Ortho Teflon - Peds

Front 1

accessory tarsal navicular

Back 1

is located at the medial tuberosity of the navicular bone. Nearly all children and adolescents who have a symptomatic accessory tarsal navicular bone become asymptomatic when they reach skeletal maturity. Initial management should include activity restrictions, shoe modification to avoid pressure over the prominent navicular, and non-narcotic analgesics. Although anecdotal, the use of arch supports may be helpful. When pain is refractory to these methods, a short period of cast immobilization may be useful. Surgery should be reserved for patients who have disabling symptoms despite a prolonged period of nonsurgical management. When surgery is indicated, simple excision of the accessory navicular is recommended.

Front 2

acetabular protrusio

Back 2

a condition that is frequently associated with Marfan syndrome. An echocardiogram is necessary to rule out the most serious consequence of this syndrome, aortic root widening, which can lead to aortic valve dysfunction or fatal aortic rupture. An electromyogram may be indicated for Charcot-Marie-Tooth disease, which is associated with acetabular dysplasia, but not protrusio. The renal ultrasound, the MRI scan, and the biopsy would be of no value in this patient. Protrusio can also be seen in patients with osteogenesis imperfecta and juvenile rheumatoid arthritis

Front 3

achondroplasia + kyphosis.

Back 3

The AP radiograph shows decreased interpedicular distance at the lower lumbar vertebrae, a feature considered to be a distinctive sign of achondroplasia. Most patients with achondroplasia have kyphosis, and this usually resolves spontaneously. When the fixed component is greater than 30°, however, brace treatment is recommended. Spinal fusion is seldom required.

Front 4

acute infection of the SI joint

Back 4

Bed rest and nonsteroidal anti-inflammatory drugs alone are insufficient to treat the problem. Staphylococcus aureus is the causative organism in most of these infections; therefore, unless there is an unusual factor in the history such as IV drug use, immune system compromise, or unusual travel, SI joint aspiration is unnecessary. It is often difficult to enter the SI joint, even under radiographic guidance. Management should consist of hospital admission and IV antibiotics. Blood cultures may be positive and should be obtained prior to starting antibiotics.

Front 5

arthrogryposis - hip dislocation

Back 5

In this age group of patients with arthrogryposis, open reduction through a medial approach is generally recommended. Open reduction through an anterior approach is reserved for patients in which a medial approach has failed or for older patients who require simultaneous femoral shortening and/or pelvic osteotomy. Closed treatment of unilateral hip dislocation in association with arthrogryposis is rarely successful. In bilateral hip dislocation associated with arthrogrypsis, the consensus is that the hips are best left unreduced because of the difficulty in obtaining excellent clinical and radiographic results bilaterally.

Front 6

Avulsion fracture of tibial spine

Back 6

Results from hyperextension/rapid deceleration in skeletally immature individuals. Equivalent to an adult ACL. Types 1 – min displaced, 2 – post hinge intact, 3 - completely displaced. Surgery for Type 3, and 2 that doesn’t reduce with knee extension.

Front 7

Becker muscular dystrophy

Back 7

Becker muscular dystrophy have an abnormality in dystrophin, but unlike patients with Duchenne muscular dystrophy, some dystrophin is present. As a result, the progression of muscle weakness is slower, with the diagnosis typically made after age 8 years. Similar to patients with Duchenne muscular dystrophy, patients with Becker muscular dystrophy have pseudohypertrophy of the calves, markedly increased creatine kinase levels, and X-linked transmission of the condition. In addition, these patients are more prone to cardiomyopathy; a condition that should be carefully evaluated if any surgery is required. Patients with spinal muscular atrophy also have proximal muscle weakness, but the onset of weakness occurs earlier in childhood. These patients also have absent deep tendon reflexes and fasciculations, but pseudohypertrophy is absent and creatine kinase levels are normal. Patients with Emery-Dreifuss dystrophy may have a similar clinical picture to Becker’s muscular dystrophy, but pseudohypertrophy is absent and creatine kinase levels are only mildly elevated. In addition, neck extension, elbow flexion, and ankle equinus contractures develop at an early age. Limb girdle dystrophy is a group of progressive muscular dystrophies that is not associated with pseudohypertrophy or a significant elevation of creatine kinase levels. Guillain-Barre syndrome is a condition associated with results from postinfectious demyelination of the peripheral nerve. These patients have the acute onset of weakness, hypotonia, and areflexia; creatine kinase levels are normal.

Front 8

Becker muscular dystrophy

Back 8

Patients with this condition have a slower rate of progression of disease compared with patients who have Duchenne muscular dystrophy, and walking may continue into the late teens. The creatine kinase level is not as high as in Duchenne muscular dystrophy, which can range from 20,000 to 30,000 IU/L. Becker muscular dystrophy is allelic to Duchenne muscular dystrophy, resulting in a mutation in the dystrophin gene. Myotonic dystrophy is characterized by a progressive inability to relax the muscles after contracture. The Gower sign is not helpful in this disease. Patients with Charcot-Marie-Tooth disease, one type of which is also known as HMSN type II, do not have elevated creatine kinase levels and usually present with a foot deformity. Spinal muscular atrophy, type II, usually presents with severe weakness in the second year of life.

Front 9

Beckwith-Wiedemann syndrome

Back 9

which consists of exophthalmos, macroglossia, gigantism, visceromegaly, abdominal wall defects, and neonatal hypoglycemia. Hemihypertrophy develops in approximately 15% of patients with BWS. Patients with hemihypertrophy that is the result of BWS have a 40% chance of developing malignancies such as Wilms’ tumor or hepatoblastoma; therefore, frequent ultrasound screening is recommended until about age 7 years. The absence of nevi and vascular markings helps to rule out other causes of hemihypertrophy, such as neurofibromatosis, Proteus syndrome, and Klippel-Trenaunay syndrome. Bone age estimations are not accurate at this young age but may become more useful later to help predict the timing of epiphysiodesis procedures

Front 10

bilateral radial clubhands

Back 10

Patients born with bilateral radial clubhands may have difficulty getting their hands to their mouth. The centralization procedure would take away that ability if there is a lack of elbow flexion

Front 11

bone infection

Back 11

The most common bacterial etiologic agent following varicella is group A beta-hemolytic streptococcus. The other organisms are much less common. Staphylococcus aureus is the most common bone infection organism. Staphylococcus epidermidis is increasingly a bone infection organism. Group B streptococcus occurs more commonly in newborns. Kingella kingae is a common joint pathogen but is not as common following varicella

Front 12

Breech positioning

Back 12

Breech positioning has been noted as the risk factor that most increases the relative risk of developmental dysplasia of the hip in multiple series and meta-analysis. All the other factors also increase the risk but to a lesser magnitude

Front 13

cerebral palsy

Back 13

cerebral palsy typically ambulate with a crouched gait characterized by excessive flexion of the hips and knees during stance. Many patients exhibit co-contracture of the quadriceps and hamstrings, causing a stiff-knee gait. Normally, the rectus femoris fires at the initiation of swing and in terminal swing through initial contact. Prolonged activity of the rectus femoris throughout the swing phase interferes with normal knee flexion. This contributes to a stiff knee during swing phase and prevents clearance of the foot. Lengthening of the hamstrings alone will not improve foot clearance. Hamstring lengthening is contraindicated when there is hyperextension during stance. Transfer of the rectus femoris to one of the knee flexors has been shown to improve knee flexion during swing by an average of 15°. This allows improved foot clearance

Front 14

Chronic dislocation of the patella

Back 14

In asymptomatic patients who are able to extend their knees, continued observation is the management of choice.

