Board review in Black andwhite

presented by: MV Pertubal, MD

Q1 A 9-year-old boy who has acquired short stature presents to the emergency department with a headache and vomiting. He had been one of the tallest boys in his class in the first grade but now is one of the shortest. His clothing size has not changed in 18 months. Review of systems is positive for frequent nocturnal urination and negative for fever, stomach pain, and diarrhea.

On physical examination, the boy appears well after vomiting, and general examination findings are within normal parameters. On neurologic examination, he converses appropriately. Visual field testing shows an apparent inability to count fingers in either lateral visual field. Extraocular movements are full, facial movements are symmetric, and tongue and palate movements are normal. Strength and reflexes are normal in all limbs. His gait is normal and not broad-based.

Brain MRI shows a midline mass with both cystic and enhancing solid components (Courtesy of M Sutton) T1-weighted sagittal MRI, as described for the boy in the vignette. There is a suprasellar mass (defined by yellow arrows). The inferior portion is solid and partially calcified ( red arrow ) and the superior portion is cystic and fluid-filled ( blue arrow ).

What is the diagnosis? A. brain abscess B. craniopharyngioma C. meningioma D. primitive neuroectodermal tumor E. subependymal giant cell astrocytoma

B. craniopharyngioma what would explain the vomiting and diarrhea?

elevated intracranial pressure what is the key finding on physical exam that would support the diagnosis? bitemporal hemianopsia what would explain the acquired short stature? growth hormone deficiency or central hypothyroidism frequent urination? deficient ADH

Craniopharyngiomas

- slow-growing tumors - often present in the first 10 years of life. - cell source is believed to be embryonic epithelial cells in the region of

Rathke’s cleft - Tx:

Surgery +/- radiation The tumor may appear hyperintense if they contain high protein, blood products, and/or cholesterol (in the classic adamantinomatous type). In the papillary variety, solid components appear isointense on T1-weighted images http://www.radpod.org/2007/06/22/craniopharyngioma/

The inferior portion is solid and partially calcified ( red arrow ) and the superior portion is cystic and fluid-filled

Why not a brain abscess? (Courtesy of D Mulvihill) Sagittal T1-weighted MRI demonstrates a low attenuation lesion in the parietal lobe, with a contrast-enhancing rim and surrounding edema

brain abscess - imaging should be done WITH CONTRAST - MRI is more sensitive than CT - Diffusion weighted MR imaging (DWI) - help differentiate brain abscess from metastatic lesions

Why not a primitive neuroectodermal turmor? - a subset of embryonal tumors (WHO classification) - 85% are located in the cerebellar vermis (MEDULLOBLASTOMA) (Courtesy of D Krueger) Sagittal T1–weighted MRI following contrast shows a heterogeneous, enhancing solid lesion in the llar vermis (arrows) consistent with a medulloblastoma.

Besides Medulloblastoma, name other posterior fossa tumors?

Gliomas : - astrocytoma - oligodendroglioma - ependymoma ** associated with cerebellar findings - ataxia, obstructive hydrocephalus Sagittal T1 post-contrast MRI of a pilocytic astrocytoma (large cystic mass with a mural nodule, arrow) in the posterior fossa

What is the most common posterior fossa tumor? Pilocytic astrocytoma What is the most common malignant brain tumor in children?

medulloblastoma

Why not subependymal giant cell astrocytomas? - found along walls of lateral ventricles - hamartomatous lesions appearing as subependymal masses - associated with tuberous sclerosis complex “tubers” (Courtesy of D Krueger) Coronal MRI demonstrating a subependymal giant cell astrocytoma. Typically in tuberous sclerosis complex and arise at the foramina of Monro where they may cause obstructive hydrocephalus.

Q2 18-month-old boy in the emergency department who appears "toxic" and is sitting uncomfortably and leaning forward in his mother's lap. His temperature is 40.0°C, heart rate is 140 beats/min, respiratory rate is 35 breaths/min, blood pressure is 90/60 mm Hg, and oxygen saturation on room air is 94% by pulse oximetry. He is drooling from the corners of his mouth, and his cry appears muffled.

lateral neck radiograph showed :

Of the following, the MOST appropriate next step in the treatment of this patient is A. administration of intramuscular penicillin B. blood cultures and a complete blood count C. emergent otolaryngology and anesthesia consultation D. intramuscular administration of dexamethasone E. throat culture

C. emergent otolaryngology and anesthesia consultation What condition is being described ?

(A) A normal epiglottis on a lateral neck radiograph, with the structures illustrated in panel B. Epiglottitis is similarly depicted radiographically (C, D).

