SURGERY BOARD REVIEWMarch 29, 2011

RESPIRATORY DISTRESS

UPPER AIRWAY

Attempt to pass NG tube Esophageal atresia Choanal atresia

Half have other congenital anomaly

CHARGE Syndrome Coloboma Heart Atresia of choanae Retarded development Genital hypoplasia Ear

CHOANAL ATRESIA

Membranous 90% Bony 10% Gavage feed until repair Maintain oral airway

MACROGLOSSIA

True enlargement of tongue Vs. Glossoptosis in which normal tongue

protrudes from abnormally small oral cavity

Focal (pebbly, vescicle-like blebs) Congenital tumors (lymphangioma,

hemangioma) One-half

Hemihypertrophy

MACROGLOSSIA

Generalized (smooth surface) Beckwith-Wiedemann, hypothyroidism

Generalized (papillary, fissured) Down syndrome

Also poor tone

Progressive Mucopolysaccharidoses

QUESTION 1

This child was most likely to have which of the following metabolic abnormalities as a newborn?

A Hyperkalemia B Hypokalemia C Hyponatremia D Hypoglycemia E Metabolic Acidosis

BECKWITH WIEDEMANN SYN

Features Macroglossia Macrosomia Hypoglycemia (islet cell hyperplasia) Hemihypertrophy Hypospadias Omphalocele

18 month old with B.W. presents with gross hematuria: Wilms Tumor

PIERRE ROBIN SEQUENCE

Unusually small mandible (micrognathia) Posteriorly displaced

tongue Upper airway obstruction U-shaped cleft palate

May be part of a larger syndrome

LARYNGEAL LESIONS

Hoarseness Faint cry Aphonia Associated dyspnea

THORACIC LESIONS

QUESTION 2

Which is the most common abnormality?

A B C D E

TE fistulae: Most common types… 1 Esophageal atresia and distal fistula 2 Esophageal atresia and no fistula 3 H-Type (only one without atresia)

Manifestation may not be as severe

1 32

TE FISTULA

Inability to pass NG tube Use to drain secretions

Resp distress secondary Fistula Reflux of pouch contents

Inability to tolerate initial feed Excessive drooling May see distension of stomach (air fistula)

Avoid positive pressure ventilation Antenatal U/S: Microgastria, polyhydramnios

PURE ESOPHAGEAL ATRESIA

Dilated proximal pouch Gasless

abdomen Scaphoid

abdomen

BRONCHOPULMONARY FOREGUT MALFORMATION

BRONCHOPULMONARY FOREGUT MALFORMATION

Congenital Lobar Emphysema Congenital cystic adenomatoid

malformation Pulmonary sequestration Bronchogenic cyst

PULMONARY SEQUESTRATION

Portion of lungs perfused by systemic arteries Lacks normal connection with tracheobronchial

tree Intralobar:

Most common lower lobes Anywhere in thorax

Extralobar: Subdiaphragmatic or retroperitoneal Associated with other anomalies

Dx: MRI or angiogram

PULMONARY SEQUESTRATION

BRONCHOGENIC CYST

Occur any point along the tracheobronchial tree Centrally located

Does not communicate, fluid fill with wall composed of tissue resembling large airways

Typically present 2nd decade of life with recurrent wheezing, cough, pneumonia

BRONCHOGENIC CYST

CONGENITAL PULMONARY AIRWAY MALFORMATION (CPAM)

Previously known as Congenital Cystic Adenomatoid Malformation (CCAM)

Hamartomatous lesion Cystic and adenomatous elements

Resp distress, recurrent PNA, asymptomatic

Severe forms can be lethal after birth Surgical resection if symptomatic

Controversial if asymptomatic

CONGENITAL PULMONARY AIRWAY MALFORMATION (CPAM)

CONGENITAL LOBAR EMPHYSEMA

Results from area of poorly developed or absent cartilage

Check-valve effect Air trapping Lung hyperexpansion

Possible acute decompensation with positive pressure ventilation

GI OBSTRUCTION

GI OBSTRUCTION

Bilious emesis is always considered a red flag for mechanical obstruction.

