperature to prevent lowering of body temperature
• A nasogastric tube is inserted at birth to prevent intestinal distention, enlarging the bowel lumen and making it more difficult to replace the bowel
• The infant should not be fed orally or allowed to suck on a pacifier until the bowel repair is complete
• Carefully observe infants for signs of obstruction (abdominal distention, constipation, diarrhea or vomiting) when they begin oral feedings.

Gastroschisis

• A condition similar to Omphalocele
• Except abdominal wall disorder is a distance from the umbilicus
• Abdominal organs are not contained by peritoneal membrane but spill freely from the abdomen
• Also, a greater amount of intestinal contents tends to herniate, increasing the potential for Volvulus and obstruction
• Surgical procedure is the same as that for Omphalocele
• Children with Gastroschisis often have decreased bowel mobility
• Even after surgical correction, they may have difficulty with absorption of nutrients and passage of stool.

Intussusception

• The invagination of one portion of the intestine into another
• Occurs in the second half of the first year of life.
• Signs and symptoms include: - Suddenly drawing up their legs and crying as if they are in severe pain - Freedom from symptoms after a peristaltic wave passes - Intense abdominal pain approximately every 15 minutes - Vomiting
  • Blood appears in the stool described as "Currant Jelly" after 12 hours.

Necrosis in Intussusception: Manifestation

• Elevated temperature
• Peritoneal irritation
• Increase White Blood Cell count
• Often rapid pulse

Intussusception Management

• Surgery to straighten the invagination portion or reduction by instillation of a water-soluble solution barium enema or air (pneumatic insufflation).

Volvulus

• A twisting of the intestine that leads to obstruction of the passage of feces
• Compromises the blood supply to the loop of intestine which is involved, usually occurs during the first 6 months of life
• Signs and symptoms include: - Intense crying and pain - Pulling up of the leg - Abdominal distention and vomiting

Diagnosing Volvulus

• Based on the history and an abdominal examination, which reveals an abdominal mass
• A sonogram or lower barium x-ray also will show the obstruction.

Managing Volvulus

• Surgery to relieve the volvulus and reattach the bowel so it is no longer free moving.

Celiac Disease

• A malabsorption sensitivity or abnormal immunologic response to the protein, gluten
• Gluten is found in grains, wheat, rye, oats and barley
• When children with Celiac Disease ingest gluten, it changes their intestinal mucosa
• This prevents the absorption of foods across the intestinal villi into the bloodstream.

Symptoms of Celiac Disease

• Steatorrhea: bulky, foul smelling, fatty stools
• Deficiency of fat soluble vitamins A, D, E, K
• Malnutrition
• Distended abdomen from fat
• Rickets: Vitamin D is not absorbed
• Hypoprothrombinemia, iron deficiency anemia, and hypoalbuminemia owing to Vitamin K deficiency
• Anorectic and irritable
• Fall behind other children their age in height and weight
• Appears skinny, with spindly extremities and wasted buttocks
• Bulky stools, malnutrition, distended abdomen and anemia (noticeable between 6 to 18 months)
• A deficiency of vitamin D or an inability to utilize vitamin D may lead to a condition called Rickets, a weakening and softening of the bones brought on by an extreme calcium loss

Diagnosing Celiac

• Can be made between 6 to 18 months after the child has started on solid foods.

Celiac Treatment

• Gluten free diet
• Administered, water-soluble forms of vitamins A and D
• Iron and Folate (to correct anemia).

Hirschsprung Disease

• Absence of ganglionic innervation to the muscles of a section of bowel
• Occurs mostly in the lower portion of the sigmoid colon just above the anus
• Lack of nerve cells means no peristalsis occurs, preventing movement of fecal material through the segment of intestine
• Generally apparent until 6 to 12 months of age

Hirschsprung Manifestation

• Chronic constipation or ribbon like stools passing through such small, narrow segments look like ribbons
• Thin and undernourished, with a large and distended abdomen.

Treatment for Hirschsprung

• Repair of a ganglionic megacolon involves dissection and removal of affected section, with anastomosis of intestine
• Generally, treated in the newborn by two-state surgery:
• 1st temporary colostomy is established
• Bowel repair at 12 to 18 months of age
• Preoperatively: For home care, help parents learn about minimal residue diets
• Omit fried and highly seasoned foods
• Give Children stool softener daily
• Postoperatively: Remove NGT (as soon as peristalsis has returned)
• Offer small, frequent feedings of fluids, such as water or gelatin, then to full fluids, a soft diet, and finally a normal diet for age
• IV infusion
• Indwelling catheter.