Front 15

Chronic granulomatous disease

Back 15

(CGD) is the most common congenital disorder of neutrophils, occurring in 1 per 200,000 to 250,000 live births. Osteomyelitis is relatively common in CGD and has some unique features in this disorder.

Front 16

Cleft hand and cleft foot malformations

Back 16

Cleft hand and cleft foot malformations are commonly inherited as autosomal-dominant traits and are associated with a number of syndromes. An autosomal-recessive and an x-linked inheritance pattern have also been described, but these are much less common and are usually atypical. In the common autosomal-dominant condition, nearly one third of the known carriers of the gene show no hand or foot abnormalities. This is known as reduced penetrance. The disorder may be variably expressed; affected family members often exhibit a range from mild abnormalities in one limb only to severe anomalies in four limbs. Variable expressivity and reduced penetrance can cause difficulty in counseling families regarding future offspring in an affected family. Many patients have a cleft hand that may be caused by the split-hand, split-foot gene (SHFM1) localized on chromosome 7q21.

Front 17

Congenital dislocation of the knee

Back 17

Congenital dislocation of the knee is an uncommon deformity that varies in presentation from simple hyperextension to complete anterior dislocation of the tibia on the femur. Treatment varies with the age at presentation and the severity of the deformity. Most authors recommend early nonsurgical management. A recent study of 24 congenital knee dislocations in 17 patients found that satisfactory results were obtained in most instances using closed treatment. Based on their findings, the authors concluded that immediate reduction or serial pcasting should be performed when the patient is seen early after birth. If the patient is seen late and correction cannot be achieved by serial casting, traction followed by closed or open reduction may be necessary. Early percutaneous quadriceps recession has been described for complex congenital knee dislocations associated with underlying disorders, such as arthrogryposis and Ehlers-Danlos syndrome.

Front 18

Congenital pseudarthrosis

Back 18

of the clavicle is a rare disorder of formation of the clavicle that is recognizable at birth. The right clavicle is most commonly affected, although bilateral involvement has been reported in approximately 10% of cases. clavicle typically displays a definite separation in its midportion, with the medial aspect lying superior to the lateral aspect, owing to muscle forces and the weight of the upper extremity

Front 19

congenital spine abnormality

Back 19

Approximately 60% of patients with a congenital spine abnormality have associated malformations outside the spinal column. Genitourinary abnormalities are probably the most common, occurring in up to 37% of patients. These are usually anatomic anomalies, such as renal agenesis, duplication, fusion, and ectopia. A genitourinary ultrasound is the least invasive screening tool. Other associated anomalies include cervical vertebral abnormalities, VATER syndrome, and intraspinal abnormalities such as diastematomyelia. An MRI scan is not recommended as part of the screening examination; however, if the patient had neurologic signs or symptoms, an MRI scan would be indicated

Front 20

congenital vertebral abnormality - Anterior failure of formation

Back 20

Anterior failure of formation results in a progressive kyphosis that may lead to cord compression and progressive neurologic deficit. Anterior failure of segmentation can also produce progressive kyphosis but usually is not severe enough to cause cord compression. Posterior failure of formation is seen in conditions such as myelomeningocele in which the neurologic deficit is generally stable. Lateral abnormalities and posterior failure of segmentation are rarely associated with progressive neurologic deficit

Front 21

congenital vertical talus

Back 21

A lateral radiograph of the foot in maximum plantar flexion is needed to demonstrate the fixed position of the deformity with malalignment of the talar-metatarsal axis. A fixed dislocation of the navicular on the talus differentiates a congenital vertical talus from the oblique talus with talonavicular subluxation

Front 22

curly toe deformity

Back 22

curly toe deformity of toe, and tenotomy of the toe flexors is highly effective for this problem

Front 23

developmental dislocation of the hip

Back 23

The natural history of complete developmental dislocation of the hip is dependent on two factors: bilaterality and the presence or absence of a false acetabulum. Patients with bilateral dislocations may have low back pain because of hyperlordosis, but they tend to have less disability than patients with unilateral dislocations who have secondary problems related to limb-length inequality. Degenerative joint disease and clinical disability are most likely to develop in patients with completely dislocated hips and well-developed false acetabula. In a 12-year-old child who has bilateral developmental hip dislocations, it would be difficult to obtain surgical treatment results that are better than the natural history of the disorder. Abductor strengthening exercises are unlikely to influence the long-term outcome in this disorder. Surface replacements are not indicated in young asymptomatic patients.

Front 24

Down syndrome

Back 24

Ligamentous laxity and muscle hypotonia seen in Down syndrome contribute to the incidence of hip subluxation and dislocation. These factors can be progressive and lead to degenerative arthritis in adults with Down syndrome.

Front 25

Down syndrome + atlanto-dens interval

Back 25

An atlanto-dens interval (ADI) of up to 4 mm in children is considered normal. Children with Down syndrome have increased ligamentous laxity, with atlantoaxial instability occurring in as many as 15% to 20% of patients. These patients are at risk for catastrophic injury following minor trauma and should be routinely screened for instability, generally beginning when the patient starts to walk. Patients with an ADI of greater than 5 mm should avoid contact sports and high-risk activities such as gymnastics, diving, the high jump, and the butterfly stroke. The American Academy of Pediatrics Committee of Sports Medicine and Fitness guidelines recommend that lateral views of the cervical spine in neutral, flexion, and extension should be obtained in all children with Down syndrome who wish to participate in sports. Patients with normal radiographs and examinations do not need repeat radiographs, although some authors suggest that instability increases with age, and therefore recommend repeat radiographs every 5 years. Cervical spine fusion in patients with Down syndrome has a high rate of complications and should be performed only on patients with symptoms and evidence of myelopathy

Front 26

Duchenne muscular dystrophy

Back 26

The presence of any curve greater than 20 degrees in a nonambulatory patient is an indication for posterior fusion with instrumentation. Because of progressive cardiomyopathy and pulmonary deficiency, waiting until the curve is larger can increase the risk of pulmonary or cardiac complications during or following surgery

Front 27

Duchenne muscular dystrophy + scoliosis

Back 27

Progressive scoliosis develops in most patients with Duchenne muscular dystrophy. The onset of spinal deformity typically follows the cessation of walking, and curves can be expected to progress about 10° per year. Posterior spinal fusion with instrumentation should be performed as soon as a curve of 25° or greater is documented and before deterioration of pulmonary function (a FVC of less than 30%) precludes surgery. Patients with kyphotic posture tend to progress more rapidly than those with lordotic posture. Brace treatment is contraindicated because it is not definitive and it may mask curve progression while pulmonary function is concomitantly worsening.

