Proper diagnosis of biomechanical abnormalities allows for more effective

treatment of this condition.

BiomechanicalManagement of

Children andAdolescentsWith DownSyndrome

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Down syndrome is the mostcommon and well-recog-nized congenital anomaly

causing mental retardation. It oc-curs in all parts of the world, in all

races, and in approximately one inevery 700 births. It is more com-mon in girls with a 3:1 female tomale ratio.26 Orthopedic problemsare common in individuals withDown syndrome. Early recognitionand treatment of these conditions

can often make a significant differ-ence in these individuals’ overallability to function and their qualityof life.

The most important deformitiesrequiring care are atlantoaxial instabil-

By Mark A. Caselli, DPM

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Welcome to Podiatry Management’s CME Instructional program. Our journal has been approved as a sponsor of Contin-uing Medical Education by the Council on Podiatric Medical Education.

You may enroll: 1) on a per issue basis (at $17.50 per topic) or 2) per year, for the special introductory rate of $99 (yousave $76). You may submit the answer sheet, along with the other information requested, via mail, fax, or phone. In the nearfuture, you may be able to submit via the Internet.

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This instructional CME program is designed to supplement, NOT replace, existing CME seminars. Thegoal of this program is to advance the knowledge of practicing podiatrists. We will endeavor to publish high quality manuscriptsby noted authors and researchers. If you have any questions or comments about this program, you can write or call us at: Podia-try Management, P.O. Box 490, East Islip, NY 11730, (631) 563-1604 or e-mail us at bblock@prodigy.net.

An answer sheet and full set of instructions are provided on pages 170-172.—Editor

ObjectivesAfter reading this article, thepodiatric physician should beable to:

1) Understand the geneticabnormalities that result inDown syndrome.

2) Recognize the generalcharacteristics of a patientwith Down syndrome.

3) Take a proper medicalhistory taking into accountboth common medical andorthopedic disorders thatmay affect ambulatory andfoot function.

4) Recognize the potential-ly serious orthopedic/neuro-logic conditions associatedwith Down syndrome.

5) Develop a lower extremi-ty treatment plan for achild/adolescent with Downsyndrome.

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B I O M E C H A N I C S & O R T H O T I C SB I O M E C H A N I C S & O R T H O T I C S

Proper diagnosis of biomechanical abnormalities allows for more effective

treatment of this condition.

BiomechanicalManagement of

Children andAdolescentsWith DownSyndrome

drome. Diamond et al.8 state, “thetreatment of painful feet in patientswith Down syndrome is imperative,because foot pain leads to relative im-mobilization and immobile retardedadults do not remain long in the com-munity”. Other frequently associatedmedical conditions include congenitalheart disease (particularly septal de-

fect) and anoma-lies of the gas-trointestinal tract(typically duode-nal atresia andHirschsprung’sdisease).

HistoryIn 1866, Lang-

don Down identi-fied Down syn-drome in a groupof mentallyhandicapped in-dividuals. He wasinterested in theethnologic classi-fication of the

mentally handicapped.16,29 Hethought that Down syndrome wasa throwback to an ancient Mongo-lian ancestor. Ten years later, theconnection between maternal ageand Down syndrome was made. Itwas thought to be the result of de-generative changes of the female re-productive tract.25,29 In 1932, Waar-denburg proposed that a chromo-some abnormality could explainDown syndrome.27 In 1959, LeJeuneidentified Down syndrome as thefirst condition to be caused by anautosomal trisomy.27

GeneticsMost often, Down syndrome is

caused by trisomy 21 in the G group.There is uneven allocation of chro-mosomes during normal reductionand division. Consequently, theovum may end up with an extra Gchromosome—the zygote having 47instead of 46 chromosomes.26 (Fig. 1)

The human genome contains ap-proximately 100,000 genes. The num-ber 21 chromosome contains less than

