American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 1 Parents bring an 18-month-old girl to your office because she has been pointing to her vulvar area and saying “owie.” She has had no fever, vomiting, diarrhea, or discharge. The parents are unaware of any trauma and have no specific concerns of child abuse. The child is at the 75th percentile for height and weight and has no breast buds, axillary hair, or pubic hair. You find no abdominal masses or tendemess. Perineal inspection reveals that about one-third of the vestibule is obscured by a thin membrane between the labia (Item Q1). Results of a urine test strip are normal except for trace leukocyte esterase, and a urine culture is negative for bacteria at 24 hours. Of the following, the MOST appropriate management of this patient is ‘A. manual separation of the fusion in your office B. oral estradiol treatment for 1 month C. referral for surgical separation D. topical antibiotic cream for 2 weeks E. topical estrogen cream for 1 month Copyright 2013 © American Academy of Pediatics 12013 PREP SA on CD-ROM American Academy of Pediatrics Question: 1 Findings as described for the girl in the vignette. (Courtesy of M Rimsza) Copyright 2013 © American Academy of Pediatrics 2American Academy é nape 2013 PREP SA on CD-ROM of Pediatrics aN x Critique: 1 Preferred Response: E Labial adhesions are common, acquired structural abnormalities of the external genitalia, affecting 0.6% to 5% of prepubertal girls. They are rare at birth and are seen most commonly before 6 years of ‘age: peak incidence is in the second year after birth. Adhesions occur when the labia minora fuse over the vestibule. Usually, only a few millimeters of posterior fusion is present and the child has few or no symptoms. However, nearly complete agglutination may lead to dysuria, urinary tract infection, or urinary obstruction. It is estimated that 80% of labial adhesions resolve spontaneously within 1 year of diagnosis, and nearly all resolve by puberty. Therefore, treatment can be reserved for patients who are symptomatic or have extensive adhesions or for patients whose parents are very concerned about physical appearance ‘Several options for treatment exist. However, estimates of efficacy are based only on case series; to date, no prospective studies are available. When treatment is considered, the choices are topical estrogen, topical corticosteroids, or surgical separation. The most frequently recommended treatment is, topical estrogen (estradiol or conjugated estrogen applied once or twice daily for 4-6 weeks). The success rate is reported to be 46% to 100%, with differences in efficacy likely related to the mechanism of application and duration of treatment. It is postulated that topical estrogen promotes cutaneous wound repair; systemic estrogen is unnecessary and is associated with significant adverse effects. While few adverse effects are reported with topical estrogen, rare adverse reactions include breast enlargement or tenderness, labial engorgement, and vulvar hyperpigmentation. Myers et al studied topical betamethasone as an alternative to estrogen with the hopes of eliminating these adverse effects. They found that 1 to 3 courses (4-6 weeks each) of twice daily topical 0.05% betamethasone successfully treated labial adhesions 68% of the time. There were no adverse events related to this treatment, Surgical separation is reserved for the management of adhesions that are unresponsive to conservative treatment. While previous approaches included gentle manual separation in the physicians office, it is now recommended that physical separation occur under general anesthesia to reduce pain and prevent psychological consequences. Unfortunately, adhesions can recur (0%-12%) with both surgical and medical approaches, and recurrences are more common after mechanical separation. However, the issue ultimately resolves with puberty. A topical antibiotic would not be indicated in the management of labial adhesions. As a result of reviewing this information, do you intend to make a change in practice to provide better patient care? Yes No SUGGESTED READING: Caglar MK. Serum estradiol levels in infants with and without labial adhesions: the role of estrogen in the etiology and treatment. Ped Dermatol, 2007;24(4):373-375 Copyright 2013 © American Academy of Pediatics 3American Academy of Pediatrics 2013 PREP SA on CD-ROM Myers JB, Sorensen CM, Wisner BO, Furness PD, Passamaneck M, Koyle MA. Betamethasone cream for the treatment of pre-pubertal labial adhesions. J Pediatr Adolesc Gynecol. 2006;19(6):407- 4n1 ‘Sugar NF, Graham EA. Common gynecologic problems in prepubertal girls. Pediatr Rev. 2008;27(6):213-223. doi: 10.1542/pir.27-6-213 Tebruegge M, Misra |, Nerminathan V. Is the topical application of oestrogen cream an effective intervention in girls suffering from labial adhesions? Arch Dis Child. 2007;92(3):268-271 Copyright 2013 © American Academy of Pediatics 4American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 2 A 14-year-old boy presents to the emergency department and complains of left-sided scrotal pain for the past few hours. He feels nauseated and has vomited once but has no urinary symptoms. On physical examination, his left testicle is high in the scrotum and no cremasteric reflex can be elicited The scrotal skin is normal, but the left testicle is slightly swollen and very tender to palpation. The results of the remainder of his examination, including his abdominal examination, are unremarkable Testicular ultrasonography with Doppler flow reveals reduced flow to the left testicle (Item Q2). Of the following, the MOST likely diagnosis for this boy is A. acute epididymitis, B. incarcerated inguinal hernia C. testicular tumor D. torsion of the appendix testes E. torsion of the testicle Copyright 2013 © American Academy of Pediatics 5American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 2 Crimi Color Doppler ultrasonography findings of the patient's Color Doppler ultrasonography of the patient's lett testis, Areas of necrosis appear as diminished ‘normal testis showing homogeneous echogenicity echogenicity (arrows), absent blood flow centrally (no and normal blood flow centrally (blue and red color). blue or red color), and diminished blood flow peripherally (Courtesy of B Wood) (Courtesy of B Wood) Copyright 2013 © American Academy of Pediatics 62013 PREP SA on CD-ROM American Academy { ES of Pediatrics t a Critique: 2 Preferred Response: E The boy described in the vignette has classic signs and symptoms of testicular torsion. This is a surgical emergency because the risk of “death” of the testicle is very high if detorsion is not performed within 12 hours. If surgical detorsion is performed within 6 hours, fewer than 5% of patients experience testicular atrophy. The reduced blood flow can be recognized by high-resolution ultrasonography with color flow Doppler. Torsion may occur at any age, but it occurs most commonly during the neonatal and adolescent years. In addition to scrotal pain, the patient may have gastrointestinal symptoms, including nausea and vomiting. There may be a vague history of trauma and past episodes of mild pain that suggest partial twists that spontaneously resolved. These patients often have testicles that lie horizontally in the scrotum with a "bell clapper” deformity that predisposes them to twist (Item C2A). The testicle moves superiorly in the scrotum when it twists, resulting in an absent cremasteric reflex; rarely, torsion can be bilateral. Since not all patients have classic symptoms and signs, itis important to perform a genital examination in males who present with gastrointestinal symptoms, especially those with nausea and vomiting because pain associated with these symptoms often points to serious underlying pathology. ‘Several disorders may mimic testicular torsion. An incarcerated inguinal hernia also produces pain, but it presents with @ mass in the groin that cannot be reduced. Tumors of the testicle are usually asymptomatic, although patients may complain of a sensation of heaviness or lower abdominal discomfort, especially if the tumor is large. Thirty percent of these patients may present with unilateral pain and tenderness of the testicle. This pain is more gradual in onset and less intense initially than that seen with testicular torsion. Examination will reveal a firm testicular mass. Epididymitis presents with scrotal swelling and pain, but the onset is less acute and the symptoms are less severe than in testicular torsion. Another cause of scrotal pain is torsion of the vestigial appendix testes or the appendix epididymis. Examination may reveal a painful small mass on the upper pole of the testis that is often seen through the scrotal skin as a blue mass and referred to as the "blue dot sign” (Item C2B). Unlike in testicular torsion, in both epididymitis and torsion of the appendix testis, the testis is oriented vertically and is not elevated in the scrotum, SUGGESTED READING: Adelman WP, Joffe A. Scrotal disorders. In: Neinstein LS, Gordon CM, Katzman DK, Rosen DS, Woods ER, eds. Adolescent Health Care: A Practical Guide. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:401-410 Gatti JM, Murphy JP. Acute testicular disorders. Pediatr Rev. 2008;29(7):235-241 doi:10.1542ipir.29-7-235 Ross A, LeLeiko NS. Acute abdominal pain, Pediatr Rev. 2010;31(4):135-144, doi:10.1542/pir.31-4- 135 Singh H, Thomas EJ, Lindsey Wilson L, et al. Errors of diagnosis in pediatric practice: a multisite ‘Copyright 2013 © American Academy of Pediatrics t2013 PREP SA on CD-ROM survey. Pediatrics. 