Front 28

Dysplasia Epiphysealis Hemimelica

Back 28

rare disorder of localized osteochondral overgrowth, involving single or multiple epiphyses or ossification centers. lesion most commonly affects the medial aspect of the epiphysis, lateral involvement, as well as involvement of entire epiphyses, has been reported. atients typically complain of painless swelling or mass on one side of a joint, limitation of motion, and occasionally locking, angular deformity, limp, regional muscle wasting, or limb-length discrepancy.. radiologic diagnosis is fairly straightforward when the lesion is fully calcified or ossified, appearing as an irregular, often multicentric, lobulated mass protruding directly from one side of the affected epiphysis or tarsal bone. Tx = sx

Front 29

epiphysiodesis for limb-length discrepancy

Back 29

Errors in timing are by far the most common in this technically safe procedure. Incomplete growth arrest has been reported in up to 15% of patients versus timing errors in 61%. Fracture through the site has been reported rarely. Neurovascular and cartilaginous injury are extremely uncommon

Front 30

Erb type of obstetrical brachial plexus palsy

Back 30

“head waiter’s” posture with the shoulder internally rotated, the elbow extended, and the wrist in flexion. The Erb type of obstetrical brachial plexus palsy involves the C5 and C6 nerve root, and occasionally, as in this child, the C7 nerve root. Obstetrical palsy is a traction injury, and is associated with a high birth weight, shoulder dystocia, cephalopelvic disproportion, or the use of forceps. Erb palsy is four times more common than injury to the entire plexus or injury to the C8 and T1 nerve roots. It results from the shoulder being depressed while the head and neck are laterally rotated, extended, and tilted in the opposite direction. Most patients recover wrist extension and elbow flexion. Patients with residual weakness of shoulder external rotation and abduction will benefit from release of the pectoralis major, latissimus dorsi, and teres major, with transfer of the latissimus dorsi and the teres major to the posterosuperior aspect of the rotator cuff. Recent studies using arthrograms and CT scans have shown a higher incidence of posterior glenoid deficiency and posterior subluxation than that observed with plain radiographs. The posterior subluxation or dislocation can be effectively reduced by tendon release and transfer procedures.

Front 31

Erb’s palsy

Back 31

classic Erb’s palsy with weakness of the muscles innervated by the fifth and sixth cervical roots. Horner syndrome, a poor prognostic indicator for recovery, is absent in this infant. All infants with brachial plexus birth palsies initially should Pbe monitored for spontaneous recovery during the first 3 to 6 months of life. During this period of observation, glenohumeral motion, especially external rotation, should be maintained. Many infants will begin to show recovery within the first 6 to 8 weeks after birth and continue on to normal function. The timing of microsurgery is controversial. A recent study found that the outcome of microsurgical repair in patients who had no recovery of biceps function within 3 months after birth was similar compared to those who had recovery of biceps function between 3 and 6 months and no microsurgical repair. The author concluded that microsurgical repair was effective in improving function in those infants who had no evidence of recovery of biceps function within the first 6 months of life.

Front 32

Focal scleroderma

Back 32

is characterized by the formation of patches of sclerotic skin, also known as morphea, or streaks of sclerosis (linear scleroderma). Systemic involvement in focal scleroderma is unusual; however, progression during childhood is common. Contracture of underlying tissues is common, often resulting in serious joint contractures. Bony changes similar to those seen in melorheostosis can be seen. This patient has characteristic skin changes, atrophy of the soft tissues, Achilles tendon contractures, and calcaneal deformities. There are no signs of arthrogryposis, which usually presents with bilateral congenital deformities, including equinovarus. Klippel-Trenaunay-Weber syndrome is characterized by venous malformation in association with focal overgrowth.

Front 33

Gaucher disease

Back 33

was caused by a deficiency of glucocerebrosidase, the lysosomal enzyme that hydrolyzes glucocerebroside, an important component of cell wall membranes. In patients with Gaucher disease, the normal necrosis of cells, especially leukocytes, causes a gradual accumulation of glucocerebroside in macrophages.

Front 34

genu varum

Back 34

The differential diagnosis of genu varum includes physiologic genu varum, Blount's disease, skeletal dysplasia, and metabolic bone disease. Children with Blount's disease are generally in the 95th percentile for height and weight, and usually multiple family members are not affected. The radiographs show widening of the physis and metaphyseal flaring. In Blount's disease, the characteristic radiographic changes involve only the tibia, and at this age, most commonly show beaking of the medial metaphysis. Skeletal dysplasias, such as chondrometaphyseal dysplasia, are associated with short stature, and the radiographic changes are similar to those seen here. However, laboratory studies in these children will be within normal limits. Children with chronic renal disease will often be of short stature, and the radiographic findings are again similar to those shown here. However, BUN and creatinine levels are elevated and phosphate levels are elevated rather than decreased in children with renal disease. The absence of dietary deficiencies and positive family history rules out vitamin D-deficient rickets. There are four types of vitamin D-resistant rickets: failure of production of 1,25-dihydroxy vitamin D, phosphate diabetes (hypophosphatemic rickets), end organ insensitivity to vitamin D, and renal tubular acidosis. All types of vitamin D-resistant rickets are resistant to treatment with physiologic doses of vitamin D. The patient’s clinical picture, family history, laboratory studies, and radiographs are most consistent with hypophosphatemic rickets. This entity is inherited as a sex-linked dominant trait

Front 35

Gorham disease

Back 35

massive osteolysis, has also been called "disappearing bone disease disappearance of contiguous bones and tapering of the bony remnants. No tx except sx +/- xrt

Front 36

hallux valgus angle

Back 36

Normal radiographic measurements are an intermetatarsal angle of less than 9 degrees, a hallux valgus angle of less than 15 degrees, and a distal metatarsal articular angle of less than 9 degrees

Front 37

Hemihypertrophy

Back 37

Hemihypertrophy or hemihypotrophy is usually idiopathic, and either the leg or the entire side of the body may be involved. In the infant or young child, it is often difficult to determine which side is abnormal if the condition is mild. Because of the association of Wilms’ tumor with hemihypertrophy, these patients should undergo a yearly renal ultrasound until at least age 5 years. Other conditions that may exhibit hemihypertrophy include Klippel-Trenaunay-Weber syndrome, Proteus syndrome, and neurofibromatosis.

