Vol.11 No. 10April 1990

AMERICAN ACADEMY OF PEDIATRICS

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291 #{149}“It Happens” - Menna

292 #{149}Pyloric Stenosis - Garcia andRandolph

297#{149}Diabetic Ketoacidosis and theRole of Outpatient Management- Chase, Garg, and Jelley

305#{149}Undescended Testis andOrchiopexy - Hawtrey

311 #{149}Cures in Childhood Cancer -

Chauvenet and Wofford

I

CONTE NTS

COMMENTARY

291 “It Happens”

Vincent J. Menna

ARTICLES

292 Pyloric Stenosis: Diagnosis and Management

Victor F. Garcia and Judson G. Randolph

297 Diabetic Ketoacidosis in Children and the Role of

Outpatient ManagementH. Peter Chase, Satish K. Garg, and David H. Jelley

305 Undescended Testis and Orchiopexy: Recent

Observations

Charles E. Hawtrey

31 1 Cures in Childhood Cancer

Allen R. Chauvenet and Marcia M. Wofford

ABSTRACTS

296 Pylonc Stenosis

304 Thumb Sucking

309 Evaluating a Child with Hypoglycemia

310 Vanoceles: To Treat or Not?

318 Cumulative Index

Cover: Breton Girls, Dancing, Pont Avon, by Paul GAUGUIN (Copyright,

National Gallery of Art, Washington, DC; Collection of Mr and Mrs PaulMellon). Gauguin was a French Symbolist and lived from 1 848 to 1903.Gauguin traveled the world as a seaman and pursued a career in bankingin Paris and Copenhagen before concentrating on his skills as a painter andsculptor. He was determined to develop a new approach to painting throughwhich to symbolically express a thought or mood, in contrast to the impres-sionist approach which sought to reproduce a scene through the exactrecording of every nuance of color and light. Completed in 1888, BretonGirls Dancing, Pont Aven is one of Gauguin’s earliest works in this newstyle. The themes of friendship, community, exercise, and appreciation ofnature depicted here are important elements in the total health and devel-opment of every child.

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�‘ The printing and production of

______ 2 Pediatrics in Review is made possible,

� R OS S � fl part, by an educational grant fromVA �T Ross Laboratories.

Answer Key: 1.B; 2.E; 3.E; 4.E; 5.A; 6.C; 7.B; 8.E; 9.D; 10.B; 11.0; 12.B; 13.B; 14.C;15.A; 16.A; 17.A; 18.D.

Vol. 11, No. 10, April 1990

Pediatricsin ReviewEDITORRobert J. HaggertyNew York Hospital-CornellMedical CenterNew York, NY

Editonal Office:The William T. Grant Foundation515 Madison Aye, 6th Floor,New York, NY 10022-5403

ASSOCIATE EDITORLawrence F. NazarianPanorama Pediatric GroupRochester, NY

Mailing Address:29 Surrey PlacePenfield, NY 14526

EVALUATION EDITORWilliam H. Milburn, Lyons, CO

MANAGING EDITORJo A. Largent, Elk Grove Wllage, IL

EDITORIAL CONSULTANTVictor C. Vaughan Ill, Stanford, CA

EDITORIAL BOARDRalph Cash, Detroit, MlDaniel D. Chapman, Ann Arbor, MlEven Chamey, Worcester, MARussell Chesney, Nashville, TNAlan L Goldbloom, Toronto, ONJ. Stephen Latimer, Bethesda, MDMarie C. McCormick, Boston, MAKurt Metzl, Kansas City, MOPhilip A. Pizzo, Bethesda, MDRobert Rennebohm, Columbus, OHWilliam 0. Robertson, Seattle, WARon Rosenfeld, Stanford, CARobert Schwartz, Providence, RIRobert J. Touloukian, New Haven, CTW. Allan Walker, Boston, MA

PUBUSHERAmerican Academy of PediatricsNancy Wachter, Copy Editor

PEDIATRICS IN REVIEW(ISSN 0191-9601)ls owned andcontrolled by the American Academy of PedIatrics. ft is

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COMMENTARY“It” Happens

No matter how hard we try, “it”happens: the mistakes that all hu-mans make, even doctors. Ourfriends, the lawyers, know this andthat is why we are often their prey.During a recent conversation with alawyer acquaintance, he mentionedthat everyone is human and makesmistakes. He related that he almostmissed the statute of limitations forone of his clients. Tears streameddown my face.

