Pediatric Surgery

Vic V. Vernenkar, D.O. St. Barnabas Hospital Dept. of Surgery

Epiglottitis
H. Influenza is most common organism A lateral xray may show edema of the epiglottis (birds 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 Fistula
A 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 common 50% of neonates with TEF have an associated anomaly (cardiovascular most common) GI malformation GU anomalies

Tracheoesophageal Fistula
Proximal and distal TEF Diagnosis Inability to pass nasogastric tube CXR to deternime length of esophageal gap Abdominal Xray with air in the stomach excludes esophageal atresia Treatment Right thoracotomy thru 4th intercostal space Proximal 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 birds 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 Ladds 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 Atresia
A newborn full term neonate with Downs 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 emesis Abdominal distension is absent

Duodenal Atresia
Check for patent anus Rule out anorectal anomalies Abdominal 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 Atresia
Abdominal distension is usually present with distal atresia. Abd. X-ray demonstrates multiple distended loops of bowel with A-F levels Contrast 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 Atresia
Caused by in utero mesenteric vascular accident. Similar to above in presentation Abdominal distention present X-rays show obstructive picture Contrast 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. (Nacetylcysteine)

Hirchsprungs 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.

Hirchsprungs Disease
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.

Hirchsprungs Disease
Diagnostic work-up includes: Contrast enema showing a contracted rectum with dilated bowel above. Failure to evacuate contrast 24h later can be diagnostic.

Hirchsprungs Disease
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

Hirchsprungs Disease
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.

NEC
A 7 day old premature infant has emesis, abdominal distension, and bloody stools. Differential includes: NEC Malrotation

NEC
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.

NEC
Surgical treatment for : Free air Abdominal wall erythema, cellulitis Worsening acidosis Hyperkalemia Palpable mass Worsening distension Overall deterioration

NEC
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 Stenosis

Mild jaundice in 5% due to reduced glucoronyl transferase activity Dx is confirmed by US Pyloric diameter > 1.4 cm Pyloric wall >0.4cm Pyloric channel > 1.6cm Ramstedt 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

Intussusception
Surgery 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) Meckels diverticulum is most common lead point In adults it is malignancy! Currant jelly stool!

Meckels Diverticulum
A healthy 2 year old presents with painless bloody stools. Patent vitelline duct True diverticulum Located on anti-mesenteric border A technetium-99 scan can assist with diagnosis and localization. Segmental resection is indicated for symptoms. Rule of twos:

Meckels 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)

Gastrochisis
Eviscerated intestines have no covering 2-5 cm defect to right of umbilicus Intestines 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 common Pericardial, sternal, diaphragmatic Musculoskeletal GI,GU Beckwith-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 left Morgnani (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!

Wilms 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

Wilms 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%) Wilms 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 puberty AFP elevated Surgical 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%)

Hernia
10-30% 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 births Incarceration very rare If 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.