Common Pediatric Surgery Problems
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common peads surg...
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COMMON PEDIA PEDIATRIC TRIC SURGERY SURGER Y PROBLEMS 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 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
Hirschsprung’s
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
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 ’
’
“
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
Chest
Clear, AE=AE
Cardiac
Normal oropharynx
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 Ladd’s 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 Ladd s 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 o nset of severe severe intermittent abdominal pain. Rectal Rectal exam is guaiac positive. What is the most likely likely diagnosis? A. Meckel s diverticulum. B. Acute appendicitis. ’
C. Intussusception. D. Intestinal Intestinal polyp.
E. Gastritis.
A. Meckel s diverticulum. B. Acute appendicitis. C. Intussusception.
’
D. Intestinal Intestinal polyp. E. 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 H 2O 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
Prevention
Breast milk
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
“
Peritoneal drainage
second-look laparotomy 24-48 hrs if needed ”
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. 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 4X greater in Down s babies ’
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
Anorectal manometry
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
anteriorly using GIA stapler
Hirschsprung s Disease ’
Hirschsprung s Disease ’
Ganglion cells
Hirschsprung s Disease ’
1. Absence of ganglion cells 2. Hypertrophic nerve trunks
Hirschsprung s Disease ’
Swenson
Soave
Duhamel
Hirschsprung s 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. 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.
E. Fluid replacement consists of ½ 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
Ultrasound is diagnostic procedure of choice
renal compensation for hypovolvemia thickness > 5 mm, channel length > 15 mm
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 Rapidity of growth Fluctuation in size Pain Infection Trauma Travel Exposure
PE
Size Multiplicity Laterality Consistency Color Mobility Tenderness Fluctuation
Case 1
Differential diagnosis
Differential Diagnosis
Congenital
Branchial cleft cysts Thyroglossal duct cyst Dermoid cyst Vascular malformation
Reactive lymphadenopathy Granulomatous disease
Inflammatory lesions
Lymphatic Hemangioma
Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele
Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid
Suppurative lymphadenitis
Noninflammatory benign Inclusion cyst Fibromatosis Keloid
Differential Diagnosis
Benign neoplasms
Malignant Neoplasm
Neurofibroma
Lipoma
Paraganglioma
Goiter
Thyroid Carcinoma
Thyroid nodule
Sarcoma
Neuroblastoma
Lymphoma Hodgkins NonHodgkins
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
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