SCORE 22-24

Urinalysis will reveal gross hematuria in 19% and microscopic heme in 25% of Wilms tumor patients.
The diagnostic test of choice is 24 hr urine collection for catecholamine byproducts:
Homovanillic acid (HVA)
Vanillylmandelic acid (VMA)
Metanephrine
Adrenaline
Noradrenaline
Vanillylglycolic acid
Bone marrow involvement can lead to anemia.
Serum LDH, ferritin, and carcinoembryonic antigen (CEA) are elevated in 25% of neuroblastoma patients
Screening marker for teratomas - AFP, HCG, CA1, inhibin , testosterone

1. Ultrasound
2. CT scan confims renal origin, evaluates for metastasis and bilaterality

1. CT scan with IV contrast
2. MIBG scan
3. Bone scan

The goal of gross total resection without endangering surrounding structures
Regional lymph node sampling for accurate staging
"Bivalving" the tumor to release and preserve encased visceral vessels

Accurate preoperative staging
Removing the tumor intact without spillage (including avoiding a preoperative biopsy)
Regional lymph node sampling

Removing the tumor intact
Ovarian preservation if possible
Performing cytology on ascitic fluid or peritoneal washings
Partial or complete omentectomy
Diaphragm inspection
Peritoneal biopsy
Regional lymph node sampling

Surgery alone is adequate for stage 1 tumors.
Higher staged tumors require adjuvant or neoadjuvant chemotherapy and radiation.

Chemotherapy
Radiation
Bone marrow transplant
Immunotherapy
Cis-retinoic acid
MIBG therapy

Obtain abdominal access, retract the liver superiorly, and deliver the pylorus into the wound without trauma.
Open the serosa sharply, bluntly divide the muscle fibers to expose the submucosa from just proximal to the hypertrophied muscle to just proximal to the pyloric vein, and return to the abdominal cavity.
Check for a leak.

Have infant supine, at end of bed.
Obtain access via umbilical incision for 5-mm port, insufflation to 8 mm Hg; make stop incisions for placement of an atraumatic grasper and extended cautery blade; score serosa with a low electric current; split muscle with cold cautery blade; and use pyloric spreader to complete myotomy.
Confirm independent rocking of divided muscle halves to ensure complete myotomy.
Perform leak test (air insufflated via orogastric or nasogastric tube)

Neonates: anal fissure, milk protein allergy, necrotizing enterocolitis, malrotation, Hirschsprung enterocolitis, coagulopathy
Infants: anal fissure, milk protein allergy, Meckel diverticulum, intussusception, infectious colitis, medical causes (ie, thrombocytopenia)
Children: polyps, Meckel diverticulum, intussusception, infectious colitis, GI duplication, vascular malformations, trauma/foreign body
Adolescents: inflammatory bowel disease, infectious colitis, Meckel diverticulum, trauma/foreign body, vascular malformations, GI duplication, hemorrhoids

Vital signs
Evaluation of the airway, breathing, and circulation to ensure stability
Assessment of mental status, including lethargy, irritability, and confusion
Head-ear-eyes-nose-throat examination to ensure there is no epistaxis or other mucocutaneous bleeding
Abdominal examination to evaluate for any hepatomegaly, splenomegaly, abdominal mass, or clinical findings of portal hypertension
Rectal examination to evaluate for fissures and polyps
Skin examination to evaluate for petechiae and ecchymoses

Complete blood count, including differential
Coagulation studies (prothrombin time, partial thromboplastin time, INR)
Basic metabolic panel (blood urea nitrogen may be elevated with GI bleeding because of reabsorption in the GI tract)
Creatinine, which may be a marker of resuscitation
Liver function tests
Stool studies (if infectious etiology of LGIB is suspected)
Plain radiography
Abdominal ultrasound
Computed tomography angiography
Meckel scan (technetium-99m pertechnetate radionuclide scan)
Tagged red blood cell scan
Endoscopy (esophagogastroduodenoscopy and colonoscopy)

5 years or older, with Meckel diverticulum as MC lead point

1. KUB - paucity of gas in RLQ , dilated bowel loops with air fluid levels
2. US - "target lesion"

1. Bowel rest, IVF
2. Radiographically guided enema reduction of intussusception

In an open reduction, a transverse right lower quadrant incision is made. The cecum and terminal ileum are delivered through the incision, and the extent of the intussusceptum is evaluated.
If the intussusceptum reaches the distal colon, extension of the incision may be necessary.
Once the leading edge of the intussusceptum is identified, it is milked slowly in a retrograde fashion while exerting minimal traction on the proximal bowel.
Care should be taken to avoid excessive pulling on the bowel as it enters the cecum because this may lead to perforation of the edematous bowel within.
This process requires constant pressure on the leading edge and patience. If successful, the bowel can be warmed with saline-soaked laparotomy sponges and closely observed when viability is questionable.

1. Bilious emesis
2. Abdominal distention
3. Peritonitis
4. Hematochezia

Perform a two-view plain radiograph of the abdomen to assess for perforation. If pneumoperitoneum is present, proceed to the operating room for exploratory laparotomy. If no pneumoperitoneum is present, proceed with the next diagnostic step.
Proceed with limited upper gastrointestinal series with oral contrast to confirm findings of malrotation.
Consult pediatric surgery if definitive findings for malrotation are observed on imaging, which may include any of the following: Misplaced duodenum, Corkscrew duodenum

No imaging is required.
Treat with fluid resuscitation.
Provide orogastric decompression.
Provide empiric antibiotic coverage that is appropriate for common bowel flora.
Proceed to the operating room for exploratory laparotomy.

young pediatric patient with bright red blood per rectum and moderate-to-high suspicion of index for Meckel diverticulum, radionucleotide scanning with technetium-99m (also known as Meckel scan) is indicated.