Surgical emergency until proven otherwise

NONBILIOUS EMESIS IN INFANTS

Most common cause is overfeeding Second most common: GE Reflux

HYPERTROPHIC PYLORIC STENOSIS

Fairly common 1:300 Unknown genetic link Vomiting starts by 2wks old

Progressively projectile Age at presentation

Most commonly b/f 1 month Remember: 2wks-2months

Male predominance Firstborn

PYLORIC STENOSIS

Clinical Manifestations: Abdomen probably will not be distended

Can feed them to look for signs Distended over stomach immediately after

feeding Peristaltic waves Palpable olive

After vomiting Epigastric b/w midline and R midclavicular line

PYLORIC STENOSIS

Diagnosis Palpable Olive (no imaging required) Hypochloremic, hypokalemic

metabolic alkalosis Ultrasonography

Pyloric muscle Thickness 3-4mm Length 15-16mm

DIAGNOSIS

Upper GI the "string" sign the "double-track" sign the "beak" sign the "shoulder" sign

PYLORIC STENOSIS

Surgical treatment (pyloromyotomy) Fluid and electrolyte correction are

most important Surgery after these are corrected

QUESTION 3

You are working up a 4 month old infant with bilious vomiting. Findings on barium enema reveal cecum located in left upper abdomen. The most likely diagnosis is: A situs inversus B malrotation C intussusception D intestinal duplication

BILIOUS VOMITING IN INFANT Malrotation with midgut volvulus

MALROTATION WITH MIDGUT VOLVULUS

Proximal obstruction at distal duodenum/ proximal jejunum Bilious emesis May not have significant abdominal

distention until late Late finding: hematemesis,

hematochezia Bowel ischemia leading to perforation

and death

MALROTATION WITH MIDGUT VOLVULUS Diagnosis

Suspect in setting of: Bilious vomiting in infant (or older child) Abdominal films:

Gastric distention and duodenal dilatation Otherwise gasless abdomen

MALROTATION

Surgical correction Ladd Procedure

Division of Ladd bands Appendectomy Widening of mesentery Securing intestines in position of

nonrotation

DUODENAL ATRESIA

Bilious vomiting shortly after birth

May occur later Annular pancreas Duodenal stenosis Duodenal web Ladd bands

Associated with Down Syn and congenital heart disease in 30-50%

MECONIUM ILEUS

20% of patients with CF Meconium ileus is CF until proven

otherwise Intraluminal obstruction by thickened

meconium Distal ileum is small, may have

microcolon Possible proximal bowel dilitation “soap bubble” appearance

MECONIUM ILEUS

Contrast enema (may be temporarily therapeutic) Microcolon Inspissated meconium “rabbit pellets”

May perforate Intraperitoneal calcifications and

ascites prenatally

QUESTION 4 You are seeing a new 9

month old who has had difficultly stooling since birth. You see in the records that this imaging has recently been performed • Which information is FALSE?• A Rectal suction biopsy should be done• B Identification of transition zone is helpful• C Development of explosive diarrhea fits

with diagnosis• D Barium enema should be performed

following GI cleanout

HIRSCHSPRUNG DISEASE

Absence of ganglion cells in distal rectum

Bilious emesis and abdominal distention

BE: proximally dilated bowel (normal) Transition zone Distal decompressed segment (abnormal)

Barium study should preceed rectal exam or enemas

Definitive: rectal suction biopsy

HIRSCHSPRUNG DISEASE

May be diagnosed in older child with chronic constipation Requiring rectal stimulation to evacuate

Lifethreatening hirschsprung-related enterocolitis Explosive diarrhea Toxic megacolon Sepsis

INTUSSUSCEPTION

6mos-2yrs Older children more likely to have lead

point Meckel Polyp Lymphoma Intestinal duplication cyst Hematoma/inflammation

Hemophilia, HSP

INTUSSUSCEPTION

Classic location: ileocolic (may be ileo-ileal if there is a lead point)

Presentation Paroxysmal bouts of severe colicky

abdominal pain Drawing up legs Period of somnolence Normal activity between episodes

Unexplained lethargy or seizure-like episode

INTUSSUSCEPTION

Emesis may turn bilious late in course Current jelly stool: late finding “sausage-shaped mass” RUQ

INTUSSUSCEPTION

Evaluation: Start with plain films

Crescent sign, target sign If suspicion high, can go straight to air

contrast or barium enema Diagnostic and therpeutic

Emergent surgery if enema unsuccessful May recur in first 12 to 24 hrs

GI BLEEDING

NEWBORN

Swallowed maternal blood Apt-downey test Mix serum with sodium hydroxide

Pink: newborn Brown: swallowed maternal blood

Vitamin K Anal stenosis (fissures) Hirschsprung Severe gastritis or AGE NEC

NEC

Acute illness of unclear etiology associated with intestinal necrosis Inflammation Invasion of enteric gas forming organisms Dissection of gas into the muscularis and portal venous

