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Inspiratory stridor is the most common indication for radiographic upper airway evaluation. The main role of imaging is to identify conditions that need to be treated emergently and/or surgically (e.g., epiglottitis, foreign bodies). Technique:
Physician capable of emergency airway intervention should accompany child
Obtain 3 radiographs:
Fluoroscope the neck if radiographs are suboptimal or equivocal.
Primary diagnostic considerations:
Infection (epiglottitis, croup, abscess)
Foreign body (airway or pharyngoesophageal)
Masses (lymphadenopathy neoplasms)
Congenital abnormalities (webs, malacia)
If upper airway is normal, consider:
Pulmonary causes (foreign body, bronchiolitis)
Mediastinal causes (vascular rings, slings)
Congenital heart disease (CHD)
Three anatomic regions:
Supraglottic region
Glottic region: ventricle and true cords
Subglottic region
Epiglottis and aryepiglottic folds are thin structures.
Glottic shoulders are seen on AP view.
Adenoids are visible at 3–6 months after birth.
Normal retropharyngeal soft tissue thickness (C1–C4) = three-quarters of vertebral body width
Common cause of stridor in the first year of life. Immature laryngeal cartilage leads to supraglottic collapse during inspiration. Stridor improves with activity and is relieved by prone positioning or neck extension. Self-limited course. Diagnosis is established by fluoroscopy (laryngeal collapse with inspiration).
Collapse of trachea with expiration. May be focal or diffuse; focal type is usually secondary to congenital anomalies that impress on the trachea, such as a vascular ring.
Most common in larynx.
Diffuse hypoplasia, 30%
Focal ring like stenosis, 50%
Funnel-like stenosis, 20%
Fixed narrowing at level of cricoid. Failure of laryngeal recanalization in utero.
Life-threatening bacterial infection of the upper airway. Most commonly caused by Haemophilus influenzae . Age: 3–6 years (older age group than with croup). Treatment is with prophylactic intubation for 24–48 hours and antibiotics.
Fever
Dysphagia
Drooling
Sore throat
Thickened aryepiglottic folds (hallmark)
Key radiographic view: lateral neck
Thickened epiglottis
Subglottic narrowing because of edema, 25%: indistinguishable from croup on AP view
Distention of hypopharynx
Other causes of enlarged epiglottis or aryepiglottic folds:
Caustic ingestion
Hereditary angioneurotic edema
Omega-shaped epiglottis (normal variant with normal aryepiglottic folds)
Stevens-Johnson syndrome
Subglottic laryngotracheobronchitis. Most commonly caused by parainfluenza virus. Age: 6 months to 3 years (younger age group than epiglottitis).
Barking cough
Upper respiratory tract infection
Self-limited
Subglottic narrowing (inverted “V” or “steeple sign”)
Key view: AP view
Lateral view should be obtained to exclude epiglottitis.
Steeple sign: loss of subglottic shoulders
Membranous croup: uncommon infection of bacterial origin (Staphylococcus aureus). Purulent membranes in subglottic trachea.
Epiglottitis may mimic croup on AP view.
Typically caused by extension of a suppurative bacterial lymphadenitis, most commonly S. aureus , group B streptococci, oral flora. Age: <1 year. Other causes include foreign body perforation and trauma.
Fever
Stiff neck
Dysphagia
Stridor (uncommon)
Most cases present as cellulitis rather than true abscess.
Widened retropharyngeal space (most common finding)
Air in soft tissues is specific for abscess.
Straightened cervical lordosis
Computed tomography (CT) is helpful to define superior and inferior mediastinal extent.
Plain radiograph findings are usually nonspecific.
Main differential diagnosis (DDx):
Retropharyngeal hematoma
Neoplasm (i.e., rhabdomyosarcoma)
Lymphadenopathy
The tonsils consist of lymphoid tissue that encircles the pharynx. Three groups: pharyngeal tonsil (adenoids), palatine tonsil, and lingual tonsil. Tonsils enlarge secondary to infection and may obstruct nasopharynx and/or eustachian tubes. Rarely, bacterial pharyngitis can lead to a tonsillar abscess (quinsy abscess), which requires drainage. Specific causes include:
Mononucleosis (Epstein-Barr virus [EBV])
Coxsackievirus (herpangina, hand-foot-mouth disease)
Adenovirus (pharyngoconjunctival fever)
Measles prodrome (rubeola)
β-Hemolytic Streptococcus (quinsy abscess)
Mass in posterior nasopharynx (enlarged adenoids)
Mass near end of uvula (palatine tonsils)
CT is useful to determine the presence of a tonsillar abscess.
Common cause of respiratory distress. Age: 6 months to 4 years. Acute aspiration results in cough, stridor, wheezing; chronic foreign body causes hemoptysis or recurrent pneumonia. Location: right bronchi > left bronchi > larynx, trachea.
Bronchial foreign body
Unilateral air trapping causing hyperlucent lung, 90%
Expiratory radiograph or lateral decubitus makes air trapping more apparent.
Atelectasis is uncommon, 10%
Only 10% of foreign bodies are radiopaque.
Chest fluoroscopy or CT should be performed if plain radiograph findings are equivocal.
Tracheal foreign body
Foreign body usually lodges in sagittal plane.
Chest radiograph (CXR) is usually normal.
Arises from a supernumerary lung bud that develops below the normal lung bud. Location and communication with gastrointestinal (GI) tract depend on when in embryonic life the bud develops. Most malformations present clinically when they become infected (communication with GI tract).