2% of the genome, orapproximately 1,000genes. The phenotypefor Down syndrome islocated on the 21q22locus of the 21st chro-mosome. The 21q22locus contains onlyabout 100 genes. There-fore, only about 100genes are responsiblefor the full phenotypeof Down syndrome.24

The genes forl e u k e m i a a n dAlzheimer’s disease arealso located on chro-mosome number 21.This accounts for thethirty-fold increased in-cidence of leukemia inDown syndrome andthe increased incidenceof Alzheimer’s disease.22

The cause of Downsyndrome is non-dis-junction of the femaleor male chromosomeduring meiotic division.Non-disjunction in thefemale accounts for75% of cases and the re-maining 25% is causedby male non-disjunc-

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Down Syndrome...

ity, dislocation of the patella,spontaneous habitual dislocation

of the hip, genu valgum, and severeflexible pes planovalgus. Fifty percentof all children with Down syndromehave gait problems.20 Painful pesplanovalgus is universal in Down syn-

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Figure 4: Severe rigid pes planovalgus in a young adult

Figure 2: Simian palmar crease seen in Down syndrome

Figure 3: Characteristic facial fea-tures and stature of adolescent fe-male with Down syndrome

Figure 1: Karyotype of female with 21 trisomy (Down syndrome)

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cases of fraternal twins, usually onetwin is not affected.3

The life span of children withDown syndrome has dramatically in-creased. In 1929, it was only 9 years. In1989, the life expectancy was reportedas 70 years.25 This is because babieswith Down syndrome used to die earlyfrom heart defects and respiratory in-fection. The heart defects are now suc-cessfully corrected by surgery and theinfections are treated with antibiotics.

CharacteristicsDown syndrome is usually appar-

ent at birth, the first signs being thepronounced muscle hypotonia andthe characteristic facies. The head isusually small, with few bony promi-nences, and brachycephalic.15

The eyes have a vertical epican-thal fold with slanted palpebral fis-sures. Normally, newborns have notrue epicanthus, a fold of skin extend-

ing from the root of the nose to themedian end of the eyebrow. The iris isspeckled on the outside with what isknown as Brushfield spots. Complica-tions involving the eyes include my-opia, cataracts, blepharitis, and ectro-pion (everted eyelids).

The nose is small, with upturnednostrils and a flat nasal bridge. The earsare small and lack distinct contour.They are round or square, while thenormal shape is oval. The upper partof the helix is folded. The lobes aresmall or absent. The ears are low-set.The mouth is small with droopy cor-ners and a protruding tongue.

Even the hands are characteristic.They are short and broad, and thenails are hyperconvex. There is a dys-plastic middle phalanx of the fifth fin-ger with clinodactyly. The fingerprintstend to consist of a preponderance ofulnar loops, a distal axial triradius(85%), and in the footprints, a hallu-cial tibial arch (50%). There is a singleline running across the palm in about

tion. The non-disjunction can occurduring any of the two phases of meio-sis. The female oocytes remain in earlyprophase I of meiosis from birth untilovulation. The oldest eggs may be sus-pended in early prophase for over 40years. These older eggs are thought tobe especially vulnerable to non-dis-junction. This accounts for the rela-tionship between Down syndromeand maternal age.3,23

One in 2,500 babies is born withDown syndrome to women under theage of 30. One in 1,200 babies is bornwith Down syndrome to women be-tween the ages of 30 and 34. Thenumber jumps to one in 200 babieswith Down syndrome born towomen between the ages of 35 and39. This is why it is recommendedthat pregnant women aged 35 orolder undergo amniocentesis.29

In addition to maternal age, othercauses of nondisjunction include virus-es, cumulative radiation damage, his-tory of the hepatitis B virus, grandpar-ent maternal age, specific genes, andhigh thyroid autoantibody.3

