what is a cleft lip?

congenital fissure in the upper lip

what is a cleft palate?

congenital fissure in either the soft palate alone or in both hard and soft palate

T/F: cleft lip and palate is the most common cranial malformation in children


when/how does a cleft lip occur?

at 6 weeks when maxillary process fails to merge with the medial nasal elevation on one or both sides

when/how does a cleft palate occur?

occurs 7-12 weeks when the lateral palatine processes fail to meet and fuse with each other, the primary palate or the nasal septum

what are factors that affect cleft lip/palate?

-maternal nutrition (folic acid deficiency)
-maternal medication in 1st trimester
-occurrence of fever and influenza in 1st trimester
-maternal prenatal cig smoking

how is a cleft lip/palate diagnosed?

-generally evident at birth
-feeding difficulties, chronic upper airway congestion/infection
-palpating palate part of newborn exam
-7-14% of infants with CL/CP have other anomalies at birth

what is the goal of cleft lip/palate repair?

normal speech, normal palate and facial growth, normal dentition

when should you ideally do a cleft lip/palate repair?

before speech develops

what are the pre-operative nursing interventions for cleft lip/palate?

Nutrition less than body requirements:
-Elevate head of bed during feeds
-Bottle: Utilize special nipples
-Gentle steady pressure on base of the bottle
-Frequents burping Special equipment: Breck feeder
Daily weights

T/F: feeding can be very difficult for an infant with cleft palate


what are the post-operative cleft lip/palate interventions?

-maintain patent airway HOB elevated
-assess for infection, bleeding, resp. distress
-protect suture line
-keep infant calm
-use elbow restrains
-support parents by accepting and treating infant as normal

why do we not breastfeed after surgery?

we have to protect the suture line and decrease crying to protect sutures

T/F: do we need to manage pain postoperatively for an infant after cleft lip/palate repair?


what is the postoperative diet for infants?

IVF and/or PO
start with clear liquids, advance as tolerated

what type of feeder can we use to feed infants with a suture line after surgery?

Brekk feeder - soft catheter goes in mouth instead of nipple
no pressure on suture line

what are the long term considerations after cleft lip/palate repair?

-language and speech (residual speech impairment)
-hearing impairment (frequent otitis media, hearing screens)
-dental problems
-social adjustment and self image

what are the psychosocial implications with cleft lip/palate?

-parental guilt/grief
-loss of expectation
-cultural beliefs
-acceptance/ reaction of others

What is a tracheoesophageal fistula/esophageal atresia?

failure of trachea to differentiate and separate from esophagus into separate distinct structures
failure of esophagus to develop as a continuous passage

describe the anatomy of a TE fistula?

there is a fistula (connection) between trachea and esophagus that connects to the stomach

what is TEF/esophageal atresia commonly seen in addition to?

cardiac anomalies
30-40% affected individuals

what are the risk factors with TEF/Esophageal Atresia?

-no definitive cause
-male = female
-increase in low birth weight
-increase in prematurity

what is polyhydramnios?

excessive fluid around the fetus

how does a baby with TEF/Esophageal Atresia present?

-inability to swallow saliva
-gastric tube/suction catheter cannot be passed!!!!!!!
-choking or cyanosis with feedings
-recurrent pneumonia
-triad of excessive secretions, reflux, respiratory distress
-frothy saliva in mouth/nose
-may become cyanotic or a

what is the classic triad of presenting symptoms with TEF/Esophegeal Atresia?

drooling, reflux, resp. distress
what is swallowed cannot go anywhere bc of blind pouch

when is it ideal to diagnose TEF/Esophageal Atresia?

before first feeding

how was TEF/Esophageal Atresia diagnosed?

X ray after passing radiopaque catheter into esophagus
do this after cannot pass an NG tube

what is the treatment for TEF/Esophageal Atresia?

surgical correction - sometimes requires staged repair - can't all be done in one surgery
aggressive surgery!!!!! create separate continuous structures

what is the prognosis for TEF/Esophageal Atresia?