Malformation | Location |
---|---|
Sequestration | |
Intralobar | 60% basilar, left |
Extralobar | 80% left or below diaphragm |
Bronchogenic cyst | Mediastinum, 85%; lung, 15% |
CPAM (formerly CCAM) | All lobes |
Congenital lobar emphysema | LUL, 40%; RML, 35%; RUL, 20% |
Recurrent infection
Lung abscess
Bronchiectasis
Hemoptysis during childhood
Nonfunctioning pulmonary tissue (nearly always posteromedial segments of lower lobes [LLs])
Systemic arterial supply: anomalous arteries from the aorta (less common branch of the celiac artery)
No connection to bronchial tree
Large (>5 cm) mass near diaphragm
Air-fluid levels if infected
Surrounding pulmonary consolidation
Sequestration may communicate with GI tract.
Result from abnormal budding of the tracheobronchial tree. Cysts contain respiratory epithelium. Location:
Mediastinum, 85% (posterior > middle > anterior mediastinum)
Lung, 15%
Well-defined round mass in subcarinal/parahilar region
Pulmonary cysts commonly located in medial third of lung
Initially no communication with tracheobronchial tree
Cysts are thin walled.
Cysts can be fluid or air filled.
Formerly called congenital cystic adenomatoid malformation (CCAM), CPAM refers to a proliferation of polypoid glandular lung tissue without normal alveolar differentiation. Respiratory distress occurs during first days of life. After neonatal period, presentations include recurrent infections and pneumothorax.
Type 0: Rare and fatal
Type 1: Most common, large cysts (2–10 cm), unilateral
Type 2: Common, multiple small cysts (0.5–2 cm), associated with other congenital abnormalities
Type 3: Uncommon, large, often solid
Type 4: Uncommon, thin walled cysts, often multifocal, high association with malignancy, specifically pleuropulmonary blastoma
Type 1 and type 4 are difficult to differentiate and carry risk of malignancy (especially type 4). Treatment is resection.
Multiple cystic pulmonary lesions of variable size
Air-fluid levels in cysts
Variable thickness of cyst wall
Progressive overdistention of one or more pulmonary lobes but usually not the entire lung. 10% of patients have CHD (patent ductus arteriosus [PDA] and ventricular septal defect [VSD]).
Idiopathic, 50%
Obstruction of airway with valve mechanism, 50%
Bronchial cartilage deficiency or immaturity
Mucus
Web, stenosis
Extrinsic compression
Hyperlucent lobe (hallmark)
First few days of life: alveolar opacification because there is no clearance of lung fluid through bronchi
May be asymptomatic in neonate but becomes symptomatic later in life
Use CT to differentiate from bronchial obstruction
Distribution
Left upper lobe (LUL), 40%
Right middle lobe (RML), 35%
Right upper lobe (RUL), 20%
Two lobes affected, 5%
Agenesis: complete absence of one or both lungs (airways, alveoli, and vessels)
Aplasia: absence of lung except for a rudimentary bronchus that ends in a blind pouch
Hypoplasia: decrease in number and size of airways and alveoli; hypoplastic PA
Special form of hypoplastic lung in which the hypoplastic lung is perfused from the aorta and drained by the inferior vena cava (IVC) or portal vein (PV). The anomalous vein has a resemblance to a Turkish scimitar (sword). Associations include:
Accessory diaphragm, diaphragmatic hernia
Bony abnormalities: hemivertebrae, rib notching, rib hypoplasia
CHD: atrial septal defect (ASD), VSD, PDA, tetralogy of Fallot
Small lung (most commonly the right lung)
Retrosternal soft tissue density (hypoplastic collapsed lung)
Anomalous vein resembles a scimitar
Systemic arterial supply from aorta
Dextroposition of the heart (shift because of hypoplastic lung)
1 in 2000–3000 births. Mortality rate of isolated hernias is 60% (with postnatal surgery) and higher when other abnormalities are present. Most reliable predictor of postnatal survival is absence of liver herniation. Respiratory distress occurs in neonatal period. Associated abnormalities include:
Pulmonary hypoplasia (common)
Central nervous system (CNS) abnormalities
Neural tube defects (NTDs): spina bifida, encephalocele
Anencephaly
Bochdalek hernia (90% of CDH): posterior
75% are on the left, 25% on right
Right-sided hernias are more difficult to detect because of similar echogenicity of liver and lung.
Contents of hernia: stomach, 60%; colon, 55%; small intestine, 90%; spleen, 45%; liver, 50%; pancreas, 25%; kidney, 20%
Malrotation of herniated bowel is very common.
Morgagni hernia (10% of CDH): anterior
Most occur on right (heart prevents development on the left).
Most common hernia contents: omentum, colon
Accompanying anomalies common
Eventration
Caused by relative absence of muscle in dome of diaphragm
Associated with:
Trisomies 13, 18, congenital cytomegalovirus (CMV), rubella arthrogryposis multiplex, pulmonary hypoplasia
Hemidiaphragm not visualized
Multicystic mass in chest
Mass effect
Kartagener syndrome (immotile cilia syndrome) is due to the deficiency of the dynein arms of cilia causing immotility of respiratory, auditory, and sperm cilia.
Complete thoracic and abdominal situs inversus
Bronchiectasis
Sinus hypoplasia and mucosal thickening
Childhood pneumonias are commonly caused by:
Mycoplasma, 30% (lower in age group <3 years)
Viral, 65% (higher in age group <3 years)
Bacterial, 5%
Causes: respiratory syncytial virus (RSV), parainfluenza
All types of bronchiolitis and bronchitis cause air trapping (overaeration) with flattening of hemidiaphragms (best seen on lateral radiograph).