There are three basic types ofDown syndrome. The classic trisomy21 type related to older maternal ageaccounts for 96% of all cases. Thetranslocation type, known as translo-cation 14/21 or translocation D, occurswhen the extra chromosome 21 is at-tached to chromosome 14. This type isnot related to maternal age. Here, thechromosome number is still 46. Phe-notypically, this type is identical to thetrisomy category. The third type ofDown syndrome is the mosaic type,which occurs in about 1% to 2% of allcases. In this group, half of the cell linehas the normal 46 chromosome kary-otype and half of the cell line has thetrisomy 47 chromosome karyotype.Individuals in this group are pheno-typically milder than those in theother two groups, and may even ap-proach normal intelligence.3

The risk of recurrence of Downsyndrome in a family after one affect-ed child is about 1% for mothers in allage groups. For certain translocationor mosaic forms, the risk is muchhigher. Females with Down syndromeare fertile; males are sterile. Femaleswith Down syndrome have a 50%chance of having children with Downsyndrome. Identical twins with Downsyndrome will both be affected. In

Down Syndrome... 50% of patients withDown syndrome, known asthe Simian crease.2,11 (Fig. 2) Chil-dren and adolescents with Downsyndrome tend to be short andheavy.(Fig. 3) They are profoundly re-tarded, with an IQ mean of about 50.27

Orthopedic DisordersThe most common orthopedic

disorders seen in Down syndromeconsist of metatarsus primus varus,with or without hallux abducto val-gus; subluxating or dislocatingpatella; severe pes planus; at-lantoaxial instability; scoliosis;slipped femoral epiphysis; genu val-gum; and acetabular dysplasia, withor without subluxating hips.8

Hypotonia, ligamentous laxity,and hyperflexibility of the joints, pre-sent in 88% of children with Downsyndrome, are probably the majorcauses of orthopedic problems.1

Pes planovalgus is the most com-mon orthopedic problem in Down syn-drome. It tends to be flexible andasymptomatic during the first twodecades but becomes more rigid andpainful during the third decade.17 (Fig. 4)It often becomes disabling and the pa-tient cannot wear shoes.7 In addition,the patient has a characteristic space be-tween the first and second toes and pro-nounced hallux abducto valgus. Club-foot and syndactyly of the second andthird digits may be present.13

KneeKnee problems are common in

Down syndrome. One study found8.3% of institutionalized patients and4% of non-institutionalized patients tohave patellofemoral instability.10 Kneedeformities often are associated withprevious foot problems. The knee prob-lem interferes with ambulation, but isnot associated with pain. The knee oftengives way, causing frequent falls. Quadri-ceps strengthening exercises are used,but often surgery is required.26 Genu val-gum is a constant finding and is associ-ated with various degrees of joint laxi-ty.(Fig. 5) Chronic patellar dislocationmay be associated with long-standinggenu valgum in patients with Downsyndrome and is not evident until thethird or fourth decade.10,17

HipThe hip in Down syndrome is

retroverted, with excessive external ro-Continued on page 80

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Fifty percent of allchildren with Downsyndrome have gait

problems.

to compression.The neurologic manifestations of

spinal compression are fatigue inwalking, gait disturbance, progressiveclumsiness, incoordination, spasticity,hyperflexion, clonus, and toe-exten-sor reflex. Onset of neck pain,headache and torticollis are indicativeof malposition of the odontoid.

Individuals with Down syn-drome must have a medical exami-nation before engaging in athleticactivities that involve much move-ment of the head and neck. In 1984,the American Academy of Pediatrics(AAP) recommended that all chil-dren with Down syndrome who par-ticipate in high-risk sports such asgymnastics, swimming, or diving bescreened with lateral radiographs inneutral, flexion, and extension be-fore beginning training or competi-tion. If they demonstrate an abnor-mal odontoid or atlantoaxial inter-val greater than 4.5 mm, despite anormal physical exam, the AAP stat-ed that they should be advised toavoid stressful sports.12

There is usually delay betweenonset of symptoms and diagnosisbecause children with Down syn-drome are retarded and do not vo-calize their complaints.

Motor Development and GaitThe motor development of the

infant with Down syndrome is de-ceptively normal for the first 6months. However, by the age of 1year, the infant is 4 to 5 months be-hind and by the age of 3 years, 10months behind.4 The child is late insitting and standing.