2010;126(1):70-79. doi:10.1542/peds.2009-3218 Srinivasan A, Cinman N, Feber KM, Gitlin J, Palmer LS. History and physical examination findings predictive of testicular torsion: an attempt to promote clinical diagnosis by house staff. J Pediatr Urol 2011;7(4):470-474. doi:10.1016/j jpurol.2010.12.010 Copyright 2013 © American Academy of Pediatics 8American Academy (GS 2013 PREP SA on CO-ROM caw A. of Pediatrics ee Critique: 2 Bell-clapper Normal deformity Torsion i Tunica vaginalis Normally (left), the tunica vaginalis attaches to the posterolateral aspect of the testis. In the bell-clapper deformity (center), the tunica vaginalis completely encircles the distal spermatic cord, epididymis, and testis, allowing the structures to swing and rotate within the tunica vaginalis (like a clapper in a bell) This abnormality predisposes to testicular torsion (right). (Courtesy of A Johnson) Copyright 2013 © American Academy of PediatricsAmerican Academy of Pediatrics 2013 PREP SA on CD-ROM Critique: 2 "Blue dot" sign of torsed testicular appendage. The testicle (T) is isolated in the lower scrotum and the torsed appendage (white arrow) appears in the upper scrotum. (Reprinted with permission from Gatti JM, Murphy JP. Acute testicular disorders. Pediatr Rev. 2008;29(7):235-241.) ‘Copyright 2013 © American Academy of Pediatrics 10American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 3 A 14-year-old boy presents with fever, joint aches, and a rash for the past 3 days. He was started on amoxicilin for a sore throat a week ago. One of his classmates had a recent diagnosis of strep throat. He has a history of being successfully treated for acute olitis media with amoxicillin a month ago and has been well in the interim. On physical examination, he has swollen lymph nodes, periarticular swelling, and dusky urticaria-like lesions on his skin (Item Q3). Of the following, these findings are MOST likely due to A. amoxicillin allergy B. infectious mononucle C. penicillin allergy D. scarlet fever E. serum sickness-like reaction Copyright 2013 © American Academy of Pediatics "American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 3 Findings as described for the boy in the vignette. (Courtesy of D Krowchuk) Copyright 2013 © American Academy of Pediatrics 122013 PREP SA on CD-ROM. American Academy é of Pediatrics aN x Critique: 3 Preferred Response: E This teenager has the symptoms of a serum sickness-like reaction to amoxicillin. Serum sickness- like reactions typically involve a constellation of signs and symptoms, which can include arthralgias, lymphadenopathy, and urticarial rash with or without fever. Fever, when present, is typically low-grade Children with serum sickness-like reactions may present with acute onset of joint pain that often leads to inability to walk. The most characteristic rash is an urticarial or serpiginous macular rash that starts in the anterior lower trunk, groin, periumbilical, or axillary regions, and spreads to the back. upper trunk, and extremities. The rash generally lasts a few days to 2 weeks. Ulcers, secondary infection, and scarring do not occur. It has been suggested that the term “serum sickness-like disease” should be replaced by “urticaria with arthritis" to describe this drug-induced syndrome, although this has not become common practice In contrast, the cardinal features of classical serum sickness are rash, fever, and polyarthralgia or polyarthritis, which begin 1 to 2 weeks after first exposure to the responsible agent and resolve within a few weeks of discontinuation. Although patients may appear very ill and uncomfortable during the acute febrile stage, the disease is self-limited and prognosis is excellent once the responsible drug is stopped. During classic serum sickness, signs of mild renal dysfunction may be evident; however, renal involvement is unusual in serum sickness-like reactions caused by medications. ‘Serum sickness may develop more rapidly and severely if a previously immunized patient is. reexposed to the culprit antigen. Rather than requiring 7 to 14 days for the development of IgM antibodies, the amnestic IgG response can begin within 12 to 36 hours. Drugs, particularly antibiotics, are the leading cause of serum sickness-like reactions, Penicillin, amoxicillin, cefaclor, and trimethoprim-sulfamethoxazole are most commonly implicated, although many drugs have been associated with these reactions. In children, serum sickness-like reactions are about 15-fold more likely with cefaclor than with other cephalosporins or amoxicillin, even though all are structurally similar B- lactam antibiotics Other drug reactions that may mimic serum sickness or serum sickness-like reactions include nonspecific exanthems, urticaria, and generalized hypersensitivity reactions, The development of an IgE-mediated drug allergy can cause the onset of urticaria during a course of therapy. Other symptoms, of Type I, IgE-mediated allergic reactions are pruritus, flushing, angioedema, wheezing, laryngeal edema, abdominal distress with emesis or diarrhea, and hypotension. Symptoms usually appear within minutes to hours of drug administration and then escalate rapidly. This child does not have symptoms of penicillin or amoxicillin IgE-mediated allergy. In general, penicillin is the most commonly reported medication allergy and is self-reported by at least 10% of patients. However, in large-scale studies, approximately 85% to 90% of these individuals are found to not be allergic and are able to tolerate penicillins. Patients may become allergic to the B- lactam ring structure that is common to all penicillins, or to the R-group side chains that distinguish different penicillins. Penicillin skin testing is the preferred method of evaluation of possible type | IGE- mediated penicillin allergy. The positive predictive value of penicillin skin testing is variable, with approximately 50% (range, 25%-100%) of skin test-positive patients developing immediate allergi reactions upon being challenged with penicillin. However, the negative predictive value of penicillin skin testing is very high. Copyright 2013 © American Academy of Pediatrics 132013 PREP SA on CD-ROM. American Academy { ES of Pediatrics t a Serious immediate-type reactions in patients challenged with penicillin after negative penicillin sI testing have not been reported. In vitro tests (radioallergosorbent tests or enzyme-linked immunosorbent assay) for penicillin, amoxicillin, and ampicillin are commercially available but of limited clinical use because their predictive values have not been defined, Some patients form IgE antibodies that can recognize the R-group side chains rather than the core ring structure. These individuals develop immediate-type reactions to amoxicillin or ampicillin but are able to tolerate penicillin; these patients are said to be selectively allergic to these aminopenicillins. It is rarely possible to distinguish these 2 types of allergy without skin testing, so patients allergic to penicillin should be advised to avoid all penicillins until they can be definitively evaluated. In the United States, however, selective IgE-mediated allergy to the aminopenicillins appears to be uncommon (1%), The child does not have features consistent with scarlet fever. The rash of scarlet fever generally ‘occurs in association with pharyngitis requiring prior exposure to Streptococcus pyogenes. The rash is described as a diffuse erythema that blanches with pressure and has numerous small (1-2 mm), papular elevations that give a "sandpaper" quality to the skin. While scarlet fever with pharyngitis can predispose to acute rheumatic fever, joint aches and pains are typically not observed during the course. Infectious mononucleosis (IM) classically occurs in adolescents and is characterized by fever, ‘severe pharyngitis, and anterior and posterior cervical or diffuse lymphadenopathy. Prominent constitutional symptoms include fatigue, anorexia, and weight loss. Patients who are treated with ampicillin or amoxicillin may develop a characteristic rash described as maculopapular (Item C3). The incidence of rash may be as high as 70% to 90%. The mechanism responsible for the rash is not well understood. Development of a drug-related rash during IM does not appear to indicate a true drug allergy because patients subsequently tolerate ampicillin without an adverse reaction. This child has been symptomatic for only a few days and his rash is urticarial rather than maculopapular, making IM less likely. SUGGESTED READING: Apter AJ, Schelleman H, Walker A, et al. Clinical and genetic risk factors of self-reported penicillin allergy. J Allergy Clin Immunol, 2008;122:152-158 Joint Task Force on Practice Parameters; American Academy of Allergy, Asthma and Immunology; American College of Allergy, Asthma and Immunology; Joint Council of Allergy, Asthma and Immunology. Drug allergy: an updated practice parameter. Ann Allergy Asthma Immunol. 2010;105(4):259-273 Knowles SR, Uetrecht J, Shear NH. Idiosyncratic drug reactions: the reactive metabolite syndromes. Lancet. 2000;356:1587-1591 Segal AR, Doherty KM, Leggott J, Zlotoff B. Cutaneous reactions to drugs in children. Pedi 2007;120:e1082-e1096. http://pediatrics. aappublications.org/content/120/4/e1082. abstract Copyright 2013 © American Academy of Pediatrics 14American Academy of Pediatrics 2013 PREP SA on CD-ROM Critique: 3 ‘Macular and papular eruption occurring in a patient who had infectious mononucleosis and received amoxicillin. (Courtesy of D Krowchuk) ‘Copyright 2013 © American Academy of Pediatrics 15American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 4 You are seeing an 11-year-old girl for the first ime in your practice. She has asthma and has been to the emergency department 3 times in the past month. Your clinics intake procedure includes. administration of the Pediatric Symptom Checklist, a mental and behavioral health screening instrument. The medical student working with you today asks why you are administering a mental health questionnaire for a child who has a chronic illness such as asthma. Of the following, you are MOST likely to explain that you use the questionnaire because A. children who have chronic iliness have an increased risk of anxiety and depressive disorders that are often undiagnosed B. exacerbations of asthma are usually triggered by anxiety C. it is just an office routine; there is no reason to specifically screen for mental or behavioral problems in this patient D. most children who have chronic illness will develop a significant psychiatric difficulty E. up to 5% of primary care office visits occur because of some type of mental or behavioral health concern Copyright 2013 © American Academy of Pediatrics 162013 PREP SA on CD-ROM American Academy { ES of Pediatrics t ey Critique: 4 Preferred Response: A Research shows that approximately 20% of primary care office appointments include mental or behavioral health concems and that frequency has been increasing over time. The routine use of mental health screening questionnaires (eg, at intake appointments or during health supervision visits) can significantly improve the rate of detection for mental or behavioral health issues. In usual care that does not include such screening tools. it is estimated that only 25% of comorbid psychiatric diagnoses are made by care providers. Children who have chronic illnesses are at an increased risk for emotional and behavioral problems that go undetected. A predictive algorithm suggested that 11% of children who have chronic ilinesses. have probable psychiatric diagnoses, compared to a 5% probability of mental illness among healthy peers. The Bergen Child Study, which evaluated more than 7,000 children ages 5 to 17 years, showed that those who had chronic illnesses demonstrated statistically significant increased rates of peer problems, emotional problems, and hyperactivity when compared with healthy, age-matched peers. Different chronic conditions entail different risks of emotional problems; for instance, asthma, congenital heart disease, and juvenile rheumatoid arthritis are all associated with higher rates of depression and anxiety (up to 1 in 3 patients), but cystic fibrosis is not Anxiety disorders have been identified in children who present with multiple-system chronic illnesses, including asthma, respiratory disorders, gastrointestinal diseases, and headaches. In an investigation of more than 1,715 children, the Child Health Questionnaire revealed that 51% of those who had anxiety disorders reported chronic illness, and among all of the children who had chronic illness, 15% had anxiety disorders. Although outcome data for treatment of comorbid conditions are limited, children who have both anxiety disorders and chronic illness are more likely to present with increased severity of somatic symptomatology, depression, and anxiety than children who have anxiety alone. Asthma exacerbations may have emotional triggers, but other triggers are more common. SUGGESTED READING: Chavira DA, Garland AF, Daley S, Hough R. The impact of medical comorbidity on mental health and functional health outcomes among children with anxiety disorders. J Dev Behav Pediatr. 