©2012 UpToDate ® Reproduced with permission from: Fleisher GR, Ludwig W, Baskin MN. Atlas of Pediatric Emergency Medicine. Philadelphia: Lippincott Williams & Wilkins, 2004. Copyright © 2004 Lippincott Williams & Wilkins.

What are the top causes of epiglottitis?

Haemophilus influenzae type b (Hib)* H. influenzae types A and F, and nontypeable strains Haemophilus parinfluenzae Streptococcus pneumoniae Staphylococcus aureus (MSSA and MRSA) Beta-hemolytic streptococci: Groups A, B, C, F, G

Q3 42-weeks’ gestation infant is delivered with moderate meconium-stained fluid. The prenatal course was unremarkable except for group B Streptococcus colonization that was treated adequately during labor. The infant requires endotracheal suctioning in the delivery room, which produces scant meconium-stained fluid from below the vocal cords.

He is taken to the special care nursery receiving blow-by oxygen, but worsening respiratory distress leads to intubation and initiation of assisted ventilation requiring an Fio 2 of 70% to maintain an O2 sat of 95%.

As you are calling the tertiary center to arrange transport, his saturations acutely deteriorate, requiring 100% oxygen to maintain his O2 saturation at 85%.

You obtain a chest radiograph which showed

Of the following, the intervention MOST likely to improve this infant’s respiratory status is A. adjustment of the endotracheal tube B. administration of exogenous surfactant C. infusion of prostaglandin E1 D. initiation of inhaled nitric oxide E. insertion of a chest tube

E. insertion of a chest tube What condition is being suspected?

Pneumothroax

What clinical findings in pneumothorax? tachypnea, grunting, flaring, retractions, and cyanosis, on inspection - asymmetric chest wall, with prominence of the affected side; shift of the point of maximum cardiac impulse.

on auscultation - diminished or absent breath sounds on the affected side.

Do all pneumothoraces require invasive treatment? Spontaneous pneumothoraces may occur upto 2% of healthy infants. Most are asymptomatic and resolve without intervention. Continuous positive airway pressure (CPAP)and positive-pressure mechanical ventilation (PPMV) further increase the risk of pneumothorax.

Q4 A 12-year-old girl who has a history of chronic rhinitis, recurrent sinusitis, and multiple pneumonias has had a productive cough for 2 months. She has had no fever or other systemic symptoms except fatigue. Bronchodilators provide limited symptomatic relief. Two previous courses of antibiotics have produced transient but limited improvement.

On physical examination, you note a slender child without clubbing. Her respiratory rate is 28 breaths/min, and faint crackles and wheezes are audible throughout her chest. She has purulent rhinitis with maxillary sinus tenderness.

A chest radiograph shows areas of linear atelectasis with thickened airways Results of sweat chloride testing and direct mutation analysis for cystic fibrosis are negative.

Of the following, the MOST appropriate next test in her evaluation is A. allergy skin testing B. echocardiography C. high-resolution chest computed tomography scan (HRCT) D. nasopharyngeal culture for virus E. sinus radiographs

C.

C. high-resolution chest computed tomography scan (HRCT) What is the case describing? Bronchiectasis (Courtesy of E Anthony) Chest computed tomography scan in bronchiectasis demonstrating cylindrical dilation of an airway (arrows).

Bronchiectasis

- is a chronic disease of the conducting airways characterized by irreversible dilation of the bronchial tree - is not a disease in itself but rather the end result of several different processes that can be classified as obstructive, infectious, defective host defense/immunologic, and congenital/genetic.

http://www.merckmanuals.com/media/home/figures/PUL_bronchiectasis.gif

Gold standard for diagnosis is HRCT diagnosis is made when the internal diameter of the airway is larger than the diameter of the adjacent artery and/or bronchi, visualized within 1 cm of the pleural surface

(Courtesy of B Wood) Lateral chest radiograph in bronchiectasis demonstrating linear atelectasi s (arrows).

Radiologic clues to cause of bronchiectasis

what is the most common cause of bronchiectasis in developed countries?

cystic fibrosis what is the most common cause of bronchiectasis in developing countries?

infectious causes (pertussis, mycobacteria, measles, and pneumococcus)

Q5 5-day-old infant, presents with bile-stained emesis. Born at term following an uncomplicated pregnancy and delivery, he weighed 3,300 g. He was sent home at 36 hours of age. Since discharge, he has been nursing every 2 to 3 hours at home, but he recently developed apparent discomfort postprandially. His mother has observed no stools since discharge. This morning, he began to spit up after feeding, and the last emesis was bile-stained.