There is a delay in independentwalking between 12 and 18 months,attributable to cerebellar dysfunctionwith slow reaction time, hypotonia,and reduced sensory and propriocep-tion input.20 Children with Down syn-drome, when institutionalized, beginto walk at an average age of 4.2 years,while those living at home begin walk-ing at an average age of 2.6 years.9

Lindsey and Drennan17 describethe gait of patients with Down syn-drome as Chaplinesque. The hips arein external rotation, the knees are inflexion and valgus, and the tibias areexternally rotated. The feet are ad-vanced with the medial longitudinalarch as the presenting aspect of thefoot and there is marked valgus andpronation of the foot.

Children with Down syndromeshow a longer period of stance thanindependent walkers, comparable tothat of the supported walking of in-fants. There is a decrease in hip exten-sion and early hip extension near theend of swing. This is seen as an at-tempt to make a flatfoot contact in-stead of the initial heel contact. Thereis a decrease in ankle sagittal plane ro-tation, and exaggerated abduction ofthe swing limb appears to be neces-

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Down Syndrome...

tation both in flexion and exten-sion, resulting in out-toe gait.13 Five

percent of children with Down syn-drome develop a dislocatable or dislo-cated hip.1 These children are usuallydelayed in walking; their hips are hy-permobile but not dislocatable until attwo to four years of age, the affectedhip spontaneously becomes dislocatedand relocated. Presenting complainsare a click in the hip, an increasinglimp or “giving way,” and refusal towalk. With recurrent dislocation, physi-cal activity diminishes. The disloca-tions are not painful. If untreated,eventually subluxation or dislocationmay become fixed. The recurring dislo-cated hip is usually treated surgically.26

Atlantoaxial InstabilityAtlantoaxial instability is an estab-

lished entity in Down syndrome.6,14,21

It occurs in 10% to 20% of these pa-tients. Atlantoaxial instability inDown syndrome is caused by liga-mentous laxity of the transverse liga-ment that holds the odontoid processclose to the anterior arch of the atlas.This instability results in loose joints,where the cervical vertebrae slip for-ward and the spinal cord is vulnerable

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Figure 7: Pre-fabricated foot orthosisenhances stability of open-toedstraight last shoe

Figure 6: The open-toed straight lastshoe improves foot and ankle stabili-ty for stance

Figure 5: Adolescent patient withjoint laxity, pronounced genu valgumand heel-ankle valgus

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of the immature foot will frequentlydecrease the external rotation of thelimb and result in development of amore appropriate gait pattern.”Rather than being aggressively treat-ed, many of the common congenitaldeformities, such as metatarsus ad-ductus and tibial torsion, are over-looked because of the patients’ manyother medical and orthopedic prob-lems, and no treatment is rendered.

The treatment modalities used inthe correction of congenital foot andtorsional abnormalities in the childwith Down syndrome are the samethat would be used in the normal pa-tient. These include serial immobiliza-tion casting, corrective shoes andsplints, and surgery. Due to the pro-longed excessive ligamentous laxityand the relatively slower foot growth,corrective modalities often are requiredfor longer periods of time in the childwith Down syndrome. Immobilizationmodalities that impede walking, suchas plaster casts or restrictive splinting,should be avoided in the older child,since these can further delay the pro-gression of neuromotor developmentin a child that already will exhibit a sig-nificant delay in learning to walk. Theuse of properly modified correctiveshoes should be encouraged when cor-recting foot pathology in children withDown syndrome who have progressedbeyond the states of sitting indepen-dently and crawling.5