Gastroesophageal Reflux (Achalasia)

• A neuromuscular disturbance where the gastroesophageal sphincter and the lower portion of the esophagus and lax in structure
• Regurgitation to gastric contents is easy
• Starts 1-week post-birth and may be associated in hiatal hernia
• Achalasia: dilated gullet (esophageal body) with structure at the bottom (LES0

Symptoms of Gastroesophageal Reflux

• Regurgitation occurs immediately after feeding
• Vomiting appears effortless and non-projectile
• The infant is irritable and can experience periods of apnea.

Managing Gastroesophageal Reflux

• Feed infants a formula, thickened by rice cereal (1 tbsp cereal per 1oz of formula or breastmilk)
• Hold the infant in an upright position.
• Keep them upright via infant chair for 1 hour after feeding.

Hiatal Hernia

• The intermittent protrusion of the stomach up through the esophageal opening in the diaphragm.
• Symptoms involve periodic, painful vomiting and shortness of breath

Treating Hiatal Hernia

• Keep in an upright position to prevent recurrence
• Use medication to reduce acid secretion
• Perform laparoscopic surgery to reduce the stomach’s ability to protrude throughthe diaphragm.

Diaphragmatic Hernia

• A protrusion of an abdominal organ (usually the stomach or intestine) through the diaphragm into the chest cavity
• Diaphragmatic hernia usually occurs on the left side, causing cardiac displacement to the right of the chest and collapse of the left lung
• In early intrauterine life, the chest and abdominal cavity are one
• At approximately week 8 of growth, the diaphragm begins to form and divide the two
• If the diaphragm does not form completely, the intestine can herniate through the diaphragm opening into the chest cavity

Assessment of Diaphragmatic Hernia

• Can be detected in utero by sonogram
• More often conditions is diagnosed, however, at birth
• Newborns with diaphragmatic hernia will have respiratory difficulty from birth, as one or more lobes of lungs cannot expand completely
• Their abdomen appears sunken because it is not as filled with intestine as a normal newborn would be

Managing Diaphragmatic Hernia

• Requires emergency surgical repair of diaphragm and replacement of the herniated intestine back into the abdomen
• The surgery typically includes a thoracic incision and/or placement of a chest tube
• The infant breathes better with the head elevated
• This allows herniated intestines to fall back as far as possible into the abdomen
• Maximizes respiratory space into chest..
• Other treatments include - Insertion of a nasogastric tube or gastrotomy tube to prevent distention of the herniated intestine. - Keeping infant NPO to reduce filling of bowel
  • Performing surgery while maintaining infant in a semi Fowler position to alleviate intra-abdominal pressure on diaphragm.

Umbilical Hernia

• A protrusion of a portion of the intestine through the umbilical ring, muscles and fascia surrounding the umbilical cord.

Umbilical Hernia: Manifestation

• Creates a bulging protrusion under the skin at the umbilicus
• Umbilical Hernias rarely be noticeable at birth (when the cord is still present) but become increasingly noticeable by health care visits. Structure is commonly a 1-2cm diameter

Features of Umbilical Hernias

• Occurs more often in girls than boys
• Structure is generally 1 to 2 cm in diameter but may be as big as an orange when children cry or strain
• If this fascia can ring is less than 2com, then closure will happen spontaneously, without repair

Managing Umbilical Hernias

• Surgery is indicated for disorders greater than 2 cm .
• Silver Dollar taping should be avoided as can cause bowel strangulation.
• Post Surgical, dressing kept in place to protect healing. Diapers should be kept folded so as not to irritate incisions.

Inguinal Hernia

• The protrusion of a section of the bowel into the inguinal ring, occurring more commony in boys
• The fold of parietal peritoneum also descends, forming a tube from the abdomen to the scrotum
• Commontypes include: -Umbilical hernia -Inguinal hernia -Femoral hernia

Inguinal Hernias: Manifestation

• Appears as a limp in the left or right groin.
• Only apparent when child is crying or when less active
• Painless