Neonatal Drug Withdrawal

Excellent article on Neonatal Drug Withdrawal found here.

CUTANEOUS LESIONS OF THE BACK

EVALUATION OF CUTANEOUS LESIONS OF THE BACK

The significance of cutaneous spinal lesions is related to there association with spinal dysraphism. This is defined as an absent or incomplete fusion of the bony spinal column. Spina bifida aperta are posterior protrusions of neural tissue such as meningomyeloceles. Occult spinal dysraphism including meningoceles and tethered cords may not be recognized on physical examination and diagnostic imaging may be necessary to detect them. Cutaneous lesions may be predictors of these occult malformations and progressive neurologic deficits may be preventable by early diagnosis and neurosurgical correction. An important part of the neworn examination is the inspection of the lumosacral region and buttocks.

Midline lumbosacral skin lesions
1. Tufts of hair
2. Lipomas. These are soft subcutaneous masses
3. Dimples. Dimples below the intergluteal crease end blindly and are not connected to the spinal cord. Some above the crease have tracts leading to the spinal cord and may lead to the development of meningitis with unusual organisms, neurologic deficits, and tethering of the cord. All patients with meningitis should have a thorough inspection of their backs.
4. Hemangiomas
5. Teleangectasias
6. Skin hypertrophy or atrophy
7. Hyperpigmentation and hypopigmention of the skin
8. Skin tags

Tethered Cords
Greater than 50% of patients with tethered cords have an isolated cutaneous lower back lesion. Tethered cords are often asymptomatic until the child is ambulatory or presents with neurologic deficits. At birth, the conus is at the L-3 level and reaches the adult level ( L1-L2) at about 3 month of age. If the cord is tehtered, it is unable to ascend normally and blood supplies to the cord are compromised and ischemic changes result . Children may present with pain in the lower extremities, a limp, atrophy of an extremity, sensory abnormalities, urinary incontinence, and bowel difficulties. The bilateral nature of the symptoms and multiple levels of involvement, may be clues to the diagnosis. Prompt neurosurgical referral is suggested.

Diagnosis of Spinal Dysraphism
1. Careful examination of midline back of all newborns
2. Palpation of the lower spine and feel for incomplete fusion of the bony spine
3. Bladder abnormalities such as failure to urinate and empty the bladder completely
4. Unexplained constipation
5. Disparity of growth of the legs or neurological findings in the lower extremities including sensory abnormalities
6. Ultrasound of the the lower back is an effective noninvasive screening tool. May follow up with a CT scan or MRI.
7. Neurosurgical referral

Prevention of Neural Tube Defects
1. It is recommened that women of reproductive age take 0.4 mg of folic acid daily to decrease the risk of defects.

Ventriculoperitoneal Shunts

Ventriculoperitoneal Shunts

The function of cerebrospinal fluid is not entirely understood.

However, cerebrospinal fluid is believed to be an ultrafiltrate of blood that cushions the brain. It allows for removal of metabolic products and the proper environment for neuro- transmission.

Most CSF is produced by the choroid plexus in the lateral, third and 4th ventricles. It moves from the point of production secondary to differences in pressure, by cilia action, blood vessel pulsation, and respiratory variations. After production, it passes from the lateral ventricle through the paired foramina of Monro into the third ventricle. CSF is resorbed in the superior sagital sinus by arachnoid villous activity in a process of active transport that is affected by CSF pressure.

Hydrocephalus

1. Overproduction of CSF- unusual and caused only by choroid plexus papilloma
2. Blockage of the normal flow of CSF.
1. Communicating or Absorptive- blockage of the resorption of CSF in the arachnoid villi, basal cisterns, or subarachnoid space. The ventricles are patent and all 4 are enlarged.
2. Non-communicating- Obstruction proximal to the foramina of Lushka and Magendie at the outlet of the 4th ventricle.
1. Tumors
2. Cysts
3. Infection and hemorrhage
4. Congenital malformations
5. Aqueductal stenosis
3. The best treatment for hydrocephalus is the placement of a extracranial shunt from the ventricles to an outside absorptive surface (i.e., ventriculoperitoneal, ventriculo-atrial, ventriculopleural, etc.)

