Pediatric Surgery

transcript

Pediatric Surgery

Vic V. Vernenkar, D.O.

St. Barnabas Hospital

Dept. of Surgery

EpiglottitisH. Influenza is most common organismA lateral xray may show edema of the epiglottis (bird’s beak)Orotracheal intubation should be performed in the OR so that an open tracheostomy can be done if needed.Never nasotracheally intubate a child because the angle between the superior and inferior glottis is too large.

Tracheoesophageal FistulaA newborn infant has excessive salivation, choking, and regurgitation with feeding.Results from abnormal ingrowth of ectodermal ridges during the 4th week of gestation.25-40% of neonates are premature, low bith weight.A maternal history of polyhydramnios is common50% of neonates with TEF have an associated anomaly (cardiovascular most common)GI malformationGU anomaliesSkeletalCNSAssociated with VACTERL (vertebral, anorectal, cardiac, TE, renal, Limb)5 types of TEFProximal esophageal atresia with distal TEF (85%)Isolated esophageal atresiaH-type TEF without atresiaProximal TEF with distal atresia

Tracheoesophageal FistulaProximal and distal TEFDiagnosisInability to pass nasogastric tubeCXR to deternime length of esophageal gapAbdominal Xray with air in the stomach excludes esophageal atresiaTreatmentRight thoracotomy thru 4th intercostal spaceProximal esophagus blood supply is from thyrocervical trunk

Tracheoesophageal Fistula

Distally supplied by more tenuous intercostals

Operation includes TEF ligation, transection, and restoration with end-to-end anastamosis.

POD 5-7 esophagram, if no leak, feed, remove drain.

Tracheoesophageal Fistula

Early complications include:Anastamotic leak, recurrent TEF, tracheomalacia.Late Complications include:Anastamotic stricture (25%), reflux (50%), dysmotility (100%).Proximal atresia with distal TEF most common.

Immunoglobulins

Which immunoglobulin is secreted in breast milk?Which immunoglobulin does not cross blood brain barrier?IGA is most common antibody in breast milk, the gut, saliva, bodily secretions.IGM is large and does not cross the placenta.

Resuscitation

An 8 year old boy presents following a bicycle crash with a ruptured spleen.What is the best indicator of early shock?Tachycardia in childhood is defined as a heart rate >150 for a neonate, >120 in the first year, >100 after one year. It is the best indicator of shock.Fluid resuscitation in children:

Resuscitation

20cc/kg crystalloid bolus for trauma

If shock persists after a second bolus, give blood at 10cc/kg

Children have a lower GFR in comparison to adults.

Malrotation

A healthy infant presents with bilious vomiting, abdominal distension, and shock.

A surgical emergency, bilious vomiting in a newborn is malrotation until proven otherwise.

Malrotation

During 6-12 week of gestation, the intestine undergoes evisceration, elongation, and eventual return to the abdominal cavity in a 270 degree counterclockwise rotation with fixation.

Malrotation is associated with abnormal rotation and fixation.

Malrotation

Ladds bands extend from the colon to the duodenum, causing duodenal obstruction and biliary emesis.Midgut volvulus refers to the narrow based mesentery twisting around the SMA (usually clockwise).This results in obstruction and vascular compromise.

Malrotation

Most develop symptoms in first month of life.

If patient stable do UGI series (gold standard).

See bird’s beak in third part of duodenum

Ligament of Treitz is right of midline.

Malrotation

Midgut volvulus is a surgical emergency.

Volume resuscitation is essential.

If patient in shock, no studies are warranted.

Malrotation

Immediate exploration to avoid loss of small bowel and resultant SBS, death.Surgical treatment is the Ladd’s procedure.This consists of division of bands, correction of malrotation, restoration of broad based mesentery, appendectomy (because it is in the wrong place in LUQ).

Duodenal AtresiaA newborn full term neonate with Down’s syndrome had bilious vomiting during the first day of life. The abdominal exam is normal.Duodenal atresia. Malrotation can present similarly but less common.Failure of recanalization during 8-10th week of gestation.Presents in first 24hrs of life.Trisomy 21 is present in about 25%Characterized by bilious emesisAbdominal distension is absent85% distal to ampulla of vater

Duodenal AtresiaCheck for patent anusRule out anorectal anomaliesAbdominal x-ray reveals double bubble sign:Air in the stomach, and 1st and 2nd portions of duodenum.If there is no distal air, the diagnosis is secure.If there is distal air, and urgent UGI needed to rule out midgut volvulus.Surgical treatment is a duodenoduodenostomy.

