COMMON PEDIATRIC SURGERY PROBLEMS

Surgery Curriculum Conference June 13, 2012

Case 1

39 week gestational age Normal pregnancy and vaginal delivery Apgars 91,105

Started breastfeeding and started to have multiple episodes of bright yellow/green emesis

Prenatal work-up
Trisomy

21 Normal ultrasound at 18weeks

Mom has negative serologies

Case 1

Clinical examination HR 160, RR 40, BP 80/50 O2 sats 100% room air HEENT: macroglossia, epicanthic fold of the eyelid, upslanting palpebral fissures Chest: Good AE=AE Cardiac: holosystolic III/VI murmur, normals S1, S2 Abdomen: non distended, soft, nontender, no erythema, no HSM Normal female genitalia MSK/Neuro: Simian crease, slight decreased muscle tone.

Case 1

Investigations
Bloodwork
Imaging Any

other diagnostic tests?

ECHO
VSD

Normal BMP, CBC, neg cultures

Neonatal Emesis DDx

Upper

GI

Duodenal atresias/webs small bowel atresias malrotation/midgut volvulus GERD Meconium ileus pyloric stenosis Inguinal hernia NEC

Neonatal Emesis DDx

Lower

GI

Colonic atresia Meconium plug Hirschsprungs Small Left Colon Syndrome Microcolon-Intestinal Hypoperistalsis Syndrome Imperforate anus

Neonatal Emesis DDx

Medical

causes

Sepsis Metabolic disorders

Hypothyroidism Electrolyte disturbances

GERD

Radiological workup

KUB/Cross-table lateral Contrast enemas for distal obstructions UGI for malrotation/proximal atresias

Duodenal atresia

Management
NGT
Resuscitate

Surgical approach
duodenoduodenostomy

Which of the following is TRUE regarding duodenal atresia?

A. It is associated with trisomy 21 in 10% cases.

B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.

Which of the following is TRUE regarding duodenal atresia?

A. It is associated with trisomy 21 in 10% cases.

B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.

Duodenal atresia

Duodenal Atresia

Failure to recanalize lumen of duodenum after solid phase of embryologic development

Associated with Downs syndrome in 30% Atresia seen in 10% of Downs patients Vomiting can be bilious or non-bilious Abdominal X-ray shows double-bubble Best repaired by bypass -> duodenoduodenostomy or duodenojejunostomy

Distal atresias are due to vascular events

no indication to divide annular pancreas

Case 2

2 day old infant in newborn nursery Sent to NICU for evaluation of bilious emesis

Physical Examination

HR 165, RR 50, O2 sats 98 RA HEENT

Normal oropharynx Clear, AE=AE

Chest

Cardiac

Normal HS, good peripheral pulses

Abdo
Nondistended, generalized tenderness Soft, no discoloration, no masses, no HSM No inguinal hernias

Work-up
Bloodwork
Imaging

Normal embryologic rotation

Abnormal rotation and nonfixation

Management of Malrotation/volvulus

Resuscitate Urgent surgery

Steps to correcting malrotation

1. Entry into abdominal cavity and evisceration (open) 2. Counterclockwise detorsion of the bowel (acute cases) 3. Division of Ladds cecal bands 4. Broadening of the small intestine mesentery 5. Incidental appendectomy 6. Placement of small bowel along the right lateral gutter and colon along the left lateral gutter

Ladd Procedure

Malrotation

Occurs in 1/200 1/500 live births Symptomatic in 1/6000 live births 30-62% have associated anomaly Up to 75% present w/in 1st month of life Classic presentation is infant with bilious emesis
May

present as pain, duodenal obstruction, malnutrition, acute abdomen/shock

Malrotation

Due to abnormal fixation of midgut to retroperitoneum leads to narrow base of mesentery which can easily twist Ladd Procedure
Reduce volvulus by rotating counterclockwise Division of Ladds bands between cecum and duodenum/right gutter Division of adhesions to widen mesentery Run bowel to r/o obstructions Appendectomy Place bowel in nonrotated position

Case 3

2 day old infant in NICU 3 Consulted for abdominal distention and bilious emesis Work-up and differential

Pathophysiology of intestinal atresias

How would you confirm diagnosis and what would you see Classification scheme

Case 4

3 day old infant Failure to pass stools, abdominal distention and bilious emesis Work-up and differential

What other tests should be done

Sweat

test for CF Genetic testing for CFTR gene mutation

Which of the following is FALSE regarding meconium ileus?

