Annual Meeting, September 16, 2012

North Carolina Pediatrics Society Annual Meeting Day #3

Welcome to the official blog of the NC Pediatrics meetings! Thanks to Blogger, we'll be going in reverse order, starting with day #3 and working our way back to day #1, like Benjamin Button, but a blog. If you're big on chronological order, just start with the September 14 post, and read up!

This meeting was perhaps the most amazing yet, and not just because Dr. Perri Klass, famous author and founder of Reach Out And Read gave the keynote address, and not just because AAP Executive Director Errol Alden came, but they did help. I've already come back to my practice with a head full of new ideas and practice changes, and I'm really excited about the moves afoot to improve care statewide. At the same time, I hope you won't skim over the policy updates. Children need our advocacy more now than ever, and I urge you to heed the calls below to get involved.

As always, I must clarify that this is not an official record of the NC Pediatric Society, just my best attempt to keep up with the amazing stream of information coming at me from what may have been the best group of speakers ever! If you notice a typo or something that doesn't look right, please let me know. For official slides and handouts please contact the speakers or the North Carolina Pediatric Society office (link at the top of the page). Enjoy!

Dr. Karen Breech turns the President’s gavel over to Dr. John Rusher, who, in his first act as President, presents Dr. Breech with a plaque and our gratitude for two amazing years of service.

Dr. Jennifer Crotty and Dr. Daniel Ostrovsky present resident poster awards from this weekend’s record-breaking set of poster presentations. “No Bones About It, An Unusual Case of Scurvy” #3. “Diagnosis and Screening Of Pediatric Hypertension In Primary Care Physician Offices” #2. “Autism Screening In Spanish-Speaking Patients.” #1.