Promote Independent Stanceand Walking

Although the delayed walking inchildren with Down syndrome is at-

tributed to cerebellar dysfunc-tion, much of it is also the re-sult of excessive ligamentouslaxity and hypotonia presentin all of these children. This lig-

sary for foot clearance.19,20

There also appears to be a relation-ship between sitting patterns and gaitpatterns of children with Down syn-drome. Clinical observation suggeststhat children with Down syndromeusually have excessive external rotationand abduction of the hip, demonstrat-ed by their sitting with widespread legs,and this excessive external rotation andabduction is displayed when they learnto sit. The wide-angled gait is caused bymarked hip retroversion, genu valgumof the knee, external tibial torsion, andexcessively pronated feet.18

It has been proven that ambula-tion performance, including bal-ance and jumping, can be signifi-cantly improved in children withDown syndrome with even mini-mal physical therapy sessions, suchas jumping classes. This type oftherapy should be encouraged.28

Considerations in the Correctionof Congenital Deformities

Early detection and treatment ofcongenital pedal deformities is im-portant in a child with Down syn-drome. Since these children are sub-ject to a multiplicity of orthopedicproblems, an aggressive program tomaintain proper skeletal alignmentcan significantly decrease the severityof these problems and allow the indi-vidual to function much more effi-ciently. In a report on managementof foot and knee deformities in thementally retarded, Lindsey and Dren-nan17 asserted that “proper alignment

Down Syndrome... amentous laxity and hypo-tonia cause severe pronationand abduction of the feet, whichgreatly reduces the child’s anterior-posterior stability.

The use of an open-toed straightlast shoe can be beneficial in main-taining foot and ankle stability,which will promote independentstance in the child with Down syn-drome. This shoe offers a rigid, flat,wide sole and a rigid heel counter,and rises to the level of the ankle,giving both foot and ankle support.(Fig. 6) The function of the shoecan be enhanced by incorporatingan orthotic device to further limitpedal pronation. (Fig. 7)

There are many types of orthosesthat can be used for this purpose,each with its own advantages and dis-advantages. Prefabricated orthosesoffer the major advantages of muchlower cost to the patient and lack ofnecessity of taking impression casts ofthe patient’s feet. Although they donot offer the level of pronation con-trol that might be available with cus-tom fabricated orthoses, there aremany devices on the market that willimprove the support offered by a cor-rective shoe alone. A prefabricated or-thotic device may be desirable for theindividual during the early stages ofstanding, when there are only shortperiods of weightbearing.

Custom orthotic devices offer thegreatest degree of versatility in elimi-nating the effects of undesirable prona-tion. The types of foot orthoses thatthe author has found the most usefulin the treatment of the young childwith Down syndrome include leatherlaminates, rigid acrylic orthoses andpolypropylene orthoses of the Univer-sity of California, Berkeley Laboratory

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Figure 8: High top sneaker with four-ply cus-tom molded leather orthosis. Note the deepheel seat and high medial and lateral flanges.

Figure 9: University of California, Berkeley Laboratory type polypropy-lene foot orthoses.

appropriate foot orthoses placed insupportive, high top, and sometimesmodified shoes.

When the use of an orthotic de-vice is contemplated in the man-agement of the severe flatfoot de-formity in Down syndrome, thepractitioner must take plaster im-pression casts of the child’s foot,being sure to accurately capture theheel and to exaggerate the correc-tion. Foam materials used in im-pression taking often will lead to animproperly fitting orthosis.

Reduction of Out-Toe Gait andGenu Valgum

Many early walkers with Down

syndrome exhibit clumsy, Chap-linesque gait patterns because of exces-sive external hip position, external tib-ial torsion, and severe pronation. Thetripping and falling resulting from thiscan be reduced by improving thechild’s anterior-posterior stability. Thiscan be accomplished by having thechild wear high top sneakers, prefer-ably rising above the ankle, with rigid,flat soles. Sneakers with soles that areconcave from medial to lateral are mostdesirable. The stability of the sneakercan be enhanced further by adding aneoprene medial buttress, which effec-tively increases the width of its weight-bearing surface.(Fig. 10) The addition ofan orthotic device will further improvethe child’s walking.