A. Underlying diagnosis is usually cystic fibrosis.

B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (soap bubble sign). E. May be relieved by water-soluble contrast enema.

Which of the following is FALSE regarding meconium ileus?

A. Underlying diagnosis is usually cystic fibrosis.

B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (soap bubble sign). E. May be relieved by water-soluble contrast enema.

Meconium Ileus

Newborn bowel obstruction secondary to inspissated meconuim in distal ileum Enema reveals microcolon -> may be therapeutic Non-operative management successful in 2/3 OR required for perforation or failed enema
may

flush bowel with N-acetylcysteine in saline

Management
Fluid resuscitaion Gastric decompression Pulmonary support as needed Contrast enema with water soluble contrast

Failure of nonoperative management

Surgery
2-4% NAC, 50% hyperosmolar agent via appendix Alternative surgical techniques involve resection, anastomosis, and temporary enterostomy through which postoperative irrigations may be delivered

Simple vs Complicated meconium ileus

Complicated
Volvulus Perforation

resulting in meconium peritonitis

adhesive meconium peritonitis giant cystic meconium peritonitis or pseudocyst meconium ascites infected meconium peritonitis

Case 5
An 8 hr old infant drools and spits up his first feed. A tube is passed into the esophagus and a film is obtained.

What is the diagnosis?

Esophageal Atresia and Tracheoesophageal Fistula

Incomplete partitioning of primitive foregut

5 types of atresias Esophageal atresia with distal TEF most common

8%

1%

85%

2%

4%

Esophageal Atresia and Tracheoesophageal Fistula

Can be part of VACTERL anomalies

vertebral, anal, cardiac, TEF, renal, limb

Atresias detected by inability to pass NGT/OGT TEF w/o atresia presents with recurrent aspiration Low-risk infants should get primary repair
long gap (>3 vertebral bodies) repair is delayed high-risk babies get gastrostomy

Post-op complications include esophageal leak, dysmotility, GE reflux, strictures

Case 6
A listless 9-month-old boy presents with acute onset of severe intermittent abdominal pain. Rectal exam is guaiac positive. What is the most likely diagnosis? A. Meckels diverticulum. B. Acute appendicitis. C. Intussusception. D. Intestinal polyp. E. Gastritis.

A. B. C. D. E.

Meckels diverticulum. Acute appendicitis. Intussusception. Intestinal polyp. Gastritis.

Intussusception

Commonly affects children 3 months to 2 yrs

severe crampy abdominal pain (every 10-20 minutes) vomiting, currant jelly stools tender, sausage-like mass in RUQ

Telescoping of terminal ileum into large intestine Contrast enema for diagnosis will reduce 80%
air pressure to 120 mmHg, barium to 100 cm H2O 10% recurrence, often within hours

OR reduction if not reduced radiographically 5% of patients need resection

Intussusception

Plain AXR
Look

for gas in cecum

Abdominal ultrasound look for target

Which of the following statements is TRUE with respect to neonatal abdominal wall defects?

A. The bowel in omphalocele is covered by a sac.

B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.

Which of the following statements is TRUE with respect to neonatal abdominal wall defects?

A. The bowel in omphalocele is covered by a sac.

B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.

