Before and after fistula repair
Tracheoesohpageal fistula
An omphalocele is an abdominal wall defect at the base of the umbilical cord (umbilicus); the infant is born with sac protruding through the defect which contains small intestine, liver, and large intestine.
Gastroschisis:
Surgical repair of abdominal wall defects involves replacing the abdominal organs back into the abdomen through the abdominal wall defect, repairing the defect if possible, or creating a sterile pouch to protect the intestines while they are gradually pushed back into the abdomen.
Anorectal malformations; Anal atresia
Imperforate anus is a relatively common congenital malformation that occurs in about 1 out of 5,000 infants.
signs & symptoms
o absence of anal opening
o misplaced anal opening
o anal opening very near the vaginal opening in the female
o no passage of first stool within 24 to 48 hours after birth
o stool passed by way of vagina or urethra
o Abdomianl distention
o vomiting if infant is fed
o Asymmetry in leg positions
o Asymmetry of the thigh fat folds
o After 3 months of age, asymmetry of rotation of the leg and apparent shortening of the affected leg
o Diminished movement in the affected side
Signs & tests
Pediatricians routinely screen all newborns and infants for hip dysplasia. There are several maneuvers that can detect a dislocated hip or a hip that is able to be dislocated. A hip that is truly dislocated in an infant should be picked up but some cases are subtle and some develop after birth, which is why multiple examinations are recommended. Some mild cases are "silent"; and cannot be picked up on physical exam.
Ultrasound of the hip is the most important imaging study and will demonstrate hip deformity. A hip X-ray joint X-ray) is helpful in older infants and children.
Treatment
In early infancy, positioning with a device to keep the legs apart and turned outward (frog-leg position) will usually hold the femoral head in the socket. If there is difficulty in maintaining proper position, a plaster cast may be applied and changed periodically to accommodate growth. Operative management may be necessary if early measures to reduce the joint (put the joint back in place) are unsuccessful, or if the defect is first detected in an older child.
Prognosis
If the dysplasia is picked up in the first few months of life, it can almost always be treated successfully with bracing. In a few cases surgery is necessary to put the hip back in joint.The older the age at diagnosis, the worse the outcome and the bigger the surgery needed to repair the problem.
Talipes equinovarus; Talipes or Clubfoot
Signs & Symtptoms:
The physical appearance of the deformity may vary.
Treatment should be initiated as early as possible.
Polydactyly
Fingers or toes (digits) may be fused together (syndactyly) or the webbing between them (inter-digital webbing) may extend far up the digits. Syndactyly is seen commonly between the 2nd and 3rd toes, and is usually associated with a syndrome.
Signs & symptoms
Treatment
Epispadias
Malignant teratoma
Inborn errors of metabolism
TYPES & OTHER NAMES:
Galactosemia - nutritional considerations; Fructose intolerance - nutritional considerations; Maple sugar urine disease (MSUD) - nutritional considerations; Phenylketonuria (PKU) - nutritional considerations; Branched chain ketoaciduria - nutritional considerations
DEFINATION:
Genetic disorders (numbering in the dozens) in which the body cannot metabolize food components normally. These disorders usually involve minute changes in the breakdown of proteins, fats and carbohydrates. The body can become malnourished even when eating a well-balanced diet. See also galactosemia, PKU, lactose intolerance, and maple syrup urine disease
RECOMMENDATION
Inborn errors of metabolism often demand diet changes. The type and extent of the changes depends the specific metabolic error. Registered dietitians and physicians can help with the diet modifications needed for each disease.
Symptoms
o jaundice (yellowish discoloration of the skin and the whites of the eyes)
o vomiting
o poor feedig
o poor weight gain
o lethargy
o irritability
o convulsions
o pupil, white spots
Signs & tests
o hepatomegaly (enlarged liver)
o hypoglycemia (low blood sugar)
o aminoaciduria (amino acids are present in the urine)
o cirrhosis
o ascites (fluid collects in the abdomen)
o mental retardation
o cataract formation
Galactosemia
PRGNOSIS:
Phenylketonuria
Causes & risks
SIGNS & SYMPTOMS OF PKU
Trisomy 18 also known as Edwards syndrome
SIGNS & SYMPTOMS OF EDWARDS
Down syndrome; Trisomy 21; Mongolism
SIGNS & SYMPTOMS OF DOWNS
Common autosomal aberrations
Before and after fistula repair.ppt
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