Incidentally discovered Meckel diverticula identified during abdominal exploration should be selectively resected because there is controversy regarding management. In general, patients without anatomic risk factors (length > 2 cm, palpable abnormality, fibrous bands) should not undergo resection. Older individuals (> 50 years) and those with medical comorbidities should not undergo resection unless there is a palpable abnormality or other significant risk factors.
Incidentally discovered Meckel diverticula without palpable abnormalities and those causing adhesive small bowel obstruction can be successfully managed with diverticulectomy.
Symptomatic Meckel diverticula or those with palpable lesions are best managed by segmental small bowel resection and primary anastomosis.

Once the diagnosis is established with ultrasound (or contrast study), the patient's degree of dehydration should be assessed.
Fluid bolus (20 mL/kg NS) should be initiated and followed by fluids containing glucose and potassium (eg, D5 ½ NS with 20 mEq KCl/L) at a rate above maintenance to help maintain hydration and correct dehydration.
If dehydration is associated with metabolic alkalosis, resuscitation should include a repeat bolus and then laboratory (electrolytes) evaluation before proceeding to the operating room.
Prior to induction of anesthesia, the stomach should be emptied with a suction catheter, and strong consideration should be given to rapid sequence intubation to minimize the risk of aspiration.

Frequently, infants continue to vomit, even as forcefully as before the operation. This is thought to be secondary to gastric atony and edema at the pylorus. This will improve over the next few days, and no workup is required. The infant still needs to be hydrated intravenously until he or she can tolerate oral feeds. Other causes are preexisting gastroesophageal reflux disease, which should be treated medically.
If the vomiting persists after a few weeks, then an incomplete pyloromyotomy should be considered and ruled out by an ultrasound. Treatment is a redo pyloromyotomy.

Asymptomatic hernias in patients younger than 4 years of age are able to be observed. Most will spontaneously close before the child reaches 4 years of age.
Smaller defects (<1 cm) are more likely to close spontaneously.
Larger defects (>1.5 cm) are less likely to close spontaneously.

consists of a remnant of the omphalomesenteric duct; treatment of this requires excision.

In a 4-year-old child who presents with a reducible 2.5-cm umbilical hernia, repair the hernia with an infraumbilical incision, perform careful dissection of the hernia sac to the level of the fascia, and close the fascia with interrupted absorbable sutures.
Adequate dissection around the umbilical stalk facilitates dissection.
The umbilical stalk is divided, and the hernia sac is resected.
All peritoneum present at the umbilical site should be removed or lightly cauterized to prevent seroma formation.
The umbilicus should be reattached to the repair area.

Patients' families should be counseled on signs such as skin changes, distension, vomiting, abdominal pain, and cessation of a bowel movements. These may be signs that the umbilical hernia is now symptomatic (incarcerated, strangulated, obstructed) and needs repair.
Patients should have the hernia monitored by their pediatrician, and repair may be considered if the hernia persists at 4 years of age.

Urachal remnants may present with drainage from the umbilicus.
Surgical exploration is indicated, and diagnostic imaging (CT, MRI, or ultrasonography) may be helpful in the diagnosis but is not necessary.
Resection of the urachal remnant should include a complete resection with a cuff of bladder (due to the risk of associated malignancy).

Omphalomesenteric remnants may persist and present as fistulas from the intestine to the umbilicus.
A contrast study via the umbilicus does not change the operative approach and is unnecessary.
Operative exploration through the umbilicus is indicated. This includes identification of all umbilical structures, division of the ductal remnant, and closure of the intestine.

Umbilical lesions may exist as remnants of the omphalomesenteric and urachal systems.
This lesion most likely represents an umbilical polyp, which may be a nuisance due to the persistent drainage but does not have any long-term risks besides infection.
Surgical excision requires identification of all the umbilical structures, including a fistulous connection to the small intestine.

Care should be taken in any umbilical hernia surgery with the surgeon aware of the possibility of a Meckel diverticulum.
A contrast agent may be injected into the lumen as a diagnostic test for a Meckel.
If a Meckel is found it should be removed. If a stalk is present then diverticulectomy alone may be sufficient.
Any remnant cyst or adhesive tissue should be taken down at the time of initial operation.

Dysphagia, drooling and postprandial regurgitation of undigested food for ingested foreign bodies
Persistent cough, tachypnea, nasal flaring, wheezing and hypoxia for aspirated foreign bodies

Immediate direct laryngoscopy is used to evaluate for object in the oropharynx. Retrieval may be attempted with McGill forceps.
An emergent surgical airway is necessary if the obstructing foreign body prevents visualization of the vocal cords on direct laryngoscopy. Tracheostomy or needle cricothyroidotomy is preferred in children. Surgical cricothyroidotomy should be avoided in children younger than 8 years of age.

Direct laryngoscopy may reveal coins in the oropharynx or proximal trachea amenable to retrieval with McGill forceps.
Rigid bronchoscopy under general anesthesia is the treatment of choice. Instrumentation options are broad, visualization is excellent, and adequate ventilation is possible through the instrument.
In older children and adolescents, flexible bronchoscopy through a laryngeal mask airway can be advantageous for small objects located in distal airways.