Shunts

1. Shunts usually consist of three parts
1. Proximal end that is radiopaque and is placed into the ventricle. This end has multiple small perforations.
2. Valve- this allows for unidirectional flow. Can adjust various opening pressures. Usually has a reservoir that allows for checking shunt pressure and sampling CSF
3. Distal end that is placed into the peritoneum or another absorptive surface by tracking the tubing subcutaneously

Shunt malfunctions

1. Median survival of a shunt (before need for revision) in a child under 2 years of age is 2 years; over two years of age is 8 - 10 years.
2. Signs and symptoms include headache, malaise, general not feeling well, vomiting, mental status alterations, increased blood pressure, head circumference increase, Cushings triad, bulging fontanel, sixth nerve palsy signs, Macewen's sign, changes in gait, and personality changes. There may also be an increase of seizures and a complaint of neck pain. The parents often know that something is wrong. Teachers may state that there has been a change of school performance
3. Obstruction- most often the proximal tip is obstructed with cells, choroid plexus, or debris. May also have kinking of the tubing and migration of the distal end.
1. Diagnosis by suspicion based on signs and symptoms and confirmed by CT scan of the head or shunt tap or lumbar puncture for CSF pressure elevation (ALWAYS RECORD A PRESSURE WHEN DOING AN LP!!!!).
4. Infection-
1. signs include fever, meningeal signs, vomiting, signs and symptoms of shunt malfunction, abdominal pain, and peritonitis.
2. There may be evidence of purulent material around the shunt insertion site and redness along the shunt tract
3. Most common organisms are S. epidermidis and S. aureus. Also gram negative organisms.
4. Diagnosis by positive blood cultures, shunt fluid cultures, or lumbar puncture cultures. Also the presence of greater than 10 cells in the fluid is suggestive of infection.

Infection peaks in the first few weeks after a shunt insertion. Infection years after shunt placement is rare unless the skin is broken over the tubing.

Treatment

1. Antibiotics including Vancomycin and Gentamycin
2. External Ventricular Drainage
3. Removal of the shunt.
4. Disconnections and breakage of tubing are another cause of malfunction, though less common than occlusion.
5. Migration into the scrotum, perforation of the bowel wall, and intussuseption are all rare complications in the peritoneum

In a child with a ventriculoperitoneal shunt, the shunt is statistically unlikely to be the cause of any specific problem. However, if family members suspect shunt malfunction or no other cause for fever, malaise, behavioral change, etc., can be found (i.e., ear infection), careful and diligent evaluation of the shunt is mandatory.

Cystic Fibrosis

Cystic Fibrosis

Cystic fibrosis is an autosomal recessive disease that is the most common cause of progressive obstructive lung disease in children and young adults. The incidence in Caucasians is 1 in 2000 to 1 in 3000. The carrier rate is approximately 1 in 25. The cystic fibrosis gene is located on chromosome 7 and this codes for the cystic fibrosis transmembrane conductance regulator (CFTR) This protein regulates electrolyte transport across cell membranes and abnormalities lead to increased viscosity of secretions and obstruction of airways and other channels. There are 500 known mutations but 70 mutations are common and identify over 90% of patients with cystic fibrosis.