A pediatric office is ripe for mis-takes because it is so busy. Everymember of the office-doctors,nurses, receptionists, and secre-taries-is anxious to resolve theirown particular problems. Doing theirjobs frequently entails interrupting aphysician. It is wise to have a policyof not interrupting someone who isbusy writing on charts or talking onthe telephone. In our office, we havea policy that a physician is interruptedin the examining room only in case ofan emergency or if another physiciancalls. If one leaves the room to an-swer a telephone call, one oftenmeets a member of the office staffwith an additional problem. Eventu-ally it is difficult to remember whichexamining room one left!

I have learned that it is a good ideato make a cursory review of a pa-

tient’s chart during each office visit,even if the chief complaint is simplyan earache. Often one finds that stud-ies ordered or consultations sug-gested were never done. In the hos-pital, it is wise to read the nurse’snotes. This is not only important tocheck the progress of patient, butalso to see if there is a discrepancybetween your evaluation and that ofthe nurse. The lawyers love to findthis. I have found that it is also ben-eficial to recheck all laboratory and x-ray reports at the time of discharge.

A colleague of mine was once toldby one of his professors that, at anacademic center, all of the tigers arein cages. In general pediatric practice,the tigers are hiding in the grass.Much of what we do on a daily basisis routine. However, a lapse of con-centration or alertness may cause usto miss the unusual or subtle case.

Given all of life’s other problemsand pressures, it is difficult to be con-stantly attentive. No matter how com-pulsive, conscientious, and dedicatedwe are, “it” can still happen. By notletting down our guard, however, wecan ensure that “it” is a rare event.

Vincent J. Menna, MDDoylestown, PA

Editor’s Comment

Dr Menna gives sound advice. Inregard to interruptions by phonecalls, I know that there are differ-ences among pediatricians regardingwhat they consider important. Someprefer accessibility by phone and takecalls whenever they come in. I wouldguess that the majority now do as DrMenna and his colleagues, however,and have “call-in hours” morning andevening. As Hercules and Charney1demonstrated in a survey of patientssome years ago, patients place dif-ferent values on availability. It is im-portant to organize one’s day in orderto offer the best care, but differentpractices may find different ways todo so. Vive Ia difference! R. J. H.

Reference

1. Hercules C, Chamey E. Availability and at-tentiveness: Are these compatible in pedi-atric practice? Clin Pediatr. 1969, 8:381 -

388

SelfEvaluation Quiz-CME Credit

As an organization accredited for continuing medical education,the American Academy of Pediatrics certifies that completion of theself-evaluation quiz in this issue of Pediatrics in Review meets thecriteria for two hours of credit in Category I of the Physician’sRecognition Award of the American Medical Association and twohours of PREP credit.

The questions for the self-evaluation quiz are located at the endof each article in this issue. Each question has a SINGLE BEST

ANSWER. To obtain credit, record your answers on your quiz replycards (which you received under separate cover), and retum thecards to the Academy. On each card is space to answer thequestions in five issues of the journal: CARD 1 for the July throughNovember issues and CARD 2 for the December through Aprilissues. To receive credit you must currently be enrolled in PREP ora subscriber to Pediatrics in Review-and we must receive bothcards by June 30, 1990.

Send your cards to: Pediatrics in Review, American Academy ofPediatrics, 141 Northwest Point Blvd, P0 Box 927, Elk Grove Village,IL 60009-0927.

The correct answers to the questions in this issue appear on theinside front cover.

EDUCATIONAL OBJECTIVE

A full review on pyloric stenosiscan be found in the preceding ar-tide by Garcia and Randolph.R.J.H.