Rigid esophagoscopy under general anesthesia is the gold standard.
For small lesions or those in the distal esophagus, flexible esophagoscopy also has good success rates.
Awake bougienage in the swaddled child can be used for coins in the distal esophagus that are lodged for less than 24 hours.
Although awake retrieval of ingested coins using Foley catheters has been reported to have a high success rates in some centers, the approach should be used only in experienced hands because it carries with it the added risk of possible aspiration after dislodgement.

Two-view chest x-rays should be performed prior to surgery to confirm that the ingested object is button battery and that it is located in the esophagus, not the trachea. Button batteries have a characteristic "double contour" sign.
Ingestion of button batteries is a surgical emergency (except in asymptomatic children with batteries in the stomach; see Learning Objective 8c).
Timing of battery removal is emergent. Once its location is confirmed, prompt removal of a battery (within 2 hours) minimizes the risk of electrolysis of tissue from external current or liquefactive necrosis from leakage of alkaline contents

For coins lodged in the esophagus, those causing symptoms should be removed urgently. If patients are asymptomatic, coins can be removed on a semiurgent basis, usually within 24 hours.
Consider glucagon to aid in passage of distal esophageal coins.
Coins located in the stomach or small bowel should be allowed to pass per rectum. A repeat x-ray should be performed at 2 weeks for retained coins, and endoscopic removal should proceed if the coins remain in the stomach.

The most common sharp objects ingested are bones and toothpicks. Expectant management on an outpatient basis should be avoided.
In infants, objects greater than 2 cm wide or 3 cm long are unlikely to pass through the pylorus and should be retrieved endoscopically.
In older children and adolescents, objects greater than 2 cm thick or 5 cm long are unlikely to clear the duodenal sweep and should be removed.

Although button batteries that are more than 20 mm in diameter are more likely to be lodged in the esophagus, particularly in children younger than 5 years of age, many pass into the stomach if under 20 mm.
A button battery in the stomach is not a surgical emergency. Outpatient management is acceptable in the asymptomatic child with a button battery in the stomach.
If the battery is not passed per rectum, an abdominal x-ray should be obtained at 48 hours for batteries more than 20 mm and in 10 to 14 days for those less than 20 mm.
If it is not passed within this time frame, or if symptoms develop, endoscopic retrieval should be performed.

Perform a physical examination.
Obtain renal and pelvic ultrasounds. Assess for an associated renal anomaly.
Obtain thoracic and abdominal x-rays. Pass a nasogastric tube to rule out esophageal atresia.
Obtain a spinal ultrasound. Evaluate for a tethered cord.
Obtain anteroposterior/lateral sacral x-rays. Assess for a hemisacrum. Calculate a sacral ratio, which helps predict the potential for bowel control.
Obtain an echocardiogram.
Order laboratory studies.

The colostomy procedure may be performed via laparoscopy or through a left lower quadrant incision.
A colostomy is ideally opened in the proximal sigmoid, and a mucous fistula is performed so the distal anatomy can be ascertained via a distal colostogram.

- prone position
Separate the rectum from the posterior vagina in a female or the urethra in a male.
- From a posterior saggital incision (if rectum si below the pubococcygeal line)
- fwith lapaorsocpy if rectum is above the pubococcygeal line
Mobilize the rectum so that it comfortably reaches the perineum and can be placed in the center of the sphincter mechanism.
- perform a colostomy

1. CBC, LFT, GGT
2. Imaging - abdominal US, HIDA scan with phenobarbital pretreatment, liver biposy may be necessary

Incision: right upper quadrant transverse versus right subcostal
Cholangiogram
Dissection of the biliary structures (scar) to the porta hepatis at the level of portal vein bifurcation
Construction of the Roux limb (45 cm)
Portoenterostomy anastomosis

1. Complete blood count and comprehensive metabolic panel, is necessary.
2. Ultrasound of the right upper quadrant should be the first diagnostic imaging study performed.
3. The ideal imaging modality is MRCP .If MRCP demonstrates a choledochal cyst or the patient presents with obstructive jaundice, ERCP or percutaneous transhepatic cholangiography should be performed because it can confirm the diagnosis and offer therapeutic intervention. A cholangiogram can be performed to fully define the biliary anatomy with both of these modalities.
CT, HIDA, ultrasound, and postcholecystectomy cholangiograms may all raise suspicion for choledochal cyst. They lack the ability to fully define the biliary anatomy and should be followed by one of the above modalities. However, ultrasound may be sufficiently diagnostic in infants.

Type I: cyst excision followed by Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy
Type II: resection of the diverticulum off the side of the common bile duct
Type III: endoscopic sphincterotomy versus transduodenal excision and sphincteroplasty
Type IV: cyst excision followed by Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy. Hepatic resection should be considered if cysts are limited to one lobe.
Type V: typically, liver transplant required to treat entire disease

More than 95% of neonates pass meconium in the first 48 hours of life, and failure to pass meconium is typical of Hirschsprung disease.
However, some infants with Hirschsprung disease do pass meconium.
In an infant who has not passed meconium in the first 48 hours of life, the possibility of Hirschsprung disease should be entertained.
Children with Hirschsprung disease lack the rectoanal inhibitory reflex (RAIR).
This reflex may be absent in other conditions as well, and some children have a false-positive test, so any patient with an absent RAIR must undergo a rectal biopsy for confirmation of the diagnosis.