Upon arrival at the emergency department, the infant appeared alert but irritable and had moderate, generalized abdominal distension. The emergency department physician obtained an upright abdominal radiograph ( Item Q52 ).

Of the following, the MOST appropriate next step is A. abdominal computed tomography scan B. contrast enema C. rectal suction biopsy D. surgical decompression E. upper gastrointestinal tract radiographic series

B. contrast enema

no passage of stools + = distal bowel obstruction dilated bowel loops

Review: Normal bowel gas pattern in neonate supine film will show a recognizable polygonal bowel gas pattern http://radiographics.rsna.org/content/27/2/285/F8.expansion

in assessing abdominal x-rays: -

D

istribution of gas -

D

ilatation of bowel

A

rrangement of loops - any

A

ir-fluid levels?

Role of water soluble contrast

enema

in distal bowel obstruction 1. identify the level of obstruction 2. relieve the obstruction Remember: oral contrast should be avoided

What would be your Top 2 differential diagnoses for distal bowel obstruction in newborns?

1. Meconium ileus 2. Hirschprung’s disease

Q6 9 year-old boy presents with worsening right and left lower quadrant abdominal pain of several months' duration. The pain occurs at any time of day or night and has awakened him from sleep on several occasions. Past history includes an appendectomy at age 6 years and a tonsilloadenoidectomy at age 8 years.

On physical examination, his abdomen appears moderately distended and, while being examined, he again begins to feel discomfort and vomits a moderate amount of yellow green fluid.

You order an abdominal plain radiograph that showed

Of the following, the MOST appropriate next step is to: A. begin intravenous hydration and bowel rest B. insert a nasogastric tube for decompression C. obtain a barium enema D. obtain abdominal computed tomography scan E. refer the boy for surgical exploration

B. insert a nasogastric tube for decompression

Q7 2-day-old baby who was born at term and was admitted to the neonatal intensive care unit due to respiratory distress. Anteroposterior chest radiography reveals multiple butterfly vertebrae (Item Q103) .

On physical examination, the baby is normally grown and formed except for hypoplastic thumbs. You request echocardiography, which reveals a large ventricular septal defect. The baby is feeding and stooling well.

Of the following, the recommendation that is MOST helpful in guiding further management for this infant is A. fluorescence in situ hybridization B. hematology consultation C. renal ultrasonography D. serum calcium quantitation E. urine organic acid analysis

C. renal ultrasonography what is being described in the vignette? VATER V ertebral anomalies A nal atresia, T racheo E sophageal fistula, R enal/Radial defects or VACTERL V ertebral anomalies, A nal atresia, C ardiac defects, T racheo E sophageal fistula, R enal anomalies, L imb [radial] anomalies) association

Q8 14-year-old boy is brought to the ED from a local skateboard park after sustaining a left elbow injury while skateboarding. He reports that he was skating down an incline at high speed when he lost his balance and fell, breaking his fall with his left arm. He had immediate pain in his left elbow and noticed that it "looked out of place." Physical examination reveals moderate swelling of the elbow with prominence of the olecranon.

Radiographs of his elbow reveal

Of the following, the MOST likely additional finding on physical examination for this boy is loss of A. sensation over the palmar aspect of the thumb and index finger B. sensation over the fifth finger C. thumb flexion D. thumb extension E. wrist extension

B. sensation over the fifth finger which neurovascular structure is being compromised? ulnar nerve

Ulnar nerve injury decreased sensation over the fifth finger loss of wrist flexion finger abduction occurs in 10% of posterior elbow dislocation Median nerve injury decreased sensation over palmar aspect of thumb and first finger decreased thumb flexion Radial nerve injury loss of wrist and thumb extension Brachial artery injuries suspect in patients who exhibit decreased radial pulse, pallor, and forearm paresthesias.

“peace sign” for ulnar nerve motor function

“Thumbs up” checks intact radial nerve motor function.

“ OK sign” to assess median nerve

Thank You!

Peace!

Sources/Referrences : PREP 2010 PREP 2011 PREP 2012 Uptodate Donnelly, L. F. (2009).

Pediatric Imaging: The Fundamentals

. Philadelphia: Saunders, Elsevier.

Haller, J., Slovis, T., & Joshi, A. (2005).

Pediatric Radiology

(3rd ed.). Berlin/Heidelberg: Springer-Verlag. doi:10.1007/b137591 http://www.ispub.com/journal/the-internet-journal-of-gastroenterology/volume-2-number 1/opening-pandora-s-box-the-role-of-contrast-enemas-in-abdominal-imaging.html