if lip fails to close. can be unilater or bilateral. the extent varies.

Dental animolies are common. Missing/deformed teeth common on the side of the cleft.

Cleft can be complete both through bone and tissue.

Bone structure but not overlying mucous membranes.

It looks normal

have to do newborn exam to tell.

Normally palate closes by 10th week gestation. Unknown causes.

Combo of genetic and environmental influences. Linked to folic acid levels, maternal alcohol ingestion, and smoking.

DX: lip-visual and ultrasound

palate-inspection/exam to palpate

S/SX:newborn has trouble sucking. no adequate suction. at risk for choking.

Surgical. Do Lip closure first usually.

Some surgeons do surgery within a couple days of birth. Usually do surgery within 3 months at the latest.

Closure of palate is done in stages. It's multiple surgeries with multiple specialists (plastic surgeon, dentists, orthodontists)

They're prone to ear infections.

Goal is to prevent trauma and infection of suture line whether it's lip/palate.

Have feeding adaptions

Careful positioning.

NEVER on abdomen. May use elbow restraints.

Logan barre-device to reduce tension on suture line for lip.

Might need scar revisions as they grow.

Abscence of normal body opening

Or abnormal closure of body passage.

Esphogaus ends in blind pouch (normally ends in stomach)

Connecting trachea and espohagus. (so air and fluids are mixing)

And in some cases there's both a fistula and atresia.

Constant secretions, lots of respiratory distress, cyanosis, choking, distention, bloated (air gets into stomach)

DX: NG tube stops (normally it'd go into stomach)

TX: prevent resp complications until surgery can be performed. Suctioning and no oral feeds.

If no other anamolies surgery will be performed within first few days of life.

Gastronomy feed if have to wait for surgery.

Once fixed normal growth and development should occur.

Obstruction of pyloric canal as a result of thickening of plyoric sphincter and narrowing of passage between stomach and duodenum.

The pylorus also elongates.

Can cause total obstruction and dilation of stomach.

Rarely seen until 2-4th week of life.

As child continues to take fluids they will start to vomit after feeds to point of projectile.

They'll become irritable, hungry, wt loss, dehydration. They're born with it, but symptoms don't present at birth.

In RUQ they will feel round mass, you can see peristaltic waves.

What's their hx? Are they throwing up? 

Palpate for mass.

Do xray to confirm or ultrasound.

Pyloromyotomy-aka Fredet Ramstedt procedure circular muscle fibers or pylorus is cut to widen the canal.

NPO, IVS, surgery then 4-6hrs after normal liquids then progress, surgical incision care, analgesics, I&O

Relaxation of cardiac sphincter.

Allows gastric contents to "reflux" up into esophagus.

More common in premies or child with neurological impairment, but can affect full term babies as well.

S/SX: Spitting up with feeds, irritation of esophagus, infant (irritable) older (heartburn), indigestion, abdominal pain, chest pain, with infants if bad will develop failure to thrive. Usually resolves in about 18 months b/c sphincter matures.

HX, feeding patterns, growth patterns

Endoscopy, barium swallow

Changing formula, adding rice to thicken formula

Small freq feeds, don't lay flat after feeds

Surgery (rare)

Kid usually grows out of it

Baby can sleep on stomach (exception!)

Teach parents about respiratory distress

Aka Congenital Aganglionic megacolon (without)

Autonomic paraympathetic ganglia in muscular wall of intestine are absent.

Leads to persistent constipation/obstruction. 

Usually shows early in life, but may not until teens.

In this case internal sphincter does not relax so constipation occurs. Everything's backed up. Area distended with feces and gas.

Common with down syndrome.

Newborn: failure to pass meconium, refusing to suck, abdominal distention, bile stained or meconium emesis

Older child: HX of abdominal distention, chronic constipation with passage of ribbon like foul smelling stools or pellet stools.

Barium studies

Rectal biopsy, which confirms absence of nerve cells.

Surgery to remove aganglionic portion of bowel to relieve obstruction. May be done in stages.

May need temporary colostomy to let the other parts of intestine rest. (depends on how bad megacolon gets)

Prior to surgery: IVs, TPN

Post OP: I&O, wound care, fluids

Proximal segment of bowel telescopes into more distal segment.

Cause of intestional obstruction in child.

Usually around 3 months to 3 years.

Most common site: ileocecal valve-the ileum slips into cecum pulling the mesentary with it.

The walls of intestine rub causing inflammation and obstruction leading to ischemia, necrosis, perforation, and hemmorrhage.

Sudden severe abdominal pain

Vomitting, palpable mass in abdomen

Currant jelly stools

Blood and mucous 

Can be life threatening! (shock symptoms!)

based on symptoms, may do barium enema

This puts pressure on bowel making it go into normal postion

Barium enema may reduce telescoping due to pressure it puts on bowel.

May require surgery to reduce telescoping.

Keep kid 24-48hrs, doesn't usually reoccur, just make sure they're stable.

Toxic levels of lead in the blood

Lead based paint is primary source.

Children absorb and retain more lead in proportion to body wt.

Lead interferes with normal cell function particulary nervous system.

Can affect also brain, blood cells, kidneys, vitamin D, calcium, metabolium.

Once in body (through GI tract) it gets deposited in bones and teeth and is slowly released.

Will take awhile for toxic levels to be normal, because still in body.