Omphalocele

Occur 1 in 5000 live births, more common in boys

over 50% have associated cardiac, GI, GU, musculoskeletal, or CNS anomalies

Herniation of abdominal contents through defective umbilical ring

overlying sac of outer amnion and peritoneum umbilical cord in continuity with sac liver involved in larger defects

High mortality (30-60%) due to other anomalies

Omphalocele

Omphalocele

Non-operative management with escharotic agent OR for reduction and closure of abdominal wall
keep intra-abdominal pressure < 20 mmHg large defects require skin flap or prosthetic Silastic silo most common, reduce daily for 3-10 days

Post-op complications include sepsis, GE reflux, inguinal hernias, abdominal wall hernia

Gastroschisis

Anterior abdominal wall defect (belly cleft)

usually to right of umbilical cord no sac or membrane covering contents exposed bowel thick, edematous, exudative peel associated intestinal atresias in 10%

Initial management
aggressive fluid replacement (2-3X normal) protection of exposed bowel w/occlusive dressing

Small bowel

Colon

Uterus + Fallopian Tube

Bladder

Stomach

Gastroschisis

Primary reduction and closure in 80-90% cases

Silastic silo if high intra-abdominal pressure may require resection if exposed bowel non-viable

Post-op complications:
abdominal compartment syndrome sepsis necrotizing enterocolitis abdominal wall cellulitis prolonged ileus short gut syndrome w/ TPN dependence

Case 7
3. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition?

A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.

Case 7

A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.

Necrotizing Entercolitis (NEC)

Idiopathic mucosal intestinal injury, may progress to transmural necrosis

1/2 patients < 1500 g (7% incidence), 80% < 2500 g at birth 90% in premature neonates

Necrotizing Entercolitis (NEC)

Signs:

feeding intolerance vomiting abdominal distention progressive sepsis autonomic instability (Apneas and Bradys) abdominal wall erythema +/- mass

Labs:

metabolic acidosis thrombocytopenia

Necrotizing Enterocolitis (NEC)

X-rays: distended loops c/w ileus, pneumatosis intestinalis May appear normal or mild ileus at first Progression demonstrates portal venous air (pathognomonic)

Necrotizing Enterocolitis (NEC)

Pathogenesis
No

single predisposing factor

milk

Prevention
Breast

Necrotizing Enterocolitis (NEC)

Medical Treatment
NPO, NGT, TPN AXR q 8 hr Usually necessitates surgery within 24 hr or not at all NPO for 10 to 14 days after radiographic evidence of disease has abated Broad spectrum Abx

Bacterial translocation Amp/Gent/Clinda or Flagyl

Necrotizing Enterocolitis (NEC)

Indications for OR are free air (absolute), fixed abdominal mass, abdominal wall erythema, failure to improve (controversial)
OR for resection of dead bowel, formation of stomas second-look laparotomy 24-48 hrs if needed Peritoneal drainage

Overall mortality 20-40% Long term complications of strictures, short bowel syndrome

Case 8
4. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis?

A. B. C. D. E.

It is more common in males. Suction rectal biopsy is rarely adequate for diagnosis. Enterocolitis is a significant cause of mortality. Disease is most often confined to the distal colon. Barium enema may be normal.

Case 8

A. B. C. D. E.

It is more common in males. Suction rectal biopsy is rarely adequate for diagnosis. Enterocolitis is a significant cause of mortality. Disease is most often confined to the distal colon. Barium enema may be normal.

Hirschsprungs Disease

Absence of ganglia in submucosal and myenteric plexuses

variable

proximal extension of aganglionosis lack of peristalsis and failure of sphincter relaxation rectosigmoid only in 75%, entire colon in 8%

1:5000 births 70 80% boys 4X greater in Downs babies

Hirschsprungs Disease

Presents as failure to pass meconium w/in 24 hrs or constipation in older child Diagnosis best made by rectal biopsy
suction

adequate if submucosa present Rectal biopsy Anorectal manometry

Hirschsprungs Disease

OR requires biopsies to confirm ganglion cells in normal bowel Pull-through operations

Swenson: complete excision, anastamosis to proximal anal canal at columns of Morgagni Soave: endorectal mucosal excision, pull through rectal muscular sleeve Duhamel: retains portion of aganglionic bowel anteriorly using GIA stapler

Hirschsprungs Disease

Hirschsprungs Disease

Ganglion cells

Hirschsprungs Disease
1. Absence of ganglion cells 2. Hypertrophic nerve trunks

Hirschsprungs Disease

Swenson

Soave

Duhamel

Hirschsprungs Disease

Enterocolitis
12

58% ? Fecal stasis Life threatening Treat with rectal irrigation and flagyl

Case 9

Newborn infant, 36 week gestational age, delivered for PROM No prenatal care Significant respiratory distress at birth requiring emergent intubation Apgars 2 and 5

Case 9

Decreased breath sounds on the left side Scaphoid abdomen Workup?