2008;29(5):394-402. doi:10.1097/DBP.0b013e3181836a5b Hysing M, Elgen I, Gilberg C, Lie SA, Lundervold AJ. Chronic physical illness and mental health in children. Results from a large-scale population study. J Child Psychol Psychiatry. 2007;48:785-792. doi:10.11114.1469-7610.2007,01755.x Kelleher KJ, Mclnerny TK, Gardner WP, Childs GE, Wasserman RC. Increasing identification of psychosocial problems: 1979-1996. Pediatrics. 2000;105(6):1313-1321. doi:10.1542/peds.105.6.1313 Copyright 2013 © American Academy of Pediatrics 72013 PREP SA on CD-ROM Tibosch MM, Verhaak CM, Merkus PJEM. Psychological characteristics associated with the onset and course of asthma in children and adolescents: A systematic review of longitudinal effects. Patient Educ Couns, 2011;82(1)11-19. doi:10.1016/j,pec.2010.03.011 Turkel S, Pao M. Late consequences of chronic pediatric illness. Psychiatr Clin North Am. 2007;30(4):819-835. doi:10.1016/j,psc.2007.07.008 Copyright 2013 © American Academy of Pediatrics 18American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 5 ‘A mother brings her 8-year-old boy who has a learning disability to your office for a health supervision visit. She has been taking her son for visual training exercises once a month for the past 6 months, but she is concerned that he is not showing marked improvement in his academic skills. She asks for your opinion regarding the efficacy of visual training exercises. Of the following, the BEST course of action is to A. advise her the exercises must be done at least once weekly for at least 6 months to demonstrate an effect B. discuss the setting in which visual training exercises may be of benefit C. explain that patterning would be more efficacious D. inform her that visual training exercises are of no value E. recommend sensory integration therapy Copyright 2013 © American Academy of Pediatrics 192013 PREP SA on CD-ROM. American Academy { ES of Pediatrics t a Critique: 5 Preferred Response: B The mother of the boy described in the vignette has been taking her son for visual training exercises for the past 6 months. When she asks about the value of these exercises, it is prudent to objectively discuss the evidence for their effectiveness rather than simply say they are of no value. However, there is no scientific evidence that subtle eye or visual problems cause learning disabilities, and there is no evidence that children who receive vision therapy benefit from visual training exercises. One possible ‘exception is the child who has convergence-insufficiency disorder, which is characterized by a failure of the eyes to converge adequately when focusing on a near object. Treating convergence insufficiency with eye-focusing exercises can make reading more comfortable, although it does not improve decoding or comprehension of reading The use of complementary and alternative medicine therapies is quite prevalent among families that have children with developmental and behavioral disorders. There is a lack of data to support the value of many of these approaches, such as chelation therapy, antiviral agents, and auditory integration therapy. Other placebo effects and anticipated improvement may be the reason some of these therapies are endorsed as improving developmental and behavioral disorders. Patterning is a series of exercises that has been advocated to treat children with neurologic impairments. The treatment is based on an outmoded and oversimplified theory of brain development; current information does not support its efficacy. Sensory integration is a form of manipulative or body- based practice that is widely used among children with developmental disorders. Such approaches may include a sensory diet, which is developed with the aim of stimulating tactile, vestibular, and proprioceptive senses by using approaches designed to alert, organize, and calm. Therapeutic body brushing is used for children who overreact to tactile stimulation. There is a need for more research in the area of sensory integration because there is limited empirical support for the therapeutic value of this method SUGGESTED READING: Agency for Healthcare Research and Quality. Therapies for children with autism spectrum disorders: ‘executive summary, Agency for Healthcare Research and Quality Web site. http://www effectivehealthcare. ahrq.gov/ehc/products/106/651/Autism_Disorder_exec-summ.pdf American Academy of Pediatrics, Section on Ophthalmology; Council on Children with Disabilities; ‘American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists. Learning disabilities, dyslexia, and vision. Pediatrics. 2009; 124(2):837-844. doi:10.1542/peds.2009-1445 Chaliman TD, Myers SM. Complementary and alternative medicine in developmental and behavioral pediatrics. In: Voight RG, Macias MM, Myers SM, eds. Developmental and Behavioral Pediatrics. Elk Grove Village, IL: American Academy of Pediatrics; 2011:chap Copyright 2013 © American Academy of Pediatrics 20American Academy of Pediatrics 2013 PREP SA on CD-ROM a Gilmour J, Harrison C, Cohen MH, Vohra S. Pediatric use of complementary and alternative medicine: legal, ethical, and clinical issues in decision-making, Pediatrics. 2011;128(sup 4)S149-S184 doi:10.1542/peds.2010-2720B Weissman L, Bridgemohan C. Autism spectrum disorders in children and adolescents: complementary and alternative therapies. UpToDate Online. http:/www. uptodate.com/contents/autism- spactrum-disorders-in-children-and-adolescents-complementary-and-alternative-therapies, Ziring PR, Brazdiunas D, Cooley WC, et al. American Academy of Pediatrics Committee of Children with Disabilities: the treatment of neurologically impaired children using patterning. Pediatrics, 1999;104(5):1149-1151 Copyright 2013 © American Academy of Pediatics 2American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 6 ‘A 16-year-old boy sustained tibial and fibular fractures of his left leg yesterday during a soccer game. In the emergency department, he underwent closed reduction of the fractures and his leg was splinted with a posterior long leg splint. His mother calls you today because, over the past 4 hours, he has been complaining of worsening pain in his lower leg that has not improved despite several doses of hydrocodone and loosening of the splint. In addition, he says itis difficult to move the toes on his left foot and that his toes are pale and “feel numb.” Of the following, the MOST common complication of this patients condition if itis left untreated is A. avascular necrosis of the tibial plateau B. equinovarus foot deformity C. fracture malunion D. leg length discrepancy E_ osteomyelitis, Copyright 2013 © American Academy of Pediatrics 22American Academy é of Pediatrics 2013 PREP SA on CD-ROM. aN x Critique: 6 Preferred Response: B The patient described in the vignette is demonstrating signs and complaining of symptoms that raise the clinical suspicion for compartment syndrome. Compartment syndrome is a potentially limb- \creased tissue pressures (typically related to muscle and soft-tissue edema) within closed osteofascial compartments cause decreased muscle perfusion and ischemia. Most commonly seen after extremity fractures, compartment syndrome may also complicate burns, snakebites, and crush injuries. The lower leg is affected most commonly, with tibial fractures, accounting for 20% to 40% of fracture-related compartment syndromes. Rapid evaluation should be performed in patients with fractures like the boy in the vignette who complain of worsening pain, pain out of proportion to the injury, distal numbness, or extremity pallor. It is important to remember that with the exception of pain, most of the classically described clinical features of compartment syndrome (the 5 Ps: pain, pallor, paresthesias, paralysis, and pulselessness) are late findings. It is also unusual for the muscle group to feel ‘tight’ to the examiner despite rising pressures. A high index of clinical suspicion should prompt the clinician to evaluate high-risk patients before neurovascular compromise is evident. If compartment syndrome is suspected, immediate orthopedic consultation is indicated, and compartment pressures should be measured. Normal muscle compartment pressure is typically less than 10 to 12 mm Hg. Subtracting the measured intracompartmental pressure from the diastolic blood pressure provides a measure of the compartmental perfusion pressure. A compartmental perfusion pressure of 30 mm Hg of less is indicative of ischemic compromise and should prompt consideration of surgical intervention to relieve the compartment pressure. Prompt release of intracompartmental pressure (within 6 to 12 hours of onset) is critical for a favorable outcome. Initial treatment of compartment syndrome involves removal of constricting dressings, splints, or casts and maintenance of the extremity at “heart level" to maximize perfusion and minimize further dependent edema. Fasciotomy of the affected compartments may be necessary to release the intracompartmental pressure and restore muscle perfusion. The most common complication of compartment syndrome is ischemic injury of the affected muscles with resultant contracture formation. When the lower leg is involved, contractures most commonly lead to equinovarus foot deformity. Other, less common complications include infection, delayed fracture healing, and malunion with or without the development of leg length discrepancy. Avascular necrosis, a condition that occurs when there is ischemia in an area of bone with tenuous vascular supply, would not be a likely complication of lower extremity compartment syndrome because it does not typically ‘occur in the tibia or fibula. SUGGESTED READING: Késtler W, Strohm PC, Siidkamp NP. Acute compartment syndrome of the limb. Injury. 2004;35(12):1221-1227. doi:10.1016/j injury.2004.04.009 Reverte MM, Dimitriou R, Kananaris NK, Giannoudis PV. What is the effect of compartment syndrome Copyright 2013 © American Academy of Pediatrics 23American Academy of Pediatrics 2013 PREP SA on CD-ROM and fasciotomies on fracture healing in tibial fractures? Injury. 