Front 38

hemophilia

Back 38

The iliacus muscle is a frequent site of hemorrhage in patients with severe or moderate hemophilia. In patients with moderate hemophilia, hemorrhage into the iliacus muscle often follows play or sporting events that include forceful contraction of the hip flexor muscles. An expanding iliacus hematoma compresses the adjacent femoral nerve, with one study reporting 60% complete femoral nerve palsy in hemophiliacs with an iliacus or iliopsoas hemorrhage. Femoral nerve compression typically includes paresthesias in the distribution of the terminal saphenous nerve branch. Hip joint hemarthrosis may occur, but this condition is not as frequent in hemophiliacs as muscle hemorrhage into the iliacus muscle. More importantly, a hip joint hemarthrosis is not associated with significant compression of the femoral nerve.

Front 39

hemophilia

Back 39

musculoskeletal problems are primarily found in patients with deficits of either factor VIII, also known as hemophilia A, or factor IX, also known as hemophilia B. Both factor VIII and factor IX deficiencies are transmitted by a sex-linked recessive gene; therefore, both disorders are largely restricted to the male population

Front 40

hemophilia

Back 40

The knee is the most common location of spontaneous bleeding in children with hemophilia. Treatment generally requires replacement to 40% to 50% of normal. For surgery, the replacement should be to 100%. The plasma level generally rises 2% for every unit (per kg body weight) of factor VIII administered

Front 41

Hypophosphatemic rickets

Back 41

Hypophosphatemic rickets is an inherited disorder that is transmitted by a unique sex-linked dominant gene. Therefore, if a male patient has a female offspring, his affected X chromosome will be transmitted and all of his female children will have hypophosphatemic rickets. All male offspring of a male patient will be unaffected. All offspring of a female patient have a 50% chance of having the disorder. Understanding the inheritance of hypophosphatemic rickets facilitates early diagnosis and early treatment. Medical treatment with phosphorus and some types of vitamin D (most authors recommend calcitriol) improves, but does not fully correct, the mineralization defect in hypophosphatemic rickets. However, if medical treatment is begun before the child begins walking, the growth plate is then adequately protected and a bowleg deformity will most likely be prevented.

Front 42

Hypothyroidism + delayed bone age

Back 42

Children with hypothyroidism have delayed bone age, reduced thickness of the physis, disorganization of the cartilage columns of the physis, and impaired differentiation of proliferating chondrocytes into hypertrophic cells. As a result, these children have severe growth retardation, and slipped capital femoral epiphysis may develop because of mechanical weakening of the physis. Thyroid hormone regulates terminal differentiation of the growth plate chondrocytes, with a resultant increase in type X collagen and alkaline phosphatase. These substances are important factors in matrix mineralization. Insulin-like growth factors and FGF-2 appear to act synergistically to stimulate mitotic activity of the growth plate chondrocytes. TGF-= and PTHrP stimulate proteoglycan synthesis and mitotic activity of the chondrocytes and inhibit type X collagen and alkaline phosphatase activity.

Front 43

JRA + valgus knee

Back 43

Children with JRA frequently have valgus in association with hypervascularity because of chronic inflammation. This is normally caused by overgrowth of the medial femoral epiphysis. Staple hemiepiphyseodesis, if done early, can reverse the deformity.

Front 44

juvenile scoliosis

Back 44

MRI has shown an association between juvenile scoliosis and intraspinal abnormalities, most often syringomyelia and Arnold-Chiari malformations. All juvenile curves greater than 20 degrees should be evaluated with MRI despite the absence of neurologic findings.

Front 45

Langerhans Cell Histiocytosis

Back 45

disease complex characterized by infiltration of one or more organs by large mononuclear cells with benign-appearing nuclei that often have a central groove

Front 46

late spinal deformity

Back 46

The incidence of late spinal deformity after complete spinal cord injury in children depends on the level of the spinal cord injury and the age of the patient at the time of injury. If a cervical level injury occurs before age 10 years, paralytic scoliosis will develop in virtually 100% of patients

Front 47

Legg-Calve-Perthes

Back 47

favorable prognosis can be expected in up to 70% of children with Legg-Calve-Perthes disease who are younger than age 6 years. Containment treatment has not been shown to alter the outcome in this age group. The goals of treatment in this patient are to reduce pain (synovitis), restore motion, and improve function. Symptomatic treatment modalities include bed rest, traction, crutches, activity modification, and nonsteroidal anti-inflammatory drugs.

Front 48

limb length

Back 48

CT scanogram is more accurate than standard scanograms for determining limb length in patients with knee flexion contractures of 30° or more. The cost and time necessary to complete the examinations are comparable, but the CT scanogram delivers only 20% of the radiation needed for standard scanograms

Front 49

Medial epicondyle fragment in pediatric

Back 49

ORIF if fragment unreduce-able or more than 1 cm displaced

Front 50

Melorheostosis

Back 50

a rare sclerosing skeletal dysplasia characterized by soft tissue contractures in childhood overlying slowly evolving linear hyperostosis. typical presentation is with painless, asymmetric joint contractures prior to 6 years of age. classic radiographic appearance of melorheostosis is asymmetrical bands of sclerosis in an irregular, linear pattern often described as molten wax flowing down the side of a candle. Surgical release.

Front 51

metatarsus adductus

Back 51

In a long-term follow-up study by Farsetti and associates, deformities that were passively correctable spontaneously resolved and no treatment was required

Front 52

Myelomeningocele

Back 52

50-90% talipes equinus

Front 53

myelomeningocele proximal to L4

Back 53

There is little evidence to support active treatment of bilateral hip dislocations in patients with

Front 54

Myositis ossificans

Back 54

refers to the acquired development of nonneoplastic heterotopic ossification within soft tissues, most often in response to localized trauma. development of heterotopic ossification has also been reported after severe thermal injury, in neurologic diseases, and posttraumatic paraplegia and brain injury, and after some orthopaedic operations, such as total hip arthroplasty

Front 55

neurofibromatosis

Back 55

The diagnosis of neurofibromatosis may be based on the presence of at least six cafe-au-lait. Neurofibromatosis occurs in 50% of patients who have an anterolateral bowing deformity of the tibia, and this bowing may be the first clinical manifestation of this disorder. The patient has anterolateral bowing of the tibia and fibula that warrants concern for a possible fracture and pseudarthrosis; therefore, the limb should be protected in a total contact orthosis to prevent fracture. In contradistinction to posteromedial bowing of the tibia and fibula, spontaneous remodeling of an anterolateral bowing deformity is not expected. Intramedullary nailing or the use of a vascularized fibula is reserved for the treatment of a congenital pseudarthrosis of the tibia.