Common Clinical Characteristics

1. Pulmonary

a. Wheezing, cough, recurrent respiratory infections and pneumonia

b. Chronic cough, increased AP diameter of the chest, clubbing

2. Gastrointestinal

a. Fatty stools due to pancreatic exocrine insufficiency

b. Poor weight gain despite large appetite

c. Hypoproteinemia, edema, anemia, Vitamin deficiency

d. Hepatomegaly

3. Newborns

a. Meconium ileus

b. Meconium peritonitis secondary to perforation

c. Inspissated bile syndrome and cholestasis, prolonged jaundice

4. Sinus disease with panopacification of the paranasal sinuses

5. Absent vas deferens with resultant azospermia

6. Rectal prolapse

7. Pancreatitis

8. Nasal Polyps

Diagnosis

1. The sweat test remains the gold standard for diagnosis of cystic fibrosis.

2. 60 meq/liter sweat chloride plus characteristic findings and + family history is diagnostic

a. Other causes of increased sweat chloride include adrenal insufficiency, mucopolysaccharidoses, Glycogen Storage Disease type I, hypothyroid, familial hypoparathyroid, malnutrition, ectodermal dysplasia, and Lab error.

3. May be difficult to collect adequate sweat in newborns and genotyping for the CFTR mutation may be performed

4. It is imperative that this difficult to perform test is done in a lab that has experience doing the test.

Management of Cystic Fibrosis

1. Mobilization of airway secretions

a. Chest physiotherapy

b. Inhaled bronchodilators

c. Mucolytic agents N-acetylcysteine (Mucomyst) and rhDnase (Pulmozyme)

2. Treatment of Infections ( Staphylococcus aureus and Pseudomonas aeruginosa

a. Antimicrobials - oral, IV, and aerosols

3. Evaluation periodically with pulmonary function testing

4. Lung transplantation.

5. Treat airway reactivity with bronchodilators such as albuterol, Theophylline, and steroids

6. Pancreatic enzyme replacement with meals and snacks.

7. High protein and caloric diets

8. Vitamin supplementation for fat soluble vitamins- A. D. and E. Vitamin K until one year of age

9. Newborns

a. Gastrograffin enemas for meconium ileus

10. Monitor for hepatic disease, biliary obstruction, gallstones, and cirrhosis

11. 30% develop diabetes mellitus by 18 years of age.

12. Genetic counseling

Prognosis

1. Median survival about 30 years

Retinopathy of Prematurity

ROP is a disorder of the developing retinal vasculature that occurs with interruption of the forming retinal vessels. Constriction and obliteration of the advancing capillary bed are followed by neovascularization of the retina, which can extend into the vitreous. The most serious and feared complication of ROP is retinal detachment. ROP has previously been known as RLF- Retrolental Fibroplasia, a very advanced form or ROP with end stage fibrosis and scarring behind the lens.

Incidence � The Cryotherapy for ROP trial, a prospective cohort study that observed 4000 infants of birth weight <>

Development of ROP: It is postulated that there are two events that occur

1. Vasoconstriction and obliteration of the capillary network in response to a vascular insult ( possibly high supplemental O2 concentration)
2. Vasoproliferation- possibly a response by the hypoxic retina to an angiogenic factor released by the insult � thought that hypoxia can cause an overexpression of VEGF that can induce abnormal retinal angiogenesis.

Etiology of ROP: multifactorial and still unclear

1. Oxygen administration originally was thought to contribute to the development of ROP. This is now being debated. The STOP ROP- Supplemental Therapeutic Oxygen to Prevent ROP study investigated whether supplemental therapeutic oxygen for premature infants reduces the proportion of infants that progress to threshold ROP. The study found that the more liberal use of oxygen actually decreased the risk of progression to threshold ROP in these infants from 48% to 41%. Threshold ROP is defined as disease progression to the point of necessitating peripheral retinal ablation therapy.
2. Prematurity- birth weight <>

Risk Factors for development of ROP:

1. Extreme Prematurity � the most significant risk factor
2. Thought to be related to oxygen administration
3. Other possible risk factors: apnea, sepsis, hypoxia, hyper or hypocapnia, IVH, Caucasian race

International Classification of ROP (ICROP)

Three components used to determine the extent of disease: the zone in which ROP occurs, the stage of ROP, and the presence or absence of plus disease.