1. The classic finding is a "transition zone" in the rectosigmoid (See image below).
2. Other findings suggestive of Hirschsprung disease include a rectosigmoid ratio of less than 1.0; retention of contrast at 24 hours; and in the case of total colonic Hirschsprung disease, a foreshortened “question mark” colon.
3. Suction rectal biopsy - Typical features of the disease include: Absence of ganglion cells, Presence of hypertrophic nerves, Abnormal pattern of cholinesterase staining, Absence of calretinin staining
- To be considered adequate, the biopsy must be taken from the rectum at least 1 cm above the dentate line and must include both mucosal and submucosal layers.
4. Determine length of aganglionic segment - Laparotomy or laparoscopy must be done, with biopsies of the colon looking for ganglion cells on frozen section. The suspected transition zone on contrast enema does not always correlate with the pathologic transition zone.

Swenson procedure: excision of the aganglionic bowel with end-to-end anastomosis
Pros: transanal approach possible, with minimal residual aganglionic rectum remaining
Cons: risk of injury to pelvic nerves and blood vessels or injury to urethra or vagina

excision of the aganglionic bowel with mucosectomy at the bottom of the rectum and pull-through of the normal bowel within a cuff of aganglionic muscle
Pros: transanal approach possible, with less risk of injury to pelvic structures, urethra, and vagina
Cons: possible development of obstruction from an overly long cuff

retention of a short segment of the aganglionic rectum with posterior pull-through of the normal bowel and a side-to-side stapled anastomosis
Pros: less pelvic dissection and lower incidence of anastomotic stricture
Cons: transanal approach not possible, and potentially higher risk of constipation due to retained aganglionic bowel

HAEC is the most common cause of death in children with Hirschsprung disease.
Early intervention with intravenous fluids, nasogastric decompression, systemic broad-spectrum antibiotics, and frequent rectal irrigations is crucial to prevent systemic sepsis in these children.
HAEC can present similarly to gastroenteritis, but because it can be fatal, all children with symptoms should be treated as if they have HAEC even if the diagnosis is not clear.

Most children with Hirschsprung disease can safely undergo a pull-through procedure performed as a single stage without a defunctioning stoma.
Colostomy should be considered as a first stage in children with severe enterocolitis or massively dilated proximal bowel and in situations where reliable frozen-section interpretation is not available.
Ileostomy should be performed as a first stage in children who present with cecal perforation or in children with total colonic aganglionosis.

Neonates who present with intestinal obstruction must be resuscitated using intravenous fluids, nasogastric decompression, and rectal irrigations. Once the obstruction resolves, infants can often be breastfed as long as rectal irrigations are continued.
Older children who present with chronic constipation should be managed with enemas to decompress the rectum prior to pull-through.

Biopsies are performed to determine where the transition zone is. These can be done laparoscopically, through an umbilical incision, or via laparotomy.
Once the transition zone has been determined using frozen section, the aganglionic colon is resected and the normally innervated bowel is anastomosed just above the anal sphincter. Certain maneuvers are required to achieve adequate length without compromising blood supply to the anastomosis or producing excessive tension.
For a rectosigmoid transition zone, the ganglionic bowel can usually be brought down to the anus without any additional maneuvers.
For more proximal transition zones, additional length can be obtained by mobilizing the left colon and taking down the splenic flexure.
For a transition zone proximal to the midtransverse colon, the middle colic vessels must be divided. The surgeon must ensure that the marginal artery is intact.

Anastomotic leak usually occurs in the first week after a pull-through operation and presents with fever, abdominal pain, and vomiting. Often, peritoneal signs are apparent on examination, and usually, free air is evident on an abdominal radiograph.
Management usually includes revision of the anastomosis and defunctioning stoma.

Usually presents in the first few months after the surgery. Mild strictures can be managed with dilatation, but severe or recalcitrant strictures may require revision of the pull-through.

Relative preoperative targets include improvements in hypoxia (ie, fraction of inspired oxygen [FiO2] < 0.5), normalization of blood pressure, adequate tissue oxygen delivery/perfusion, and stabilization of pulmonary hypertension. The Congenital Diaphragmatic Hernia module contains a detailed discussion of this condition.
Optimal timing of surgical repair, particularly in relation to extracorporeal membrane oxygenation (ECMO) support, remains controversial. Both minimally invasive surgery (MIS; usually via thoracoscopy) and open surgery (usually via subcostal laparotomy) have defined and theoretical advantages and disadvantages.
In patients who require ECMO support who have not undergone previous repair, a defined early or late repair strategy is reasonable. Preoperative optimization of anticoagulation and the addition of aminocaproic acid minimize the bleeding risk

ultrasound and magnetic resonance imaging (MRI) are the two dominant modalities used to diagnose and risk-stratify fetuses with CDH.
Fetal diagnosis occurs in 50% to 80% of cases, usually at the 20-week screening ultrasound. The lung-to-head ratio (LHR) and liver location should be identified and are important for risk stratification.
Fetal MRI can provide even higher quality fetal pulmonary imaging and is used to obtain the fetal lung volume, an additional measurement for risk stratification.