201 1;42(12):1402-1407 doi:10.1016) injury.2011.09.007 Thati S, Carlson C, Maskill JD, Anderson JG, Bohay DR. Tibial compartment syndrome and the cavovarus foot. Foot Ankle Clin. 2008;13(2):275-305. doi:10.1016/.fel.2008.02.001 Wheeless CR Ill. Compartment syndrome resulting from tibial frx. In: Wheeless CR Ill, ed. Wheeless Textbook of Orthopaedics. Durham, NC: Duke University; 2011 http://www wheelessoniine.com/ortho/compartment_syndrome_resulting_from_tibial_frx Copyright 2013 © American Academy of Pediatrics 24American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 7 ‘A resident who is rotating with you in the newborn nursery asks for advice on the management of a 4.1-kg, 1-day-old male newborn whose heel stick specimen revealed an initial glucose value of 45 mg/dl. (2.5 mmol/L). Results from repeat testing after the infant was fed revealed a blood glucose concentration of 82 mg/dL. (4.6 mmol/L). A third glucose test performed 24 hours after birth revealed a blood glucose concentration of 95 mg/dL (5.3 mmol/L). The resident reports that on physical examination the infant is sleepy but easily aroused and in no acute distress. The remainder of the results of his physical examination are normal except that his penis appears to be less than 2 cm in length Of the following, the MOST appropriate next step in the management of this infant is to A. begin an intravenous glucose infusion B. measure gonadotropin and growth hormone concentrations, C. obtain a complete blood cell count and blood and urine culture D. obtain a stretched penile length measurement E. obtain head magnetic resonance imaging Copyright 2013 © American Academy of Pediatrics 252013 PREP SA on CD-ROM. American Academy { ES of Pediatrics t ey Critique: 7 Preferred Response: D The infant described in the vignette has hypoglycemia and a penis that appears to be less than 2 em in length. These findings should suggest the possibility of panhypopituitarism (eg, growth hormone, gonadotropin, and corticotropin deficiency). Infants who have panhypopituitarism may present with both hypoglycemia due to lack of insulin counterregulatory hormones (growth hormone and cortisol) and micropenis due to inadequate exposure in utero to growth hormone and gonadotropins (luteinizing hormone and follicle-stimulating hormone). With the immediate concer for hypoglycemia alleviated, the most appropriate next step in this boys evaluation is to determine whether he has micropenis. Many children who are slightly large for gestational age will have a generous suprapubic fat pad anterior to the pubic symphysis. This fat pad can give the false impression that the childs penis is inappropriately small. Thus, inspection is not an adequate technique for assessing penile size. Instead, a stretched penile length (SPL) measurement should be obtained. SPL is performed by placing a tongue blade at the dorsal base of the penis and gently pushing down to the pubic symphysis while extending the penis along the length of the blade. The length of the penis can be accurately assessed by drawing a horizontal line on the tongue blade just above the glans of the stretched penis and subsequently measuring the distance from the end of the blade to the marked line. Children who have an SPL less than 1.8 cm (2.5 standard deviations less than normal) are considered to have micropenis and would require additional workup to determine the cause Although head magnetic resonance imaging and gonadotropin and growth hormone measurements would be indicated if the infant was confirmed to have micropenis and persistent hypoglycemia, these tests are not indicated for the infant described in the vignette. Because the infants hypoglycemia responded well to feedings, there is no need to begin an intravenous glucose infusion. A glucose infusion would be indicated if the infant was unable to take adequate carbohydrates by mouth to normalize blood glucose concentrations, A complete blood cell count and blood and urine culture is not indicated because there are no signs of infection. If repeat testing had demonstrated glucose concentrations of less than 45 mg/dL (2.5 mmol/L), additional workup for metabolic disturbances would have been indicated and a complete sepsis evaluation might have been considered. SUGGESTED READING: Grumbach MM. A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant. J Clin Endocrinol Metab. 2005;90(5):3122-3127. doi:10.1210/je.2004-2465 Rozancea PJ, Hay WW Jr. Describing hypoglycemia - definition or operational threshold? Early Hum Dev. 2010;86(5):275-260. doi:10.1016/j,earlhumdev.2010.05.002 Urbach S, OGorman C, Alabdulrazzaq D. Case 2: hypoglycer hormonal red flags. Paediatr Child Health. 2009;14(7):453-456 and micropenis in the newborn - Copyright 2013 © American Academy of Pediatrics 26American Academy nape 2013 PREP SA on CD-ROM of Pediatrics aN z Question: 8 A 14-year-old girl presents to the emergency department for evaluation of severe headache, fever, and lethargy. The parents note that she first became ill with an acute upper respiratory illness with congestion and low-grade fever. The parents also report that over the past few days the girl has had an elevated temperature of 39.1°C, complaints of increasing frontal headaches (greater on the lef side), and increased sleepiness. On physical examination, the girl is uncomfortable and awake but sleepy. On ‘examination of head, eyes, ears, nose, and throat, there is tenderness over the frontal sinuses. Her neck is supple, Neurologic examination reveals a left sixth cranial nerve palsy. The remainder of her ‘examination is unremarkable. Computed tomography scan with contrast reveals pansinusitis with meningeal enhancement over the left frontoparietal lobe and an epidural collection of approximately 2 cm. Of the following, the BEST choice for empirical antibiotic therapy, pending neurosurgical evaluation, ampicillin and gentamicin cefazolin and gentamicin ceftriaxone and vancomycin metronidazole and vancomycin moo pM > nafcillin and vancomycin Copyright 2013 © American Academy of Pediatics ar2013 PREP SA on CD-ROM. American Academy é of Pediatrics aN x Critique: 8 Preferred Response: C The child described in the vignette has a brain abscess documented on computed tomography scan. Brain abscesses can arise as a complication of a number of infections through direct extension (eq, sinusitis, mastoiditis, and odontogenic infections) or hematogenous spread (eg, endocarditis), especially in children with cyanotic congenital heart disease. Trauma and surgical procedures are other predisposing factors for development of brain abscesses. These infections are frequently polymicrobial. Treatment generally involves a combination of antimicrobial therapy and surgical drainage. Initial tic therapy depends on the focus of origin and the likely associated infectious agents. In this case, the epidural abscess arose as a direct extension from frontal sinusitis, so the likely organisms reflect the pathogens of sinusitis: Streptococcus pneumoniae, Haemophilus influenzae, other streptococcal species, anaerobes, and, less likely, Staphylococcus aureus. Of the listed regimens, ceftriaxone and vancomycin provides coverage for these organisms, and these drugs adequately cross the blood-brain barrier. ‘Ampicillin and gentamicin would not provide adequate coverage for B-lactamase positive H influenzae, S aureus, or penicillin-resistant S pneumoniae. Additionally, gentamicin does not adequately cross the blood-brain barrier. Similarly, first-generation cephalosporins such as cefazolin do not adequately cover H influenzae and do not cross the blood-brain barrier. The combination of metronidazole and vancomycin or nafeillin and vancomycin does not provide adequate coverage, especially for H influenzae. The duration of antibiotic therapy for brain abscesses is typically 6 to 8 weeks according to clinical response. In addition to antibiotic therapy, treatment for brain abscesses includes consultation with the appropriate specialists and surgical drainage if indicated SUGGESTED READING: Al Masalma M, Armougom F, Scheld WM, et al. The expansion of the microbial spectrum of brain abscesses with use of multiple 16S ribosomal DNA sequencing. Clin Infect Dis, 2009;48(9):1 169-1178 doi:10.1086/597578 Southwick FS. Treatment and prognosis of brain abscess. UpToDate Online 20.7. 2012 http://www, uptodate.com/contents/treatment-and-prognosis-of-brain- abscess ?source=search_result&search=treatment+and+prognosis+of+brain+abscess&selectedTitle=1 %67E134 ‘Copyright 2013 © American Academy of Pediatrics 28American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 9 A 14-year-old girl who has a history of juvenile idiopathic arthritis is brought to the emergency department with severe epigastric abdominal pain. She has been taking ibuprofen to control her joint pains. Physical examination demonstrates a well-developed, well-nourished girl in moderate distress from pain. The remainder of the general physical examination shows direct tenderness in the epigastrium without rebound. The rectal examination shows normal sphincter tone and a scant amount of dark brown, hemoccult-positive stool in the rectal vault, Initial laboratory data include the following: + White blood cell count, 10,500/pL (10.5 * 109/L) + Hemoglobin, 11.7 g/d. (117 g/L) + Erythrocyte sedimentation rate, 32 mmvh + Serum electrolytes, normal + Blood urea nitrogen, normal + Creatinine, normal Of the following, the MOST likely mechanism responsible for her symptoms is A. decreased pepsinogen production B. duodenal stress ulceration C. gastritis induced by peptidoglycans D. hypersecretion of gastric acid E.in ition of prostaglandin synthesis Copyright 2013 © American Academy of Pediatrics 29American Academy é nape 2013 PREP SA on CD-ROM of Pediatrics aN x Critique: 9 Preferred Response: E The girl described in the vignette presents with abdominal pain, hemoccult-positive stools, and anemia. These findings indicate chronic gastrointestinal blood loss, and the presence of epigastric tendemess strongly suggests a lesion in the upper gastrointestinal tract (ie, proximal to the ligament of Treitz). Although the differential diagnosis of chronic upper gastrointestinal bleeding is broad and includes erosive esophagitis, Helicobacter pylori infection, and Crohn Disease, the most likely diagnosis is gastritis or peptic ulcer disease caused by her use of ibuprofen. Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, inhibit prostaglandin synthesis. Prostaglandin E stimulates gastric mucus production and helps maintain the protective mucin glycoprotein-bicarbonate layer, without which gastric mucosa is exposed to the deleterious effects of gastric acid as well as to the proteolytic effects of chief cell-produced pepsin, which can result in gastritis or peptic ulcers. NSAIDs are highly effective anti-inflammatory agents that act via inhibition of the enzymes, cyclooxygenase-1 (COX-1) or cyclooxygenase-2 (COX-2) or both. The NSAIDS that inhibit both COX-1 and COX-2 are termed nonselective cyclooxygenase inhibitors and include aspirin, ibuprofen, and naproxen. These agents are associated with a significantly higher risk for both gastrointestinal and renal toxicity when compared with selective COX-2 inhibitors (eg, celecoxib). However, the COX-2 inhibitors have been associated with an increased incidence of deleterious cardiovascular effects in adults, To counteract the adverse gastrointestinal effects of long-term use of NSAIDs, patients are often prescribed so-called cytoprotective agents such as proton-pump inhibitors to prevent the occurrence of gastritis and ulcers. In some cases, the synthetic prostaglandin misoprostol may also be used for this, purpose. However, because misoprostol may also induce uterine contractions, itis rarely used in adolescent female patients such as the girl in the vignette. The proteolytic enzyme pepsin is produced by the gastric chief cells and represents a potentially noxious agent for the unprotected epithelium. Reduced production of pepsins proenzyme, pepsinogen, would therefore not be expected to induce gastric ulceration. Stress ulcerations occur in severely ill and in postoperative patients, particularly in the intensive care setting. These lesions are single or multiple, typically noninflammatory in nature, and occur with equal frequency in the stomach and duodenum. Stress ulcers may be due to epithelial cell ischemia, While reduction in the gastric mucus layer from NSAID-induced prostaglandin inhibition may potentiate gastric damage in the ICU setting, the girl described in the vignette does not represent a typical scenario for stress ulceration Peptidoglycans are polymers consisting of sugars and amino acids that form a mesh-like layer outside the plasma membrane of bacteria. The peptidoglycans of Helicobacter pylori have additional virulence factors, but their precise role in H pylori-associated gastritis remains unclear. While this entity should certainly be considered in any patient with suspected gastritis, the prevalence of H pylori infection in the United States is relatively low in the first 2 decades of lif. Gastric acid hypersecretion results from a basal increase in gastric acid output and occurs in disorders marked by hypergastrinemia (eg, Zollinger-Ellison Syndrome) and hyperhistaminemia. In the Pediatric population, rebound gastric acid hypersecretion may occur after the use of proton-pump Copyright 2013 © American Academy of Pediatrics 20American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Ses inhibitors; acid hypersecretion has also been described during cysteamine treatment in children with cystinosis. In the present case, however, the effects of NSAIDs are far more likely to be responsible for the clinical findings SUGGESTED READING: Collier DN. Non-steroidal antiinflammatory drugs and abdominal pain. Pediatr Rev. 2007:28(2):75- 77. doi:10.1542/pir.28-2-75 Hawkey CJ, Karrasch JA, Szezepanski L, et al. Omeprazole compared with misoprostol for ulcers associated with nonsteroidal antiinflamatory drugs. N Engl J Med. 1998;338(11):727-734 Lanza FL. A guideline for the treatment and prevention of NSAID-induced ulcers. Am J Gastroenterol. 1998:93(11):2037-2046 ‘Stupnicki T, Dietrich K, Gonzélez-Carro P, et al. Efficacy and tolerability of pantoprazole compared with misoprostol for the prevention of NSAID-related gastrointestinal lesions and symptoms in theumatic patients. Digestion. 2003:68(4):198-208, Weaver AL, Gitlin N. Ulcer prevention in long-term users of nonsteroidal anti-inflammatory drugs. Arch Intern Med. 2002:162(19):2248-2249 Copyright 2013 © American Academy of Pediatics aAmerican Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 10 ‘An 18-month-old boy is brought to your clinic because of redness of his left cheek that his mother noticed yesterday. He has been otherwise well and has had no fever. Physical examination findings reveal a tender, erythematous area of slight induration on his left cheek (Item Q10). His mother tells you that the boy recently started sucking ice chips. Of the following, the MOST likely diagnosis is A. cellulitis B. cold panniculitis C. dental abscess D. juvenile xanthogranuloma E. lipoma Copyright 2013 © American Academy of Pediatrics 22American Academy of Pediatrics 2013 PREP SA on CD-ROM & Question: 10 Erythematous plaque as described for the boy in the vignette, (Courtesy of M Rimsza) Copyright 2013 © American Academy of Pediatrics 332013 PREP SA on CD-ROM. American Academy { ES of Pediatrics t ey Critique: 10 Preferred Response: B Cold panniculitis, also known as “popsicle panniculit in caused by prolonged exposure to cold objects. Sucking on popsicles or ice chips results in the most common presentation on the cheeks, but any area of the body exposed to cold can be affected. The mechanism of pathogenesis is similar to that of fat necrosis and likely occurs because in infants the subcutaneous fat is more apt to. solidify than in adults Typical lesions are bluish red nodules that may be painful. They usually arise within 1 to 2 days of cold exposure, and they may persist for several weeks. Although rarely required for diagnosis, histologic examination reveals histiocytic and lymphoid cells within fat lobules. Because the lesions resolve within weeks, no treatment is necessary. However. it is advisable to avoid cold exposure if possible. Cellulitis is suppurative inflammation of the skin caused by bacteria, such as Streptococcus pyogenes and Staphylococcus aureus. A child who has cellulitis is often febrile, and his skin is very tender, erythematous, and warm to the touch. A dental abscess could cause painful swelling of the cheek, but fever and tendemess over the gingiva and affected tooth would be expected. Juvenile xanthogranuloma is characterized by yellowish or brownish nodules on the skin (Item C10), and the lesions are typically present from birth or early infancy. A lipoma, a benign tumor of fat cells, may be found anywhere on the body but typically does not cause tenderness or skin discoloration SUGGESTED READING: Morelli JG. Panniculitis and erythema nodosum. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:2282-2284 Torrelo A, Hernandez A. Panniculitis in children, Dermatol Clin. 2008;26:491-500 Copyright 2013 © American Academy of Pediatrics 34American Academy of Pediatrics 2013 PREP SA on CD-ROM Critique: 10 ‘A juvenile xanthogranuloma is an orange or yellow-brown nodule. (Courtesy of D Krowchuk) Copyright 2013 © American Academy of Pediatrics 36American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 11 A full-term male newborn is admitted to the regular nursery after an uneventful delivery. Apgar scores are 8 at 1 minute and 9 at 5 minutes. He feeds well initially, but 32 hours after birth you are called by the nurse because he is feeding poorly and seems “lethargic.” You arrange for transfer of the newborn to the intensive care nursery, where the resident on duty obtains a blood culture and provides appropriate antibiotic coverage. Of the following, if sepsis is ruled out, the test MOST likely to be diagnostic in this newborn is ‘A. a complete blood cell count with differential B. an electroencephalogram C. head ultrasonography D. liver function tests E. serum ammonia and urine organic acids Copyright 2013 © American Academy of Pediatrics 26American Academy é ican, 2013 PREP SA on CD-ROM of Pediatrics aN x Critique: 11 Preferred Response: E The child in this vignette is a healthy, full-term newborn who has developed mental status changes within 48 hours after birth. Although sepsis is the most likely diagnosis in this scenario, the newborns presentation is also typical of an inborn error of metabolism, such as a urea cycle defect or organic acidemia. Therefore, serum ammonia and urine organic acid testing should be considered and done in parallel with blood cultures and antibiotic administration. Early identification of an inborn error with immediate treatment may minimize potential neurologic sequelae from complications such as severe metabolic acidosis or coma secondary to hyperammonemia. Mild hyperammonemia with metabolic acidosis is frequently seen with an organic acidemia, whereas severe hyperammonemia and respiratory alkalosis are often found with a urea cycle defect. Initial treatment for these metabolic disorders, revolves around optimal hydration, protein restriction, and avoidance of a catabolic state. Once the diagnosis is established, use of special metabolic formulas, and sometimes cofactors specific to a particular metabolic pathway, are indicated. Although expanded newborn screening may result in earlier detection of one of these life-threatening inborn errors of metabolism, the results of these screening tests may not come back before the appearance of clinical symptoms, as in the case of the newborn in the vignette. Immediate recognition and intervention are keys to minimizing harm to the newborn. ‘A complete blood cell count with differential may show some mild, nonspecific changes in the case of a metabolic derangement, but it may also be completely normal. An electroencephalogram is certainly a consideration in the case of a newborn with mental status changes, but the absence of fluctuating mental status or other features of seizures, such as abnormal movements or lip smacking, makes seizures less likely. Head ultrasonography is also a reasonable consideration to rule out an intracranial bleed or hydrocephalus, but there are no other indications that these clinical findings are related to an intracranial process. Finally, liver function tests would be helpful to rule out liver failure associated with secondary hyperammonemia, but the newborn has no other findings to suggest a hepatic process such as jaundice, ascites, or abdominal distension SUGGESTED READING: Burton KB. Inborn errors of metabolism and infancy: a guide to diagnosis, Pediatrics. 1998;102(6):e69. doi:10.1542/peds.102.6.069 Enns GM, Packman S. Diagnosing inborn errors of metabolism in the newborn: clinical features. NeoReviews. 2001;2(8):e183-e191. doi:10.1542/neo.2-8-e183 Talele SS, Xu K, Pariser AR, et al. Therapies for inborn errors of metabolism: what has the orphan drug act delivered. Pediatrics. 2010;126(1):101-106. doi:10.1542/peds.2009-3246 Copyright 2013 © American Academy of Pediatrics aAmerican Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 12 ‘A 2-year-old boy you have followed up since birth presents to your office for a health supervision visit. He was bom at term and has had normal growth and development. The mother recently noticed that he looks “cross-eyed” at times. There is no family history of eye abnormalities. On physical examination, his left eye is deviated nasally with a white pupillary reflex (Item (12). There is no proptosis, conjunctival injection, or pain with eye movements. The remainder of results for the physical examination are normal. Results of the complete blood cell count are within normal limits. Of the following, the MOST likely diagnosis for this patient is, bilateral retinoblastoma congenital cataract leukemia with chloroma neuroblastoma moo p > unilateral retinoblastoma ‘Copyright 2013 © American Academy of Pediatrics 38American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 12 White pupillary reflex as described for the child in the vignette (Courtesy of RG Weaver Jr) Copyright 2013 © American Academy of Pediatrics 39American Academy of Pediatrics 2013 PREP SA on CD-ROM. Ses Critique: 12 Preferred Response: E One of the most common causes of leukocoria (white pupillary reflex) in children is retinoblastoma (47% of cases); therefore, all children with a new finding of leukocoria should be referred immediately to an ophthalmologist who is experienced in examining children. Other causes include persistent fetal vasculature, retinopathy of prematurity, cataracts, optic disc abnormalities, uveitis, or vitreous hemorrhage. Retinoblastoma is the most common intraocular tumor of childhood, occurring in