RSV, Mycoplasma, and parainfluenza virus are the most common agents that cause radiographic abnormalities (in 10%–30% of infected children).
Any virus may result in any of the five different radiographic patterns.
The following three pathogens are the most common:
Pneumococcus (age 1–3 years)
S. aureus (infancy)
H. influenzae (late infancy)
Consolidation
Alveolar exudate
Segmental consolidation
Lobar consolidation
Other findings
Effusions
Pneumatocele
Complications
Pneumothorax
Bronchiectasis (reversible)
Swyer-James syndrome (acquired pulmonary hypoplasia/postinfectious bronchiolitis obliterans), radiographically characterized by small, hyperlucent lungs with diminished vessels (focal emphysema)
Usually age <8 years
Pneumococcal pneumonia in early consolidative phase
Pneumonia appears round because of poorly developed collateral pathways (pores of Kohn and channels of Lambert).
With time, the initially round pneumonia develops into a more typical consolidation.
Cystic fibrosis (CF)
Recurrent aspirations
Rare causes of recurrent infection:
Hypogammaglobulinemia (Bruton disease; DDx clue: no adenoids or hilar lymph nodes)
Hyperimmunoglobulinemia E (Buckley syndrome)
Immotile cilia syndrome (Kartagener syndrome)
Other immunodeficiencies
BFM
Aspiration pneumonia results from inhalation of swallowed materials or gastric content. Gastric acid damages capillaries causing acute pulmonary edema. Secondary infection or acute respiratory distress syndrome (ARDS) may ensue.
Aspiration caused by swallowing dysfunction (most common cause)
Anoxic birth injury (common)
Coma, anesthesia
Aspiration caused by obstruction
Esophageal atresia (EA) or stenosis
Esophageal obstruction
Gastroesophageal (GE) reflux, hiatal hernia
Gastric or duodenal obstruction
Fistula
Tracheoesophageal fistula (TEF)
Recurrent pneumonias; distribution: aspiration in supine position: upper lobes (ULs), superior segments of LL; aspiration in upright position: both LLs
Segmental and subsegmental atelectasis
Interstitial fibrosis
Inflammatory thickening of bronchial walls
Pulmonary manifestations (pneumonia, acute chest syndrome, and pulmonary fibrosis) are the leading causes of death. Children with acute chest syndrome may present with one or multiple foci of consolidation, fever, chest pain, or cough. Causes: infection (higher incidence), fat emboli originating from infracting bone, pulmonary thrombosis.
Consolidation
Pleural effusion
Fine reticular opacities (pulmonary fibrosis)
Large heart in severe anemia
H-shaped vertebral bodies
Osteonecrosis, bone infarct in visualized humeri
Respiratory distress in the newborn is usually due to one of four disease entities:
Respiratory distress syndrome (hyaline membrane disease [HMD])
Transient tachypnea of the newborn (TTN)
Meconium aspiration
Neonatal pneumonia (NP)
Disease | Lung Volume | Opacities | Time Course | Complication |
---|---|---|---|---|
RDS | Low | Granular | 4–6 days | PIE, BPD, PDA |
Transient tachypnea | High or normal | Linear, streaky a | <48 hours | None |
Meconium aspiration | Hyperinflation | Coarse, patchy | At birth | PFC, ECMO |
Neonatal pneumonia | Anything | Granular | Variable |
RDS (formerly known as HMD) is caused by surfactant deficiency in premature infants. Surfactant diminishes surface tension of expanding alveoli. As a result, acinar atelectasis and interstitial edema occur. Hyaline membranes are formed by proteinaceous exudate. Symptoms occur within 2 hours of life. The incidence of RDS depends on the gestational age (GA) at birth.
Birth at Gestational Age (wk) | Incidence (%) |
---|---|
27 | 50 |
31 | 16 |
34 | 5 |
36 | 1 |
Any opacity in a premature infant should be regarded as RDS until proven otherwise.
Lungs are opaque (ground-glass) or reticulogranular (hallmark).
Hypoaeration (atelectasis) leads to low lung volumes: bell-shaped thorax (if not intubated).
Bronchograms are often present.
Absence of consolidation or pleural effusions
In contrast to other causes of RDS in neonates, pleural effusions are uncommon.
Treatment with surfactant may result in asymmetric improvement.
CXR signs of premature infants:
No subcutaneous fat
No humeral ossification center
Endotracheal tube present
In most cases of RDS, the diagnosis is made clinically but may initially be made radiographically. The role of the radiologist is to assess serial CXRs.
Complications of RDS:
Persistent PDA (signs of congestive heart failure [CHF]); the ductus usually closes within 1–2 days after birth in response to the high P o 2 content.
Barotrauma from treatment: pneumothorax, pneumomediastinum, PIE, and acquired lobar emphysema
Diffuse opacities (whiteout) may be due to a variety of causes:
Atelectasis
Progression of RDS
Aspiration
Pulmonary hemorrhage
CHF
Superimposed pneumonia
PIE refers to an accumulation of interstitial air in peribronchial and perivascular spaces. Most common cause: positive-pressure ventilation. Complications:
Pneumothorax
Pneumomediastinum
Pneumopericardium
Tortuous linear lucencies radiate outward from the hilar regions.
The lucencies extend all the way to the periphery of the lung.
Lucencies do not change with respiration.
Caused by oxygen toxicity and barotrauma of respiratory therapy. BPD is uncommon in larger and more mature infants (GA >30 weeks or weighing >1200 g at birth).
