COMMON
COMMON
PEDIA
PEDIA
TRIC
TRIC
SURGER
SURGER
Y PR
Y PR
OBLEMS
OBLEMS
Surgery Curriculum ConferenceSurgery Curriculum Conference
June 13, 2012
Case 1
Case 1
39 week gestational age
39 week gestational age
Normal pregnancy and
Normal pregnancy and vaginal delivery
vaginal delivery
Apgars 9
Apgars 9
1,1,10
10
55
Started breastfeeding and started to have multiple
Started breastfeeding and started to have multiple
episodes of
Prenatal work-up
Trisomy 21
Normal ultrasound at 18weeks
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
Case 1
Investigations
Bloodwork
Imaging
ECHO
VSD
Neonatal Emesis DDx
Upper GI
Duodenal atresias/webs
small bowel atresias
malrotation/midgut volvulus
GERD
Meconium ileus
pyloric stenosis
Inguinal hernia
NEC
Lower GI
Colonic atresia
Meconium plug
Hirschsprung’s
Small Left Colon Syndrome
Microcolon-Intestinal Hypoperistalsis Syndrome
Imperforate anus
Medical causes
Sepsis
Metabolic disorders
Hypothyroidism Electrolyte disturbances GERD
Neonatal Emesis DDx
Radiological workup
KUB/Cross-table lateral
Contrast enemas for distal obstructions
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,
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,
Duodenal Atresia
Failure to recanalize lumen of duodenum after
solid phase of embryologic development
Distal atresias are due to vascular events
Associated with Down
’s syndrome in 30%
Atresia seen in 10% of Down
’s patients
Vomiting can be bilious or non-bilious
Abdominal X-ray shows
“double-bubble
”
Best repaired by bypass ->
duodenoduodenostomy or duodenojejunostomy
Case 2
2 day old infant in newborn nursery
Physical Examination
HR 165, RR 50, O2 sats 98 RA
HEENT
Normal oropharynx Chest
Clear, AE=AE Cardiac
Normal HS, good peripheral pulses
Abdo
Nondistended, generalized tenderness Soft, no discoloration, no masses, no HSM No inguinal hernias
Work-up
Bloodwork
Imaging
Management of Malrotation/volvulus
Resuscitate
Steps to correcting malrotation
1. Entry into abdominal cavity and evisceration (open)
2. Counterclockwise detorsion of the bowel (acute
cases)
3.
Division of Ladd’scecal bands
4. Broadening of the small intestine mesentery
5. Incidental appendectomy
6. Placement of small bowel along the right lateral
Ladd Procedure
Malrotation
Malrotation
Occurs in 1/200
Occurs in 1/200
–
–
1/500 live births
1/500 live births
Symptomatic in 1/6000 live births
Symptomatic in 1/6000 live births
30-62% have associated anomaly
30-62% have associated anomaly
Up to 75% present w/in 1st month of life
Up to 75% present w/in 1st month of life
Classic presentation is infant with bilious emesis
Classic presentation is infant with bilious emesis
May present as pain, duodenal obstruction,
May present as pain, duodenal obstruction,
malnutrition, acute abdomen/shock
malnutrition, acute abdomen/shock
Malrotation
Malrotation
Due to abnormal fixation of midgut to
Due to abnormal fixation of midgut to
retroperitoneum
retroperitoneum
– –leads to narrow base of
leads to narrow base of
mesentery which can easily twist
mesentery which can easily twist
Ladd Procedure
Ladd Procedure
Reduce volvulus by rotating counterclockwiseReduce volvulus by rotating counterclockwise
Division of LaddDivision of Ladd’’s bands between cecum ands bands between cecum and
duodenum/right gutter duodenum/right gutter
Division of Division of adhesions to widen mesenteryadhesions to widen mesentery
Run bowel to r/o obstructionsRun bowel to r/o obstructions
AppendectomyAppendectomy
Case 3
2 day old infant in NICU 3
Consulted for abdominal distention and bilious
emesis
Pathophysiology of intestinal atresias
How would you confirm diagnosis and what would
you see
Case 4
3 day old infant
Failure to pass stools, abdominal distention and
bilious emesis
What other tests should be done
Sweat test for CF
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).
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).
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
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
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.
Esophageal Atresia and
Tracheoesophageal Fistula
Incomplete partitioning of primitive foregut 5 types of atresias
Esophageal atresia with distal TEF most common
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,
Case 6
Case 6
A listless 9-month-old boy presents with acute o
A listless 9-month-old boy presents with acute onset
nset
of sev
of severe
ere intermittent
intermittent abdominal
abdominal pain.
pain. Recta
Rectall
exam
exam is
is guaiac positive.
guaiac positive. What
What is
is the
the most lik
most likely
ely
diagnosis?
diagnosis?