Pylonc Stenosis

PIR 296 pediatrics in review #{149}vol. 11 no. 10 april 1990

hypertrophic pyloric stenosis and hepaticglucuronyl transferase. Pediatr Surg.1974; 9:359

14. Franken EA. Gastrointestinal Radiology inPediatrics. Hagerstown, MD: Harper andRow; 1975: 82-87

15. Menussen T, Shaff J. Roentgen examina-tion of the pyloric canal of infants withhypertrophic pyloric stenosis. Am J DisChild. 1934; 48:1304

16. Teele RL, Smith EH. Ultrasound in thediagnosis of idiopathic hypertrophic py-loric stenosis. N Engi J Med 1977;296:1149

17. Bowen A’D. The vomiting infant’s recentadvances and unsettled issues in imaging.Radiolog Clin N Am. 1988; 26:377

18. Blumhagen J, Machen L, Krauter 0. Son-ographic diagnosis of hypertrophic pyloricstenosis. Am J Roentgoenol. 1988;150:1367

19. Galladay ES, Broadwater JR, Mallett DL.Pyloric stenosis-a timed perspective.Arch Surg. 1987; 122:825

Self-Evaluation Quiz

1. Among the following, the least likely find-ing in an infant with infantile hypertrophic

pylonc stenosis (IHPS) is:A. Jaundice.B. Bile-stained vomitus.

C. Constipation.0. Blood-stained vomitus.E. Female sex.

2. The pattern of familial occurrence of IHPSis:

A. Autosomal dominant with low pane-

trance, favoring males.B. Autosomal recessive.

C. X-Iinked dominant with low penetrance.0. x-Iinked recessive.E. Multifactonal.

3. A 4-week-old infant has had increasingly

severe vomiting for 1 week, culminating to-day in projectile vomiting. His mother hasgenerally refed him after he vomits; refeed-ings have usually been accepted. He is wellhydrated, hungry, and active. A 1-cm massfeeling somewhat like a cherry pit is feft inthe epigastrium, just to the right of the mid-line. Penstaltic waves are seen to formunder the left costal margin and to movetoward the mass. Electrolyte levels are: so-dium, 130 mEq/L; potassium; 3.5 mEq/L;chloride, 98 mEq/L; HCO2, 24 mEq/L Amongthe following, the most appropriate nextstep in the management of this infant is:

A. Abdominal ultrasonography.B. Examination of the pyloric area with bar-

ium.

C. Computed tomography of the abdomen.

0. Magnetic resonance imaging.

E. Pyloromyotomy.

4. A 3-week-old male infant has had in-creasing vomiting for the past 2 weeks, oc-casionally projectile in the past 1 or 2 days.Findings from a physical examination are

normal except for moderate dehydration.The infant is alert and fussy. Attempts tofind a palpable mass in the pyloric area have

been unsuccessful. Family history recordsthat a sibling is doing well after surgery at Imonth of age for IHPS. Serum electrolytelevels are: sodium, 122 mEq/L; chloride, 80mEq/L, potassium, 7.1 mEq/L; HCO2, 15mEq/L Among the following, the proceduremost likely to lead to a definitive diagnosisis:

A. Abdominal ultrasonography.

B. Examination of the pyloric area with bar-ium.

C. Reexamination of the abdomen after re-hydration.

D. Chromosomal analysis.

E. Measurement of the serum 1 7-OH-pro-

gesterone level.

Pyloric Stenosis

Pylonc Stenosis: Congenital or Acquired. Rollins MD, et al. Arch Dis Child.1989;64:138.

The pyloric muscle dimensions of 1400 consecutive newborns were measured atbirth (ie, prospectively) by ultrasonography. Nine infants subsequently developedpyloric stenosis. All diagnoses were proved at surgery. The pyloric measurements ofthe infants with pyloric stenosis did not differ from normal at birth, but there wassignificant increase at the time of diagnosis. “We conclude that infantile hypertrophicpyloric stenosis should no longer be referred to as ‘congenital’ pylonc stenosis,although the true etiology of this acquired condition remains to be elucidated.” (RichardH. Rapkin, MD, Children’s Hospital of New Jersey)

Diabetic Ketoacidosis

PIR 304 pediatrics in review #{149}vol. 11 no. 10 april 1990

lone JI, Donnelly WH: Cerebral edemacomplicating diabetic ketoacidosis in child-hood. J Pediatr. 1980;96:357-361

1 7. Krane EJ, Rockoff MA, Wallman JK,Wolfsdorf JI. Subclinical brain swelling inchildren during treatment of diabetic ke-toacidosis. N EngI J Med. 1 985;31 2:1147-1151

18. Scibilia J, Finegold 0, Dorman J, BeckerD, Drash A. Why do children with diabetesdie? Acta Endocrinol. 1986;1 13(suppl):326-333

19. Duck SC, Wyatt DT. Factors associatedwith brain hemiation in the treatment ofdiabetic ketoacidosis. J Pediatr. 1988;113:10-14