Early: Irritability, hyperactive, poor concentration, aggression, impulsiveness

May also develop learning disabilities, hearing impairment, and growth delays.

Can lead to encephalopathy or degenerative disease of brain.

VERY SERIOUS PERMANENT CONSEQUENCES IF NOT RESOLVED.

DX: Blood test to measure lead levels.

TX: Chelation therapy give meds to bind/pull lead out. Helps increase rate of excretion.

Doctor prescribes based on individuals blood test.

Early prognosis is encouraging but not certain. Follow ups for long period of time. Find and remove source!

Viral or bacterial inflammation of intestinal tract.

Diarrhea or vomitting can lead to dehydration

TX: to stop diarrhea including antibiotics if bacterical.

REHYDRATE!

Disorder affecting body's ability to use protein

Child lacks liver enzyme, Phenlalanine hyadroxlyaze needed to convert phenylalanine into tyrosine.

Can lead to mental retardation.

Test done on newborns, must be treated.

Have to have had milk before test b/c they need protein in system.

Malabsorption syndrome.

Child unable to digest gluten. (protein found in wheat, barley, oats, and rye)

Doesn't appear until foods ingested. Leads to villi being destroyed.

Experience abdominal distention, vomitting, diarrhea, can develop organic failure to thrive, 

Gluten free diet required.

Prescence of gluten

(In celiac disease pt)

Causes immune system damage/destroys villi

Absorption will then not take place.

No wheat, rye, barley, oats for life, (ketchups, mayo, soups all contain gluten)

Life long diet. 

At risk for growth impairment and possibly GI cancer as an adult.

When uretheral opening is along surface of penis isntead of the tip.

Commonly seen with chordee-ventral curvature of penis.

DX: at birth, sometimes ultrasound

Surgical: extend urethra in some cases.

6-18 months most common time. Often several stages.

When infant presents will not circumsize at birth because will use skin as part of repairing process.

Urerethral stints often placed.

Urethral opening on dorsal or upper surface of penis.

Often seen with extrophy of bladder (when bladder is on external surface of abdomen)

Surgery done immediately within 48hours if extrophy!

Cryptorchidism

Congenital Disorder

Undescended Testicle(s)

If not corrected can result in sterility.

S/SX: newborn exam palpate scrotum can be unilateral/bilateral.

TX: wait and see sometimes they descend on their own. Administer HCG to facilitate descent. 

Will do orichiopexy by age 2 (surgery)

Can carry increased risk of testicular cancer.

Wilm's Tumor/Nephroblastoma

Noncongenital Disorder

Highly metastatic cancerous tumor in kidney region.

Usually detected in children 2-5 yrs old.

Thought to be genetic link. Thought to come from bits of leftover embryonic tissue.

May be none. Mass detected on routine exam.

Hematuria and HTN. Swelling and mass within abdomen.

Abdomen MUST NOT be palpated once mass is discovered b/c it can rupture.

Mass will be one sided, firm, non tender initially.

Ultrasound, CT, MRI

Will evaluate for metastasis to liver, lungs, and bone.

Surgery followed by chemo within 48hrs of diagnosis.

Removed kidney and any affected lymph nodes.

Also radiation if larage tumor or extensive metastasizes.

Watch I&O, fluid and electrolyte balance

Offer plenty of emotional support.

Clinical state characterized by edema, proteinuria, hypoalbuminemia, hyperlipidemia, altered immunity.

Two kinds:

Primary & Secondary

AKA minimal change nephrotic syndrome (MCNS)

Affects ONLY the kidneys

And frequently follows infection such as pyleonephritis.

Result/tied in with a systemic illness.

More involved often with lupus/diabetes/sickle cell anemia.

Is altered in both primary & secondary nephrotic syndrome.

As a result of alteration albumin moves from blood to urine (not good!) and results in hypoalbuminemia and proteinurea.

This changes osmotic pressure of blood and as a result kidneys start absorbing sodium and water and causes edema.

The low osmotic pressure stimulates the liver to make lipoproteins leading to the hyperlipidemia.

Edema-periorbital, lower extrem, scrotum, abdomen (can lead to ascites) all happens gradually

HTN & anorexia, irritability

Possible respiratory distress

Increased suceptibility to infection.

Urine anaysis (U/A) 

Albumin levels will be high and protein in urine

Possible renal biopsy

Chronic disorder that can last from 12-18months.

Tend to have periods of remissions and exacerbations.

Prone to getting it again if it's secondary.

Corticosterioids to reduce edema.

Cytoxan (chemo drug) helps to promote long term remission, but compromises immunity even more.

Diuretics reduce edema. Good skin care.

Frequent urine testing. I&O. Low sodium diet.

Inflammation of glomeruli and nephrons of the kidneys.

Thought to be an allergic reaction to certain infection most commonly group A beta strep.

Antigen antibody reaction that blocks to glomeruli allowing RBCs and protein to get into urine (oh no!)

1-3 weeks after strep infection

Hematuria, tea colored urine

Oliguria, proteinuria

Periorbital edema, anorexia, h/a

malaise, fever, irritability

sometimes a slightly elevated BP

DX: Based on U/A and look to see if they had a strep infection.

TX: bedrest, antibiotics, diuretics, I&O, wts, urine testing, low sodium diets

Usually resolves completely. There's potential for this to be a chronic form if s/sx persist for more than a year.