Michelle Trager Cabrera, MD, Assistant Professor of Pediatric Ophthalmology UNC

Understanding Amblyopia and Strabismus

• Review of eye anatomy: cornea, iris, lens, anterior chamber, angle & trabecular meshwork, ciliary body, sclera, retina, choroid layer, optic nerve (CNII), vitreous cavity/posterior chamber, conjunctiva, episclera (under the conjunctiva)
• Afferent visual pathways: optic nerve signals cross to lateral geniculate nucleus, prject to striate cortex with monocular and binocular cells. 70% of the cells in the striate cortex are binocular.
• Binocular vision arises from simultaneous perception of separate but slightly dissimilar images arising in each eye, appreciating them as a single image. Process is called “fusion”.
• Binocular vision allows a single image, stereopsis, enlargement of the visual field, compensation for the blind spot.
• Monocular cues can give clues to depth and distance, so some function is preserved even with monocular vision.
• Newborns have poor visual acuity, around 20/800 or worse. By 4 months 20/400, 8 months 20/100, 1 year 20/50. 6 month old babies still see quite blurry images.
• Amblyopia is decreased visual acuity NOT attributable to organic eye disease. Caused by damage to the lateral geniculate nucleus and striate cortex during the sensitive period of visual development.
• Amblyopia is a spectrum of vision loss, from mild to severe. Defined as >2 lines of eye chart difference. Most common cause of vision loss in children. Much more common with premies, low birth weight, developmental delay, positive family history
• About half are associated with strabismus.
• Pattern deprivation: something blocking the entrance of light to the retina.
• Strabismus: eyes are mis-aligned.
• Optical defocus: child needs glasses, but is not wearing them, so receives a blurry image, worse with more difference between the two eyes.
• Window of effective therapy is between age 7 and 14, around age 9 on average
• How do we test vision in a pre-verbal child?
• Light-wince reflex in newborns.
• Fix-and-follow response over age 3 months (toy or light must be silent). Check one eye at a time as well as both together.
• Research: visual evoked potential for pre-verbal children. 
• Preferential looking test in the ophthalmology clinic, uses card with stripes of varying widths. Child prefers stripy side rather than gray box.
• Verbal child: optitype testing with pictures, letters. Use heel of hand, not fingers for one-eye testing!
• Refer for lack of wince to light response, failure to fix and follow by age 3 months,  vision worse than 20/40 in young kids, worse than 20/30 if over age 6, difference in eyes greater than 2 lines of vision chart.
• Treatment: provide clear retinal image, occlude the good (sound) eye with patch or drops.
• Exotropia: “wall” eyes, Esotropia: “cross” eyes. Hypertropia: one eye looks upward.
• Strabismus in children is asymptomatic due to suppression of the wandering eye!
• Why treat early? Strabismus could suggest treatable underlying disease, could cause amblyopia, could signify life-threatening condition, could save binocular vision, can prevent social and developmental problems in school.
• Negative social attitudes toward strabismus patients starts around age 5 years (dolls used to guage behavioral responses). Esotropia and exotropia elicited a lot of negative responses.
• Children with strabismus show increased rates of depression, anxiety, and social phobia.
• Often waits until school age for surgical repair, since that’s when social consequences begin.
• Corneal light reflex test: shine light straight at the child, look at where the light falls in relation to the pupil. Center it in one eye, then glance at the other eye for comparison. If light falls on iris rather than the pupil, child has strabismus.
• Cover testing: difficult, especially in uncooperative child. Get a good fixation target. Cover one eye with the heel of your hand, not your fingers, watch the uncovered eye for movement, a quick flick movement, will occur in the other eye when you switch. The movement is in the opposite direction of the gaze abnormality (compensating)
• Ophthalmologists can use prisms to neutralize strabismus, measuring just how many degrees of strabismus are present.
• Binocular vision test: Worth 4 dot test. Stereopsis testing. 
• Pseudostrabismus: looks like esotropia due to wide nasal bridge or prominent epicanthal folds. Doesn’t mean child cannot also have true strabismus.
• Harder to judge strabismus with darker iris color. Look at the whole iris in those cases.
• Causes of strabismus: visual deprivation, refractive error, idiopathic (developmental delay, prenatal drug/EtOH exposure), congenital disease, orbital disease, rare syndromes.
• Refractive error is also called accomodative esotropia. Can be fully corrected with glasses. Accomodative convergance mechanism is simply too strong.
• Infantile esotropia starts before age 6 months.
• Intermittent esotropia: starts around age 2. Tend to have good vision, good stereopsis.
• Head tilt: cranial nerve 4 palsy. Try pulling the child’s head the other direction to see what happens to his eyes!
• Strabismus can be normal at birth, should resolve by 2-3 months of age, when child begins to fix and follow.
• When to refer? Any child >3 months old with strabismus on your exam or based on caretaker’s history, even if you don’t see it. Children with strong risk factors such as genetic syndromes, prematurity, cerebral palsy.
• Strabismus surgery: lateral rectus, medial rectus insert anteriorly on the surface of the sclera, covered by conjunctiva. Superior rectus elevates, inferior rectus, lateral rectus, medial rectus, superior oblique, inferior oblique. Superior oblique turns in, depresses globe. Inferior oblique extorts and elevates the eye.
• Most common procedure: rectus muscle resession, which removes muscle from globe, re-attaches so it’s more slack. 
• Resection is a strengthening procedure, shortens the muscle.
• Q&A: visual evoked potentials are probably not a useful routine screening tool. May just pick up normal-variant delayed visual maturation in infancy.
• Q&A: Red reflex, look for symmetry from one side to the other. Difference may be from anisometropia as well as cataracts, tumors. Leukocoria is reason for urgent referral.
• Q&A: mild refractive error in the school years can often be followed annually without corrective lenses. With age, child is more likely to notice visual error, usually around age 9 years. Myopia likely to worsen with progressive eye growth.
• Q&A: photo-screeners probably work reasonably well. Evaluate relative red reflex.  Pick up strabismus, anisometropia.

Dr. Amina Ahmed, Pediatrics, Pediatric Infectious Diseases, Carolinas Medical Center/Levine Children’s Hospital