approximately 1 in 15,000 live births. The annual incidences is 11 per 1 million children from birth to 4 years old and 0.6 per 1 million children 5 years old and older. The average age at the time of diagnosis is 2 years in unilateral cases and 1 year in bilateral cases. Leukocoria and strabismus are the most common presenting findings. Other findings may include decreased vision, ocular inflammation, vitreous hemorrhage, hyphema, orbital cellulitis, proptosis, glaucoma, eye pain, fever, and a family history of retinoblastoma The diagnosis of retinoblastoma is usually made on the basis of clinical examination, and the presence of calcification on computed tomography or ocular ultrasonography In this vignette, the age of the child, the lack of findings in right eye, and the relatively higher frequency of unilateral disease over bilateral disease make unilateral retinoblastoma the most likely diagnosis in this patient. Leukemia with chloroma, or intraocular leukemic infiltration, is a less likely diagnosis given the normal blood cell count. Neuroblastoma can present in this age group but is not associated with leukocoria. Ocular findings typically seen in neuroblastoma include Horner syndrome (miosis, ptosis, enophthalmos, and anhydrosis), periorbital hemorrhage, proptosis, papilledema, retinal hemorrhage, strabismus, and opsoclonus. Congenital cataracts are also a common cause of leukocoria but are usually apparent at birth or early infancy (Item C12). Cataracts can be associated with congenital infections or various systemic disorders (eg, diabetes mellitus, galactosemia, Turner syndrome, Down syndrome, peroxisomal disorders). SUGGESTED READING: Hurwitz RL, Shields CL, Shield JA, et al. Retinoblastoma. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:809- 837 Kaufman PL, Saunders RA. Approach to the child with leukocoria, UpToDate Online. 2012. http://www, uptodate.com/contents/approach-to-the-child-with- leukocoria?source=search_result&search=%22approach+to+the+child+with +leukocoria%22aselectedTi tle=1%7E150 ‘Tamboli A, Podgor MJ, Horm JW. The incidence of retinoblastoma in the United States: 1974 through 1985. Arch Ophthalmol, 1990;108(1):128 Copyright 2013 © American Academy of Pediatics 40American Academy eeaae 2013 PREP SA on CD-ROM of Pediatrics Critique: 12 NS rn Bt ‘A congenital cataract is a cause of a white pupillary reflex. (Courtesy of M Rimsza) Copyright 2013 © American Academy of Pediatrics 4American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 13 You are evaluating a 4-month-old boy for complaints of fussiness and decreased feeding for the past 24 hours. You have not previously seen the patient, but the mother states that he has had no previous illnesses, did not receive his immunizations, and is not taking any medications. On physical examination, the boy has a temperature of 37.6°C, a heart rate of 160 beats/min, and a respiratory rate of 55 breaths/min, Physical examination findings are remarkable only for a lethargic infant who has a full anterior fontanelle and widened cranial sutures. Of the following, the MOST appropriate initial test is ‘computed tomography of the head electroencephalogram lumbar puncture plain radiograph of the skull moo p > urine toxicology screen ‘Copyright 2013 © American Academy of Pediatrics 422013 PREP SA on CD-ROM American Academy { ES of Pediatrics t ey Critique: 13 Preferred Response: A Increased intracranial pressure is a medical emergency that must be recognized promptly to prevent potentially permanent neurologic injury or death. Initial symptoms of increased intracranial pressure in infants can include irritability, lethargy, and vomiting (especially in the morning). Physical examination findings include full or bulging fontanelle, widened sutures, increasing head circumference, sluggish or unequal pupils, impaired or absent upward gaze of the eyes ("sunsetting”), and papilledema. Changes in motor tone with eventual asymmetric movements and progression to decerebrate or decorticate posturing can be seen as well. Increased heart rate and blood pressure, which may improve cerebral blood flow, can occur. The respiratory rate also may increase, although the exact cause of this central hyperventilation and its effect on intracranial pressure is not clear. Cushing triad (hypertension, bradycardia, and abnormal respiration) is a late finding that usually indicates imminent brainstem herniation. The infant described in the vignette exhibits several signs consistent with increased intracranial pressure, including lethargy, a full anterior fontanelle, widened sutures, and elevations of the heart and respiratory rates. Computed tomography scan or magnetic resonance imaging (MRI) of the head is the first priority in evaluating suspected increased intracranial pressure after initial stabilization. There is no indication for a plain radiograph of the skull in this patient. Computed tomography - although it delivers ionizing radiation and provides less anatomical detail when compared to MRI - is often used in the acute setting because of its greater availability, speed in determining potential need for surgical intervention, and value in narrowing the diagnosis and initiating treatment. It is important to remember that the presence of increased cranial pressure cannot be accurately determined on a computed tomography study unless there is evidence of effacement of the basilar cisterns or lateral ventricles, Lumbar puncture is indicated when infectious causes are of concern. However, for the infant in the vignette, the absence of fever and other symptoms and signs consistent with infection do not support the need for lumbar puncture. In addition, lumbar puncture should be deferred in the presence of significant neurologic findings that might be consistent with impending brainstem herniation. Electroencephalography or urine toxicology might be indicated in a child who presents with a change in mental status, but the presentation of the infant in the vignette makes these tests less likely to be informative. SUGGESTED READING: Avner JR. Altered states of consciousness. Pediatr Rev. 2006;27(9):331-338. doi:10.1542/pir.27-9- 331 Bell MJ, Kochanek PM, Neurologic emergencies and stabilization. In; Kliegman RM, Stanton BF, St Geme JW Ill, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 201 1:296-304. Oliver WJ, Shope TC, Kuhns LR. Fatal lumbar puncture, fact versus fiction: an approach to a clinical Copyright 2013 © American Academy of Pediatics 43American Academy of Pediatrics 2013 PREP SA on CD-ROM ® dilemma. Pediatrics. 2003;112:6174-€176. http://pediatrics.zappublications.org/content/1 12/3/6174 abstract Copyright 2013 © American Academy of PediaticsAmerican Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 14 Just before halftime at a high school football game, a 17-year-old boy who plays wide receiver is. undercut by a tackler while leaping to catch a pass. He drops the ball and, after getting to his feet, is confused and walks to the opposing teams side of the field. He remains confused while walking into the locker room for halftime but then seems normal by the start of the second half. However, he continues to have some amnesia for the details of the play in which he was tackled. Of the following, the recommendation you are MOST likely to make as team physician is A. daily neurologic examinations for 2 weeks B. emergency department evaluation and clearance before returning to play C. return to play at the start of the second half D. return to play in 30 minutes if symptoms resolve E. return to play in 7 to 10 days if symptoms resolve Copyright 2013 © American Academy of Pediatics 45American Academy of Pediatrics 2013 PREP SA on CD-ROM. Ses Critique: 14 Preferred Response: E The boy in the vignette has experienced a concussion characterized by transient cognitive symptoms of amnesia and disorientation after a fall. In this setting, it is best to disallow return to play during this game. He should be evaluated in the office but does not need daily neurologic examinations for 2 weeks. He may return to play in 7 to 10 days if symptoms completely resolve. The symptoms described are mild enough (no loss of consciousness) that urgent referral to the emergency department is not warranted. However, he should be sent to the emergency department for evaluation if he experiences repeated vorniting, severe or progressively worsening headache, a seizure, unsteady gait, weakness or numbness, slurred speech, unusual behavior, signs of a basilar skull fracture, or worsening mental status Guidelines regarding concussion and sports continue to evolve as they are informed by research on traumatic encephalopathy in contact sports. The most important feature of current guidelines for return to play is that assessment needs to be careful and that return to play should not occur until all cognitive symptoms have resolved. An experienced physician should examine the student and ascertain that all symptoms have resolved at rest and with exercise before the student returns to play. Provocative ‘exercise may include a 40-yard sprint, 5 pushups, 5 situps, and 5 knee bends; the examiner evaluates for emergence of headache, dizziness, nausea, visual symptoms, and mental or emotional changes. Neuroimaging is not indicated according to this presentation. However, if headache or cognitive symptoms worsen over the next week or if seizures or focal neurologic deficits are identified, imaging should be considered. Findings on imaging of brain swelling or contusion would be an indication to terminate participation for the rest of the season. SUGGESTED READING: Grubenhoff JA, Kirkwood M, Gao D, Deakyne S, Wathen J. Evaluation of the standardized assessment of concussion in a pediatric emergency department. Pediatrics, 2010;126(4):688-695. doi:10.1542/peds.2009-2804 Halstead ME, Walter KD; American Academy of Pediatrics Council on Sports Medicine and Fitness. Clinical report: sport-related concussion in children and adolescents. Pediatrics, 2010;126(3):597-615. doi:10.1842/peds.2010-2005 Copyright 2013 © American Academy of Pediatics 46American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 15 ‘A 6-year-old girl who has corrected congenital heart disease and a history of furunculosis presents to the emergency department with fever and increased work of breathing. Physical examination reveals a temperature of 39.5°C, heart rate of 130 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 85/55 mm Hg, Her oxygen saturation is 90% in room air. A new blowing murmur is noted on cardiac examination, and auscultation of the chest reveals diffuse crackles bilaterally. She has hepatosplenomegaly and a capillary refill of 3 to 4 seconds, Her skin examination yields normal results. Her white blood cell count is 30.0 * 103/uL (30.0 x 109/L), with 71% polymorphonuclear leukocytes, 23% lymphocytes, and 6% monocytes. Chest radiograph shows diffuse pulmonary interstitial edema Of the following, the MOST appropriate initial antibiotic therapy for this patient is A. ampicillin-sulbactam B. ceftazidime C. doxycycline D. trimethoprim-sulfamethoxazole E. vancomycin Copyright 2013 © American Academy of Pediatics 