100% in otherwise healthy children

what are pre-operative interventions for TEF/Esophageal Atresia?

-monitor resp. assessment
-position HOB UP significantly to decrease chance of aspirating stomach contents
-frequent suctioning to keep blind pouch empty
-NPO, IVF hydration
-gastric decompression

what are post-operative interventions for TEF/Esophageal Atresia?

-airway management (surgery on airway)
-thermoregulation (open radiant warmer)
-gastric decompression (to low wall suction)
-suction: with extreme caution - may be interfering with new surgical site

what are other post-operative for TEF/Esophageal Atresia?

-chest tube
-pain control (pharm and non-pharm)
-comfort (touch, eye contact)
(g-tube feeds started slowly, oral feeds started slowly after healing from surgery)

are feeding difficulties common after TE esophageal atresia repair?

yes!!!! begin G-tube feedings slowly, then PO feeds

what is a hernia?

a protrusion of organ through an abnormal opening; become dangerous when constriction leads to impaired circulation

when do abdominal wall defects occur?

3rd week gestation


herniation of abdominal contents thru umbilical ring; intact peritoneal sac


herniation of abdominal contents lateral to umbilical ring; no peritoneal sac

what do omphalocele and gastroschisis have in common? what is the difference?

both are herniation of abdominal contents; start around 3rd week of gestation
omphalocele: thru umbilical ring, intact peritoneal sac
gastroschisis: lateral to umbilical ring, no peritoneal sac

what is the therapeutic management of gastroschisis or omphalocele?

-cover with moist (saline soaked) sac with sterile wrap
-IVF and antibiotics
-surgery closure: contents reduced into abdomen and abdominal wall closed
-silo: used when a staged repair is needed

why is thermoregulation a big deal in management?

we have to provide a heat source to keep baby warm bc it takes energy - we do not want them to expend any extra energy!!!!!

what are main goals for therapeutic management for gastroschisis or omphalocele?

prevent infection
prevent fluid loss - watch for drying out of the sac
go to surgery quickly!

what is the goal of surgical repair for omphalocele or gastrochisis? when should you do the surgery?

reduce contents into abdomen and close abdominal wall
typically in 1st 24 hours of life
ideally in 1 repair but they have a narrow, scaphoid abdomen so may need more

if we are not able to reduce contents in one surgery, what do we typically use in the next surgery?
what do we need to monitor during this procedure?

silo - sterile intact tent, intestines gathered and push intestines back into abdomen
watch for respiratory distress!!!!! to determine how quickly we can reduce this

what are the pre-operative nursing considerations for gastrochisis or omphalocele?

-infection ppx (IV antibiotics)
-fluid management (risk of fluid loss)
-NG tube for decompression
-TPN/lipids (nutrition)
-family support

what are post-operative nursing considerations for gastrochisis or omphalocele?

assess resp. status s/t increased abdominal pressure
assess bowel function
TPN/IL s/t prolonged ileus (nutritional support)

what is an umbilical hernia?

Incomplete fusion of umbilical ring where umbilical vessels leave abdominal wall
Most common type of hernia!!!!!

describe the incidence of umbilical hernia?

-AA > Caucasians
-LBW/premature > full term
-usually an isolated defect

T/F: complications are common with umbilical hernias

FASLE - they are rare - only if there is incarceration of hernia

when should surgeries be performed for umbilical hernias?

if still present at 2-3 years
usually resolves on its own

T/F: you can grow and thrive without a repair of an umbilical hernia

true - reassure family that they are common and will usually resolve on own

what is a congenital diaphragmatic hernia?

Diaphragm not completely formed allowing intestines and/or organs to enter thoracic cavity

how is the heart affected by a diaphragmatic hernia?

intestinal contents in chest wall - compromised lung growth and expansion
the heart has been pushed over

T/F: diaphragmatic hernia can be diagnosed prenatally

true - using a prenatal anatomical scan

what is the manifestation of CDH?