Classic findings on chest x-ray of newborns with CDH include intrathoracic bowel loops, cephalic gastric displacement (as seen when the orogastric tube hooks toward the thorax), and mediastinal shift away from the defect.
Early echocardiography can identify anatomic cardiac defects, general cardiac function, and presence and severity of pulmonary hypertension

initial resuscitation include early intubation, ventilator management (see below), intravenous access, arterial access, and orogastric decompression.
To guide resuscitation, chest radiography, echocardiography, vital signs/oxygenation monitoring, and serial arterial blood gas monitoring are useful.
It is paramount that ventilator management and principles of ventilation are understood. Conventional ventilation, including pressure-limited ventilation and permissive hypercapnia, is the optimal initial approach. Targets should be established and defined limits specified.
Adjuncts such as high-frequency oscillatory ventilation, followed by extracorporeal life support or extracorporeal membrane oxygenation (ECMO), may be necessary.
Patients who do not stabilize with ventilator support are candidates for ECMO. Both venovenous and its venoarterial approaches are acceptable

appropriate positioning, laparotomy incision (usually subcostal), reduction of herniated contents, exposure of the posterior rim of the diaphragm, assessment of the defect, primary closure versus fashioning/fixation of the patch/muscle flap, and abdominal closure, with careful monitoring throughout the case.

open approach while on ECMO includes preoperative preparation focused on optimization of coagulation/anticoagulation, meticulous hemostasis, minimal dissection, and tube thoracostomy.

Inguinal canal (from external ring to the upper scrotum)
Base of the penis
Thigh
Opposite hemiscrotum
Perineum

Inguinal hernia
Epididymal abnormalities
Hypospadias
Prune belly syndrome
Posterior urethral valves
Anomalies of the upper urinary tract

An undescended testis is either not palpable or is palpable but cannot be brought into the scrotum on physical examination. If it can be brought to the scrotum, it does not remain there without constant traction.
A retractile testis resides in the upper inguinal canal, where it is palpable. Once a retractile testis has been manipulated into the scrotum, it will remain in the scrotum without traction.
A retractile testis will descend in response to administration of human chorionic gonadotropin (hCG), whereas an undescended testis will not.

In a patient with an undescended testis that cannot be repositioned into the scrotum nonoperatively, surgical intervention should begin with a reexamination under anesthesia in case of a nonpalpable testis.
Should the testis remain nonpalpable, it is appropriate to proceed with either an inguinal exploration or diagnostic laparoscopy with supervision and minimal assistance from the staff surgeon.
In a patient with an intra-abdominal undescended testis, one may use the Fowler-Stephens approach for division of the testicular vessels and preservation of the vasculature arising from the peritoneum surrounding the vas deferens as a vascular pedicle to the testicle when it is placed into the scrotum.
For more information, see Orchiopexy.

In an individual with an undescended testis, orchiopexy should be performed after 6 months of age and definitely by one year of age to mitigate the effects on spermatogenesis which start to appear after 12 months of age.
Orchiopexy should be recommended for an individual with either a primary or acquired undescended testis that will not remain in the scrotum without traction.

Repeat examination under anesthesia
Diagnostic laparoscopy. This is appropriate when the undescended testis is not palpable in the extra-abdominal tissue.
Diagnosis of atrophic testis occurs when a blind-ending vas deferens and atretic testicular vessels are found on diagnostic laparoscopy.
Dissection of the likely hernia sac
Creation of a subdartos pouch
Possible inferior epigastric vessel ligation. This is indicated when the spermatic cord is not long enough to reach the scrotum without division of the vessels.
Guidance of the testis into the scrotum
Securing the testis
Testis biopsy and measurement
Closure

Diminished fertility is likely.
There is an increased risk of testicular malignancy, and self-examinations are mandatory.

A: Isolated esophageal atresia without TEF, 8%
B: Esophageal atresia with proximal TEF, 2%
C: Esophageal atresia with distal TEF, 85%
D: Esophageal atresia with proximal and distal TEF, 1%
E: Isolated TEF without esophageal atresia (H-type), 4%

made based on the inability to place an orogastric tube into the stomach (usually beyond 10 cm), with coiling of the tube in the thoracic inlet demonstrated on "babygram."
Equivocal cases may benefit from adjunct testing, such as a contrast esophagram (pouchogram), to rule out iatrogenic injury and to delineate the anatomy.
Additional studies, such as CT, MRI, esophagoscopy, and bronchoscopy, are rarely indicated to make the diagnosis of esophageal atresia.
Initiation of a workup for the condition known as VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities), with an echocardiogram, renal ultrasound, spinal ultrasound, and radial limb evaluation, is mandatory in all confirmed cases.

A right thoracotomy approach is made because the aortic arch is usually left-sided.
The incision is made at the fourth intercostal space.
Extrapleural dissection is used to gain access to the posterior mediastinum.
The azygous vein is ligated.
The distal fistula is identified at its insertion into the trachea and transected.
The tracheal opening is closed with interrupted sutures.
The upper pouch is mobilized from the thoracic inlet and anastomosed to the lower pouch using interrupted sutures.
Minimally invasive techniques of repair with thoracoscopy are being increasingly applied

Low birth weight (eg, < 1500 g, controversial)
Long-gap esophageal atresia (type A or B)
Instability secondary to a major cardiac anomaly

A distal tracheoesophageal fistula is located on the posterior membranous wall of the trachea.
A distal fistula at the carina suggests a longer gap than a fistula to the distal trachea.
If possible, an endotracheal tube should be positioned distal to the fistula to minimize insufflation of the gastrointestinal tract.
A proximal tracheoesophageal fistula located in the upper trachea is much less common and usually more subtle in appearance.