47American Academy of Pediatrics 2013 PREP SA on CD-ROM. Ses Critique: 15 Preferred Response: E The patient described in the vignette presents with clinical sepsis, and her history of corrected congenital heart disease and furunculosis in conjunction with fever and a new murmur on physical examination strongly suggests acute infectious endocarditis (IE). In children with a fulminant presentation of IE, Staphylococcus aureus is the most common cause. Therefore, prompt initiation of antimicrobial therapy with a bactericidal agent such as vancomycin is warranted. Vancomycin is a glycopeptide antibiotic that inhibits bacterial cell wall synthesis by binding tightly to peptidoglycan precursors (D-alanyl-D-alanine portion) and blocking polymerization. This disturbance of cell wall synthesis leads to lysis of the bacteria. Therefore, this bactericidal agent is most appropriately used for suspected or confirmed serious gram-positive infection in patients seriously ill or allergic to B- lactam antibiotics. Vancomycin should be continued only in patients in whom there is documentation or strong suspicion of a gram-positive organism resistant to oxacillin and cephalosporins and susceptible to vancomycin. One such example is methicillin-resistant S aureus (MRSA). Vancomycin can also be used for prophylaxis when prosthetic device implantation requires major surgery or in patients who are seriously allergic to B-lactams; it can also be used enterally for the treatment of Clostridium difficile colitis. Despite the existence of guidelines for appropriate vancomycin use, misuse of the drug is widespread and is associated with the emergence of vancomycin-resistant enterococci and, although rare, vancomycin-intermediate and vancomycin-resistant S aureus. Vancomycin can stimulate histamine release, resulting in the “red man syndrome” (also known as “red neck syndrome’), in which pruritus and erythema of the head and neck is common. This is a rate-dependent infusion reaction and not a true drug allergy. Slowing the drug infusion rate and administering diphenhydramine can abort or prevent this common reaction. Ampicillin-sulbactam is a B-lactamase-resistant, semisynthetic penicillin that has activity against anaerobes, susceptible aerobic gram-positive organisms, and respiratory tract gram-negative pathogens. It does not have activity against MRSA, so it would not be appropriate empirical therapy for the patient described in the vignette. The expanded-spectrum cephalosporins (third and fourth generations), such as ceftazidime, have activity against enteric gram-negative bacilli, which rarely cause IE in children. Ceftazidime does not have activity against staphylococci, is not the agent of choice for treating streptococci, and therefore would not be an appropriate choice for the treatment of IE in children. Doxycycline can be used to treat uncomplicated skin and soft-tissue infections caused by susceptible strains of S aureus in children older than 7 years of age, and trimethoprim- sulfamethoxazole is also effective against susceptible strains of S aureus, but neither is appropriate for the treatment of a life-threatening invasive staphylococcal infection SUGGESTED READING: American Academy of Pediatrics. Staphylococcal infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. 2012 Red Book: Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:653-668. http://aapredbook.aappublications, Copyright 2013 © American Academy of Pediatics 43American Academy of Pediatrics 2013 PREP SA on CD-ROM org/content/1/SEC131/SEC263.body Bradley JS, Sauberan J. Antimicrobial agents. In: Long SS, Pickering LK, Prober CG, eds. Principles and Practice of Pediatric Infectious Diseases. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2008:1420- 1452 Kaplan SL, Hulten KG, Mason EO. Staphylococcus aureus infections (coagulase-positive Staphylococci). In: Feigin RD, Cherry JD, Demmier-Harrison GJ, Kaplan SL, eds. Feigin & Cherrys Textbook of Pediatric Infectious Diseases. 6th ed. Philadelphia, PA: Saunders Elsevier, 2009:1197- 1213 Liu C, Bayer A, Cosgrove SE, et al. Clinical practice guidelines by the Infectious Diseases Society of ‘America for the treatment of methicillin-resistant Staphylococcus aureus infection in adults and children. Clin Infect Dis. 2011;52(3):1-38. doi:10.1093/cid/ciqi 46 Copyright 2013 © American Academy of Pediatics 49American Academy of Pediatrics 2013 PREP SA on CD-ROM Question: 16 The parents of a 1-month-old infant who was found to have a solitary kidney in utero are asking questions about the status of the infants kidney function. The infant is otherwise healthy. Upon the insistence of the family, a serum creatinine and ultrasonography are performed. The serum creatinine is 0.5 mg/dL. Renal ultrasonography demonstrates a 5.5 cm kidney on the right side of the body and absent kidney on the left. Of the following, the statement that BEST characterizes the renal status of the infant is ‘A. nephrotoxic agents should be minimized because of decreased nephron mass B. this infant is at higher than usual risk for dehydration in the setting of gastroenteritis C. this infantis likely to require dialysis by adolescence D. this infant will require annual ultrasonography to screen for renal malignancy E. toilet training may be more challenging in the future for this infant ‘Copyright 2013 © American Academy of Pediatrics 502013 PREP SA on CD-ROM American Academy { ES of Pediatrics t ey Critique: 16 Preferred Response: A The renal status of newborns can be challenging to predict because of the dynamic nature of the glomerular fitration rate (GFR) in the first 2 years after birth. Typically, newborns are born with 2 kidneys, each composed of approximately 1 million nephrons. The GFR increases progressively from day 1 after birth up to near adult-level function (120 cc/min/1.73 m2) by 2 years of age. The most frequently used means to estimate the GFR of a child is via a serum creatinine concentration. The serum creatinine may be elevated for the first 10 days after birth, reflecting maternal levels, which have not yet been eliminated by the kidneys of the newborn. By approximately 2 to 4 weeks of age, an infants serum creatinine should be approximately 0.3 to 0.4 mg/dL. By 3 months of age, the creatinine should decrease to a concentration of approximately 0.2 to 0.3 mg/dL, where it should remain until approximately 1 to 2 years of age. After 2 years of age (when adult level of GFR has been reached), the serum creatinine concentration may begin to rise slightly because creatinine concentration is also a reflection of increasing muscle mass in a growing child. The normal creatinine concentration ranges from 0.8 to 1.1 mgidL for young adult females to 1.0 to 1.3 mg/dL. for young adult males, with higher values corresponding with the muscle mass of the patient The vignette describes a 1-month-old infant with a single kidney and a serum creatinine of 0.5 mg/dL. This infant has unilateral renal agenesis, which has an estimated incidence of 1 in 2.900 newboms. Infants with unilateral renal agenesis have 50% of the expected nephron number. Because of a compensatory increase in the single nephron GFR (SNGFR), the serum creatinine should continue to fall to normal levels for the childs age over the first 1 to 2 years of life until reaching an expected level of 0.3 mg/dL. Moreover, this single kidney is fully functional and has normal urine concentrating abilities; therefore, an infant with gastroenteritis would not be expected to be at high risk of dehydration in this setting, It is unusual for the doubling of the SNGFR to result in a hyperfiltration injury to the nephron mass and result in proteinuria. As the single kidney should be structurally and functionally normal, screening for hypertension should be done beginning at age 3 years during health maintenance visits. Periodic urinalysis also should be considered to screen for the development of proteinuria. Avoidance of nephrotoxic medications such as nonsteroidal anti-inflammatory drugs, intravenous radiocontrast, and aminoglycoside antibiotics should be considered in children with a single kidney because of the reduced renal reserve in these patients. Also, newborns with renal agenesis have an approximately 30% risk of vesicoureteral reflux (VUR) into their kidneys, therefore, performance of a voiding cystourethrogram to evaluate for VUR is recommended in these patients. As this child has normal urinary concentrating ability and bladder function, this child should develop daytime and nighttime continence at the same age as other children who have 2 kidneys. Bladder dysfunction would not be expected in this setting. Infants with renal agenesis are not at an increased risk of malignancy. This is unlike children with genetic syndromes such as Beckwith-Wiedemann syndrome, in which ultrasonography every 3 months until 8 years of age is recommended to screen for the development of Wilms tumor. SUGGESTED Copyright 2013 © American Academy of Pediatrics 5American Academy of Pediatrics 2013 PREP SA on CD-ROM READING: Finney H, Newman DJ, Thakkar H, Fell JM, Price CP. Reference ranges for plasma cystatin C and creatinine measurements in premature infants, neonates, and older children. Arch Dis Child. 2000;82(1):71-75 Robson WL, Leung AK, Rogers RC. Unilateral renal agenesis. Adv Pediatr. 1995;42:575-592 Copyright 2013 © American Academy of Pediatics 52American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 17 ‘A 2-year-old girl who has developmental delay presents to urgent care after a cyanotic episode at daycare. She was noted to have a look of fear and then confusion associated with slow breathing, a choking sound, and diminished awareness. Afterward, she Seemed “out of if’ and unlike herself. The parents believe that this is similar to several previous episodes, although the duration today was longer. Physical examination reveals that the child has small growth parameters, including head circumference. She sits but does not stand, and she has no language and poor eye contact. She has truncal unsteadiness and brings her hands to her mouth frequently. Of the following, the MOST likely diagnosis is A. acute cerebellar ataxia B. ataxia telangiectasia C. Joubert syndrome D. Niemann-Pick disease type C E. Rett syndrome Copyright 2013 © American Academy of Pediatrics 532013 PREP SA on CD-ROM. American Academy é of Pediatrics aN x Critique: 17 Preferred Response: E The girl in the vignette presented for medical attention after experiencing a s parents, it appears that several previous, unprovoked seizures have occurred, which is consistent with a diagnosis of epilepsy. In girls who have small growth parameters, developmental delay, truncal unsteadiness, and stereotypic midline hand behaviors, Rett syndrome should be considered. Rett syndrome is an X-linked dominant disorder caused most often by a mutation in the MECP2 gene. The diagnosis of Rett syndrome is made clinically and is the entity most likely responsible for the symptoms and signs exhibited by the girl in the vignette. Although many children who have recurrent seizures have an idiopathic form of epilepsy, symptomatic (secondary) epilepsy