Orthopedic Problems in the Newborn

General Principles in Assessing Musculoskeletal System

1. posture, position, gross abnormalities
2. discomfort from bone/joint movement
3. ROM
4. muscle size, symmetry, strength
5. configuration and motility of back

Prenatal History

1. Intrauterine environment can alter fetal growth, movement or position
2. Oligohydramnios, breech, abnormal growth patterns, exposure to teratogens can affect maturation of musculoskeletal system
3. Birth trauma or neurological insult: duration of labor, signs of fetal distress, vaginal vs C/S... can affect conditions ie CP, brachial palsy, torticollis
4. Higher incidence of congenital hip dysplasia in 1stborn children

General inspection

1. any tremors present after 4 days of life are abnormal
2. extremities: unequal length of circumference is associated with intrabdominal neoplasms
3. breech baby: will lie w/ flexed, abducted hips, with extended knees
4. vaginal baby: lower extremities externally rotated, bowed, everted feet.

Physical exam

1. failure to move an extremity may indicate spinal cord injury or brachial plexus palsy
2. after the clavicle, the humerus is the bone most often fractured during birth
3. elbow: the normal newborn has a mild flexion contracture that doesn't disappear until a few weeks p birth
4. simian crease may be nl. however the following findings together suggest down syndrome: short fingers, incurved pinky, low set thumb, simian crease.
5. macrodactyly (large finger or toe) may be nl or sign of neurofibromatosis
6. overlapping of 2nd over 3rd finger may suggest trisomy 18
7. note position of scapule while in prone position. a winged/elevated scapule = Sprengel deformity.
8. scoliosis in nbn in rare, but if occurs, usually assoc w/ a structural anomaly of vertebral column. Usually female, may be familial. lifting baby by armpits makes scoliosis more obvious
9. hips of newborn generally have a flexion contracture, normally 25-30 degrees between thigh and bed
10. r/o congenital hip dysplasia
    • asymmetry of skin folds in gluteal/femoral regions suggest dislocation (place infant on back and pull up legs (hips flexed, legs extended up)
    • Ortolani: attempts to reduce a dislocated femoral head into the acetabulum. a palpable clunk is a positive result. higher pitched clicks and snaps can be heard/felt, usually due to tendones, ligaments, fluid in hip joint.
    • Barlow: attempts to dislocated the femoral head. A 'clunk' indicates that the femoral head has slipped over the lateral edge of the acetabulum and demonstrates an unstable joint that is dislocatable
11. femoral length: galeazzi or alli's sign: femurs aligned, feet flat on bed, big toes aligned, infant's knees flexed, observe height of knees.
12. Tibial torsion
13. Tibia: lateral bowing is nl in newborn. anterior bowing is abnl, consult ortho.
14. Flatfoot
15. ankle and forefoot adduction is a positional deformity from utero, ddx from congenital equinovarus (clubfoot). after placing the foot in the midline and dorsiflexing, an clubfoot will not resist dorsiflexion and not have full rom.

Musculoskeletal anomalies

Anomalies of Neck

• Klippel Feil Syndrome
  • a malformation sequence: fusion of cervical vertebrae; shorter appearing neck and ltd range of motion in all directions; low hairline (Although features of both  Noonan Syndrome and Turner syndrome can include a low hairline and short neck, neither is associated with fused vertebrae or facial asymmetry.)
  • a sporadic occurrence, although rare families have been described in which there was an autosomal dominant form with variable expression; more commonly in females
  • may be accompanied by hemivertebrae, may cause webbed neck, torticollis, and facial asymmetry
  • may occur as part of a more serious abnormality in neural tube development, such as cervical  myelomeningocele -> associated neurologic deficits also will be evident, including paraplegia or hemiplegia and cranial or cerebral nerve palsies.
  • may be assoc w other malformations ie Sprengel's, rib deformities, scoliosis, cleft palate,  CNS defects/MR/posterior fossa dermoid cysts,  cardiac/pulmonary/renal anomalies;  deafness in up to 30% of affected individuals,,
  • Therefore, affected infants must be evaluated carefully for these associated defects via audiologic evaluation, echocardiography, and renal ultrasonography.
  • lateral flexion-extension radiographs must be obtained to identify patients who have hypermobility of the upper cervical segment and are at risk for developing neurologic impairment; may be an incidental finding on XR if mild
  • If hypermobility is present, the child should be evaluated annually and avoid contact sports.
  • tx: ROM exercises or bracing may be done to improve mobility or correct deformity. Surgery (except for cosmesis) rarely indicated
• Torticollis