-acute respiratory distress in newborn (dyspnea & cyanosis)
-scaphoid abdomen
-decreased cardiac output to shock

what are pre-natal signs/presentation on scan for CDH?

mediastinum shift
loops of bowel in the chest (typically left chest)

describe the post-birth management for congenital diaphragmatic hernia?

-respiratory support (no bag mask ventilation - could cause air in stomach, but can use blow by or intubation)
-GI decompression via NGT
-HOB elevated
surgical repair: early repair to return abdominal contents to abdomen and repair defect

why do we never bag valve mask a child with congenital diaphragmatic hernia?

can get air into GI system

what things should you look for in a newborn assessment to examine if we don't know if they have congenital diaphragmatic hernia? if known?

-respiratory distress that does not respond to NRP resuscitation
-if known CDH, intubate to prevent air getting into intestines

what are post-operative considerations for CDH?

-close observation for respiratory distress, infection, respiratory support
-gastric decompression- TPN/lipids
-manage pain, comfort, decrease stimulation and decrease stress
-developmental needs of newborn

what is an inguinal hernia?

protrusion of small intestines/fluid into groin through a weakness or tear in the abdominal wall

describe the incidence of inguinal hernia

males > females
premie > term

what does an inguinal hernia look like?

mass in groin, can present as painless inguinal swelling (can be painful)
they will cry/scream if painful

why would an inguinal hernia be painful?

incarcerated hernia - cutting off blood supply/O2 to bowel - causing build up
there will be extreme pain and crying/screaming

when will sac manifest with an inguinal hernia?

sac is present at birth (undescended testicles get stuck in scrotum)
-there is a female pathway also
may not manifest until 2-3 months!!!!!!

what is the management of inguinal hernia?

surgical repair - sooner rather than later due to risk of incarceration
usually bilateral
left testicle descends before right - common to see it in right

what should you teach parents of child with incarcerated, painful inguinal hernia?

teach them how to reduce it
-get them in warm bath, to calm them, relax them, then push it in
if reduced, then we do not have to go surgery now, can wait

what happens if you are unable to reduce an inguinal hernia?

you have to go into surgery immediately

how can you tell the difference between an inguinal hernia and an undescended testicle?

evaluate the scrotum
if you can feel testicle in the scrotum then you know that bulge is inguinal hernia
would see under-developed scrotum if it was undescended testicle

what should you do pre-operatively and post-operatively for inguinal hernia?

-manage pain/educate parents
-monitor the wound
-look for s/sx infection
-manage pain
-decrease activity

what is Hirschsprung's disease???

congenital anomaly that results in mechanical obstruction from inadequate motility in part of the intestine
(large intestines absorb water and bulk up stool)

what does the colon look like in a person with HD? how does it happen?

portion of descending colon that has no nerves and no peristalsis, causes back up proximal to aganglionic portion and now we have a megacolon!!!!!!

describe the incidence of HD

account for 1/4 of all cases of neonatal obstruction

how does HD present in a newborn?

-abdominal distention
-no passage of meconium in first 48 hours
-respiratory distress
-presents as acute, life threatening event

what are the signs of HD we can see in a older infant/child?

smaller portion of children present in childhood
-FTT (failure to thrive)
-chronic constipation,
****foul smelling "ribbon like" stools
-abdominal distention

what do you need to confirm diagnosis of HD?

rectal biopsy - see no nerves

what is the therapeutic management of HD?

3 stages of surgery are required

what is stage 1 of HD surgery?

surgical removal of aganglionic portion of colon
give them temporary colostomy

what is stage 2 of HD surgery?

(8 months-1 year later or weigh approximately 20 lbs)-
pull through - Involves "pulling" the end of the intact bowel down to a part of the rectum for re-anastomosis
still have colostomy

what is stage 3 of HD surgery?

closure of colostomy (if performed) usually 3 months after stage II surgery

what can you do for patients with HD who have mild symptoms? very small portion of population

conservative therapy for chronic constipation
-occasional enemas
-develop regular pattern of defecation
-at continued risk for development of enterocolitis

what is pre-operative care for HD surgery?