Severe genu valgum associatedwith joint laxity and severe prona-tion is also a common problem inDown syndrome. A goal for treat-ment modalities used for this prob-lem is their ability to maintain thelower extremity in a position per-pendicular to the ground. Methodsof accomplishing this include theuse of high top oxford shoes or,more practically, supportive sneak-ers, which can be modified by split-ting the soling material and addingvarus wedging. This modification,in combination with foot orthoses,can yield a significant improve-ment in lower limb alignment.

Reduction of Effects of PedalStructural Disorders

The pedal structural problems as-sociated with Down syndrome are thesame as those associated with otherpatients who exhibit severe ligamen-tous laxity and excessive pronation.These include an increased incidenceof severe hallux valgus and hammertoe deformities, as well as plantarfasciitis, fatigue, and early onset ofpedal arthritis associated with severeflatfoot. These problems usually beginto manifest themselves in adolescenceand early adulthood.

It is not within the scope of thisarticle to discuss the many treatmentmodalities available for these prob-lems, since they are the same aswould be applied to any other pa-tient. It is important, though, to con-sider that many patients with Downsyndrome are aware of and con-cerned about shoe style, and are in-volved in athletic events. The authorhas observed that many youngwomen with Down syndrome wantto wear stylish shoes. In these cases,the consideration of a fashion-typeorthosis may be appropriate. Manypatients with Down syndrome arealso involved in sports activities.Over 28 percent of the participantsin the Special Olympics have Downsyndrome. These activities must alsobe considered in the overall biome-chanical approach to treatment. ■

References1 Aprin H, Zink WP, Hall JE: Manage-

ment of dislocation of the hip in Downsyndrome. J Pediatr Orthop 5: 428, 1985.

2 Bodmer EJ, Evancho G, Sweeney JM:Dermatoglyphics: a study and its signifi-

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(UCBL) type.Leather laminated orthoses, if

used, should be ordered four-plywith a deep heel cup and high medi-al and lateral flanges. The leather or-thoses offer the advantage of beingmoderately rigid with some lateralcompression and expansion availableto compensate for the fit of eachshoe used and the ability of the or-thosis-shoe combination to controlthe individual’s pronation. Whenused in snug-fitting supportive shoes,the shoes will improve the functionof the orthoses. If used in a moreflexible or loose-fitting shoe, ratherthan being uncomfortable, theleather orthoses will expand laterallyand offer a comfortable fit. Leatherlaminate orthoses also offer the ad-vantage of being easily modified andadjusted. (Fig. 8)

Rigid acrylic devices offer excel-lent pronation control. They allowminimal tolerance in casting, fabri-cation, and shoes. Since they exhib-it no lateral compression, if the de-vice is only slightly wider thanideal, the acrylic orthoses actuallycan detract from the support af-forded by the shoes alone.

The polypropylene orthosis(UCBL) is an excellent device, andmost commonly prescribed for con-trolling pronation in the severe flexi-ble flatfoot present in Down syn-drome. It can be fabricated with adeep heel cup and high medial andlateral flanges. It is very thin andrigid, and yet is somewhat laterallycompressible.(Fig. 9) The major disad-vantage of this device is its high cost.

Supramalleolar ankle-foot or-thoses can also be used. These devicesrequire more elaborate impression-taking techniques and are expensive.The author has found that in manycases they offer little advantage over

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Figure 10: High top sneakers with medial buttress for added stability

Pes planovalgus is themost common

orthopedic problem inDown syndrome.

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cance to podiatry. JAPA 69: 665, 1979.3 Burns GW: The Science of Genetics, 3rd Ed, Macmillan Publish-

ing, New York, 1976.4 Carr J: Mental and motor development in young Mongol chil-

dren. J Ment Defic Res 14: 205, 1970.5 Caselli MA, Cohen-Sobel E, Thompson J, Adler J, Gonzalez L:

Biomechanical management of children and adolescents with Downsyndrome. JAPMA 81:119, 1991.