Zone 1- the most posterior � an area within twice the distance from the optic nerve head to the fovea

Zone 2- ROP outside of zone 1

Zone 3- ROP only present on the temporal side of the eye

• Stage 1- a line of demarcation develops from the vascularized region of the retina and the avascular zone
• Stage 2- the line becomes a ridge that protrudes into the vitreous. Histological evidence of an A-V shunt
• Stage 3- Extra-retinal vascular proliferation occurs with the ridge. Neovascular tufts can be found posterior to the ridge.
• Stage 4- Scarring and fibrosis can occur when the neovascularization extends into the vitreous. This can cause traction on the retina, leading to retinal detachment.
• Stage 5 � Indicates total retinal detachment.
• Plus Disease � can occur when vessels posterior to the ridge become dilated and tortuous.

Diagnosis of ROP:

National (AAPOS, AAP, AAO) recommendations for ROP screening exams in premature infants:

1. Infants <1500> 1500g with poor clinical course � dilated eye exams at 4-6 weeks of age. Exams are to continue every 2-4 weeks until retinal maturity is reached.
2. Infants with ROP or immature retinal vessels are to have exams every 1-2 weeks until vessels are mature.

Treatment of ROP � Treatment is initiated when there is ROP in zone I or II, with five contiguous or eight cumulative clock hours of stage 3, and with plus disease � where severe visual loss occurs approximately 50% of the time. (Threshold Retinopathy)

1. Cryotherapy- an attempt the prevent further progression of the disease by destroying the cells that may release angiogenic factors. If both eyes have threshold ROP, only one eye is treated due to the risk of vitreous hemorrhage. If there is a very high risk of bilateral retinal detachment, the procedure can be performed in both eyes.
2. Photocoagulation � laser photocoagulation procedure done to destroy cells that could lead to disease progression. In a meta analysis of four photocoagulation trials, this procedure was found to be at least as effective as cryotherapy.
3. Retinal reattachment � an attempt to treat stage 5 disease, has a low success rate.

Prognosis of ROP � 90% of Stage 1 and Stage 2 disease regresses spontaneously. Approximately 50% of Stage 3 disease can regress spontaneously. Prognosis for stage 4 and 5 disease is poor, with a high incidence of visual problems and retinal detachment.

Neonatal Intestinal Obstruction

NEONATAL INTESTINAL OBSTRUCTION

99% of healthy full-term infants pass their first stool or meconium within 24 hours of birth and all healthy term neonates should do so by 48 hours.

With preterm infants the length of time can extend up to 9 days.

Neonatal intestinal obstruction occurs in 1/1500 live births. Etiologies are from intrinsic developmental defects, abnormalities of peristalsis or abnormal intestinal contents, or from insults in utero after the formation of normal bowel. Failure to recognize neonatal bowel obstruction can result in aspiration of vomit, sepsis, mid-gut infarction or enterocolitis.

Differential Diagnosis for failure to pass meconium:

1. Disorders of the small intestine
1. Duodenal atresia
2. Jejunoileal atresia
3. Malrotation and volvulus
4. Meconium ileus
2. Disorders of the large intestine
1. Meconium plug syndrome
2. Anorectal malformation
3. Hirschsprung's disease
4. Small left colon syndrome
3. Other causes
1. Narcotics
2. Electrolyte abnormalities; hypermagnesemia, hypokalemia, hypercalcemia
3. Hypothyroidism
4. Sepsis
5. Congestive heart failure

Meconium plug syndrome: Meconium plug syndrome is the most common form of functional bowel obstruction in the newborn, with an incidence of 1/500.

It is a transient form of distal colon or rectal obstruction caused by inspissated and dehydrated meconium, the etiology for which is unknown.

Diagnosis is made through barium enema revealing the outline of meconium plug. Barium enema can also be therapeutic along with rectal stimulation in inducing passage of the meconium.

Generally, infants with meconium plug syndrome have normal bowel function after passing the meconium plug.

Anorectal malformation: The incidence of anorectal malformation is 1/4000 live births, including anal stenosis, imperforate anus, and fistula.

Malformations are caused by a defect in embryonal development where the urorectal septum, lateral mesoderm and ectodermal structures combine to form the normal rectum and lower urinary tract.