Front 56

Neurofibromatosis type 1

Back 56

Neurofibromatosis type 1 (NF-1) is an autosomal-dominant disorder affecting about 1 in 4,000 people. NF-1 causes tumors to grow along various types of nerves and affects the development of non-nervous tissues, such as bone and skin. The gene for NF-1 is located on the long arm of chromosome 17 and codes the protein neurofibromin. Research indicates that NF-1 acts as a tumor-suppressor gene and, as such, plays an important role in the control of cell growth and differentiation. Axillary and inguinal freckling is considered a good diagnostic marker for NF-1. The hyperpigmented spots that measure from 2 mm to 4 mm may be congenital, but these typically appear and increase later in life. Scoliosis is the most common musculoskeletal disorder of NF-1. The curves are frequently dystrophic, kyphotic, and have a high risk of pseudarthrosis following spinal fusion. Anterior and posterior spinal fusion with rigid posterior segmental instrumentation is the treatment of choice.

Front 57

OCD

Back 57

This skeletally immature patient has a small OCD lesion that appears stable, and he has not undergone any treatment. Therefore, a trial of immobilization until pain resolves is the best initial choice. Thereafter, cessation of sport activities for 4 to 6 months may allow healing of the lesion. Surgical treatment of juvenile OCD lesions is reserved for unstable lesions, patients who have not shown radiographic evidence of healing and are still symptomatic after 6 months of nonsurgical management, or patients who are approaching skeletal maturity. Good results with stable in situ lesions that have failed to respond to nonsurgical management have been reported with both transarticular and retroarticular drilling. Results after excision alone are poor at 5-year follow-up, and it is unclear if microfracture will improve the long-term outcome. Mosaicplasty may be the next best option for patients who remain or become symptomatic after excision of the fragment and microfracture

Front 58

Osteogenesis imperfecta

Back 58

Osteogenesis imperfecta (OI) is a genetically determined disorder of type I collagen synthesis that is characterized by bone fragility. Type I OI is the mildest and most common form. Inheritance is autosomal-dominant; however, as in this patient, new mutations are frequent. Type I is subclassified into the A type (absence of dentinogenesis imperfecta) and B type (presence of dentinogenesis imperfecta). The sclerae are blue, and the first fractures usually occur in the preschool years after walking has begun. Cells from individuals with type I OI largely demonstrate a quantitative defect of type I collagen; they synthesize and secrete about half the normal amount of type I procollagen. Qualitative mutations that lead to an abnormal type I procollagen molecule result in more severe types of the disorder.

Front 59

Osteoid osteomas

Back 59

are painful bone lesions, with radiographs revealing a dense sclerotic cortex surrounding a small radiolucency or nidus. Symptoms often are worse at night but usually are not activity related. While treatment in the past has consisted of open en bloc excision, current means of removal include percutaneous drilling under CT guidance and percutaneous radiofrequency coagulation

Front 60

Osteonecrosis of femoral head

Back 60

in adolescents, 1-2% have OFN when piriformis fossa entry used for IMR (lateral ascending vessels of femoral a.)

Front 61

Osteopathia Striata

Back 61

or Voorhoeve disease, is another of the sclerosing bone dysplasias and is characterized by dense linear striations in bone

Front 62

osteopetrosis

Back 62

The condition results from defective resorption of immature bone by osteoclasts. There are three distinct clinical forms: (1) infantile-malignant, which is autosomal recessive and fatal in the first few years of life if untreated; (2) intermediate autosomal recessive; and (3) autosomal dominant. These conditions do not follow a malignant course, and patients have normal life expectancy with orthopaedic problems and anemia. In the malignant form, the clinical features include frequent fractures, macrocephaly, progressive deafness and blindness, hepatosplenomegaly, and severe anemia beginning in early infancy or in utero. Deafness and blindness are generally thought to represent effects of pressure on nerves and usually occur later in life. The anemia is caused by encroachment of bone on marrow, resulting in obliteration, and the hepatosplenomegaly is caused by compensatory extramedullary hematopoiesis. Dental caries and abscesses, as well as osteomyelitis of the mandible, are also seen. Most patients have normal intelligence. Treatment of the malignant form includes high dose 1,25 dihydroxy vitamin D with a low-calcium diet to stimulate bone resorption, not because there are vitamin deficiencies. Bone marrow transplant has also been successful.

Front 63

Osteopetrosis

Back 63

The radiographs show the characteristic features of osteopetrosis. The condition results from defective resorption of immature bone by osteoclasts. There are three distinct clinical forms: (1) infantile-malignant, which is autosomal recessive and fatal in the first few years of life if untreated; (2) intermediate autosomal recessive; and (3) autosomal dominant. These conditions do not follow a malignant course, and patients have normal life expectancy with orthopaedic problems and anemia. In the malignant form, the clinical features include frequent fractures, macrocephaly, progressive deafness and blindness, hepatosplenomegaly, and severe anemia beginning in early infancy or in utero. Deafness and blindness are generally thought to represent effects of pressure on nerves and usually occur later in life. The anemia is caused by encroachment of bone on marrow, resulting in obliteration, and the hepatosplenomegaly is caused by compensatory extramedullary hematopoiesis. Dental caries and abscesses, as well as osteomyelitis of the mandible, are also seen. Most patients have normal intelligence. Treatment of the malignant form includes high dose 1,25 dihydroxy vitamin D with a low-calcium diet to stimulate bone resorption, not because there are vitamin deficiencies. Bone marrow transplant has also been successful

Front 64

Osteopoikilosis

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, or osteopathia condensans disseminata, is an uncommon, autosomal dominant, sclerosing bone dysplasia characterized by numerous small foci of increased radiodensity in the periarticular regions. articular discomfort with a joint effusion. osteosclerotic nodules are well–defined, homogeneous, bilateral, circular- to ovoid–shaped from 1 to 15 mm, and are located in the metaphyses and epiphyses of long bones, the carpus, the tarsus, the pelvis, and the scapulae

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Pauciarticular juvenile rheumatoid arthritis

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is the most common subgroup of JRA. It typically presents between the ages of 2 to 4 years with a mild swelling of one to four joints. Girls are affected four times more often than boys. The ankle and knee are commonly involved, and limping is typically worse in the morning and after extended activity. The diagnosis of pauciarticular JRA is typically one of exclusion because laboratory studies, including erythrocyte sedimentation rate and rheumatoid factor, are usually within normal limits. Pauciarticular JRA has the highest incidence of chronic uveitis, and in the subgroup with elevated antinuclear antibody (ANA) titers, the incidence is 75%. In JRA, uveitis usually occurs after the onset of synovitis but may precede the joint symptoms. At the early stage of uveitis, the patient is asymptomatic. If the eye condition is not detected and treated, progressive loss of vision may occur. Orthopaedic surgeons may be instrumental in making the diagnosis of pauciarticular JRA. Pauciarticular JRA is not associated with conditions that require input from the other specialists

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Pauciarticular juvenile rheumatoid arthritis (JRA)

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most common subgroup of JRA. It typically presents between the ages of 2 to 4 years with a mild swelling of one to four joints. Girls are affected four times more often than boys. The ankle and knee are commonly involved, and limping is typically worse in the morning and after extended activity. The diagnosis of pauciarticular JRA is typically one of exclusion because laboratory studies, including erythrocyte sedimentation rate and rheumatoid factor, are usually within normal limits. Pauciarticular JRA has the highest incidence of chronic uveitis, and in the subgroup with elevated antinuclear antibody (ANA) titers, the incidence is 75%. In JRA, uveitis usually occurs after the onset of synovitis but may precede the joint symptoms. At the early stage of uveitis, the patient is asymptomatic. If the eye condition is not detected and treated, progressive loss of vision may occur.

