common pediatric surgery problems

7/28/2019 Common Pediatric Surgery Problems

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COMMON PEDIATRICSURGERY PROBLEMSSurgery Curriculum Conference

June 13, 2012


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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


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Prenatal work-up

Trisomy 21

Normal ultrasound at 18weeks

Mom has negative serologies


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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.


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Case 1

Investigations

Bloodwork

Imaging

Any other diagnostic tests?


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ECHO

VSD

Normal BMP, CBC, neg cultures


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Neonatal Emesis DDx

Upper GI

Duodenal atresias/webs

small bowel atresias

malrotation/midgut volvulus GERD

Meconium ileus

pyloric stenosis Inguinal hernia

NEC


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Lower GI

Colonic atresia

Meconium plug

Hirschsprungs

Small Left Colon Syndrome

Microcolon-Intestinal Hypoperistalsis Syndrome

Imperforate anus

Neonatal Emesis DDx


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Medical causes

Sepsis

Metabolic disorders

Hypothyroidism

Electrolyte disturbances

GERD

Neonatal Emesis DDx


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Radiological workup

KUB/Cross-table lateral

Contrast enemas for distal obstructions

UGI for malrotation/proximal atresias


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Duodenal atresia

Management

NGT

Resuscitate

Surgical approach

duodenoduodenostomy


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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.


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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.


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Duodenal atresia


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Duodenal Atresia

Failure to recanalize lumen of duodenum aftersolid phase of embryologic development Distal atresias are due to vascular events

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 no indication to divide annular pancreas


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Case 2

2 day old infant in newborn nursery

Sent to NICU for evaluation of bilious emesis


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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


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Work-up

Bloodwork

Imaging


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Normal embryologic rotation


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Abnormal rotation and nonfixation


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Management of Malrotation/volvulus

Resuscitate

Urgent surgery


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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


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Ladd Procedure


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Malrotation

Occurs in 1/2001/500 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


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Malrotation

Due to abnormal fixation of midgut toretroperitoneumleads to narrow base ofmesentery which can easily twist

Ladd Procedure Reduce volvulus by rotating counterclockwise

Division of Ladds bands between cecum andduodenum/right gutter

Division of adhesions to widen mesentery Run bowel to r/o obstructions

Appendectomy

Place bowel in nonrotated position


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Case 3

2 day old infant in NICU 3

Consulted for abdominal distention and bilious

emesis

Work-up and differential


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Pathophysiology of intestinal atresias

How would you confirm diagnosis and what would

you see

Classification scheme


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Case 4

3 day old infant

Failure to pass stools, abdominal distention and

bilious emesis

Work-up and differential


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What other tests should be done

Sweat test for CF

Genetic testing for CFTR gene mutation


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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.


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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.


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Meconium Ileus

Newborn bowel obstruction secondary toinspissated 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


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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 irrigationsmay be delivered


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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


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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?


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Esophageal Atresia and


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Tracheoesophageal Fistula

Incomplete partitioning of primitive foregut

5 types of atresias

Esophageal atresia with distal TEF most common

8% 1% 85% 2% 4%


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E h l At i d


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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


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Case 6

A listless 9-month-old boy presents with acute onsetof 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.


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A. Meckels diverticulum.

B. Acute appendicitis.

C. Intussusception.

D. Intestinal polyp.

E. Gastritis.


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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


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Intussusception

Plain AXR

Look for gas in cecum

Abdominal ultrasoundlook for target


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Which of the following statements is TRUE with respect


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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.



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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.


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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 defectiveumbilical 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


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Omphalocele


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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


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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


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Uterus +Fallopian Tube

Bladder

Stomach

ColonSmall bowel


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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


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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, NGTdrainage, 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

C 7


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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


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Necrotizing Entercolitis (NEC)

Idiopathic mucosal intestinal injury, may progress totransmural necrosis 1/2 patients < 1500 g (7% incidence), 80% < 2500 g

at birth

90% in premature neonates


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Necrotizing Entercolitis (NEC)

Signs: feeding intolerance

vomiting

abdominal distention

progressive sepsis

autonomic instability (Apneas and Bradys)

abdominal wall erythema +/- mass

Labs: metabolic acidosis thrombocytopenia

N ti i E t liti (NEC)


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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)

N i i E li i (NEC)


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Pathogenesis

No single predisposing factor

Prevention

Breast milk


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N ti i E t liti (NEC)


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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



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Indications for OR are free air (absolute), fixedabdominal 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 bowelsyndrome


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Case 8


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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

C 8


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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.


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Hirschsprungs Disease

Absence of ganglia in submucosal and myentericplexuses

variable proximal extension of aganglionosis

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

1:5000 births

7080% boys

4X greater in Downs babies

Hirschspr ngs Disease


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Hirschsprung s Disease

Presents as failure to pass meconium w/in 24 hrs orconstipation in older child

Diagnosis best made by rectal biopsy

suction adequate if submucosa present Rectal biopsy

Anorectal manometry


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OR requires biopsies to confirm ganglion cells in

normal bowel

Pull-through operations

Swenson: complete excision, anastamosis to proximalanal canal at columns of Morgagni

Soave: endorectal mucosal excision, pull through rectal

muscular sleeve

Duhamel: retains portion of aganglionic bowelanteriorly using GIA stapler



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p g



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p g

Ganglion cells



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p g

1. Absence ofganglion cells

2. Hypertrophic nerve

trunks



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p g

Swenson Soave Duhamel



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Hirschsprung s Disease

Enterocolitis 1258%

? Fecal stasis

Life threatening Treat with rectal irrigation and flagyl

Case 9


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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


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Case 9

Decreased breath sounds on the left side

Scaphoid abdomen

Workup?