70% of infants with anorectal malformation have associated anomalies. The mnemonic VACTERL is used to describe the combination of vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal defects and radial limb hypoplasia.

Anal stenosis accounts for 20% of anorectal anomalies and treatment is with dilatation.

The mortality rate for patients with anorectal malformation is directly related to associated anomalies.

Malrotation and volvulus: Malrotation of the midgut is caused by a failure of normal bowel rotation. The mid-gut does not complete its normal 290° counterclockwise rotation during embryologic development, resulting in abnormal placement and fixation of the small bowel. This can cause obstruction and sometimes infarction of the small and large bowel, known as volvulus. Volvulus usually occurs within the first week of life.

Upright abdominal films reveal the classic double bubble sign, showing air in the stomach and proximal duodenum.

Treatment is with the Ladd's procedure involving counterclockwise reduction of the volvulus, release of adhesive bands (Ladd's bands) to mobilize the duodenum, and appendectomy. Recurrence of mid-gut volvulus after the Ladd's procedure can occur in up to 10% of cases.

Meconium ileus: This disorder is differentiated from meconium plug syndrome by location of the stool. In meconium ileus, the thick, tenacious bowel is most commonly found in the ileum but occasionally occurs in the jejunum or proximal colon.

90%-95% of patients with meconium ileus have cystic fibrosis. But, only 15% of cystic fibrosis patients will have meconium ileus as neonates. Associated anomalies such as volvulus, jejunoileal atresia, or bowel perforation occur in over half of infants with meconium ileus.

Duodenal atresia: The etiology of duodenal atresia is from failure to recanalize the lumen after the solid phase of intestinal development. This occurs between the 4th and 5th week of gestation. 40% of the patients with duodenal atresia have Down Syndrome. Diagnosis can be made in utero if polyhydramnios is present. Plain films can reveal the double bubble sign and an upper GI series might be necessary to distinguish between malrotation and duodenal atresia. Treatment is with duodenoduodenostomy.

Hirchsprung's disease: Hirchsprung's disease, or congenital aganglionic megacolon, is a motor disorder of the colon that causes a functional intestinal obstruction. It occurs in 1/5000 infants with a male to female predominance of 4:1. The pathogenesis of the disease is failure of migration of the neural crest cells that form the colonic ganglion cells. Without parasympathetic innervation, the colon cannot relax or undergo peristalsis, resulting in a functional obstruction.

The aganglionic segment is limited to the rectosigmoid in the majority of patients. 10% have full colonic involvement and in 10% more, there is lack of ganglion cells into the small bowel.

Diagnosis of Hirchsprung's disease can be made with barium enema, revealing a transition zone between the constricted aganglionic segment and the proximal, normally dilated segment.

Confirmation of the diagnosis is made with rectal suction biopsy.

The treatment of Hirchsprung's disease is through surgical resection of the aganglionic bowel.

The major complications of the disease, even after surgical resection, are bowel obstruction and enterocolitis.

Full article found here.

Hypoglycemia in the Neonate

The definition of hypoglycemia in infants is a current medical controversy, as asymptomatic hypoglycemia is generally not related to significant morbidity, and many healthy neonates have been found to have transiently low blood glucose concentrations. However, due to uncertainty about the level and duration of hypoglycemia that causes brain damage, an operational threshold has been defined. Currently, it is standard practice to treat and evaluate newborns with a plasma glucose concentration less than 40 mg/dl on the first day of life, and less than 40 to 50mg/dl after 24 hours of age.