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phenytoin

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phenytoin induces osteomalacia, or rickets, in growing children, through interference with metabolism of vitamin D. Oral supplementation of vitamin D can minimize this effect in patients who are undergoing prolonged treatment with phenytoin.

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Plexiform neurofibromas

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Plexiform neurofibromas are lesions found in patients with NF1. Clinical reports show the prevalence of plexiform neurofibroma to be 20% to 30% but increases to 40% when imaging studies are routinely obtained. The lesions are characterized by diffuse hypertrophy of the involved nerves but with preservation of the nerves’ fascicular organization. The lesions may involve the dermis or may arise in the deeper structures. Palpation of a dermal lesion provokes an image of a “bag of worms.” Plexiform neurofibromas may cause disfigurement and hyperpigmentation of the overlying skin. The lesions also can cause diffuse hypertrophy of the soft tissue and bone, with resultant changes ranging from a relatively minor limb-length discrepancy to gigantism of the entire extremity. Dural ectasia is frequently found in patients with NF1. Therefore, MRI should be obtained prior to planning spinal procedures in these patients; however, dural ectasia is not the cause of limb overgrowth. Lisch nodules are benign hamartomas of the iris. The lesions are uncommon during early childhood but are found in all adults with NF1. Juvenile xanthogranuloma has a low occurrence rate in patients with NF1; its presence is associated with juvenile chronic myeloid leukemia. Malignant peripheral nerve sheath tumors, formally called neurofibrosarcoma, result from malignant degeneration of a plexiform neurofibroma. This condition occurs in up to 4% of patients with NF1. Localized pain, an enlarging mass, or progressive neurologic symptoms suggest a malignant peripheral nerve sheath tumor in a patient with NF1. However, progressive neurologic symptoms also may occur with benign growth of a plexiform neurofibroma

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Progressive diaphyseal dysplasia

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or Camurati-Engelmann syndrome, is a sclerosing bone dysplasia characterized by progressive diaphyseal thickening and sclerosis, bone pain and weakness. plain radiographs demonstrate symmetric, periosteal and endosteal diaphyseal sclerosis and diaphyseal widening. The tibia is most frequently involved, but all the long bones in the upper and lower extremities, and the clavicle, can be affected early in the course of the disease. Corticosteroid administration results in reduced pain and fatigue and better function, such as the ability to run

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Proteus Syndrome

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is a sporadic vascular, skeletal, and soft tissue disorder characterized by vascular anomalies, macrodactyly, exostoses, asymmetric hypertrophy, subcutaneous tumors, scoliosis, and other anomalies. The cutaneous vascular malformations include capillary, lymphatic, capillary-venous, and capillary-lymphatic-venous types. Macrodactyly. Hemihypertrophy. Angular upper- and lower–limb deformities.

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radioulnar synostosis

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Patients with this disorder frequently have duplication of sex chromosomes. Synostosis is often seen in females with 48-XXXX or 49-XXXXX in association with delayed development and mental retardation. In males, it can be associated with 48-XXXY or 49-XXXXY

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rectus femoris tendon transfer

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rectus femoris muscle spans two joints and is active during running, sprinting, and walking at a fast pace during the preswing and early swing phase of gait. In these situations, the muscle helps to generate power to initiate hip flexion while absorbing or controlling the rate of knee flexion during early swing phase. Quadriceps activity, including the rectus femoris, is not normally needed when walking at a routine cadence. However, rectus femoris activity is commonly noted during preswing and the swing phase in patients with cerebral palsy, particularly those with diplegia. In an effort to initiate swing phase, the rectus femoris is “overactive.” As a result, the knee flexion that commonly occurs at terminal stance and initial swing is restricted. Instead of achieving the normal 50 to 60 degrees of flexion during early swing, this patient’s knee flexion is limited to 15 degrees. The goal of treatment is to retain rectus femoris activity for initiation of hip flexion but to diminish its restraint on knee flexion. Studies have shown that transfer of the distal rectus femoris tendon provides more flexion of the knee during the swing phase of gait than simply releasing the tendon

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Recurrent patellar dislocations

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One study found that in patients who had a patellar dislocation between the ages of 11 to 14 years, 60% had a recurrent dislocation. The incidence of recurrent dislocation dropped to 33% in patients who had a patellar dislocation between the ages of 15 to 18 years. Predisposing signs included passive lateral hypermobility of the patella, a dysplastic distal third of the vastus medialis obliquis muscle, and a high and/or lateral position of the patella. A second study found that the risk of redislocation was considerably higher in patients who were in their teens at the first episode of dislocation compared to older patients. There are no studies linking either a patella baja or a bipartite patella to an increased risk of redislocation.

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recurvatum deformity of the proximal tibia

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The radiograph shows a recurvatum deformity of the proximal tibia with growth arrest of the tibial tubercle apophysis. This deformity has been described in association with femoral shaft fractures in children and has been attributed to a clinically silent, concommitant injury to the proximal tibial physes and also to iatrogenic injury associated with a proximal tibial traction pin.

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Reflex sympathetic dystrophy

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1.

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2.

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Continuing pain, allodynia, or hyperalgesia in which the pain is disproportionate to the inciting event.

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3.

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Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the painful region.

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4.

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The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction

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Rhizotomy

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While other surgical and nonsurgical options exist for management of spasticity, the criteria originally laid out by Peacock and associates describe the most appropriate candidate for rhizotomy as a patient with spastic diplegia who is between the ages of 4 to 8 years and has a stable gait pattern that is limited by lower extremity spasticity. Rhizotomy is not recommended in patients with athetosis because of unpredictable results. In addition, rhizotomy should be avoided in nonambulatory patients with spastic quadriplegia because it is associated with significant spinal deformities.

