Before and after fistula repair

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

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Neonatal Surgery

Neonatal Surgery
By:Juan E Gonzalez, CRNA, MS, ARNP
Based on prior lecture by
John P. McDonough, CRNA, Ed.D., ARNP
Professor & Director
Anesthesiology Nursing

Anatomical Differences
Pedi vs. Adult Airway
More Vertical
Less vertical
R mainstem bronchus
V-shaped
U-shaped
Shape of Epiglottis
Glottis
Cricoid
Narrowest Point
C3-C6
C2-C4
Laryngeal location

Head Position
Visual Alignment of Oral/Pharyngeal/Laryngeal axes
Attempt to achieve “sniffing” position will OBSTRUCT pt
Intubation
To intubate or not to intubate…
Is that a question??
Choice of Intubating Technique
Choice of Intubating Technique
(patient factors)
Blood Pressure Control
Emergence
Surgeries in the First Week of Life
* Congenital Diaphragmatic Hernia (CDH)
* Omphalocele & Gastroschisis
* Tracheoesophageal Fistula (TEF) (hrs-days to diagnose)
* Intestinal Obstruction (hr-days to diagnose)
* Meningomyelocele
Confounding Factors
* Prematurity
* Associated Congenital Anomalies
Maternal Cocaine Use in Pregnancy
Congenital Diaphragmatic Hernia
Embriologic features of CDH
CDH scenarios
CDH Clinical Presentations
Surgery & CDH
Anesthesia & CDH
Omphalocele
Gastroschisis
Delivery Room Management of Gastroschisis
Gastroschisis Periop Concerns
Postop Care of Gastroschisis & Omphalocele
Tracheoesophageal Fistula (TEF)
TEF Clinical Presentation
TEF Anesthetic Considerations
Tracheoesophogeal Fistula
“VATER” syndrome
Vertebral defects
Anal atresia
TEF
Esophageal atresia
Renal dysplasia
Intestinal Obstruction (Upper GI obstruction)
Intestinal Obstruction (Lower GI obstruction)
Lower GI obstruction
Meningomyelocele
Meningomyelocele
Hydrocephalus
Hydrocephalus Anesthetic Approach
Surgical Procedures in the First Month of Life
Inguinal Hernia Repair (IHR)
Inguinal Hernia Repair Anesthetic Techniques
Inguinal Hernia Repair Post Op Apnea in Premies
Ligation of PDA
Placement of Central Venous Catheter
References

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Abdominal Wall Defects: Omphalocele vs. Gastroschisis

Abdominal Wall Defects: Omphalocele vs. Gastroschisis
By:Joanna Thomson,
Surgery Clerkship

Embryology Review
* The Midgut gives rise to:
o Duodenum distal to the bile duct
o Jejunum
o Ileum
o Cecum
o Appendix
o Ascending colon
o Hepatic flexure of the colon
o Proximal two-thirds of transverse colon.

Physiological Umbilical Herniation
* As a result of rapid growth and expansion of the liver, the abdominal cavity temporarily becomes too small to contain all the intestinal loops.
* The intestinal loops enter the extraembyronic cavity within the umbilical cord during the sixth week of development.
* As herniation occurs, the loop undergoes a 90 degree counterclockwise rotation around the superior mesenteric artery.

Return to Abdominal Cavity
* During 10th week of development, herniated intestinal loops begin to return to the abdominal cavity.
* Undergoes additional 180 degree counterclockwise rotation about the superior mesenteric artery.
* Factors responsible for this return are not precisely known... It is thought that regression of the mesonephros (kidney), reduced growth of the liver, and expansion of the abdominal cavity all play roles.

Omphalocele
* Herniation of abdominal viscera through an enlarged umbilical ring.
o Failure of the bowel to return to the body cavity following physiological umbilical herniation. Defective mesodermal growth causes incomplete central fusion and persistent herniation of the midgut.
* Extruded viscera may include LIVER, small and large intestines, stomach, spleen, or bladder.
* Covered by amnion and peritoneum

Gastroschisis
* Herniation of intestinal loops through the anterior abdominal wall.
* Defect lateral to the umbilicus (right>left)
o Abnormal involution of the right umbilical vein or vascular accident involving the omphalomesenteric artery causes localized abdominal wall weakness.
* No sac covers the extruded viscera.

Prenatal Diagnosis
* Elevated maternal serum alpha fetoprotein
* Ultrasound
Omphalocele Gastroschisis

Epidemiology
* Prevalence:
o Omphalocele: 1/5,000 births
o Gastroschisis: 1/10,000 births
+ Increasing in frequency, especially in young women.
* Mortality:
o Omphalocele: 25%
+ Related directly to presence of chromosomal and other abnormalities
o Gastroschisis: <5%

Omphalocele Associated Anomalies
* Chromosomal abnormalities (50%)
* Neural tube defects (40%)
* Beckwith-Wiedemann syndrome
* Pentalogy of Cantrell

Gastroschisis Associated Anomalies
* Additional gastrointestinal problems
Initial Management
* Acute management aimed at maintaining circulation to bowel and preventing infection while stabilizing infant (temperature/fluids) :
o Cover the defect with sterile dressing soaked in warm saline to prevent fluid loss
o Nasogastric decompression
o IV fluids with glucose
o Antibiotics

Surgical Treatment
* Surgery performed to return the viscera to the abdominal cavity and close the defect.
o Primary Surgical Closure: Success dependent on size of the defect and size of the abdominal and thoracic cavities.

o Staged Closure: Gradual reduction of the contents into the abdominal cavity using an extra-abdominal extension of the peritoneal cavity (termed a silo) and using gentle pressure. Usually requires 1-3 weeks, after which the defect is then primarily closed.

Abdominal Wall Defects.ppt

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