Risk Factors

1. Premature infants
2. Infants of diabetic mothers
3. Infants who are small or large for gestational age (SGA or LGA)
4. Infants with sepsis or history of birth asphyxia
5. Infants of mothers treated with hypoglycemic or beta-adrenergic agents (tocolytics)
6. Insulinomas
7. Hypopituitarism and adrenal insufficiency
8. Erythroblastosis fetalis
9. Inborn errors of metabolism- glycogen storage diseases, aminoacidurias, organic acidemias, fatty acid oxidation defects, galactosemia

Common Symptoms

1. Tremors, jitteriness
2. Change in level of consciousness (irritability, stupor, lethargy)
3. Apnea, bradycardia, cyanosis, tachycardia, tachypnea
4. Hypothermia
5. Poor feeding or poor suck
6. Seizures
7. OR, infants may be asymptomatic and diagnosed because fall into high risk group

Diagnosis

1. Maternal history of drugs, diabetes, preterm or post term delivery

2. Growth parameters abnormal

3. Septic appearing

4. Family history of metabolic disorder

When a patient presents acutely with hypoglycemic symptoms, it is imperative to rapidly assess blood glucose measurements at the bedside, often using a reflectance meter or a Chemstrip. These values should be confirmed by laboratory measurement.

If hypoglycemia persists and does not respond to routine therapies, then the neonate must be evaluated for hyperinsulinemia, endocrinopathies, and inborn errors of metabolism.

Complications

Prolonged or recurrent low blood glucose levels are well known to cause seizures and neruologic sequelae. Unfortunately, the level of blood glucose and the duration of time that it remains low enough to cause damage are unknown. For this reason, in order to prevent the terrible sequelae that may result from prolonged hypoglycemia, it is imperative that it is discovered early and quickly corrected. It is unknown if asymptomatic hypoglycemia can cause brain damage.

Treatment

1. Introduce early enteral feedings with formula

2. If not taking oral fluids, IV glucose should be administered

3. Follow up to make sure that normal glucose levels are maintained

4. Corticosteroids

5. Glucogon if there are adequate liver stores of glycogen

Full article found here.

Abdominal Masses in Neonates

Abdominal Masses in the Neonatal Period

The discovery of an abdominal mass on physical examination in the newborn period causes concern and the need for a rapid diagnosis. The incidence of an abdominal mass is 1/1000 live births.

With the introduction of fetal ultrasound, many abdominal masses are diagnosed in utero. Many dilatations of the urinary tract diagnosed in utero resolve spontaneously. In utero procedures to correct most anomalies are still experimental . The majority of masses are of benign origin and greater than 50% are of renal origin. The majority will be diagnosed with a good history, physical examination, and ultrasound evaluation.

History

1. Was there a prenatal ultrasound performed?
2. Are there any GI symptoms such as vomiting and poor feeding?
3. How much amniotic fluid was present?
4. Any family history of masses or renal disease?

Physical Examination

1. Location of the mass- flank, mid-abdomen, or suprapubic
2. Is the mass solid, cystic, smooth, or tender?
3. Is there hepatosplenomegaly?
4. Other physical findings unrelated to the mass- facies, rectal, lung exam, other anomalies.

Common Etiologies of Abdominal Masses

1. Renal (55%)
1. Multicystic dysplastic kidney- usually a flank mass and irregular surface. If bilateral, usually some intrauterine obstruction. On ultrasound hypoechogenic. Usually removed surgically prior to development of hypertension.
2. Hydronephrosis- neonates are usually asymptomatic and present with a flank mass. Most are secondary to an obstruction at the ureteropelvic junction. May also be associated with reflux. Surgically repair and may need nephrostomy to decompress first.
3. Polycystic Disease
1. Infantile is inherited as autosomal recessive and associated with hepatic cysts and pulmonary hypoplasia. Poor prognosis.
2. Adult Polycystic Disease- autosomal dominant inheritance and rarely seen in childhood
4. Posterior Urethral Valves -may have enlarged kidneys or bladder
5. Renal Vein Thrombosis- history of dehydration and hemoconcentration. May have hematuria and Proteinuria.
6. Mesoblastic Nephroma-a benign hamartoma that may have associated hematuria. Remove and pathology will differentiate from rare Wilm's tumor.
2. Adrenal Masses and other retroperitoneal(10%)
1. Hemorrhage- associated with birth trauma.
2. Neuroblastoma- may have calcifications on plain film of the abdomen
3. Enlarged liver - (5%) there may be cysts, tumors, and hemangiomas. Choledochal cyst of the gall bladder often presents with jaundice
4. Duplications of the Gastrointestinal tract-and other GI lesions (15%) Duplications most commonly in the ileocecal area and most do not communicate with the intestines.
5. Pelvic and Genital Tract (15%)
1. Ovarian cysts
2. Hydrometrocolpos
3. Distended bladder
4. Teratomas
5. Anterior meningomyeloceles