Spinal Deformities

1. Congenital scoliosis (Further discussion of scoliosis)
   • undetectable to very severe; curvature of spine in lateral plane
   • lateral curve is associated with vertebral rotation (thoracic spine), chest wall deformity (rib hump), or flank prominence (lumbar spine). May also see shoulder assymetry, unilateral scapular prominence, waist assymetry, small chest, paralumbar bumps.
   • In infants, hold baby prone on examiners hand.
   • Adam's forward-bending test can be done in kids starting from age 6, until the end of puberty.
   • affected infants rapidly develope plagiocephaly with flattening of the head on the concave side of the curve and corresponding prominence on the opposite side of the head.
   • 5% of all scoliosis cases are congenital. 85% idiopathic, 5% neuromuscular (CP, polio, spinal muscular atrophy, muscular dystrophy, myelomeningocele, spinocerebellar degeneration, spinal anomalies: hemivertebrae), 5% misc (marfan, ehlers-danlos, neurofibromatosis, etc)
   • embryonic defect, not chromosomal or inherited; failure of vertebral formation, segmentation, or both
   • neurologic function can be affected
   • measure by cobb method
   • must eval for GU tract anomalies esp unilateral renal agenesis (increased incidence 20-30%, with congenital vertebral anomalies)
   • may also see Klippel-Feil syndrome, Sprengel deformity of scapula, neuromuscular conditions (above)
   • Tx in adolescents:
     • Curve < 25 degrees: follow, refer to ortho
     • Spinal bracing and exercise program: Idiopathic curves of 25-40 degrees or lesser curves showing rapid progression:
     • Surgery if curvature > 40 degrees or rapid progression despite bracing.
     • untreated curvatures > 60 degrees inevitably suffer sig secondary cardiopulmonary problems (decreased vital capacity, shunting, decreased o2 sat, cor pulmonale)
   • Functional scoliosis: the curve disappears when child is seated. May be due to poor posture, limb-length inequality, flexion contracture of hip or knee. May also be seen w/ paraspinous muscle spasm after back injury, splinting because of pain (pyelo, appy, pna), or herniated disk and secondary root pain.
2. Myelomeningocele
   • congenital neural tube defect
   • failure of closure of caudal end
   • skin disruption not always present; examine closely any soft mass noted over spine or just off midline; examine muscle function of lower extremities