20. Franklin B, Liu J, Ginsberg-Fellner F. Ce-rebral edema and ophthalmoplegia re-versed by Mannitol in a new case of in-sulin-dependent diabetes mellitus. Pediat-rics. 1982;69:87-90

21. Chapman J, Wright AD, Nattrass M, Fitz-gerald MG. Recurrent diabetic ketoaci-dosis. Diabetic Med. 1988;5:659-661

22. Chase HP, Rainwater NG. Missed insulininjections: A common syndrome. PractDiabetol. 1989;8:20-23

Self-Evaluation Quiz

5. Each of the following is a counterregula-tory hormone which is in excess during di-abetic ketoacidosis (DKA), except:

A. Insulin.

B. Glucagon.

C. Epinephnne.D. Corticosteroids.E. Growth hormone.

6. A 7-year-old girl has DKA secondary to aviral infection. Home management has beeninitiated using the phone. Which of the fol-lowing would not suggest that she shouldbe seen by a physician right away?

A. Deep respirations.B. Inability to stand.

C. Ketones in urine.0. Persistent vomiting.E. Unreliable family.

7. A 4-year-old boy has been hospitalizedfor DKA which has lasted 12 hours. Whichof the following blood tests would be mostlikely to be useful?A. Calcium concentration.

B. Venous pH value.

C. White blood cell count.0. Creatinine level.E. Phosphorus concentration.

8. Each of the following is a contraindica-tion to the intravenous administration of p0-tassium to a child with DKA, except:

A. pH 7.0.B. Peaked T waves on electrocardiogram.

C. Central line only, venous access.0. Serum potassium concentration not

known.E. Normal serum potassium concentration.

9. An 8-year-old girl with DKA, who hasreceived standard intravenous therapy for 4hours, develops a headache, drowsiness,and dilated pupils. Which of the followingwould not be appropriate treatment at thistime?

A.B.C.D.E.

Elevation of the head of the bed.Hyperventilation.Restriction of fluids.

Dexamethasone.Mannitol.

10. The result of which of the following isleast likely to be important before initiatingrehydration and intravenous insulin therapyin a child with DKA?

A. Clinical assessment of hydration.B. Serum osmolality.

C. Venous pH value.0. Urine ketone levels.E. Blood glucose level.

Thumb Sucking

Thumb Sucking: Pediatrician’s Guidelines. Firman PC, et al. Clin Pediatr. 28:438,1989.

In the United States, 19% of the children still suck their thumbs at 5 years of age.Some risks accompany this behavior, including malocclusion, alopecia, accidentalpoisoning, digital hyperextension, paronychia, and “risks to psychological health.” Thelast is true “because many parents dislike it and at least some routinely criticize,ridicule, and/or punish its practice. . .many children also dislike thumb sucking [inothers!] and do not want thumb-sucking children as friends. . .or classmates.”

Thumb sucking should be treated only if it is chronic, if the child is more than 4years of age, and if there is another (eg, dental) problem. Methods of therapy whichwork well include motivational systems (rewards), bitter taste treatments (which workonly with the child’s approval), dental appliance installation, and “planned ignor-ing. . .most thumb sucking in childhood is harmless, self-limited, and does not requiredirected intervention.”

Comment: lllingworth says, “The danger of thumb sucking lies not in the thumbsucking but in what the parents do about it.” Even the malocclusion question isunresolved; the great majority of persistent thumb suckers do not develop malocclu-sion. The author’s suggestions are sound, and the article is worth reading in itsentirety. (Richard H. Rapkin, MD, Children’s Hospital of New Jersey)

TABLE. Algorithm for Management of Undescended Testes

At birth:Multiple congenital anom-

aliesAmbiguous genitalia

Assess anomalies and give priority to thosethat threaten life

KaryotypingGenitogramGonadal biopsyPanendoscopySurgical management

After 1 y of ageBilateral undescentPalpable testisManipulable testis

Bilateral maldescent (non-palpable)

Open extemal ring

Unilateral undescentPalpable testisManipulable testis

Counsel familyHormonal stimulation

1500 IU human chonionic gonadotropin/M22timesperwkfor2to5wk

1 .2 mg of gonadotropin-releasing hor-mone given intranasally (response prob-ability of 6% to 19%)