Evaluation

1. History and Physical examination
2. Abdominal radiograph- will show gas pattern, displacement of organs may identify location of the mass. May also show calcifications associated with neuroblastoma, meconium peritonitis, and hepatoblastoma.
3. Ultrasound- Will differentiate solid from cystic and locate which organ the mass is located in.
4. CT and MRI- will provide more anatomic detail.
5. Include the obstetrican, surgeon, and urologist in the process of evaluating abdominal masses.

Full article found here.

INTESTINAL OBSTRUCTION

INTESTINAL OBSTRUCTION
A. Diagnosis and preoperative management:
•Intestinal obstruction should be suspected with maternal history of
polyhydramnios, large amount (>20 mL) of gastric fluid at birth, bilious or nonbilious
emesis, or progressive abdominal distension.
•Common causes include duodenal, jejunal, ileal, or colonic atresia, malrotation
with mid gut volvulus, meconium ileus with associated cystic fibrosis, meconium
plug, Hirschsprung’s disease, imperforate anus, and hypoplastic left colon.
•Infants with bowel atresia may pass meconium.
•The higher the obstruction, the more prominent is the vomiting. The lower the
obstruction, the more prominent is the distension.
•Make infant NPO, start IV, and monitor electrolytes, urine output and weight.
•Place Replogle tube to continuous suction and measure output.
•Obtain KUB looking for
-“double bubble” sign of duodenal atresia. If present, no further GI workup is
needed and patient should go to surgery when stable.
-multiple dilated loops of bowel indicating a more distal obstruction
-intraperitoneal calcifications suggestive of perforation with meconium ileus
-air throughout bowel to the rectum suspicious for Hirschsprung’s disease
-bubbly-appearing stool filling the bowel suggestive of meconium ileus and
cystic fibrosis
•Upper GI contrast study (with dilute Hypaque™or Gastrograffin™) may be
required to assess for malrotation and possible volvulus.
•Contrast enema using Gastrografin™ or dilute Hypaque™ may be done to identify
an area of obstruction or to relieve meconium plug or meconium ileus.
•Suspect acute volvulus secondary to malrotation if the baby has signs of shock,
metabolic acidosis or peritonitis. If there are signs suggesting volvulus,
emergency operation is indicated since gut viability may be threatened.
•Suspect Hirschsprung’s disease with repeated episodes of abdominal distension
or very delayed passage of meconium. Diagnosis can be made with suction rectal
biopsy. If no ganglion cells are seen, a surgical biopsy will confirm the diagnosis.
•Infants with Hirschsprung’s disease are at risk for development of fatal toxic
megacolon until the bowel has been decompressed by corrective surgery or
colostomy. Surgeons may choose to decompress initially with rectal irrigation.
This is different from simple enemas.
•Imperforate anus may be the sole abnormality or may be part of the VATER
association. Look carefully for evidence of recto-vaginal, recto-urethral or
perineal fistula. Ultrasound may help determine if the defect is low (and easily
repaired) or high (requiring colostomy drainage). These patients will need
eventual workup for tethered spinal cord and urinary tract anomalies.
B. Post operative management:
•IV fluid replacement at maintenance levels with parenteral nutrition (see P. 136)
starting within 2d of operation. Intermittent fluid boluses may be required in the
first 48h to maintain adequate urine output and to treat hypotension and
hypoperfusion. Consider early use of low-dose dopamine (3-5 mcg/kg/min).
•If there has been extensive bowel manipulation, the baby may require baseline
fluid administration 1.5 times normal (i.e., 150 mL/kg/d) because of capillary
leak. Use Lactated Ringer’s Solution with 5% or 10% dextrose for at least the
first 24h after operation.
•Maintain Replogle tube to continuous suction and measure output. If drainage is
more than 10 mL/kg per 12h shift, replace volume loss with an equal volume of
0.45% NaCl.
•Replogle tube may be removed when drainage is minimal and non-bilious.
•After the baby has passed stool, start feedings with small volumes and advance
slowly over the next 48h to ensure that baby is not developing abdominal
distension secondary to postoperative ileus or to stricture at the anastomotic site.