Diagnostic Criterion | Gestational Age Less than 32 wk | Gestational Age Greater than 32 wk |
---|---|---|
Time point of assessment | 36 wk PMA a or discharge to home, whichever comes first; treatment with >21% oxygen for at least 28 days plus | >28 days but <56 days postnatal age or discharge to home, whichever comes first; treatment with >21% oxygen for at least 28 days plus |
Mild BPD | Breathing room air at 36 wk PMA b or discharge, whichever comes first | Breathing room air by 56 days postnatal age or discharge, whichever comes first |
Moderate BPD | Need a for <30% oxygen at 36 wk PMA or discharge, whichever comes first | Need a for <30% oxygen at 56 days postnatal age or discharge, whichever comes first |
Severe BPD | Need a for ≥30% oxygen and/or positive pressure (PPV a or nasal CPAP) at 36 wk PMA or discharge, whichever comes first | Need a for ≥30% oxygen and/or positive pressure (PPV or nasal CPAP) at 56 days postnatal age or discharge, whichever comes first |
a Using a physiologic test (pulse oximetry saturation range) to confirm the oxygen requirement.
Complications of BPD include asthma-like disease, pulmonary artery hypertension (HTN) (with or without cor pulmonale), sepsis, and neurodevelopmental impairment.
Meconium (mucus, epithelial cells, bile, debris) is the first stool that is evacuated within 12 hours after delivery. In fetal distress, evacuation may occur into the amniotic fluid (up to 10% of deliveries). However, in only 1% does this aspiration cause respiratory symptoms. Only meconium aspirated to below the vocal cords is clinically significant. Meconium aspiration sometimes clears in 3–5 days. CXR nearly always returns to normal by 1 year of age. History is key for distinguishing between other forms of neonatal respiratory distress.
Patchy, bilateral opacities, may be “rope-like”
Atelectasis
Hyperinflated lungs
Pneumothorax, pneumomediastinum, 25%
Mortality (25%) from persistent fetal circulation
Transplacental infection
Toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus (TORCH)
Pulmonary manifestation of TORCH is usually less severe than other manifestations.
Perineal flora (group B streptococci, enterococci, Escherichia coli )
Ascending infection
Premature rupture of membranes (PROM)
Infection while passing through birth canal
Patchy asymmetric opacities in a term infant represent NP until proven otherwise.
Hyperinflation
TTN (wet lung syndrome) is a clinical diagnosis. It is caused by a delayed resorption of intrauterine pulmonary liquids. Normally, pulmonary fluids are cleared by:
Bronchial squeezing during delivery, 30%
Absorption, 30%: lymphatics, capillaries
Suction, 30%
Cesarean section, premature delivery, maternal sedation (no thoracic squeezing)
Hypoproteinemia, hypervolemia, erythrocythemia
Fluid overload (similar appearance to noncardiogenic pulmonary edema)
Prominent vascular markings
Pleural effusion
Fluid in fissure
Alveolar edema
Lungs clear in 24–48 hours.
Mainly utilized in neonates in cases of persistent pulmonary hypertension of the newborn (PPHN), which is an abnormal persistent right-to-left shunt of blood through the fetal circulation, leading to severe hypoxemia.
Etiologies for ECMO use:
Meconium aspiration
Pneumonia
RDS
CDH
Prenatal asphyxia
Idiopathic
Exclusion criteria for ECMO include:
<34 weeks GA
>10 days of age
Serious intracranial hemorrhage
Patients who require epinephrine
Late neurologic sequelae; developmental delay, 50%
Intracranial hemorrhage, 10%
Pneumothorax, pneumomediastinum
Pulmonary hemorrhage (common)
Pleural effusions (common)
Catheter complications
The ratio of thymus to body weight decreases with age. Thymus is routinely identified on CXR from birth to 2 years of age. Size and shape of the thymus are highly variable from person to person.
Anterior
Thymic hyperplasia and thymic variations in shape and size (most common)
Teratoma
Malignant lymphoma
Cystic hygroma
Thyroid extension
Thymomas are extremely rare.
Middle
Adenopathy (leukemia, lymphoma, tuberculosis [TB])
BFM
Vascular malformations
Posterior
Neuroblastoma
Ganglioneuroma
Neurofibromatosis (NF)
Neurenteric cysts
Meningoceles
Extramedullary hematopoeisis
Any pediatric anterior mediastinal mass is considered thymus until proven otherwise.
Posterior mediastinal masses are the most common abnormal chest masses in infants.
Further detailed in Chapter 1 . A unique radiographic sign in infants is the Spinnaker sign or elevated thymus sign.
Airway obstruction (foreign body)
Mechanical ventilation
Obstructive lung disease—asthma (Macklin effect)
Trauma
Vomiting (e.g., in patients with bulimia)
Spontaneous
Infection (pneumonia, meconium aspiration)
The GI tract develops from three embryologic precursors, each of which has a separate vascular supply: foregut, midgut, hindgut.
Origin | Vascular Supply | Derivatives |
---|---|---|
Foregut | Celiac artery | Pharynx, lower respiratory tract, esophagus, stomach, liver, pancreas, biliary tree, duodenum |
Midgut | SMA | Distal duodenum, small bowel, ascending colon |
Hindgut | IMA | Transverse colon, rectum, bladder, urethra |
Bowel is formed in three steps ( Fig. 11.17 ):
Rotation
Fixation
Canalization
Duodenojejunal loop (first 270-degree loop) brings stomach into horizontal axis and puts liver into right and spleen into left abdomen. The common bile duct (CBD) is associated with the ventral pancreas and becomes located dorsally because of the rotation. Before the rotation, the first loop rotation results in the duodenojejunal junction being located to the left.