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Rib penciling & NF

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Neurofibromatosis can progress very rapidly. Rib penciling is the only singular prognostic factor. Significant progression has been observed in 87% of the curves with three or more penciled ribs

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Rickets

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causes widening of the hypertrophic layer of the physis because of the failure of mineralization and vascular invasion. The other zones of the physis may be altered in other disease conditions but remain relatively unchanged in rickets

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sacral agenesis

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Clinical signs include the classic dimpling over the buttocks and the characteristic lower extremity deformities. Imperforate anus is often associated with this disorder. Although motor function correlates with the level of vertebral defect, sensation is usually intact. This is important therapeutically, because patients are not as prone to pressure sores as are those with myelodysplasia. Kyphosis may develop in many patients with lumbosacral agenesis, but lordosis is unusual. Latex allergy and progressive neural deterioration may occur in patients with either myelodysplasia or sacral agenesis but is more common in the former.

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sacral agenesis

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Clinical signs include the classic dimpling over the buttocks and the characteristic lower extremity deformities. Imperforate anus is often associated with this disorder. Although motor function correlates with the level of vertebral defect, sensation is usually intact. This is important therapeutically, because patients are not as prone to pressure sores as are those with myelodysplasia. Kyphosis may develop in many patients with lumbosacral agenesis, but lordosis is unusual. Latex allergy and progressive neural deterioration may occur in patients with either myelodysplasia or sacral agenesis but is more common in the former.

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SCFE

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According to the classification system based on physeal stability, an unstable SCFE is one in which the patient is unable to walk, even with crutches. Ishemic necrosis, or osteonecrosis, of the femoral head is the most devastating complication of SCFE. One study found a 47% incidence of ischemic necrosis following unstable slips. This complication is most likely the result of vascular injury associated with initial femoral head displacement rather than the result of either tamponade from joint effusion or gentle repositioning prior to stabilization

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SCFE

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Vascular invasion, histologically similar to granulation tissue, has been noted between the columns in the zone of hypertrophy, leading to the theory of microtrauma as an etiology

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SCFE

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Vascular invasion, histologically similar to granulation tissue, has been noted between the columns in the zone of hypertrophy, leading to the theory of microtrauma as an etiology. SCFE is also associated with conditions that increase the height of the zone of hypertrophy, including the adolescent growth spurt and endocrinopathies. The perichondral ring has been shown to decrease in thickness with age. Normal undulations in the growth plate also decrease during this time, possibly further destabilizing the physis. Abnormal accumulations of proteoglycan have been reported.

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Schmid metaphyseal dysostosis

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Bowlegs associated with very wide physes, particularly noted at the hips. The widening of the growth plates is a classic sign of rickets; however, the normal levels of calcium, phosphorus, and alkaline phosphatase rule out both nutritional and hypophosphatemic rickets. Patients with nutritional rickets or hypophosphatemic rickets have hypophosphatemia and increased alkaline phosphatase levels. Jansen metaphyseal dysostosis has very severe radiographic findings that are not found in this patient; however, these radiographic findings are classic for Schmid metaphyseal dysostosis. This disorder is caused by a mutation in the gene for type X collagen, which is found only in the growth plates of growing children. Laboratory studies show a calcium level of 9.5 mg/dL (normal 9.0 to 11.0 mg/dL), a phosphorus level of 4.2 mg/dL (normal 3 to 5.7 mg/dL), and an alkaline phosphatase level of 305 IU/L (normal 104 to 345 IU/L).

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Sickle Cell Disease

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Bone and joint problems in sickle cell disease include the sickle cell crises, dactylitis- Dactylitis, or hand–foot syndrome, is secondary to infarction of a bone in the hand or foot, and frequently is the first clinical manifestation seen after hemoglobin F has been replaced by hemoglobin S. The typical patient is a child younger than 2 years of age, who presents with an acutely swollen hand or foot, osteonecrosis, osteomyelitis, septic arthritis, reactive arthritis, and leg ulcers. In addition, hemolytic anemia in these patients causes erythroid hyperplasia of the bone marrow, with resultant increased size of the medullary spaces, and osteopenia. The spine is more likely to demonstrate the effect of marrow hyperplasia. Thinning of the cortices and trabeculae lead to collapse, with development of a biconcave "fish" vertebra and mild structural changes

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Sindig-Larsen-Johansson

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fragmentation of the inferior pole of the patella. This finding, along with the clinical presentation, is most consistent with Sindig-Larsen-Johansson disease. This is an overuse syndrome commonly seen in boys ages 9 to 11 years. The differential diagnosis includes bipartite patella and patellar sleeve fracture. Like most overuse syndromes, Sindig-Larsen-Johansson disease responds to activity modification and nonsteroidal anti-inflammatory drugs. While symptoms usually resolve with short periods of activity restriction, radiographic findings may persist.

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sleeve fracture

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Fractures through the cartilage on the inferior pole of the patella, the so-called sleeve fracture, are often difficult to diagnose because of the paucity of ossified bone visible on the radiographs. If the fracture is missed and the fragments are widely displaced, the patella may heal in an elongated configuration that may result in compromise of the extensor mechanism function. The treatment of choice is open reduction and internal fixation using a tension band wire technique to achieve close approximation of the fragments and restore full active knee extension.

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sleeve fracture

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The radiograph shows an avulsion fracture, or “sleeve fracture,” of the distal pole of the patella. The distal fragment is much larger than it appears on the radiograph because it largely consists of cartilage; therefore, excision of the fragment is contraindicated. The treatment of choice is open reduction and tension band fixation to correct patella alta and restore the extensor mechanism

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slipped capital femoral epiphyses

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slipped capital femoral epiphyses is based on the following temporal criteria: acute (symptoms that persist for less than 3 weeks); chronic (symptoms that persist for more than 3 weeks); or acute on chronic (acute exacerbation of long-standing symptoms). A newer classification differentiates between a stable slip where weight bearing is possible, and an unstable slip if it is not. Reduction of an unstable slip often occurs unintentionally with induction of anesthesia and positioning of the patient for surgery. The rate of satisfactory results is lower primarily because of a much higher incidence of osteonecrosis following internal fixation of an unstable slip.

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spastic diplegic cerebral palsy

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Children with spastic diplegic cerebral palsy often have contractures of multiple joints. Because the gait abnormalities can be complex, isolated surgery is rarely indicated. To avoid compensatory deformities at other joints, it is preferable to correct all deformities in a single operation. Isolated heel cord lengthening in the presence of tight hamstrings and hip flexors will lead to progressive flexion at the hips and knees, thus worsening a crouched gait. Split posterior tibial tendon transfer is used for patients with hindfoot varus, which is not present in this patient.