Avoidance of positive pressure ventilation (including endotracheal intubation) if possible
Use of 10F Replogle tube in the upper esophageal pouch on continuous suction
Head of the bed at 45 degrees
Use of proton pump inhibitors (aspiration prophylaxis: controversial)
Use of intravenous broad-spectrum antibiotics (aspiration prophylaxis: controversial)

Esophageal anastomotic stricture: esophagram
Recurrent/missed tracheoesophageal fistula: prone pull-back esophagram and rigid bronchoscopy
Gastroesophageal reflux disease: upper gastrointestinal contrast study, pH probe, and impedance study
Vocal cord dysfunction: laryngoscopy
Laryngeal cleft: rigid bronchoscopy
Tracheomalacia: rigid bronchoscopy under spontaneous ventilation
Tracheal pouch: rigid bronchoscopy

Gastroschisis is a defect often to the right of the umbilicus, whereas omphalocele is a defect through the umbilicus.
Gastroschisis has no covering membrane, whereas omphalocele is characterized by an intact membrane. However, the membrane may rupture.
Gastroschisis often involves only bowel, whereas omphalocele can include herniated liver, stomach, bowel, and other organs.

Patients with omphalocele do not have dysmotility and will likely tolerate feeds sooner than those with gastroschisis.
Enteral feeds can be started once bowel function returns.
If there is no bowel function 4 to 6 weeks after gastroschisis closure, a contrast study is considered to evaluate for an intestinal atresia.

Nearly all patients with abdominal wall defects have intestinal rotational anomalies; however, volvulus is rare. Patients may present with adhesive bowel obstruction later in life.
Infants with gastroschisis are at increased risk for associated intestinal atresias.
Gastroesophageal reflux disease is present in 90% of infants with gastroschisis and 43% of those with omphalocele.
Of newborns with gastroschisis, 39% have undescended testicles. Half descend spontaneously in the first year of life.

For infants with NEC and no sign of intestinal necrosis or perforation (stages I-IIIA), nonoperative management is the recommended treatment. This includes prompt initiation of broad-spectrum antibiotics, discontinuation of enteral feeds, bowel rest/decompression, and nutritional support with parenteral nutrition and intravenous fluid resuscitation.
The antibiotic regimen should cover the most common organisms seen in NEC as well as nosocomial nursery flora. Common regimens include vancomycin and gentamicin or a third-generation cephalosporin. Fungal coverage should be considered in infants with a protracted course of disease.
Infants should be monitored with frequent physical examinations, serial platelet/leukocyte counts, and two-view x-rays every 6 to 8 hours. As they improve, the frequency of radiographs can be decreased.
Infants should remain nothing-by-mouth (NPO) and on broad-spectrum antibiotics for 7 to 14 days.

Absolute indications: stage IIIB disease (pneumoperitoneum), abdominal compartment syndrome, or positive paracentesis
Relative indications: stages IIB and IIIA disease with failure to improve despite medical management (clinical deterioration), as well as abdominal wall erythema, portal venous gas, fixed loop, or complex ascites on ultrasound

Primary peritoneal drain in a very low-birth-weight neonate (less than 1.5kg) with pneumoperitoneum
Resection of focal disease with bowel diversion
Resection of all frankly necrotic bowel in multifocal disease with temporary closure and repeat laparotomy
Closure of the abdomen and discussion with the family and health care team in the setting of NEC totalis, a variant where gangrene destroys the majority of the small bowel.

A transverse supraumbilical incision in an infant provides wide access to the entire peritoneal cavity.
The presence of free succus or purulent peritonitis is an indication of intestinal perforation that will require resection. Although peritoneal cultures often grow Klebsiella species, Escherichia coli, Enterobacter species, and yeast, culture information rarely guides antibiotic therapy, and the utility of peritoneal cultures is questionable.
The terminal ileum is most frequently involved, followed by the colon. However, an inspection of the entire intestinal tract should be undertaken to determine the extent of intestinal involvement.
In unstable patients with large areas of marginal intestine, resection of frankly perforated intestine and temporary abdominal closure is often necessary to increase the likelihood of bowel preservation.
Primary peritoneal drainage is achieved by making an approximately 1-cm full-thickness incision in the right lower quadrant of the abdomen and placing a long ¼-inch Penrose drain after manually expressing any stool and pus from the peritoneal cavity and irrigation with warm saline. The drain may also be brought out on the contralateral side to create a “bucket-handle” drainage system.

Resection with primary anastomosis can be performed in carefully selected patients who have focal, sharply localized disease with a normal appearance of the rest of the bowel and hemodynamic stability.
Resection with diversion is appropriate for patients who are unstable but have a focal region of necrosis or perforation.
Patchy, interrupted regions of bowel that appear nonviable are frequently encountered. The "clip-and-drop" technique involves resection of perforated intestine or regions of intestine with significant pneumatosis and retention of all marginal-appearing bowel. Individual segments can be loosely approximated or aligned over a catheter with plans for a close-interval second-look operation to allow for better delineation of viable versus nonviable bowel.