A Decade Of newborn hearing Screening in North Carolina

• At Carolinas they have been screening children for congenital CMV infection, then following up with regular hearing screening, as up to 15% develop hearing loss.
• Many of the babies who refer based on hearing screen never make it to follow up!
• Congenital hearing loss is the most common birth defect, affects 1-2:1,000 live births, up to 3/1000 by age 5 years. Many babies have delayed-onset hearing loss, often from congenital CMV
• Most common cause at birth is genetic, usually non-syndromic. Then CMV, perinatal infections. Usually asymptomatic.
• As kids age, CMV takes on a larger percentage of the overall hearing loss diagnoses.
• Half of children with hearing loss are NOT easily identified at birth.
• The earlier you intervene in hearing loss, the better the clinical outcomes!
• In the 1980’s, it became clear we had good screening tests. In 1993 the NIH consensus statement on screening came out. In 1994 the JCIH statement came out, and in 2000 the AAP endorsed and promoted universal newborn hearing screening.
• Data clearly endorse the efficacy of hearing intervention prior to age 6 months. Outcomes are clearly better.
• Early Hearing Detection and Intervention Program (EHDI), state by state.
• Both to identify and to TRACK these babies, make sure they have a medical home.
• Back in 2000, the evidence supporting intervention was not completely clear. By 2008 there was no question based on evidence-based literature to support early intervention.
• Screening newborns is now standard of care throughout the US. US Preventive Services Task Force supports recommendations.
• Mean age of diagnosis is now 2-3 months instead of 2-3 years!
• 2007 JCIH revised statement: identify hearing loss by age 1 month, refer by age 3 months, start intervention by age 6 months.
• High-risk babies should all see audiology: NICU grads, caregiver concern, positive family history, use of ototoxic medication, exchange transfusion, in-utero infections, facriofacial anomalies including ear pits, physical findings suggesting syndrome, head trauma, chemotherapy, neurodegenerative disorder, post-natal infections like HSV.
• Otoacoustic emissions test (OAE). Put noise into the ear, look for cochlear response.
• aABR is automated auditory brainstem response, tests the nerve, catches more disease. More expensive, harder to perform.
• A child who fails aABR should NOT then be cleared by a positive OAE!
• Results are either “pass” or “refer”. Many children who “refer” actually have normal hearing. Depends on operator of hearing test, they need good training.
• Now referral rate is 2-4% from most hospitals.
• Can use two-stage approach: re-test within two weeks at the birth hospital. We need to track whether these babies are making it back!
• The older kids are, the harder it is to perform a BAER test, so refer to audiology as early as possible!
• At age 6-36 months testing is behavioral audiometry, OAE, tympanometry, ABR.
• Looking for causes of hearing loss consider genetic testing early. The ENT is probably not referring to genetics.
• Congenital CMV is the most common congenital infection, usually asymptomatic, but up to 15% go on to have hearing loss, which usually develops later than the newborn screen.
• Who gets lost to follow-up? Babies who transfer hospitals, transfer MD’s, don’t have medical homes, whose moms didn’t hear about the importance of follow up. Poverty appears to play a big role in who follows up. Rural settings are more challenging.
• NC is meeting the benchmarks for newborn screening, but we don’t appear to be doing a good job of getting children to their 3-month follow up visits when the refer from screening.
• The MD/provider should ideally be the person to reinforce the message of how important it is for parents to follow up.
• Refer to AUDIOLOGY before referring to ENT! Audiology should respond faster, and they will get the ENT involved if the hearing loss is confirmed, may be able to avoid a lot of unnecessary ENT referrals!
• www.ncnewbornhearing.org
• Q&A: If in the future we can get congenital CMV screening at birth, we may be able to flag high-risk children for repeat hearing screening at age 3 months, but we’re not there yet.
• Q&A: Gerri Mattson, MD, MPH. All the birth hospitals are now part of a statewide network of hearing screening tracking. Now interest in giving primary care providers access to the system as we have for newborn screening and immunizations

Dr. Kenneth Roberts

AAP Clinical Guideline: The Diagnosis and Management of the Initial Urinary Tract Infection in Febrile Infants and Young Children* Unanswered Questions - Unquestioned Answers