system 1-3/1000 live births Incidence decreases with increasing gestational

age 13% occur in term infants

Preexisting illness is common Mortality rate 20-30%

NEC

Presentation Nonspecific systemic signs in a fed, well infant

Apnea Respiratory failure Lethargy Poor feeding Temp instability Abdominal signs

Distention Gastric retention Tenderness Vomiting Diarrhea Gross bleeding

NEC

Diagnosis Abdominal distention Rectal bleeding Abdominal radiographs

Early - abnormal gas pattern with dilated loops c/w ileus

Pneumatosis intestinalis Bubbles of gas in the

bowel wall Portal air: late finding If perforation suspected

Cross-table lateral Left lateral decubitus

Sentinel loop Abdominal ultrasound

NEC

Diagnosis Nonspecific

Blood studies CBC Coags

DIC Chemistries

Stool analysis Occult blood

Sepsis evaluation 20-30%

NEC

Management

Supportive care Bowel rest and decompression with intermittent suction TPN Fluid replacement CV support Resp support Hematologic support Metabolic support

Surgical intervention Reserved for perforation, obstruction

NEC

Management Antibiotic therapy

Vanc, gent, clinda Vanc, gent, flagyl Vanc, gent, zosyn Term infants

Amp, gent and clinda 10-14 day course

Unless complication

TODDLER GI BLEEDING

Painless rectal bleeding Meckel diverticulum

Can have massive bleeding Dx: technetium-99m pertechnetate isotope

scan False negatives possible

May present with complications: Volvulus Internal hernia Intussusception Acute inflammation (similar to appendicitis)

ESOPHAGEAL VARICES

Massive upper GI bleeding Portal HTN

Portal vein thrombosis h/o UVC

Hepatic cirrhosis Chronic liver failure

TPN-related liver disease Biliary atresia s/p Kasai Cystic fibrosis a1- antitrypsin deficiency Viral hepatitis

ESOPHAGEAL VARICES

Treatment Endoscopy

Direct visualization Banding Sclerosant techniques

Transjugular intracaval portosystemic shunting

DON’T FORGET

HSP (Henoch-Schonlein Purpura) Vasculitic conditions Arthralgias Palpable purpuric rash

Buttocks Lower extremities

NORMAL CBC HUS (Hemolytic Uremic Syndrome)

Renal failure, anemia, thrombocytopenia Very sick!!

OLDER CHILDREN

Benign hamartomatous juvenile polyps Age 2-8 Painless, low volume, streaks on stool Autonecrosis and slough into stool May prolapse through anus Surgical or endoscopic resection is

therapeutic

QUESTION 5

This 10 y/o child has had recurrent lower GI bleeding. What is your diagnosis? A Peutz-Jeghers B Familial adenomatous polyposis C Gardner’s Syndrome D Hereditary Hemorrhagic

Telangectasias

FAMILIAL POLYP DISORDERS Gardner’s

Polyps of small and large bowel: premalignant

Extra teeth Osteomas AD inheritance Surgical resection

Peutz-Jeghers Hamartomatous polyps:

premalignant Melanotic mucocutaneous

pigmentation lips, gums AD inheritance Surgical resection

DON’T FORGET

Maintain high index of suspicion for appendicitis Rare in toddlers, but 75% rupture prior to dx Don’t expect them to fit classic picture Obturator and psoas signs may help

In Children, classic features (pain followed by vomiting, fever, anorexia, migration to RLQ, rebound) neither sensitive nor specific Lack of migration to RLQ in 50% Absence of anorexia 40% No rebound tenderness 52%

DON’T FORGET

Imitators of appendicitis Yersinia Mesenteric adenitis Sickle crisis

Caution in adolescent female!!! Other causes of abdominal pain:

Lower lobe pneumonia DKA

ABDOMINAL MASSES

ABDOMINAL MASSES

What is it? Age Location Characteristics

Firm Cystic Mobile Fixed

Initial diagnostic studies History usually no significance

ABDOMINAL MASSES

Location Most important

factor Characteristics

Cystic Usually benign

Solid Predominantly

malignant

Age Second most

important factor

ABDOMINAL MASSES

Work up Plain films

Displacement Obstruction Calcification

Ultrasound Initial diagnostic

tool of choice Solid vs cystic

ABDOMINAL MASSES

Work up CT

Study of choice Extent of local disease Sites of distant

metastases Character of the lesion Contrast

Careful analysis of abdominal and pelvic structures

MRI

QUESTION 6

On examination of a newborn female, you palpate an abdominal mass. Ultrasound reveals a 6cm ovarian cyst. What should you tell these anxious parents?

A. Most ovarian cysts resolve spontaneously and therefore no treatment is needed

B. Large cysts >5cm are at risk for torsion and should be aspiratedC. These are very rare and have a high risk for malignant transformationD. They are caused by hormones produced by the fetus in utero