A prenatal ultrasound demonstrating a double-bubble sign and polyhydramnios is often associated with duodenal atresia.
Jejunoileal atresia is more difficult to diagnose on prenatal ultrasound because findings are nonspecific, such as polyhydramnios or dilated loops of bowel.
Pathognomonic findings for meconium pseudocyst on prenatal ultrasound include dilated loops of bowel, a meconium-filled pseudocyst, and intra-abdominal calcifications (due to prior intestinal perforation or necrosis)

duodenal atresia, malrotation with midgut volvulus, jejunoileal atresia, meconium ileus, and more distal forms of obstruction, such as Hirschsprung disease or imperforate anus.

midgut volvulus is an emergency laparotomy and Ladd procedure, whereas the management for duodenal atresia is a diamond duodenoduodenostomy, which can be safely delayed in children with complex heart disease and performed electively when these patients are hemodynamically stable.

. Babies with simple meconium ileus present with obstruction of the distal ileum secondary to the thick inspissated meconium. Complicated meconium ileus refers to an intrauterine perforation causing either diffuse peritonitis (meconium peritonitis) or a large cyst filled with air trapped by adhesions (meconium pseudocyst). Simple meconium ileus may be managed nonoperatively with water-soluble contrast enemas, whereas complicated meconium ileus often requires a laparotomy.

. Babies with simple meconium ileus present with obstruction of the distal ileum secondary to the thick inspissated meconium. Complicated meconium ileus refers to an intrauterine perforation causing either diffuse peritonitis (meconium peritonitis) or a large cyst filled with air trapped by adhesions (meconium pseudocyst). Simple meconium ileus may be managed nonoperatively with water-soluble contrast enemas, whereas complicated meconium ileus often requires a laparotomy.

A Kocher maneuver must first be performed to free the proximal and distal ends of the duodenum.
A catheter should be gently inserted into the distal duodenum and flushed with normal saline to rule out concomitant distal intestinal atresia (incidence is approximately 1%-3%).
The diamond duodenoduodenostomy should be formed so it is posterior, easily draining, and tension free.
Laparoscopic duodenoduodenostomy can be safety performed.
A tapering duodenoplasty with suture plication or resection using a gastrointestinal anastomosis (GIA) stapler may be performed when there is gross dilatation of the proximal duodenal segment. The tapering should be positioned along the antimesenteric border of the bowel to avoid the ampulla.
Assess for second, distal obstructions.

Resection of the large, dilated proximal bowel is one option to decrease a large size mismatch if it will not result in short bowel syndrome.
Tapering enteroplasty is an option for a large size mismatch that will preserve bowel length.
A Cheatle slit along the antimesenteric border of the distal segment is another option in a large size mismatch.
Assess for second or multiple atresias or webs.

, identification of the obstruction, enterotomy with intraluminal irrigation using warmed saline or 2% to 4% N-acetylcysteine. Options for closure vary depending on the situation and include primary closure of the enterotomy, creation of a proximal stoma with a distal mucus fistula for continued irrigations postoperatively, or creation of a catheter enterostomy, which also allows for continued irrigations postoperatively but avoids a second operation for stoma takedown.

may be treated with laparotomy and usually initially require source control, often with the placement of multiple peritoneal drains and, occasionally, creation of a stoma, if possible. It is important to assess the residual intestinal length and conserve bowel length to avoid short bowel syndrome. However, contamination and peritonitis are often quite significant at the first operation, and therefore intestinal length may be better assessed at the next operation.

Simple meconium ileus may be successfully treated with a water-soluble contrast enema. Diatrizoate meglumine and diatrizoate sodium solution (Gastrografin) have remained the standard. Treatment success is variable but is as high as two-thirds of patients. Treatment may require more than one enema.
Care must be taken to aggressively resuscitate the patient with fluid before, during, and after a Gastrografin enema because transient osmotic diarrhea and osmotic diuresis will occur.
Complications of this nonoperative technique include worsening obstruction and intestinal perforation. The surgeon should have a low threshold for operative intervention in patients who do not respond well.

Type I:
Mucosal (membranous) atresia with intact bowel wall and mesentery
Type II:
Two blind ends are separated by a fibrous cord with intact mesentery.
Type IIIa:
Two blind ends are completely separated, and the mesentery has a V-shaped defect.
Type IIIb:
This is similar to type IIIa; however, the distal bowel and mesentery are twisted on themselves in an "apple-peel" deformity.
Type IV:
Multiple atresias, sometimes referred to as a "string of sausages"

Initially, the stoma and mucus fistula should be irrigated daily with N-acetylcysteine until bowel function returns. Then the irrigations may be stopped, the Replogle tube removed, and enteral feeds initiated.
Breast milk is preferred, but if it is unavailable, formula may be given. Enteral feeds are usually started at low volume (10-20 cc/kg/day) and advanced to goal over several days.
The pediatric pulmonology team should be consulted. The patient should undergo appropriate testing for cystic fibrosis, and if it is confirmed, pancreatic enzymes should be initiated with enteral feeds.
The stoma may be taken down 6 to 8 weeks postoperatively if the child is clinically well and thriving.

A thorough history and physical that focuses on tolerance of feeds, the presence and timing of abdominal distention, and the color and frequency of emesis should be performed.
An exam should evaluate the degree of distention, abdominal tenderness, color of the abdominal wall, as well as the existence of the anal opening. The presence or absence of inguinal hernias should be evaluated.
A nasogastric tube should be inserted for decompression, and the quality of the output should be checked.
Laboratory tests should include electrolytes and acid-base balance and guide fluid resuscitation as appropriate.
Imaging includes plain abdominal radiographs in both supine and left lateral decubitus positions to evaluate for free intraperitoneal air as well as any fixed intestinal loops. An upper gastrointestinal series can evaluate for intestinal malrotation.
If the obstruction is thought to be more distal, a contrast enema can distinguish between a distal intestinal atresia, Hirschsprung disease, and nonsurgical causes of obstruction, such as a meconium plug.
The radiological findings that establish the diagnosis of Jejunoileal atresia are dilated intestinal loops on plain films and a micro-colon on a contrast enema.