Upper Extrem anomalies

1. Sprengel deformity (congenital elevation of scapula)
   • one of the more common deformities of shoulder girdle; familial predisposition;
   • elevated scapula: usually unilateral. B/L in 1/3 of cases.
   • scapula is small/hypoplastic, and malrotated; vertebral border is more superior and horizontal than normal
   • confirm with radiography
   • may be attached to c-spine by a band of fibrous tissue or bone (omovertebral bone)
   • due to failure of normal scapular descent during fetal life
   • affected side of neck appears shorter, broader, may look like torticollis
   • limitation of shoulder abduction and flexion
   • internal and external rotation only slightly affected
   • freq assoc with congenital spinal abnormalities ie scoliosis, torticollis, rib deformations, renal anomalies, Klippel-Feil syndrome
   • Tx: stretching and ROM exercises rarely successful. Surgery usually for cosmetic/functional reasons, usually: excision of the prominent superior aspect of clavicle OR release and reduction of scapule by placing in inferiorly on the chest wall (brachial plexus injury may occur with the latter)
2. Cleido-cranial dysostosis
   • complete or partial absence of clavicles
   • autosomal dominant
   • excessive scapulothoracic motion
   • complete absence accompanied by defective ossification of cranium, large fontanels, delayed closure of sutures
   • little functional disability
   • no treatment required
3. Brachial Palsy
   • prolonged, difficult labor ending in traumatic delivery
   • most often in large babies prone to stretching injuries
   • paralysis of upper arm more common than lower arm or whole arm
   • upper arm paralysis: arm adducted and internally rotated, elbow extended, forearm pronated, wrist flexed (waiter's tip); moro absent on this side; grasp present
   • if nerve roots not affected, infants regain neurologic function within several days, as hemorrhage and edema in area resolve
   • at 3 m/o, close exam reveals tightness of shoulder on internal rotation, difficulty supinating forearm and abducting shoulder
4. Congenital absence of radius
   • hand and wrist are deviated 90 degrees or more
   • b/l in 50%
   • shortened forearm w/ bowing of ulna
   • absent or hypoplastic thumb
   • limited elbow movement
   • pediatric hand surgeon referral
   • 4 entities assoc with absent or hypoplastic radius
     • congenital thrombocytopenia-absent radius (TAR) syndrome
     • VACTERL (formerly VATER): vertebral, anus (imperforate), cardiac origin, TE fistula, radii (absent), renal origin, limbs.
     • Fanconi anemia
     • Holt-Oran Syndrome (assoc w/ secumdum atrioseptal defect)

Lower Extrem anomalies

1. Developmental dysplasia of the hip DDH
2. Congenital absence of tibia or fibula
   • long bone absence is rare; but the most common absent one is the fibula
   • no tibia? shortened leg, mild to marked. Unstable knee w/ flexion contracture. Nl or varus foot. 20% b/l
   • no fibula? shortened leg, medially-anteriorly bowed tibia, severe valgus foot.
   • ped ortho referral early
3. Genu recurvatum
   • rare. congenital disloc or hyper-extension of knee
   • due to frank breech in utero of prenatal dev defect
   • may be assoc w/ oligo, congen absence of quadriceps muscle, or syndrome ie sacral agenesis
   • usually b/l, usually females
   • early tx by peds ortho prevents further deformity or interference w nl function
4. Metatarsus adductus
5. Clubfoot (talipes equinovarus)
6. Causes of intoeing
7. Pes planus
8. Tibial Torsion, internal/external

Conditions affecting upper and/or lower extrem

1. Congenital Constricting Bands (Streeter Dysplasia)
   • varies: shallow indentations of soft tissue to severe, amputating constrictions
   • due to amniotic bands
   • severe bands are emergent, esp if vascular/lymphatic obstruction - surgery consult
   • assoc with polydactyly
2. Syndactyly
   • one of the most common congenital anomalies; familial
   • failure of digits of hand or feet to separate; more common and disabling in upper extrem.
   • usually B/L.
   • the more severe the ~, the greater likelihood of bony abnormalities too
   • of toes: no tx necessary, cosmetic only
   • if multiple fingers affected: function may deterioriate as fingers grow. Early correction
   • May be assoc w/ other congenital anomalies, esp Apert's syndrome, and Streeter's dysplasia.
3. Polydactyly
   • common, familial
   • more often in AA vs caucasians
   • most common type: floppy digit or skin tag on radial or ulnar side of hand
   • may involve duplication of a nl digit: functional 6 fingers
   • consult to determine therapeutic decisions as well as functional considerations
   • management:
     • usually w/ extra digit on little finger, tx in office immediately following birth or anytime thereafter using fine suture ligation e.g 4-0 black silk at base
     • Most other extra digits, incl thumb/toes, are complex. Need ped ortho or plastics; surgery is done at 9-12 mos of age (early enough to have minimal impact on infant, late enough for the tissue planes to be better developed). from Consultant for Pediatricians Jan 2009.