Cecocolic loop (second 270-degree loop). The small bowel (SB) develops from the dorsal mesentery and then rotates 270 degrees so that the cecum lies near the right upper quadrant (RUQ). It later descends to the right lower quadrant (RLQ).
Fixation is incomplete at birth. Abnormal fixation of the colonic mesentery to the posterior abdominal wall results in potential spaces into which the bowel may herniate (paraduodenal hernias). Abnormalities of fixation include:
Increased mobility (cecal bascule)
Internal hernia (paraduodenal, paracecal)
After forming a primitive tube, the bowel lumen solidifies and then recanalizes to form the viable lumen. Anomalies in this step may result in atresia or duplication.
Atresia
Duodenal atresia results from failure of recanalization of the foregut (10 weeks) and is frequently associated with other abnormalities (malrotation, 50%; Down syndrome, 30%; EA).
Jejunoileal atresia results from an ischemic insult, not a failure of recanalization; occurs later and is usually an isolated phenomenon.
Duplication
Abnormal recanalization: small intramural duplication cysts may develop.
Notochordal adhesion: a portion of bowel remains adherent to the notochord and a diverticulum may develop.
Traverses the UA, the internal iliac artery, and the infrarenal aorta. Distinguishable from an umbilical venous line because the UA line travels caudally in the UA before it reaches the iliac artery and travels cranially. The tip of the line should be located above the renal arteries between levels T8 and T12. An alternative location of the catheter tip is below the renal arteries between levels L3 and L4.
The UV line should traverse the UV, portal sinus (junction of left and right portal vein), ductus venosus (which closes at 96 hours of life), and IVC and end in the right atrium (RA). The line can easily be misplaced in a portal or hepatic branch (the tip then projects over the liver).
Spectrum of anomalies involving esophagus and trachea. There are two clinical presentations:
Early presentation in EA with a pouch (N-type, 85%): vomiting, choking, difficulty with secretions
Late presentation of recurrent pneumonias if there is a fistula between esophagus and trachea (H-type, 5%)
TEF, N-type fistula, 85%
Pure EA without fistula, 10%
TEF, H-type fistula (no atresia), 1%
Other forms
VACTERL
V ertebral anomalies
A norectal anomalies
C ardiovascular anomalies
T racheal anomalies
E sophageal fistula
R enal anomalies: renal agenesis
L imb anomalies: radial ray cardiac anomalies
VSD
Ductus arteriosus
Right aortic arch
EA is associated with other atresias and/or stenosis
Duodenal atresia
Imperforate anus
Plain radiograph findings
Gas-filled dilated proximal esophageal segment (pouch)
Gasless abdomen (EA-type)
Excessive air in stomach (H-type)
Aspiration pneumonia
Procedures
Pass 8-Fr feeding tube through nose to the level of atresia; distal end of tube marks level of atresia; inject air if necessary
Definite diagnosis is established by injection of 1–2 mL of liquid barium; this, however, should be done only at a large referral institution.
Videotape the injection.
Main DDx
Traumatic perforation of posterior pharynx
Pharyngeal pseudodiverticulum
Immaturity of lower esophageal sphincter during first 3 months of life (usually resolves spontaneously)
Congenital short esophagus
Hiatal hernia
Chalasia (lower esophageal sphincter fails to contract in resting phase; differentiate from achalasia in adults)
Gastric outlet obstruction
Barium swallow
Determine presence of reflux
Exclude organic abnormalities
GE scintigraphy
Most sensitive technique to determine reflux and GE emptying
Semiquantitative method
Anatomy only poorly visualized
Foreign bodies in the esophagus may cause airway symptoms by:
Direct mechanical compression of airway
Periesophageal inflammation
Perforation and abscess
Fistulization to trachea
Foreign body lodges in coronal plane
Esophagogram may show inflammatory mass.
Fluoroscopic removal with Foley catheter in select instances (e.g., nonembedded)
Caused by hypertrophy of the circular musculature of the pylorus (differentiate from pylorospasm). Incidence: 1 : 1000 births. Male-female ratio = 4 : 1. Unknown cause. Treatment is with pyloromyotomy.
Projectile nonbilious vomiting after each feeding
Peaks at 3–6 weeks after birth
Never occurs after 3 months
Palpable antral mass (olive-sized)
Weight loss
Dehydration
Jaundice
Alkalosis
EA, TEF, hiatal hernia
Renal abnormalities
Turner syndrome
Trisomy 18
Rubella
Plain radiograph ( Fig. 11.22 )
Gastric distention (>7 cm)
Peristaltic waves result in a “caterpillar” appearance to the distended stomach.
Decreased air in distal bowel
Thick antral folds
Ultrasound (US)
HPS appears as target lesion (anechoic mass in RUQ with central echoes because of gas).
Scan in right posterior oblique (RPO) projection (move fluid into antrum)
Size criteria are used to establish the diagnosis of HPS:
Pyloric muscle thickness >3.5–4 mm
Pyloric length >15–18 mm
Useful as first imaging modality
Upper gastrointestinal (UGI) technique ( Fig. 11.23 )
Insert an 8-Fr feeding tube to decompress the stomach and drain gastric contents before administration of contrast agent.
Use right anterior oblique (RAO) view (to visualize the pylorus)
Instill 10–20 mL of barium.
Wait for pylorus to open; obtain spot views
Get air contrast view by turning patient supine
If there is no pyloric stenosis, get lateral and AP views to exclude malrotation.