6 Committee on Sports Medicine: Atlantoaxial instability inDown syndrome. Pediatrics 74: 152,1984.

7 Diamond LS: Management of inherited disorders of the skele-ton. Course Lect 25: 107, 1976.

8 Diamond LS, Lynne D, Sigman B: Orthopedic disorders in pa-tients with Down’s syndrome. Orthop Clin North Am 12: 57, 1981.

9 Donoghue EC, Kirman BH, Bullmore GHL, et al: Some factorsaffecting age of walking in a mentally retarded population. Dev MedChild Neurol 12: 781, 1970.

10 Dugdale TW, Renshaw TS: Instability of the patellofemoraljoint in Down syndrome. J Bone Joint Surg 68A: 405, 1986.

11 Erlick NE, Engel ED, Davis RH: Dermatoglyphics: a diagnostic tool forchromosomal abnormalities in podiatric medicine. JAPA 71: 409, 1981.

12 Gersoff WK, Federico DJ: Head and neck injuries. In Reider B(ed) Sports Medicine, The School-Age Athlete, 2nd Ed, WB SaundersCompany, Philadelphia, 1996.

13 Goldberg MJ, Ampola MG: Birth defect syndromes in which ortho-pedic problems may be overlooked. Orthop Clin North Am 7: 385, 1976.

14 Hreidarsson S, Magran G, Singer H: Symptomatic atlantoaxialdislocation in Down syndrome. Pediatrics 69: 568,1982.

15 Jaswal S, Jaswal IJS: Malformation of the head in Down syn-drome. Indian Pediatr 25: 373,1988.

16 Kirman BH: General aspects of Down’s syndrome. Physiothera-py 62: 6, 1976.

17 Lindsey RW, Drennan JC: Management of foot and knee defor-mities in the mentally retarded. Orthop Clin North Am 12: 107,1981.

18 Lydic JS, Steel C: Assessment of the quality of sitting and gait pat-terns in children with Down’s syndrome. Phys Ter 59: 1489, 1979.

19 Parker AW, Bronks R: Gait of children with Down syndrome.Arch Phys Med Rehabil 61: 343, 1980.

20 Parker AW, Bronks R, Snyder CW: Walking patterns inDown’s syndrome. J Ment Defic Res 30: 317, 1986.

21 Pueschel SM, Scola FH: Atlantoaxial instability in individu-als with Down syndrome: epidemiologic radiographic, and clini-cal studies. Pediatrics 80: 555, 1987.

22 Rao GP: Late and delayed problems of Down syndrome pa-tients. Indian J Pediatr 55: 353, 1988.

23 Roberts JAF: An Introduction to Medical Genetics, 6th Ed,Oxford University Press, New York, 1973.

24 Smith GF, Warren ST: The biology of Down syndrome.Ann NY Acad Sci 450: 1, 1985.

25 Stratford B, Steele J: Incidence and prevalence of Down’s syndrome:a discussion and report. J Ment Defic Res 29: 95, 1985.

26 Tachdjian MO: Pediatric Orthopedics, 2nd Ed, WB Saunders Com-pany, Philadelphia, 1990.

27 Thompson JS, Thompson MW: Genetics in Medicine, WB SaundersCompany, Philadelphia, 1966.

28 Wang WY, JuYH: Promoting bal-ance and jumpingskills in childrenwith Down syn-drome. Percept MotSkills 94: 443, 2002.

29 WinchesterAM: Human Genetics,2nd Ed, Charles E.Merrill, Columbus,OH, 1975.

Down Syndrome...

1) Down syndrome is the most common con-genital anomaly. Which of the following is theoverall birth incidence of Down syndrome?

A) One in every 50 birthsB) One in every 200 birthsC) One in every 700 birthsD) One in every 3000 births

2) Most often, Down syndrome is caused by:A) Trisomy 18B) Trisomy 21C) Translocation 14/21D) Young maternal age

3) The life-span of children with Down syndromehas dramatically increased primarily due to:

A) Advances in cardiac surgeryB) Early recognition of orthopedic deformitiesC) Decline in institutionalizationD) Early identification of affected infants

4) Which one of the following is not a commonfinding in Down syndrome?