Jejunoileal Atresia

A 3 day old infant has bilious vomiting, abdominal distension.

Differential includes:

Duodenal atresia

Malrotation

Meconium ileus

Imperforate anus

Hirchsprungs

Jejunoileal Atresia

Jejunoileal atresia is caused by an in utero vascular accident.

Presents within first 2-3 days

Associated with Cystic Fibrosis in 10%

Jejunoileal AtresiaAbdominal distension is usually present with distal atresia.Abd. X-ray demonstrates multiple distended loops of bowel with A-F levelsContrast enema demonstrates a micro colon and no reflux into dilated intestines.Multiple areas of involvement in 10%.Surgical correction involves end-to –end anastamosis. Preserve length to prevent SBS.

Colonic AtresiaCaused by in utero mesenteric vascular accident.Similar to above in presentationAbdominal distention presentX-rays show obstructive pictureContrast enema shows microcolon with a cut off in proximal colon.Surgical correction involves end-to-end anastamosis.Intestinal atresia can be associated with gastrochisis.

Meconium Ileus

A newborn with cystic fibrosis presents with mild abdominal distension. An X-ray demonstrates a ground glass appearing mass on the right side of the abdomen.

A gastrograffin enema may be all that is needed to treat meconium ileus, complicated cases may need surgery.

Caused by obstruction of terminal ileum with meconium.

Meconium Ileus

15% have CF

Simple MI can be treated with enemas.

Complex MI requires enterotomy, resection, evacuation.

Mucomyst enemas can be used for this. (N-acetylcysteine)

Hirchsprung’s Disease

A full-term neonate has bilious emesis during first and second days of life. The abdomen is distended. X-rays show dilated loops of small bowel. A contrast enema reveals a narrow rectum, compared to the sigmoid. The baby failed to evacuate the contrast the following day.

A bedside suction rectal biopsy at least 2cm above dentate line is the gold standard test.

Failure of the normal migration of neural crest cells.

Absent ganglia in the myenteric and submucosal plexus.

The absence always occurs in the distal rectum and extends proximally.

80-85% localized to rectosigmoid.

Diagnostic work-up includes:

Contrast enema showing a contracted rectum with dilated bowel above.

Failure to evacuate contrast 24h later can be diagnostic.

Rectal biopsy is required to confirm absence of ganglion cells and nerve hypertrophy.

Surgical treatment:

Soave endo-rectal pull through with removal of the diseased distal bowel with coloanal anastamosis

Children who present acutely ill may need staged procedure with colostomy.

Need to do intraoperative frozen section to help determine the anatomic location of transition zone.

Imperforate Anus

Anus absent or misplaced

Usually form anterior fistulous tract

Associated with coloanal deformity

Imperforate Anus

Divided into high and low malformations with respect to the levators.

High: fistula to bladder, vagina, or urethra, are treated with colostomy and posterior sagital anorectoplasty (PSARP), and genitourinary reconstruction if necessary.

Low: PSARP

Imperforate Anus

Preop anal dilatation may be needed to prevent stricture.

A colostomy is generally not needed to treat a low (below levator) imperforate anus.

A 7 day old premature infant has emesis, abdominal distension, and bloody stools.

Differential includes:

Malrotation

More common in premature infants after initiation of feeding.Abdominal x-ray often reveals pneumotosis intestinalis or portal vein air.Treatment is conservative: NPO, NGT, antibiotics, serial x-rays.Medical management is successful in 50% of cases.

Surgical treatment for :

Free air

Abdominal wall erythema, cellulitis

Worsening acidosis

Hyperkalemia

Palpable mass

Worsening distension

Overall deterioration

Surgery often involves resection of affected intestine and creation of and end ileostomy and mucous fistula.

If the neonate survives, reverse around 4-6 weeks later after a contrast study rules out strictures.

NEC is the most common cause of SBS in childhood

Abdominal erythema is an indication for surgery!