UGI findings of HPS:
Indented gastric antrum (shoulder sign)
Compression of duodenal bulb
Narrow and elongated pylorus: string sign
Intermittent findings of pyloric stenosis. Treatment is with antispasmodic drugs.
Adrenogenital syndrome
Dehydration
Sepsis
Pyloric musculature is of normal thickness.
Prominent mucosa (echogenic)
Exclude secondary causes of pylorospasm (e.g., ulcer).
Mesenteroaxial volvulus: pylorus lies above GE junction (GEJ).
Occurs with eventration of left diaphragm or diaphragmatic hernia
Acute syndrome: obstruction, ischemia
Organoaxial volvulus: rotation around long axis of stomach
Rare in children
Associated with large hiatal hernia
Lesser curvature is inferior and greater curvature lies superior.
Associated gastric outlet obstruction
Inherited genetic disorder (X-linked and autosomal recessive [AR]) leading to dysfunctional nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in phagocytic cells (leukocytes and monocytes), preventing normal respiratory burst. Usually presents at less than 5 years of age.
Recurrent bacterial and fungal infections: Recurrent pneumonia (80%), osteomyelitis (30%, especially small bones of the feet and hand)
Lymphadenitis, abscess, and granuloma formation
Hepatosplenomegaly
GI manifestations from granuloma infiltration: esophageal strictures and antral narrowing (characteristic), which may lead to gastric outlet obstruction
Results from failure of recanalization (around 10 weeks). Incidence: 1 : 3500 live births. Atresia: stenosis = 2 : 1. Common cause of bowel obstruction. Bilious vomiting occurs within 24 hours after birth. Treatment is with duodenojejunostomy or duodenoduodenostomy.
30% have Down syndrome.
40% have polyhydramnios and are premature.
Malrotation, EA, biliary atresia, renal anomalies, imperforate anus with or without sacral anomalies, CHD
Enlarged duodenal bulb and stomach (double-bubble sign)
Small amount of air in distal SB does not exclude diagnosis of duodenal atresia (hepatopancreatic duct may bifurcate in “Y” shape and insert above and below atresia).
Variant of duodenal stenosis caused by an obstructive duodenal membrane. The pressure gradient through the diaphragm causes the formation of a diverticulum (“wind sock” appearance).
Uncommon congenital ring-like position of pancreas surrounding the second portion of duodenum. Results from abnormal rotation of embryonic pancreatic tissue. The annular pancreas usually causes duodenal narrowing. Diagnosis is made by endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP).
Rare. Well-defined, expansile, less infiltrative compared with adult pancreatic tumors.
Pancreatoblastoma (most common, young children <10 years old): heterogeneous large multilocular septated masses which enhance
Solid pseudopapillary tumor (adolescent girls): low malignant potential, cystic and solid, hemorrhagic, may have calcification.
Islet cell tumors (older children): insulinomas tend to be smaller compared with gastrinomas
Normally, the rotations place the ligament of Treitz to the left of the spine at the level of the duodenal bulb. Distal end of mesentery is in RLQ. In malrotation the mesenteric attachment is short, allowing bowel to twist around the superior mesenteric vessels ( Fig. 11.25 ). Superior mesenteric vein (SMV) compression (edematous bowel) is followed by superior mesenteric artery (SMA) compression (gangrene).
Gastroschisis
Omphalocele
Diaphragmatic hernia
Duodenal or jejunal atresia
Plain radiograph
Suspect diagnosis in the setting of bowel obstruction and abnormally positioned bowel loops.
A normal position of the cecum does not exclude malrotation but makes it much less likely.
US
Reversal of SMA (right) and SMV (left) location may occasionally be diagnosed by color Doppler imaging.
Distended proximal duodenum
Usually not useful
UGI (gold standard)
Abnormal position of duodenojejunal junction because of absence of ligament of Treitz
Beaking at site of obstruction
Spiraling of SB as it twists around SMA (corkscrew) ( Fig. 11.26 )
Edema of bowel wall
Early diagnosis is essential to prevent bowel necrosis.
Barium enema (BE)
A normal BE will rule out 97% of malrotation.
Can determine if there is a concomitant cecal volvulus
CT
SMA to right of SMV
Gastric outlet obstruction
Spiraling of duodenum and jejunum around SMA axis
Peritoneal bands in patients with malrotation. The bands extend from malplaced cecum to porta hepatis and may cause duodenal obstruction.
Treatment for malrotation. Involves counterclockwise derotation of the intestinal twist, widening of the mesenteric root, positioning of the SB on the right hemiabdomen and large bowel on the left, and prophylactic appendectomy.
As a result of in utero ischemia rather than recanalization failure. Ileum is most commonly affected, followed by jejunum and duodenum; may involve multiple sites. Associated with polyhydramnios in 20%–40%.
Dilated SB
Microcolon
In 10% of patients with CF, meconium ileus is the presenting symptom. Thick, tenacious meconium adheres to the SB and causes obstruction, usually at the level of the ileocecal valve. Meconium ileus can be uncomplicated (i.e., no other abnormalities) or complicated (50%) by other abnormalities, such as:
Ileal atresia
Perforation, peritonitis
Stenosis
Volvulus
Plain radiograph
Neuhauser sign: “soap bubble” appearance (air mixed with meconium)
SB obstruction
Calcification because of meconium peritonitis, 15%
Enema with water-soluble contrast medium
Microcolon is typical: small unused colon
Distal 10–30 cm of ileum is larger than colon.
Inspissated meconium in terminal ileum
Hyperosmolar contrast may stimulate passage of meconium.