Orchiopexy if response to hormonal therapyis limited or absent

Diagnostic localizationLaparoscopy to identify chord structure or

intra-abdominal testesHuman chorionic gonadotropin stimulationOrchiopexy if hormonal stimulation fallsCounsel familyHormonal stimulation

1500 IU human chorionic gonadotropin/M22timesperwkfor2 to5wkorintramuscularly daily for 28 d

Orchiopexy if response to hormonal therapyis limited or absent

Diagnostic localizationLaparoscopyThallium scanComputed tomography or magnetic reso-

nance imagingOrchiopexyOrchiopexyCounsel family regarding:

Decreased likelihood of fertilityContinuation of hormonal functionIncreased likelihood of testicular dys-

genesis at or near puberty, at whichtime orchiectomy becomes an option

Unilateral maldescent(nonpalpable)

Testis reascentUnilateral undescent (after

1 y of age)

Undescended Testis

PIR 308 pediatrics in review #{149}vol. 1 1 no. 10 april 1990

nithm for the management of childrenwith undescended testis is outlined inthe Table.

REFERENCES

1. Scorer CG. The descent of the testis. ArchDis Child 1964;39:605-609

2. Batata MA, et al. Cryptorchism and testic-ular cancer. J Urol 1980;124:382-387

3. Johnson DE, et al. Cryptorchism and tes-ticular tumongenesis. Surgery. 1 968;63:919-922

4. Baumrucher GO. Incidence of testicularpathology. US Army Med Dept. 1946;5:312

5. Pike MC. Boys with late descendingtestes: the source of patients with “retrac-tile” testes undergoing orchidopexy?” BritMed J. 1 986;293:789-790

6. Atwell JO. Ascent of the testis: fact orfiction. Br J Urol. 1 985;57:474-477

7. Alpert PF, Klein RS. Spermatogenesis inthe unilateral cryptorchid testis after or-chidopexy. J. Urol. 1 983;1 29:301-302

8. Albescu JZ, et al. Male fertility in patientstreated for cryptorchidism before puberty.Fertil Steril. 1971 ;22:829-833

9. Knorr 0. Fertility after hCG-treatment ofmaldescended testes. PediatrAdolesc En-docrinol. 1 979;6:21 5

10. Rajfer J, et al. A randomized, double-blindstudy comparing human chononic gonad-

otropin and gonadotropin releasing hor-mone. N EngI J Med. 1 986;31 4:466-470

1 1 . de Muinck Keizer-Schrama SMPF, etDouble-blind placebo-controlled study ofLHRH nasal spray in treatment of unde-scended testes. Lancet 19;1 :876

1 2. Hinman F Jr. Alternatives to orchiopexy. JUrol. 1 980;1 23:548-551

1 3. Kogan SJ. Fertility in cryptorchidism. In:Hadziselimovic F, ed. Cryptorchidism:Management and Implications. New York:Springer-Verlag; 1983:71-82

Self-Evaluation Quiz

1 1. Which of the following is least likely tobe a true statement?

A. 0.8% of 1-year-old boys have an unde-

scended testis.B. 0.2% of male military recruits have an

undescended testis.C. Over 20% of premature male infants

weighing less than 2500 g have an un-

descended testis.0. A very premature 900-9 male newborn

with bilateral undescended testes is un-likely to have descended testes at 1 year

of age.E. Most individuals with undescended testes

are identified in childhood.

12. Each of the following is a true statementpertaining to testicular neoplasms, except:

A. About 1 1 % of males with undescendedtestes develop neoplasms.

B. Orchiopexy reduces the risk of testicularneoplasia to no more than that of the

normal population.C. Seminomas are the most frequent type of

testicular neoplasm.0. 8% of men with an undescended testis

develop a neoplasm in the contralateral

testis.E. The risk of malignant transformation in an

undescended testis is higher for an intra-abdominal testis than for a scrotal testis.

13. Which of the following isleast likely tobe a true statement?

A. Over 70% of men treated for bilateralcryptorchism have azoospermia.

B. Men treated for unilateral undescendedtestes are as as likely to be fertile as menwith bilaterally normally descended

testes.C. Medical or surgical treatment for unde-

scended testes is best done at about 1to 2 years of age.

0. Controlled studies show no clear benefitof hormonal treatment of truly unde-scended testes.

E. Appropriate surgical treatment of unde-scended testes requires close attention

to details of technique.