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Congenital Diaphragmatic Hernia

CDH


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CDH

Primary physiologicdisturbance: pulmonary hypoplasia

Pulmonary hypertension most important (reversible)

Prenatal: Polyhydramnios

Interventions Not proven to improve

outcomes

CDH Post natal Treatment


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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

SURGERYonce physiolgically stable


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ECMO CANNULATION

ECMO CANNULATION


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VENO-ARTERIAL CANNULATION

ECMO CANNULATION


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VENO-VENOUS CANNULATION

ECMO Circuit


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ECMO Circuit

CDH - Survival


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CDH Survival

Prognosis:

Pulmonary recovery: Overall reported survival variesamong 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.

Case 10


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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 NS + KCL

Case 10


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A 5-wk-old boy presents with 3 days of non-bilious projectilevomiting 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 NS + KCL

P l i St i


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Pyloric Stenosis

1 in 600 births, male: female ratio 4:1, 3-12weeks

Gastric outlet obstruction due to hypertrophy ofpyloric 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 mm

Repair via Fredet-Ramstedt pyloromyotomy

Pyloromyotomy


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Pyloromyotomy


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Case 11


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A 6-wk-old infant presents with jaundice. A sonogramappears 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.

Case


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A 6-wk-old infant presents with jaundice. An abdominalUSG 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


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Biliary Atresia

Fibrous obliteration of extrahepatic bile ducts 1 in 10-15 thousand births

Jaundice, conjugated hyperbilirubinemia, firmhepatomegaly 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 ductproliferation

Kasai Portoenterostomy


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Roux-en-Y limb of jejenum sutured to porta whereatretic bile ducts exit hepatic parenchyma

Results depend on age (10 weeks), anatomy andhistology 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 Kasaiprocedure

Biliary Atresia


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Biliary Atresia

Biliary Atresia


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Biliary Atresia



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Congenital Lung lesions


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Which statement is FALSE regarding extrapulmonarysequestration?

A. The parenchyma is not connected to thetracheobronchial tree

B. Arterial blood supply is systemic

C. Venous blood supply is pulmonary

D. Most frequently in males

E. Commonly associated with other anomalies

g g


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Which statement is FALSE regarding extrapulmonarysequestration?

A. The parenchyma is not connected to thetracheobronchial 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

M lf ti


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Malformation

Pulmonary Sequestration


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y q

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 supply95%

Systemic venous drainage>80%

Pulmonary Sequestration


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y q

Usually located b/w LLL and diaphragm Extrapulmonary may also be found connected to gi

tract

Associated anomalies65% Pulmonary hypoplasia 25%, CDH 16%


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Congenital Lobar Emphysema


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g p y

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 managementlow vent pressure/volume,

positioning Resection provides definitive treatment


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PEDIATRIC HEAD ANDNECK MASSES

Case 1


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18mos old female Presents to your office with a mass above her left

eyebrow

What next?

Differential diagnosis

Evaluation of mass


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H&P Age

Onset

Rapidity of growth

Fluctuation in size

Pain

Infection

Trauma Travel

Exposure

PE

SizeMultiplicity

Laterality

Consistency

ColorMobility

Tenderness

Fluctuation

Case 1


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Differential diagnosis


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Differential Diagnosis


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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

Keloid

Differential Diagnosis


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Benign neoplasms Neurofibroma

Lipoma

ParagangliomaGoiter

Thyroid nodule

Malignant Neoplasm Lymphoma

Hodgkins

NonHodgkins

Thyroid Carcinoma

Sarcoma

Neuroblastoma

Case 2


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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


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Mass is anterior to sternoclavicular musle Less than 5 mm

Small skin opening

Branchial cleft anomalies


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Branchial cleft anomalies


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Branchial arches


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Case 3


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12 year old girl Mass in the anterior neck

Case 3


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An 8 y.o. boy has a recurrent painful swelling in a 2cmmass 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


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An 8 y.o. boy has a recurrent painful swelling in a 2cmmass 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


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Arise from duct formed when developing thyroidpasses from lingual foramen cecum through/near

hyoid bone to neck

Most common midline neck mass in kidsMay be lateral (within 2cm) in 25% of cases

Can extend to pyramidal lobe

Contain aberrant thyroid tissue in 1%

Thyroglossal Duct Cyst


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May contain papillary or mixed papillary/follicularadenocarcinoma in 1%

Sistrunk procedure

Excise entire duct to level of foramen cecum, includingpart of hyoid bone to prevent recurrence

Periop antibiotics unnecessary, 4% infection rate

Sistrunk


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Sistrunk


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common pediatric surgery problems

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