Neonatal intestinal obstruction as a result of colonic inertia (in contradistinction to meconium ileus where there is accumulation of meconium in SB). Because of the inertia, the colon usually requires an enema to stimulate peristalsis. Usually occurs in full-term babies and infants of diabetic mothers. In the spectrum with small left colon syndrome. No association with CF.
As a result of prenatal perforation of bowel. The inflammatory response may cause peritoneal calcification as early as 12 hours after perforation. Can also observe calcifications in testes.
Occurs in older patients with CF, usually patients who do not excrete bile salts. The inspissated fecal material causes intestinal obstruction.
Invagination of a segment of bowel into more distal bowel.
Ileocolic
Ileoileocolic
Ileoileal
Colocolic
Ileocolic and ileoileocolic intussusception make up 90% of all intussusceptions. 90% of all pediatric intussusceptions have no pathologic lead point (hypertrophy of lymphoid tissue); in the remaining 10%, the lead point is due to:
Meckel diverticulum
Polyp or other tumors (lymphoma)
Intramural hematoma
Mesenteric adenitis
Henoch-Schönlein purpura
Usually in the first 2 years of life (40% from 3–6 months), rarely in neonates
Colicky crampy pain, 90%
Vomiting, 90%
Sausage-shaped mass, 60%
Blood per rectum (currant jelly stool), 60%
Bowel obstruction
Intestinal ischemia
Perforation
Sepsis
Dehydration
Plain radiograph
Frequently normal (50%)
Intraluminal convex filling defect in partially air-filled bowel loop (commonly at hepatic flexure)
US
Target or doughnut sign
Alert pediatric surgeon (in case of perforation).
Preliminary kidney, urethra, bladder (KUB) to rule out free air.
Insufflate air rapidly, maintaining air pressure below 120 mm Hg. Alternatively, use dilute contrast (e.g., Cysto-Conray 17%) in bag placed 3 feet above table top. Rectal tube may be taped to reduce air leak.
Maintain constant hydrostatic pressure but no longer than 3 min against the nonmoving intussusception mass. If patient evacuates around tube, repeat filling may be performed up to 3 tries before surgery is contemplated. (Rule of 3s: bag 3 feet high, 3 min per try, and 3 tries)
Successful reduction is marked by free flow of air/contrast into the terminal ileum. Fluoroscopy is used to evaluate for a pathologic lead point. A postevacuation and a 24-hour radiograph are obtained.
Contraindications to reduction:
Perforation
Peritonitis
Henoch-Schönlein purpura (predisposes to perforation)
Complications
Recurrence in 6%–10% (half within 48 hours after initial reduction)
Perforation during radiographic reduction is rare (incidence: 1 : 300 cases)
Small vessel vasculitis presenting with rash, subcutaneous edema, abdominal pain, bloody stools, and arthritis.
Intramural hemorrhage of SB
Intussusception, typically ileoileal
Gallbladder (GB) hydrops
Echogenic kidneys
Location: SB (terminal ileum) > esophagus > duodenum > jejunum > stomach (incidence decreases from distal to proximal, skips the stomach). Duplication cysts typically present as an abdominal mass. SB duplications are most commonly located on the mesenteric side; esophageal duplications are commonly located within the lumen.
Round fluid-filled mass displacing adjacent bowel
May contain ectopic gastric mucosa (hemorrhage)
Calcifications are rare.
Communicating vertebral anomalies (neurenteric cysts; most common in esophagus)
Omphalomesenteric duct anomalies are due to persistence of the vitelline duct, which connects the yolk sac with the bowel lumen through the umbilicus. Spectrum:
Meckel diverticulum
Patent omphalomesenteric duct (umbilicoileal fistula)
Omphalomesenteric cyst (vitelline cyst)
Omphalomesenteric sinus (umbilical sinus)
Persistence of the omphalomesenteric duct at its junction with the ileum. Rule of 2s:
Occurs in 2% of population (most common congenital GI abnormality)
Complications usually occur before 2 years of age
The diverticulum is located within 2 feet of the ileocecal valve.
20% of patients have complications.
Hemorrhage from peptic ulceration when gastric mucosa is present within lesion
Inflammation and ulcer
Obstruction
Difficult to detect because diverticula usually do not fill with barium.
Pertechnetate scan is the diagnostic imaging modality of choice to detect ectopic gastric mucosa (sensitivity 95%).
False-positive pertechnetate studies may occur in:
Crohn disease
Appendicitis
Intussusception
Abscess
Age: >4 years (most common cause of SB obstruction) (see Chapter 3 ).
Plain radiograph
Mass in RLQ
Obliterated properitoneal fat line
Sentinel loop
Fecalith
US
May be useful in children
Thickened appendiceal wall shadowing appendicolith, RLQ abscess
BE (rarely done)
A completely filled appendix excludes the diagnosis of appendicitis.
15% of normal appendices do not fill with contrast.
Signs suggestive of appendicitis:
Beak of barium at base of appendix (mucosal edema)
Irregularity of barium near tip of cecum
Deformity of cecum (abscess, mass effect)
CT
Helical CT with opacification of the GI tract achieved through the oral or rectal administration of 3% diatrizoate meglumine solution is useful to diagnose or exclude appendicitis and to establish an alternative diagnosis.
Most common GI emergency in premature infants. Precise causes unknown (ischemia? antigens? bacteria?). Develops most often within 2–6 days after birth.
Indication for surgery:
Pneumoperitoneum
Increased incidence in:
Premature infants
Neonates with bowel obstruction (e.g., atresia)
Neonates with CHD
SB dilatation: adynamic ileus (first finding), unchanging configuration over serial radiographs
Pneumatosis intestinalis, 80% (second most common sign)
Gas in portal vein may be seen transiently (US more sensitive than plain radiograph); this finding does not imply as poor an outcome as it does in adults.