Hypertrophic Pyloric Stenosis

A 4 week old infant presents with non-bilious vomiting and hypochloremic, hypokalemic, metabolic alkalosis.

Idiopathic thickening and elongation of the pylorus causing GOO.

Age is 3-6 weeks (1 month of age)

Initially fed normally then projectile vomiting.

An “olive” is palpated in 50%

Hypertrophic Pyloric StenosisMild jaundice in 5% due to reduced glucoronyl transferase activityDx is confirmed by USPyloric diameter > 1.4 cmPyloric wall >0.4cmPyloric channel > 1.6cmRamstedt pyloromyotomy is treatment of choice (open or laparoscopic).Surgery is not an emergency (resuscitate).

Intussusception

A healthy 11 month boy presents with sudden emesis, crampy abdominal pain, bloody stools.

Intussusception is most common cause of intestinal obstruction in early childhood and classically present between ages 3 months and 3 years.

Air contrast enema is diagnostic and therapeutic.

Intussusception

Most occur before age of two.

Etiology is thought to be lymphoid hyperplasia in terminal ileum after a viral illness.

Proximal bowel (intussusceptum) invaginates into the distal bowel (intussuscipien) causing swelling, obstruction, and possible vascular compromise.

Controlled air contrast enema is successful in 90%

Intussusception

Indications for surgery:

Enema not success

Third episode

Peritonitis

IntussusceptionSurgery involves reduction, appendectomy, and bowel resection for pathology.Recurrence after radiographic or surgical treatment is 5%.Lead point present in 10% of cases (increases with age)Meckel’s diverticulum is most common lead pointIn adults it is malignancy!Currant jelly stool!

Meckel’s DiverticulumA healthy 2 year old presents with painless bloody stools.Patent vitelline ductTrue diverticulumLocated on anti-mesenteric borderA technetium-99 scan can assist with diagnosis and localization.Segmental resection is indicated for symptoms.Rule of twos:

Meckel’s Diverticulum

2% of population

2% symptomatic

2 times more in males

2 feet from valve

2 years of age or less

2 presentations (bleeding or obstruction)

2 tissue types (pancreatic, gastric)

Most common cause of bleeding in children.

Biliary Atresia

A 1 month old infant has acholic stools and persistent jaundice.

Jaundice in the newborn that persists >2 weeks is no longer considered physiologic.

Early diagnosis is critical (before 2 months) to prevent progressive liver damage.

Biliary Atresia

Hallmarks:

Bile duct proliferation

Cholestasis with plugging

Inflammatory cell infiltrate

Progression to cirrhosis

Biliary Atresia

Must rule out hepatocellular dysfunction due to infectious, metabolic, hematologic, or genetic disoders.

Elevated conjugated bilirubin?

Elevated unconjugated bilirubin?

Biliary Atresia

Ultrasound helps to determine bile duct size and if a gallbladder is present.

Bile ducts are not enlarged in atresia

If atresia is suspected do mini RUQ incision for local exploration and biopsy.

Initial goal is to establish diagnosis!

Biliary Atresia

If gallbladder is present do cholangiogram.

If cholangiogram demonstrates a patent but hypoplastic biliary system, the incision is closed.

Biliary Atresia

If a patent GB or biliary tract cannot be identified, the incision is elongated and a Kasai procedure is performed: portoenterostomy.

The fibrotic GB and extrahepatic biliary tree is dissected to the porta hepatis and resected.

Biliary Atresia

A Roux-en-Y is created and the roux jejunal limb is sutured to the porta hepatis to help reestablish bile flow from the minute bile ducts.

Liver transplant is reserved for progression to liver disease, failed Kasai, cases where delayed diagnosis.

Gastrochisis

A neonate is born with an abdominal waal defect to the right of the umbilicus. The eviscerated intestines appear thickened and do not have a peritoneal covering.

Caused by intrauterine rupture of umbilical vein. (1 vein 2 arteries)

GastrochisisEviscerated intestines have no covering2-5 cm defect to right of umbilicusIntestines are thickened, edematous and foreshortened.Associated anomalies are uncommon (except intestinal atresia in 10-15%)Perioperative management includes volume, NGT, confirmation of bowel viability and protective dressings.