• Technical report: http://pediatrics.aappublications.org/content/128/3/e749.full
• Guidelines themselves: http://pediatrics.aappublications.org/content/128/3/595.full
• Read the guidelines, technical report if you’d like (above)
• Idea from the 1960’s: smoldering pyelonephritis eating away at our children’s kidneys without symptoms. This idea never made any sense, but it survived until the 1990’s, reflected in the 1999 guidelines. It does not seem to be true, and it never was.
• Yes, this is a paradigm shift from the last forty years, which is probably a good thing!
• Population addressed: infants and young children 2 months to 24 months of age with unexplained fever. Around 5% have UTI, and rates of scarring are higher than in older children.
• Can’t really say what this means for children under 2 months of age, and these guidelines do not address that age group. Committee is meeting this month to consider this population.
• Risk levels also appear to be lower in older kids: Miralax is probably the best UTI prophylaxis in this age group!
• There are 7 key action statements, 8 areas for suggested further research.
• #1 If a clinician decides a child is going to need antibiotics for fever, please get a catheterized urinalysis for UA and culture BEFORE starting antibiotics! This is a high-quality, level A recommendation.
• Suprapubic aspiration is the “gold standard” but no one does them any more. Catheterization is comparable. If a catheter goes into the vagina, leave it there and use it as traction and don’t use it again!
• Bag urine is NOT suitable for culture. It is essentially a vaginal wash. False positives are 88% overall, 99% in circumcised boys!
• #2 If a child is not so ill as to need antibiotics, then assess likelihood of UTI. If likelihood is low (1-2%), then you can follow clinically. If likelihood is higher, then get catheterized specimen or, alternately, get any kind of urine for UA, then get a cath if UA appears possible.
• Risk factors: Girls: white race, age <12 month, temp >39 C, fever > 2 days, absence of another source of fever.
• Boys: non-black race, temp >39 C, fever >24 hours, absence of another source of fever, UNCIRCUMCISED (risk over 1% regardless of other risk factors)
• #3 UTI diagnosis requires both positive culture (>50,000 CFU of a true uropathogen) AND a positive urinalysis.
• A positive urinalysis has positive leukocyte esterase and/or nitrites, microscopy has white cells and/or bacteria.
• Asymptomatic bacteruria is not a disease, just a condition of white girls and women due to bacterial attachment, lead to positive cultures with NEGATIVE urinalyses! This is why we want both culture and UA to be positive.
• #4 Initial antimicrobial treatment can be oral or parenteral, they are equivalent!
• Choice of drug is based on local sensitivity. Be aware that sensitivity reports from local labs are based not on individual patients, but on individual samples, meaning that chronically ill, hospitalized patients skew the results in hospital labs.
• Duration of treatment: 7-14 days, data are lacking to say just how long.
• #5 Febrile infants with UTI should undergo renal and bladder imaging, but this is a weak recommendation (level C). Yield of abnormal findings worth doing anything about is quite low, even though abnormal findings themselves are quite common.
• #6 VCUG are not recommended for routine performance after the first UTI if the ultrasound is normal!
• Based on meta-analysis of six studies of whether prophylaxis versus no prophylaxis and whether it prevents renal scarring in the future. Meta-analysis showed NO benefit!
• Urologists thought the meta-analysis was inappropriate since studies were not all using the same methodologies, inclusion criteria. So Dr. Roberts wrote the six authors of those studies to try and obtain comparable data from their data sets.
• All six agreed, providing data set of 1,091 infants. Prophylaxis clearly made no difference, but the higher the grade of reflux, the higher the rate of recurrence, regardless of whether you’re on prophylaxis. Therefore a recurrence of UTI is in and of itself a good indicator of the presence of high-grade reflux.
• Is there harm for waiting until the second UTI to get VCUG? Rates of renal scarring take off around the 3rd to 4th UTI.
• British guidelines already recommended a similar approach, and follow up studies showed no increase in the rates of recurrent UTI in this age group.
• Active treatment of VUR does not reduce the occurrence of chronic kidney disease based on large prospective follow up studies.
• If you look at the original studies that got us to treating VUR in the first place, they were badly flawed by confounding co-morbidities.
• #7 Following a confirmed UTI, parents should be instructed to seek prompt medical evaluation for future febrile illnesses to insure that recurrent infection can be detected and treated promptly.
• Areas for future research: what is the true relationship between UTI’s and reduced renal function/HTN? What alternatives are there to invasive collection of urine? What is the role of VUR and VCUG in disease in the first place. What is the role of prophylaxis in VUR? Are there genetic or racial differences in what we should do? What is the proper duration of treatment for UTI?
• Q&A: Is the imaging recommendation the same for boys and girls? What about posterior urethral valves? Prenatal ultrasound, timely newborn voiding, absence of UTI by age 2 months probably screens out most of the cases of posterior urethral valves. Kids whose valves are diagnosed later probably also have a better prognosis.
• Q&A: is a negative dipstick urine a true negative urinalysis in the absence of microscopy? The white cells damage the kidneys, not the E. coli. Microscopy is better, but LE is a pretty sensitive indicator of the presence of white cells in the urine. May send a child out without treating based on negative dipstick, but if symptoms persist another day you probably need to get that child back that day and re-evaluate. Not sure what to do with a positive culture in the setting of negative UA.
• Q&A: if we have the prenatal ultrasound results, do we still need an ultrasound after the first UTI? Data are being accumulated, probably not able to answer that question just yet. May depend in part on the quality and site of the prenatal ultrasound.
• Q&A: Is there a source of quantitative data on WBC’s and bacteria in pediatric urinalyses? Good studies from Hoberman on bacteruria in children. 
• Q&A: Word of caution from Duke urologist: agree with guidelines, thinks they’re great, but be aware that some kids may move, seek care in multiple locations, and MD’s may not be aware that their “first” febrile UTI is not really their first episode. Be aware of that risk. Also, remember this guideline is for kids aged 2 months to 24 months, does not cover children who may have dysfunctional voiding, etc. Don’t apply these guidelines to children who are outside the covered age group.