ESOPHAGEAL ATRESIA WITH OR WITHOUT TEF

ESOPHAGEAL ATRESIA WITH OR WITHOUT TRACHEO-ESOPHAGEAL FISTULA (TEF):
A. Diagnosis and preoperative management:
•Esophageal atresia may often be suspected prior to the first feeding by a history of
polyhydramnios or observation of copious oral secretions than require very
frequent suctioning.
•Attempt to pass feeding tube with radiopaque line into the stomach. If the tube
does not pass, leave in place and obtain chest x-ray and KUB.
•Do not obtain contrast study. This may result in aspiration.
•If the tube curls up in blind esophageal pouch and there is no air in bowel, assume
a diagnosis of esophageal atresia.
•If the tube curls up in blind esophageal pouch and there is air in the distal bowel,
assume a diagnosis of esophageal atresia with distal TEF.
•Keep infant in a position with the head up to prevent aspiration.
•Place Replogle tube on continuous suction to drain the blind pouch.
•Avoid bag and mask ventilation and nasal CPAP to prevent over-distension of the
stomach. If the baby needs respiratory assistance, intubate the infant.
•If the baby has severe lung disease and a distal TE fistula, ventilation of the lungs
may be extremely difficult because of the low resistance through the fistula into
the stomach and bowel. Notify surgery immediately as the baby may need
immediate closure of the fistula or an emergency gastrostomy with placement of a
distal esophageal balloon to facilitate adequate ventilation.
•Examine infant carefully for other anomalies associated with VATER or
CHARGE, including vertebral abnormalities, radial anomalies, choanal atresia,
imperforate anus, renal abnormalities, congenital heart disease, coloboma or
evidence of Down syndrome.
B. Post operative management:
•Regular maintenance IV fluids with extra boluses of normal saline as needed for
oliguria, hypotension, or poor perfusion. If infant requires >15 mL/kg of extra
fluid, consider starting dopamine at 5 mcg/kg/min to ↑ blood pressure and
perfusion to kidneys.
•If a chest tube is in place draining the area of the anastomosis, do not connect the
pleuravac to suction without consulting with the Attending Surgeon. The chest
tube is usually in place for 7-10d until x-ray studies show no leak at the
anastomosis.
•If the anastomosis is under tension, the surgeons will often want to keep the baby
on muscle relaxants postoperatively for a few days to a week, to prevent
disruption of the anastomosis.
•Do not extubate until the baby is extremely stable on very low ventilatory settings,
because positive pressure mask ventilation must be avoided to prevent
transmission of pressure to the esophagus, which may rupture the anastomotic
suture line.
•If the baby needs to be reintubated, the most experienced person should do this.
Faulty (i.e., esophageal) intubation could result in injury to the anastomosis.
•Leave the orogastric or nasogastric tube in place until x-ray studies show no leak at
the anastomotic site, and Pediatric Surgery agrees to removal of the tube. If the
tube accidentally comes out, do not reinsert tube without consulting with the
Attending Pediatric Surgeon, as you may damage the anastomosis.
•X-ray contrast study should be done at approximately 10 days postoperatively to
assess for leakage at the anastomotic site prior to starting oral feedings.
Gastrostomy tube feedings and NG tube feedings may be started earlier.