Gastrochisis

Treatment:Attempt primary reduction, if unsuccessful, use a silo.A silo allows for a bedside staged closure to be followed by primary closure.During exploration confirm presence or absence of atresia.Post-op ileus common, TPN life saving.

Omphalocele

A baby is born with an abdominal wall defect. The exposed intestines have an intact peritoneal covering and appear normal.Omphalocele is associated with 40-80% incidence of another congenital anomaly!Defect thru umbilicus, 4-10cm defect.Covered, normal appearing.

Omphalocele

Associated anomalies Cardiac most commonPericardial, sternal, diaphragmaticMusculoskeletalGI,GUBeckwith-Weidmann syndrome (omphalocele, hyperinsulinema, macroglossia)

Omphalocele

Perioperative management includes volume, NGT, ID other anomalies.

Primary reduction is optimal, but if defect too large, then staged closure.

Outcomes related more to associated anomalies than to the omphalocele itself.

Congenital Diaphragmatic Hernia

A few hours after birth, a newborn develops dyspnea, cyanosis, retractions.Two types:Bochdalek (posterolateral) most common, 80% on leftMorgnani (anteromedial)CXR shows loops of intestine or gastric bubble in chest. NGT helps in diagnosis.

Congenital Diaphragmatic Hernia

Resuscitate, stabilize first!

May require ECMO

Beware of contralateral PTX due to aggressive ventilation.

Surgical repair is done through subcostal incision, with reduction of organs, closure of defect.

Primary repair is optimal but may need patch to avoid tension.

Neuroblastoma

A 2 year old boy complains of belly pain and lack of appetite. Physical reveals a large abdominal mass.

Most common malignant solid tumor in children.

Derived from neural crest tissue

May arise anywhere along sympathetic ganglia.

Most common in adrenal medulla (50%)

Neuroblastoma

Age 1-2 years

Extends across midline

Ocular involvement may present as raccoon eyes.

Calcifications on x-ray

Elevated catecholamines, VMA, metanephrines.

Due to production of hormones, children may present with flushing, HTN, watery diarrhea, periorbital ecchymosis.

Neuroblastoma

Age at presentation is major prognostic factor.

Less than 1 year >70% survival

Older than 1 year <35% survival

Good prognostic features:

Tumors with <10 copies of N-myc gene

Aneuploid tumors

Low mitosis index

Normal LDH and catecholamine levels.

Neuroblastoma

Rarely mets

If able, surgical excision is treatment of choice, chemo may be beneficial.

The N-myc gene is associated with neuroblastoma!

Wilm’s Tumor (Nephroblastoma)

Second most common solid tumor in children.

Mass palpable and HTN, hematuria

Derived from kidney

3-4 years of age

Rare to extend across midline

Wilm’s Tumor (Nephroblastoma)

No x-ray calcifications

Prognosis based on tumor grade

Mets to bone and lung

Primary surgical excision very important

Neoadjuvant chemo for large tumors

Overall survival is good (85%)

Wilm’s tumors replace renal parenchyma on CT scans whereas Neuroblastoma displaces it.

Hepatoblastoma

A 7 year old boy presents with precocious puberty and elevated Alfa fetal protein.Most common liver tumor in children.Liver cancer variant (better prognosis than hepatocellular ca in adults)Beta HCG release results in pubertyAFP elevatedSurgical resection is treatment of choice.

Mediastinal Masses in Children

A 7 year old girl presents with dysphagia. A CT scan reveals a mediastinal mass.

Most common is T-Cell lymphoma

Teratoma

Tumor (neuroblastoma, neurofibroma, neuroganglionoma, germ-cell tumors).

Most Common Malignancy in Children?

Leukemia

Hernia

A 14 month old presents with incarcerated right inguinal hernia. What do you do?

Reduce, admit, do semielective repair in future.

High ligation and division of sac.

Elective repair to avoid reincarceration (16%)

Hernia10-30% incidence of contralateral inguinal hernia.Watch out for a normal ovary in an inguinal hernia in females.Umbilical hernias occur in 10-30% of live birthsIncarceration very rareIf less than 2cm, frequently close spontaneously, closure and repair should be delayed until age of 4.If larger than 2cm, repair at time of diagnosis.

Pediatric Surgery

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