A) Ligamentous laxityB) Spastic diplegiaC) HyperflexibilityD) Hypotonia

5) The most common foot problem found inDown syndrome is:

A) Hallux abducto valgusB) HammertoesC) Pes cavovarusD) Pes planovarus

6) Patellofemoral instability is common in Downsyndrome. Which one of the following is a com-mon finding in this condition?

A) Less common in institutionalized patientsB) Often very painfulC) Results in frequent fallingD) Surgery rarely required

7) A child with Down syndrome demonstratingan increasing limp should be evaluated for:

A) Flexible flatfoot

E X A M I N A T I O N

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Dr. Caselli is StaffPodiatrist at theVA Hudson ValleyHealth Care Sys-tem and AdjunctProfessor, Depart-ment of Orthope-dic Sciences, NewYork College ofPodiatric Medi-cine.

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proper fit of appliancesC) Delayed ambulationD) Severity of deformities

13) Which one of the followingcauses for delayed walking inchildren with Down syndromecannot be helped with orthope-dic shoe gear and foot orthoses?

A) HypotoniaB) Ligamentous laxityC) Acetabular dysplasiaD) Severe pronation

14) Which one of the followingtypes of orthopedic shoe gear isrecommended to promote inde-pendent stance in the child withDown syndrome?

A) Sable clubfoot shoeB) Custom molded shoeC) High top bootD) Open-toed straight lastshoe

15) Which one of the followingis not an advantage of a prefab-ricated foot orthosis?

A) Low costB) Better fitC) No need for impressioncastingD) More readily obtained

16) The advantage of usingleather laminate orthoses overthose made from a more rigidmaterial is that leather:

A) Lasts longerB) Offers more shock absorp-tionC) Compensates for fit inshoesD) More durable

17) The most commonly recom-mended foot orthoses for thetreatment of the severe flexible

B) Genu valgumC) ScoliosisD) Dislocated hip

8) A child with Down syndromedemonstrating progressive clum-siness, spasticity, toe extensor re-flex and uncoordination shouldbe evaluated for:

A) Atlantoaxial instabilityB) Severe pedal pronationC) Peroneal spasmD) Foot fracture

9) Motor developmental delay inDown syndrome occurs:

A) At birthB) After 6 months of ageC) After 3 years of ageD) In early adolescence

10) The common gait pattern inDown syndrome is that of:

A) Intermittent toe-walkingB) Mild in-toeC) Severe out-toeD) Scissor gait pattern

11) Early treatment of foot de-formities is often not initiated inchildren with Down syndromesince:

A) Children are mostly insti-tutionalizedB) There are many othermedical and orthopedicproblemsC) The foot is not deemedimportantD) The deformities are mild

12) Immobilization modalitiesare required to be used forlonger periods of time in Downsyndrome due to:

A) Ligamentous laxity andslow growthB) Difficulty in obtaining

flatfoot seen in Down syndromeis the:

A) University of California,Berkeley Laboratory (UCBL)typeB) Leather laminate typeC) Acrylic typeD) Pre-fabricated type

18) The best method of takingan impression of a child’s footfor the fabrication of orthoses is:

A) Paper tracingB) Plaster castC) Foam impressionD) In shoe impression

19) Adding all but which one ofthe following can enhancesneaker support and stability?

A) Medial buttressB) Varus sole wedgingC) Outer sole wedgeD) Foot orthosis

20) When treating foot patholo-gy in a patient with Down syn-drome:

A) The multiple orthopedicand medical problems mustbe consideredB) Because of the severemental retardation, feedbackfrom the patient is of littlevalueC) Orthopedic interventionhas little effect on ambulato-ry outcomeD) Down syndrome is toorare to be of medical signifi-cance

E X A M I N A T I O N(cont’d)

SEE INSTRUCTIONSAND ANSWER SHEETON PAGES 170-172