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spastic quadriparesis

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Progressive hip subluxation occurs in up to 50% of children with spastic quadriparesis. The subluxation is the result of chronic muscle hypertonicity, especially in the adductor muscle group. In time, the constant muscle tension will lead to dislocation, dysplastic changes in the acetabulum, and erosive changes in the cartilage of the femoral head. Many of these children will experience pain. Two recent studies have shown that early soft-tissue releases can successfully prevent progressive subluxation in children who are younger than age 4 years and who have a Reimers index (migration index) of less than 40%. Botulinum toxin A injections may reduce tone in the adductors for 4 to 6 months, but it is difficult to inject into the iliopsoas. Additionally, there are no long-term studies documenting the efficacy of botulinum toxin A to treat progressive hip subluxation in patients who have spastic quadriparesis. In general, proximal femoral osteotomy, combined with soft-tissue release as necessary, is indicated in older children (older than age 4 years) with progressive subluxation. Although selective dorsal rhizotomy has been used in nonambulatory patients, outcomes are less well documented than in ambulatory patients. There are no studies documenting the effect of selective dorsal rhizotomy on progressive hip subluxation in nonambulatory children.

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spina bifida + bilateral hip dislocation

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Children with spina bifida and bilateral symmetrical dislocation of the hips usually do not require treatment. A level pelvis and good range of motion of the hips are more important for ambulation than reduction of bilateral hip dislocations. Because the patient has good sitting and standing balance and good range of motion, maintenance of that range of motion and symmetry is more important than reduction. Surgery is not recommended

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Spinal cord injury in skeletally immature patients

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almost always leads to the development of paralytic spinal deformity. The age at injury is the most important factor affecting the development of scoliosis. Spinal cord injury that occurs more than 1 year prior to skeletal maturity is almost always followed by the development of scoliosis. In one study, scoliosis developed in 100% of children who were younger than age 10 years at the time of spinal cord injury. Scoliosis can occur after injury at any level. Spasticity is often a contributing factor. Up to two thirds of patients who have paralytic scoliosis prior to skeletal maturity will eventually require surgery for curve control.

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spinal muscular atrophy

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Children with this condition appear normal at birth but are not able to walk. The disorder affects anterior horn cells. Fasciculations may be present, but deep tendon reflexes are typically absent. The development of scoliosis is almost universal with this type of spinal muscular atrophy. More than 90% of patients with spinal muscular atrophy have deletions in the telomeric survival motor neuron gene. Peripheral myelin protein 22 is abnormal in Charcot-Marie-Tooth type IA. Connexin 32 is abnormal in the X-linked type of Charcot-Marie-Tooth disease. Neurofibromin is affected in neurofibromatosis type 1. Friedreich’s ataxia is secondary to a disorder of frataxin.

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Spinal muscular atrophy

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is caused by an abnormal survival motor neuron gene that prevents apoptosis of the motor nerves. Spinal fusion results in better sitting balance, stabilized or improved pulmonary function, and high parental satisfaction, but it may result in at least temporary loss of upper extremity function

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Spinal muscular atrophy

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Spinal muscular atrophy is caused by an abnormal survival motor neuron gene that prevents apoptosis of the motor nerves. Spinal fusion results in better sitting balance, stabilized or improved pulmonary function, and high parental satisfaction, but it may result in at least temporary loss of upper extremity function

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split posterior tendon

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Isolated split posterior tendon transfer alone is best performed in a patient with cerebral palsy who is between the ages of 4 and 7 years and has a flexible equinovarus foot. Rigid deformities often must be managed with a combination of soft-tissue and bony procedures. Patients with out-of-phase activity may be best managed with a transfer of the posterior tibialis to the dorsum of the foot, while those with continuous activity are better candidates for an isolated split posterior tendon transfer

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surgical treatment of spondylolisthesis

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Indications for surgical treatment of spondylolisthesis include pain and/or progression of deformity. Specifically, surgery is necessary when there is persistent pain or a neurologic deficit that fails to respond to nonsurgical therapy, there is significant slip progression, or the slip is greater than 50%. For patients with mild spondylolisthesis, in situ posterolateral L5-S1 fusion is adequate. In patients with more severe slips (greater than 50%), extension of the fusion to L4 offers better mechanical advantage. Postoperative immobilization may be achieved with instrumentation, casting, or both. In patients with a slip angle of greater than 45°, reduction of the lumbosacral kyphosis with instrumentation or casting is desirable to prevent slip progression. Laminectomy alone is contraindicated in a child. Nerve root decompression is indicated if radiculopathy is present clinically.

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talipes equinovarus

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In this technique, the primary goal is to rotate the foot laterally around a talus that is held fixed by the manipulating surgeon’s hands. While the navicular may be rotated anterolaterally with this technique, the primary focus is on the calcaneus. The calcaneus is rotated laterally and superiorly, not translated. Some dorsiflexion of the calcaneus can be obtained by manipulation, but the primary focus is on the rotational relationship of the talus and calcaneus, not the degree of calcaneal dorsiflexion

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Thalassemia

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Normal hemoglobin is a tetramer consisting of two a-like and two b-like globin polypeptides

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Tillaux & triplane fx of adolesc.

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Surgery indicated if more than 2 mm displacement

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Triplane fracture of the ankle

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In adolescence, closure of the distal tibial physis starts peripherally at the anteromedial aspect of the medial malleolus and extends posteriorly and laterally. The anterolateral quadrant of the physis is the last to close, making this region the most susceptible to separation. When the foot is twisted into external rotation, the anterolateral portion of the epiphysis is avulsed by the pull of the anterior tibiofibular ligament. When this fragment alone is avulsed, the result is a juvenile Tillaux fracture. When the fracture extends to involve the remainder of the physis and posterior metaphysis, as in this patient, the result is a triplane fracture.

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valgus stress injury

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In the skeletally mature individual, a valgus stress injury to the knee while playing football will often result in a sprain of the medial collateral ligament. In skeletally immature patients, the same mechanism can cause a fracture of the distal femoral physis. If the fracture is nondisplaced, the plain radiographs may show only soft-tissue swelling or effusion. While the MRI scan may show edema in the soft tissues on the medial side of the knee and even an abnormality of the physis, stress radiographs provide a quicker and less expensive means of making the diagnosis. Arthroscopy and arthrography would not be helpful in making the diagnosis. Arthroscopy may result in further displacement of the injury.

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X-linked agammaglobulinemia

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is a sex-linked recessive disorder manifested by recurrent bacterial infections that typically begin after 6 months of age, when maternal immunoglobulins have essentially disappeared

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zone of Ranvier

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provides the earliest increase in strength of the physis. During the first year of life, the zone spreads over the adjacent metaphysis to form a fibrous circumferential ring bridging from the epiphysis to the diaphysis. This ring increases the mechanical strength of the physis. The zone also helps the physis grow latitudinally. In turn, the increased width of the physis helps the physis further resist mechanical forces. The change in shape of the physis to its progressively more undulating form is also a factor in increasing physeal strength, but this occurs over a longer period of time, as the child’s activity level increases. The undulations of the physis seen in some growth plates also add to stability but to a lesser extent. The other changes contribute little toward increasing physeal strength.