Congenital Diaphragmatic Hernia

CDH

Primary physiologic disturbance:

pulmonary hypoplasia Pulmonary hypertension

most important (reversible)

Prenatal:
Polyhydramnios Interventions

Not proven to improve outcomes

CDH Post natal Treatment

Gentle ventilation nitric oxide surfactant high frequency, oscillating ventilation muscle paralysis, induced alkalosis spontaneous respiration, permissive hypercapnea perfluorocarbon ventilation combinations of the above extracorporeal life support SURGERY once physiolgically stable

ECMO CANNULATION

ECMO CANNULATION
VENO-ARTERIAL CANNULATION

ECMO CANNULATION
VENO-VENOUS CANNULATION

ECMO Circuit

CDH - Survival

Prognosis: Pulmonary recovery: Overall reported survival varies among institutions. When all resources, including ECMO, are provided, survival rates range from 4069%. Long-term morbidity: Significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, and neurodevelopmental delay, may occur in survivors.

Case 10
A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition?

A. Immediate laparotomy is warranted.

B. C. D. E.

UGI series is the diagnostic procedure of choice. Delay in diagnosis leads to metabolic acidosis. Most commonly seen in females. Fluid replacement consists of NS + KCL

A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition?

A. Immediate laparotomy is warranted.

B. C. D. E.

UGI series is the diagnostic procedure of choice. Delay in diagnosis leads to metabolic acidosis. Most commonly seen in females. Fluid replacement consists of NS + KCL

Pyloric Stenosis

1 in 600 births, male: female ratio 4:1, 3-12 weeks Gastric outlet obstruction due to hypertrophy of pyloric muscle Progressive, projectile non-bilious vomiting Hypochloremic, hypokalemic metabolic alkalosis

renal compensation for hypovolvemia thickness > 5 mm, channel length > 15 mm

Ultrasound is diagnostic procedure of choice

Repair via Fredet-Ramstedt pyloromyotomy

Pyloromyotomy

Case 11
A 6-wk-old infant presents with jaundice. A sonogram appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management?

A. List for liver transplant.

B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.

A 6-wk-old infant presents with jaundice. An abdominal USG appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management?

A. List for liver transplant. B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.

Biliary Atresia

Fibrous obliteration of extrahepatic bile ducts

1 in 10-15 thousand births

Jaundice, conjugated hyperbilirubinemia, firm hepatomegaly due to biliary cirrhosis Lab work up should include LFTs, Alpha-1 antitrypsin, TORCH infections, sweat test, hepatitis Sono shows no extrahepatic ducts, tiny gallbladder HIDA scan reveals no emptying into the duodenum Liver biopsy reveals cholestasis and bile duct proliferation

Kasai Portoenterostomy

Roux-en-Y limb of jejenum sutured to porta where atretic bile ducts exit hepatic parenchyma Results depend on age (10 weeks), anatomy and histology of atretic bile ducts, ? degree of cirrhosis
overall: 1/3 fail immediately Long term survival in 25% of those that have drainage Results of liver transplantation not affected by Kasai procedure

Biliary Atresia

Biliary Atresia

Kasai Portoenterostomy

Congenital Lung lesions

Which statement is FALSE regarding extrapulmonary sequestration? A. The parenchyma is not connected to the tracheobronchial tree B. Arterial blood supply is systemic C. Venous blood supply is pulmonary D. Most frequently in males E. Commonly associated with other anomalies

Which statement is FALSE regarding extrapulmonary sequestration?