should be considered when developmental delay is present. Symptomatic epilepsy may be caused by structural problems in the brain that produce both the developmental impairments and the predisposition to seizures. Loss of skills over time is not expected in most symptomatic epilepsies unless the seizures are severe and refractory to treatment. ‘A small proportion of children who have symptomatic epilepsies have a degenerative neurologic disease or recognizable genetic disorder. A degenerative neurologic disease should be considered when epilepsy and developmental delay are accompanied by the loss of previously acquired skills. The latter may be assessed by asking a parent “Is there anything that your child once was able to do that she can no longer do?” ‘Suspicion of Rett syndrome or other neurodegenerative diseases should generally prompt a comprehensive, stepwise evaluation to precisely identify the cause. Online resources like Online Mendelian Inheritance in Man (http:/’www.ncbi.nlm.nih.gov/omim) can be extremely helpful. Given the complexity and expense of these diagnostic workups, early referral to a child neurologist is indicated Neurologists may use clinical clues to stratify diseases into categories, such as predominately cerebral gray matter, white matter, or cerebellar. The truncal unsteadiness described for the git! in the vignette may be truncal ataxia, which can result from many conditions affecting the cerebellum. These conditions include congenital structural malformations, genetic degenerative diseases, or an acquired process such as a toxic ingestion or a posterior fossa neoplasm. The Web site for the National Institute of Neurological Disorders and Stroke (http:/www.ninds.nih.gov) is an excellent resource for the other responses in the vignette (ie, ataxia telangiectasia, Joubert syndrome, and Niemann-Pick type C disease). Congenital structural malformations include problems like Joubert syndrome, which is characterized by underdevelopment or absence of the cerebellar vermis. Typically, cerebellar malformation syndromes do not present with seizures. Genetic degenerative diseases affecting the cerebellum are numerous and may or may not present early with seizures. Ataxia telangiectasia presents initially in toddlers with choreic and dystonic dyskinesias and gait and speech coordination problems but not with seizures. Niemann-Pick disease type C is a degenerative disease involving lipid storage. It can present in childhood with progressive tremor, gait disturbance, dementia, and, eventually, seizures, Splenic enlargement may be present. However, seizures are not an early finding, Acute cerebellar ataxia is an acquired disorder with subacute (hours to days) onset and progression in a previously normal child. It may be postviral. It Copyright 2013 © American Academy of Pediatrics 54American Academy nape 2013 PREP SA on CD-ROM of Pediatrics does not present with seizures, but nystagmus may be present. This diagnosis is easy to confuse with ‘opsoclonus-myocionus-ataxia syndrome. Expert consultation is recommended for suspected ataxia and structural or degenerative cerebellar conditions. SUGGESTED READING: Cakir B, Teksam M, Kosehan D, Akin K, Koktener A. Inborn errors of metabolism presenting in childhood. J Neuroimaging. 2011:21(2):e117-133. doi:10.11114,1852-6569.2011.00575.x Erlandson A, Hagberg 8. MECP2 abnormality phenotypes: clinicopathologic area with broad variability. J Child Neurol. 2005;20(9):727-732. doi:10.1177/08830738050200090501 Gropman AL, Batshaw ML. Epigenetics, copy number variation, and other molecular mechanisms underlying neurodevelopmental disabilities: new insights and diagnostic approaches. J Dev Behav Pediatr. 2010;31 (7):582-591 Kolodny EH, Lyon G. Degenerative Disorders. In: Maria BL, ed. Current Management in Child Neurology. 3rd ed. Hamilton, Ontario: BC Decker; 2005: 268-276 Kurian MA, McNeill A, Lin JP, Maher ER. Childhood disorders of neurodegeneration with brain iron accumulation (NBIA). Dev Med Child Neurol. 2011;53(5):394-404 Kwon JM. Neurodegenerative disorders of childhood. In: Kliegman RM, Stanton BF, St Geme JW Ill Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011: 2089-2076 Copyright 2013 © American Academy of Pediatrics 55American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 18 During a health supervision visit, the parents of an 8-year-old girl mention that she runs with an unusual gait. She had a history of developmental hip dysplasia that was treated conservatively with a Pavlik harness, but she has not seen her orthopedic surgeon for 4 years. Otherwise, she has been healthy and has normal growth parameters and development. Her examination results are normal except for a Trendelenburg gait, spine curvature with pelvic tlt, and slight limitation of hip abduction on the left side. She has normal strength bilaterally. Of the following, the BEST next step in evaluating this patient is to A. obtain radiographs to evaluate degree of scoliosis B. order a complete blood cell count and sedimentation rate to assess for an inflammatory process C. measure her leg lengths to assess for discrepancy D. refer her to a neurologist for evaluation for muscle disorders E. schedule hip ultrasonography to monitor hip dysplasia Copyright 2013 © American Academy of Pediatrics 56American Academy é nape 2013 PREP SA on CD-ROM of Pediatrics aN x Critique: 18 Preferred Response: C Leg length inequality in children can be the result of a number of conditions, including trauma, infection, neuromuscular diseases, tumors, and congenital malformations. Among the more common insults leading to slowed growth of an extremity and leg length discrepancy are developmental hip dysplasia, Legg-Calvé-Perthes syndrome, cerebral palsy, hip or femur fracture, and septic hip. In addition, there may be angular or rotational deformities such as coxa brevis or coxa vara Patients who have leg length inequality may present because of an abnormal, awkward gait, often with a Trendelenburg gait pattem (the pelvis drops toward the unaffected side with heel strike on the affected side) due to weakness of hip abductor muscles. They may also circumduct the longer limb when walking or maintain that knee in flexion. They may toe walk on the shorter limb. As for the girl in the vignette, a pelvic tit accompanied by compensatory or functional scoliosis may be observed. Once the pelvic tit is leveled, the apparent scoliosis resolves and scoliosis films are not needed. Limb length discrepancies less than 2 cm usually do not cause significant gait disturbance or symptoms. If the leg length discrepancy is greater than 3.7% of the length of the longer limb, gait disturbance is visually obvious. If there is a difference greater than 5.5%, the patient must use greater mechanical work when walking, leading to muscle fatigue and hip, lower back, knee, or ankle pain The most accurate method of determining the amount of leg length discrepancy in the clinical setting is to place wooden blocks of known thickness under the foot of the shorter leg until the pelvis levels. Measuring the lower extremities is an alternative approach, but it has a higher error rate than the block method. Radiographic imaging also may be used to measure the legs. A standing radiograph from hip to ankle that uses a single long cassette demonstrates both mechanical alignment and angular deformities but lacks accuracy because of magnification and distortion. A scanogram prevents, distortion, but the child must hold perfectly still for 3 separate exposures. This method also does not provide an accurate depiction of angular deformities. A computed tomography scanogram is accurate and allows measurement in the face of fixed hip and knee contracture, but again, the patient must remain still throughout the procedure, Treatment depends on both the amount of discrepancy and the potential growth remaining. There are several methods for predicting leg length inequality at maturity that aid in deciding inequality type and timing of intervention. For inequalities less than 2 cm, a lift within the shoe is usually sufficient to ‘overcome symptoms. For predicted discrepancies at maturity of 2 to 5 cm, epiphysiodesis of the longer limb slows growth on the unaffected side, allowing more equal lengths. For differences greater than 5 ‘om, leg-lengthening procedures on the affected limb are usually recommended. ‘Since the girls past medical history and physical findings suggest that leg length discrepancy is likely responsible for the observed spinal curvature, radiographs to evaluate for scoliosis would not be the next step in evaluation. In the absence of fever or signs of inflammation, a complete blood cell count and sedimentation rate would not be useful, Referral to a neurologist would be indicated if the girl exhibited signs of a myopathy, but such is not the case. Ultrasonography of the hip is not useful as a mean of monitoring hip dysplasia in a girl of this age. Copyright 2013 © American Academy of Pediatrics 87American Academy of Pediatrics 2013 PREP SA on CD-ROM. SUGGESTED READING: Beaty JH. Congenital anomalies of the lower extremity. In: Canale ST, Beaty JH, eds. Campbells Operative Orthopedics. 11th ed. Philadelphia, PA: Mosby Elsevier; 2007 http://www mdconsult. com/books/page.do?eid=4-u1.0-B978-0-323-03329-9..X5001-68isbn=978-0-323- 03329-98sid=1255591747&unigld=313181919-172#4-u1.0-B978-0-323-03329-9..X5001-6 Friedman JE, Davidson RS. Leg-Length Discrepancy. In: Kliegman RM, Stanton BF, St Geme JW. Schor NF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:2351 Friend L. Widmann RF. Advances in management of limb length discrepancy and lower limb deformity. Curr Opin Pediatr. 2008;20(1):46-51 Inan M, Chan G, Bowen JR. The correction of leg-length discrepancy after treatment in developmental dysplasia of the hip by using a percutaneous epiphysiodesis. J Pediatr Orthop B. 2008;17(1):43-46 Copyright 2013 © American Academy of Pediatrics 58American Academy nape 2013 PREP SA on CD-ROM of Pediatrics Question: 19 You are reviewing with a resident the history and physical examination findings of a 13-year-old girl who complains of intermittent, crampy, lower abdominal pain for almost a year. The resident reports that acetaminophen and ibuprofen do not relieve the girls pain, and she has recently begun to have back pain and difficulty urinating. She has no history of weight loss or gastrointestinal symptoms, and she has not started menstruating or ever been sexually active. Family history reveals that the girls mother began menstruating at age 12 years. On physical examination, her vital signs are normal. She has a sexual maturity rating of 5, and the resident reports that results of the girls abdominal examination are normal except for a possible small mass above her pubic symphysis. Of the following, the BEST next step in the management of this girl would be to A. conduct an external genital examination B. induce menses with a progesterone challenge C. order abdominal ultrasonography D. order follicle-stimulating hormone and luteinizing hormone levels E. send a urine sample for analysis and culture Copyright 2013 © American Academy of Pediatrics 59