Perform visual inspection of the bowel. Ensure that the bowel is viable with adequate blood supply. Malrotation of the bowel should also be ruled out by inspection, and a concurrent Ladd procedure should be performed if malrotation of the bowel is found.
Rule out additional obstructions or atretic segments. Multiple atretic segments may be present and should be addressed concurrently. Passing a catheter to ensure luminal patency can help avoid a missed atresia. Approximately 5% of cases have second atresias.
Colonic patency should be confirmed either preoperatively by contrast enema or intraoperatively.
Bowel length should be measured before resection of dilated or atretic segments. Should the length place the patient at risk for short bowel syndrome, the surgeon must consider whether segments would be amenable to tapering enteroplasties, Cheatle incisions, or other techniques that can minimize the length of resected bowel.
Appropriately modify the operative plan based on lesion location, length of bowel remaining, and condition of the patient and bowel. A type I atresia, in which there is only a mucosal atresia and intact bowel wall and mesentery, will be repaired differently from a type IIIb atresia, which is defined by a large mesenteric defect with apple-peel deformity.
Consider placement of a feeding gastrostomy tube, if short bowel syndrome is a concern since these patients often require continuous gastric feedings during their intestinal rehabilitation.

lower border of the sternocleidomastoid muscle, through the carotid bifurcation, and over the glossopharyngeal and hypoglossal nerves to enter the lateral pharyngeal wall.

Initial treatment with antibiotics and possible aspiration is preferred to incision and drainage.
Antibiotics covering skin flora should offer sufficient coverage, unless connection to the oropharynx is suspected.
Any infection present should be treated prior to surgery to minimize the risk of damage to nearby structures or incomplete resection.

CT or MRI of second branchial cysts may show a characteristic beak sign between the external and internal carotid arteries.
Barium esophagram may be particularly helpful in identifying a third or fourth branchial fistula or sinus and may be used in conjunction with CT. An opening in the pharynx may be identified by endoscopy which should be included in the evaluation of any lesion thought to be third or fourth branchial anomaly.
Patients with first branchial cleft anomalies and hearing impairment should be evaluated for renal malformations, because first branchial cleft anomalies may be associated with hearing impairment and as part of the branchio-otorenal syndrome or the branchio-otic syndrome.

Proper head positioning
Marking or cannulation of the tract if possible. A lacrimal probe or monofilament suture may help to cannulate the tract.
Optimal planning of skin incision. The recommended skin incision is a horizontal (along Langer lines) elliptical incision around the cutaneous opening.
Dissection along the tract
Extent of the fistula. Manual palpation with a finger, or use of a bougie in the pharynx, may facilitate identification of the upper extent of the fistula. This should be ligated with an absorbable suture.

While performing a thyroglossal duct cyst resection (Sistrunk procedure), be able to make an infrahyoid transverse skin incision directly over the cyst and dissect the cyst, tract, and hyoid bone for en bloc excision up to the foramen cecum.

It is necessary to independently suture ligate the tract at the base of the tongue.
To facilitate dissection up to the foramen cecum, the anesthesiologist can apply downward pressure on the tongue base.
A visual depiction of the procedure may be seen here: American Pediatric Surgical Association, Pediatric Surgery Not A Textbook (NaT): Thyroglossal Duct Cyst Excision.

Obtain a chest CT scan.
Calculate the Haller index of a patient with pectus excavatum, given the dimensions of the chest on CT scanning.
Calculate the correction index in a patient with pectus excavatum.
Order pulmonary function tests routinely. A patient with cardiac symptoms should have an electrocardiogram and an echocardiogram.
Eval metal allergy, connective tissue diseases

Mark the chest at the deepest point of the excavatum as well as the rib spaces to determine where to enter and exit the chest.
Create lateral transverse incisions near the anterior axillary line (or lateral curvilinear inframammary incisions in a female patient), and perform dissection towards the marked entrance and exit sites on both sides.
Utilize thoracoscopy (or a subxiphoid incision) to guide the bar passer and bar across the anterior mediastinum.
In patients where thoracoscopy is used, the bar passer enters the chest under direct vision at the previously marked site on the right and is used to create a space between the pericardium and the posterior surface of the sternum. The bar passer is made to exit the left chest at the previously marked exit site.
Umbilical tapes or a chest tube are attached to the end of the bar passer and brought back out through the right side.
The previously bent pectus bar is passed across the mediastinum along the same dissection path as the bar passer, and the bar is flipped into place, correcting the deformity.
Stabilization techniques should be used to keep the bar in place during excavatum surgery. These techniques may include transverse stainless steel stabilizers, sutures around the ribs, or other methods to ensure the bar does not rotate or migrate.

Make a transverse inframammary incision in the center of the chest.
Create skin flaps that are superior to the angle of Louis and inferior to the xiphoid.
Create pectoralis major flaps superiorly to expose the highest affected costal cartilage.
Remove affected costal cartilages while preserving the perichondrium.
Perform a sternal osteotomy to move the sternum more posteriorly. Stainless steel wires may be used to hold the sternum in this new position.
Close muscles and skin over a drain.