-NG placement
-Strict I & O (meconium passage)
-Measurement of abdominal girth
-vital signs!!!!
quick colostomy training for parents

what is post-operative care for HD surgery?

-IV fluid maintenance
-dressing changes
-foley care
-discharge education

what are the GI obstructive disorders?

1. hypertrophic pyloric stenosis
2. intussusception

what is the manifestation of an acute intestinal obstruction?

1. abdominal pain
2. distension
3. N/V
4. change in stool

what is pyloric stenosis?

A narrowing of the pyloric sphincter at the outlet of the stomach, obstructing the flow of food into the small intestines

who commonly gets pyloric stenosis?

1st born caucasian males

what causes pyloric stenosis?

muscle of pyloric sphincter becomes thick and elongated, narrowing the pyloric channel

what are the clinical manifestations/presentation of pyloric stenosis?

-early nonbilious vomiting beginning around 3 weeks of age, progressing to projectile vomiting
-olive shaped mass in epigastrum
-we will see dehydration, weight loss (baggy skin), metabolic alkalosis
-will be ravenously hungry and losing weight (thin)

how is the diagnosis of pyloric stenosis made?

-made by history and PE
-possible metabolic alkalosis and severe volume depletion

when do we treat pyloric stenosis?

we send them immediately to surgery- pyloromyotomy
we need to correct metabolic alkalosis!!!!!

what is the surgical repair of pyloric stenosis called?


what are pre-operatively considerations of pyloric stenosis?

-correct metabolic alkalosis

what are the post-operative considerations for pyloric stenosis?

-start to feed 4-6 hours post op
-vomiting post-op is not uncommon (educate)
-pain management

what is intussusception?

telescoping of bowel upon itself
most common cause of intestinal obstruction in children younger than 3

what age groups get intussusception? more common in girls or boys? risks?

3 months to 3 years
have a prior intussusception puts you at risk for future

where in bowel does intussusception most commonly occur?

right abdomen

what is the acute triad of intusseception?*******

1) pain - sudden onset of crampy abdominal pain
2) sausage shaped abdominal mass
3) currant jelly stool (bloody mucus)

what are the chronic manifestations of intusseception?

diarrhea, anorexia, weight loss, vomiting, periodic pain
once you have it, you are predisposed to have it again

how is intusseception diagnosed?

diagnosis and treatment are 1 in the same
barium enema put in through rectum and hydrostatic pressure un-telescopes the bowel

if intusseception does not resolves after barium enema, what do you do? if it is not treated and causes death to part of the bowel, it may require what?

reduction via surgical manipulation
may require surgical resection of dead part

T/F: intusseception is fatal without treatment


what is celiac disease?

Malabsorption syndrome: second to CF in cause of malabsorption in children
Permanent intestinal intolerance to dietary wheat and related proteins that produce mucosal lesions in genetically susceptible individuals

what are symptoms of celiac disease?

diarrhea, FTT, abdominal distention, anorexia, irritability

how is a diagnosis of celiac disease made?

withdraw gluten from diet - have full remission of symptoms

what is the treatment of celiac disease?

dietary management

what are the clinical manifestations of GER?

common - cardiac sphincter cannot keep food in stomach bc it is underdeveloped - stomach contents is going back into esophagus

what is parent education/treatment for GER?

-burp often
-small feeds more frequently
-sit them up 30 mins after they eat
they could use medication, pay attention to if it progresses!!!!!!!

what are the clinical manifestations of GERD? gastroesophageal reflux disease

-FTT!!!!! not meeting growth milestones
-anorexia, irritability
-recurrent pneumonia - aspirate into lungs

kids with recurrent pneumonia r/t GERD often have co-morbid _______________________________

neurological disorder

how do you make GERD diagnosis? treatment?

barium swallow study
pH probe - check pH
may need surgical correction - nissen fundoplication

explain nissen fundoplication for GERD?

fundus of stomach becomes backup to cardiac sphincter
often have to have a G tube also