Pneumoperitoneum (20%) indicates bowel perforation: football sign (floating air and ascites give the appearance of a large elliptical lucency in supine position).
Barium is contraindicated; use water-soluble contrast if a bowel obstruction or Hirschsprung disease needs to be ruled out.
Acute
Perforation
Later in life
Bowel stricture (commonly near splenic flexure)
Complications of surgery: short SB syndrome, dumping, malabsorption
Complications of associated diseases common in premature infants:
HMD
Germinal matrix hemorrhage
Periventricular leukomalacia
Absence of the myenteric plexus cells (aganglionosis, incomplete craniocaudal migration of embryonic neuroblasts) in distal segment of the colon causes hypertonicity and obstruction. Clinical: 80% (male-female ratio = 6 : 1) present in the first 6 weeks of life with obstruction, intermittent diarrhea, or constipation. Diagnosis is by rectal biopsy. Treatment is with colostomy (Swenson, Duhamel, Soave operations), myomectomy. Associated with Down syndrome.
Intestinal obstruction (in neonates)
Perforation
Enterocolitis 15%, cause uncertain
Bowel gas pattern of distal colonic obstruction on plain radiograph
BE is normal in 30%
Transition zone between normal and stenotic colonic segment
Rectosigmoid ratio is abnormal (<1) because of the narrowed segment.
Sawtooth appearance of colon on contrast enema
Significant barium retention on the 24-hour BE radiograph may be helpful.
Transition zone in rectosigmoid, 80%
Failure of descent and separation of hindgut and genitourinary (GU) system in second trimester.
High (supralevator) malformations
Rectum ends above levator sling
High association with GU (50%) and cardiac anomalies
Low (infralevator) malformations
Rectum ends below levator sling
Low association with GU anomalies (25%)
Male | Female | |
---|---|---|
High malformation | Fistula to the urethra or less commonly to the bladder | Rectovaginal fistula, hydrometrocolpos |
Low malformation (bowel passes through levator sling) | Anoperineal fistula | Fistula to lower portion of urethra, vagina perineum |
Associated anomalies are very common:
Sacral anomalies, 30% (tethered cord)
Currarino triad: anorectal anomaly, sacral bone abnormality, and presacral mass
GU anomalies, 30%
VACTERL
GI anomalies: duodenal atresia, EA
Plain radiograph
Low colonic obstruction
Gas bubbles in bladder or vagina
Determine location of the gas-filled rectal pouch to the M-line (line drawn through junction of upper two-thirds and lower one-third of ischia; line indicates level of levator sling).
Upside-down views can be obtained to distend distal rectal pouch.
Magnetic resonance imaging (MRI)
Determine location of rectal pouch with respect to levator and to determine if the malformation is supralevator or infralevator type.
Facilitates the detection of associated spinal anomalies
Unknown cause (congenital, autoimmune with perinatal viral infection, toxins?). More common in East Asians. Most common indication for liver transplantation in children.
Type I: Atresia restricted to CBD
Type II: Atresia including common hepatic duct
Type III: Atresia involves left and right hepatic ducts and porta hepatis, most common (>90%)
Kasai operation (hepatoportoenterostomy): bridge to liver transplant to help relieve jaundice and delay or stop liver fibrosis. Earlier age of surgery (before 60 days) and high volume surgical center are most important prognostic factors.
Main differential is neonatal hepatitis, which is often idiopathic (known causes include viral hepatitis, CMV, rubella, and other viruses). Neonatal hepatitis is a diagnosis of exclusion after other causes of cholestasis have been ruled out.
US
Normal GB in 20%
Failure to detect extrahepatic bile ducts
Hepatic iminodiacetic acid derivative (HIDA) scan
No visualization of bowel at 24 hours
Visualization of GB is not helpful and can be seen in 20%.
Good hepatic visualization within 5 minutes excludes diagnosis.
Preprocedural phenobarbital (5 mg/kg per day × 5 days) improves sensitivity of hepatobiliary scans. The finding of normal (≥1.5 cm) or enlarged (≥3 cm) GB is more supportive of diagnosis of hepatitis.
Main DDx is neonatal hepatitis. Findings of neonatal hepatitis include:
Bowel activity present at 24 hours
Decreased and slow hepatic accumulation of tracer
GB may not be seen
One must exclude the presence of CF in patients thought to have biliary atresia. Inspissated bile in CF can be indistinguishable from biliary atresia by US or nuclear scan.
Hemangioma | Hemangioendothelioma | |
---|---|---|
Age | Older children | <6 months |
Size | <2 cm | 2–15 cm |
Location | Right lobe | Both lobes |
Symptoms | No | Hepatomegaly, CHF |
Ultrasound | Well-defined hyperechoic lesion | Variable |
Malignant potential | No | Rare |
Most common benign pediatric liver tumor. 85% present at <6 months. Associated cutaneous hemangiomas in 50%.
CHF because of arteriovenous (AV) shunt, 15%
Intraperitoneal hemorrhage
Disseminated intravascular coagulopathy
Thrombocytopenia (Kasabach-Merritt syndrome)
Complex, hypoechoic mass by US
Similar signal intensity/contrast characteristics as adult hemangioma
Occurs during first 10 years of life
Large multiloculated cystic lesion
Large cysts >10 cm may be seen.
10% are exophytic.
Most common primary malignant liver tumor in children. Age: <4 years. Elevated alpha-fetoprotein (AFP).
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