A. The parenchyma is not connected to the tracheobronchial tree B. Arterial blood supply is systemic C. Venous blood supply is pulmonary D. Most frequently in males E. Commonly associated with other anomalies

Congenital Pulmonary Airway Malformation

Pulmonary Sequestration

Cystic mass of nonfuctioning primitive lung tissue not connected to tracheobronchial tree

Extrapulmonary

usually diagnosed in first year due to other anomalies

Intrapulmonary (90%)

Usually diagnosed later childhood/adolescence

Males 3-4:1 Systemic arterial supply 95% Systemic venous drainage >80%

Pulmonary Sequestration

Usually located b/w LLL and diaphragm

Extrapulmonary

may also be found connected to gi

tract

Associated anomalies 65%

Pulmonary

hypoplasia 25%, CDH 16%

Congenital Lobar Emphysema

Air trapped in the lobe Leads to adjacent lobe atelectasis Shifts mediastinum to opposite side More common in the upper lobes CXR for diagnosis Nonop management low vent pressure/volume, positioning Resection provides definitive treatment

PEDIATRIC HEAD AND NECK MASSES

Case 1

18mos old female Presents to your office with a mass above her left eyebrow What next? Differential diagnosis

Evaluation of mass

H&P
Age Onset

Size Multiplicity Laterality

Rapidity

of growth Fluctuation in size Pain Infection Trauma Travel Exposure

Consistency
Color Mobility

Tenderness
Fluctuation

PE

Case 1

Differential diagnosis

Differential Diagnosis

Congenital
Branchial cleft cysts Thyroglossal duct cyst Dermoid cyst Vascular malformation

Inflammatory lesions
Reactive lymphadenopathy Granulomatous disease

Lymphatic Hemangioma

Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid

Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele

Suppurative lymphadenitis

Noninflammatory benign
Inclusion cyst Fibromatosis Keloid

Differential Diagnosis

Benign neoplasms
Neurofibroma
Lipoma Paraganglioma

Malignant Neoplasm
Lymphoma
Hodgkins NonHodgkins

Goiter Thyroid

Thyroid

nodule

Carcinoma Sarcoma Neuroblastoma

Case 2

2 year old male Mass on side of neck Noticed recently and slowly has increased in size
One

episode where it was erythematous and tender Treated with antibiotics and resolved

Case 2

Mass is anterior to sternoclavicular musle Less than 5 mm Small skin opening

Branchial cleft anomalies

Branchial cleft anomalies

Branchial arches

Case 3

12 year old girl Mass in the anterior neck

Case 3

An 8 y.o. boy has a recurrent painful swelling in a 2cm mass in the midline of his neck below the hyoid bone. Which is TRUE?

A. Ectopic thyroid is present in 50% of cases

B. surgical excision includes the pyramidal lobe of the thyroid C. the structure originates at the foramen cecum D. Fistula tracts drain laterally at the inferior border of the sternoclaidomastoid E. Simple excision can be done with local anesthesia

An 8 y.o. boy has a recurrent painful swelling in a 2cm mass in the midline of his neck below the hyoid bone. Which is TRUE?

A. Ectopic thyroid is present in 50% of cases

B. surgical excision includes the pyramidal lobe of the thyroid C. the structure originates at the foramen cecum D. Fistula tracts drain laterally at the inferior border of the sternoclaidomastoid E. Simple excision can be done with local anesthesia

Thyroglossal Duct Cyst

Arise from duct formed when developing thyroid passes from lingual foramen cecum through/near hyoid bone to neck Most common midline neck mass in kids
May

be lateral (within 2cm) in 25% of cases

Can extend to pyramidal lobe Contain aberrant thyroid tissue in 1%

Thyroglossal Duct Cyst

May contain papillary or mixed papillary/follicular adenocarcinoma in 1% Sistrunk procedure

Excise

entire duct to level of foramen cecum, including part of hyoid bone to prevent recurrence Periop antibiotics unnecessary, 4% infection rate

Sistrunk

Sistrunk