A. Meckel
A. Meckel
’’s diverticulum.
s diverticulum.
B.
B. Acute
Acute appendicitis.
appendicitis.
C. Intussusception.
C. Intussusception.
D
D.
. Intesti
Intestinal
nal polyp.
polyp.
A. Meckel
A. Meckel
’’s diverticulum.
s diverticulum.
B.
B. Acute
Acute appendicitis.
appendicitis.
C. Intussusception.
C. Intussusception.
D
D.
. Intesti
Intestinal
nal polyp.
polyp.
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
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
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
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
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,
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)
Uterus + Fallopian Tube Bladder Stomach Colon Small bowel
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
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.
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
Prevention
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
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
4. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis?
A. It is more common in males.
B. Suction rectal biopsy is rarely adequate for diagnosis. C. Enterocolitis is a significant cause of mortality.
D. Disease is most often confined to the distal colon. E. Barium enema may be normal.
Case 8
A. It is more common in males.
B. Suction rectal biopsy is rarely adequate for diagnosis. C. Enterocolitis is a significant cause of mortality.
D. Disease is most often confined to the distal colon. E. Barium enema may be normal.
Hirschsprung
’s 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
Hirschsprung
’s 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
Hirschsprung
’s 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
Hirschsprung
’s Disease
Hirschsprung
’s Disease
1. Absence of ganglion cells
2. Hypertrophic nerve trunks
Hirschsprung
’s Disease
Hirschsprung
’s Disease
Enterocolitis
12
–58%
? Fecal stasis
Life threatening
Case 9
Newborn infant, 36 week gestational age,
delivered for PROM
No prenatal care
Significant respiratory distress at birth requiring
emergent intubation
Case 9
Decreased breath sounds on the left side
Scaphoid abdomen
CDH
Primary physiologic
disturbance:
pulmonary hypoplasia Pulmonary hypertension
most important (reversible)
Prenatal:
Polyhydramnios Interventions
Not proven to improve
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
ECMO CANNULATION
ECMO CANNULATION
CDH - Survival
Prognosis:
Pulmonary recovery: Overall reported survival varies
among institutions. When all resources, including
ECMO, are provided, survival rates range from
40-69%.
Long-term morbidity: Significant long-term morbidity,
including chronic lung disease, growth failure,
gastroesophageal reflux, and neurodevelopmental
delay, may occur in survivors.
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. UGI series is the diagnostic procedure of choice. C. Delay in diagnosis leads to metabolic acidosis. D. Most commonly seen in females.
E. Fluid replacement consists of ½ 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. UGI series is the diagnostic procedure of choice. C. Delay in diagnosis leads to metabolic acidosis. D. Most commonly seen in females.
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
Ultrasound is diagnostic procedure of choice
thickness > 5 mm, channel length > 15 mmA 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
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
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
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
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%
Pulmonary Sequestration
Usually located b/w LLL and diaphragm
Extrapulmonary may also be found connected to gi
tract
Associated anomalies
–
65%
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
PEDIATRIC HEAD AND
NECK MASSES
Case 1
Case 1
18mos old female
18mos old female
Presents to your office with a mass above her left
Presents to your office with a mass above her left
eyebrow
eyebrow
What next?
What next?
Evaluation of mass
Evaluation of mass
H&P
H&P
Age
Age
Onset
Onset
Rapidity of growth
Rapidity of growth
Fluctuation in size
Fluctuation in size
Pain
Pain
Infection
Infection
Trauma
Trauma
Travel
Travel
Exposure
Exposure
PE
PE
Size
Size
Multiplicity
Multiplicity
Laterality
Laterality
Consistency
Consistency
Color
Color
Mobility
Mobility
Tenderness
Tenderness
Fluctuation
Fluctuation
Case 1
Differential Diagnosis
Congenital
Branchial cleft cysts Thyroglossal duct cyst Dermoid cyst Vascular malformation Lymphatic Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele
Inflammatory lesions
Reactive lymphadenopathy Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lymphadenitis Noninflammatory benign
Inclusion cyst Fibromatosis KeloidDifferential Diagnosis
Benign neoplasms
Neurofibroma
Lipoma
Paraganglioma
Goiter
Thyroid nodule
Malignant Neoplasm
Lymphoma
Hodgkins NonHodgkins Thyroid 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
Case 3
12 year old girl
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
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
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
Thyroglossal Duct Cyst
May contain papillary or mixed papillary/follicular
adenocarcinoma in 1%
Sistrunk procedure