This document provides information on Blount's disease (infantile tibia vara) including its definition, etiology, anatomy, pathophysiology, and radiologic findings. Specifically, it describes:
1) Blount's disease is characterized by an abrupt angulation just below the proximal tibia physis with an irregular physeal line and wedge-shaped epiphysis.
2) The exact cause is unknown but it is considered a developmental disorder that is sometimes familial.
3) It provides detailed anatomy of the tibia, fibula, and the muscles of the leg that are involved.
4) The pathophysiology involves irregular physeal cell columns and disrupted endochond
This document provides information on Blount's disease (infantile tibia vara) including its definition, etiology, anatomy, pathophysiology, and radiologic findings. Specifically, it describes:
1) Blount's disease is characterized by an abrupt angulation just below the proximal tibia physis with an irregular physeal line and wedge-shaped epiphysis.
2) The exact cause is unknown but it is considered a developmental disorder that is sometimes familial.
3) It provides detailed anatomy of the tibia, fibula, and the muscles of the leg that are involved.
4) The pathophysiology involves irregular physeal cell columns and disrupted endochond
This document provides information on Blount's disease (infantile tibia vara) including its definition, etiology, anatomy, pathophysiology, and radiologic findings. Specifically, it describes:
1) Blount's disease is characterized by an abrupt angulation just below the proximal tibia physis with an irregular physeal line and wedge-shaped epiphysis.
2) The exact cause is unknown but it is considered a developmental disorder that is sometimes familial.
3) It provides detailed anatomy of the tibia, fibula, and the muscles of the leg that are involved.
4) The pathophysiology involves irregular physeal cell columns and disrupted endochond
By: Sri Hardianti C111 08 202 Nor Anisah bt Abu Hanipah C111 08 780 Muhammad Fadzhil Bin Amran C 111 09 841 Izzad bin Azlan C111 08 793 ReynaldoMailoa C111 09 131 Aditya Wisnu Pratama C111 09 296
Advisors: dr. Jecky C. dr. Rico A. dr. Sebastian M. dr. M. Horeb
Created as Clinical Student Assignment Department of Orthopaedic & Traumatology Medical Faculty of Hasanuddin University Makassar 2014
TEXTBOOK I) DEFINITION Infantile tibia vara, first described by Erlacher in 1922, is best known as Blount's disease after the classic description by Blount in 1937. Blount characterized the deformity as an abrupt angulation just below the proximal physis, an irregular physeal line, and a wedge-shaped epiphysis with a beak at the medial metaphysis. Apparent lateral subluxation of the proximal end of the tibia is often present. II) ETIOLOGY Several authors have reported a familial occurrence of the conditionand one report of infantile tibia vara in a family suggested that the disease may be inherited as an autosomal dominant condition with variable penetrance.However, as noted by Langenskild and Riska,
because the radiographic features of infantile tibia vara have never been seen in patients younger than 1 year and rarely in patients younger than 2 years, the condition is considered a developmental disorder and not a congenital one. Other studies have found no evidence of an inherited condition and have concluded that the etiology is multifactorial. III) ANATOMY The tibia and fibula are the bones of the leg. The tibia articulates with the condyles of the femur superiorly and the talus inferiorly and in doing so transmits the body's weight. The fibula mainly functions as an attachment for muscles, but it is also important for the stability of the ankle joint. The shafts (bodies) of the tibia and fibula are connected by a dense interosseous membrane composed of strong oblique fibers.
A) Tibia Tibia is located on the anteromedial side of the leg, nearly parallel to the fibula, the tibia is the second largest bone in the body. The proximal end widens to form medial and lateral condyles and there is tibial plateau, which articulate with the lateral and medial condyles of the femur and the lateral and medial menisci intervening. Separating the upper articular surfaces of the tibial condyles are anterior and posterior intercondylar areas lying between these areas is the intercondylar eminence. (3,4)
The shaft of the tibia is triangular in cross section, presenting three borders and three surfaces. Its anterior and medial borders, with the medial surface between them, are subcutaneous. At the junction of the anterior border with the upper end of the tibia is the tuberosity, which receives the attachment of the ligamentum patellae. The anterior border becomes rounded below, where it becomes continuous with the medial malleolus. The lateral or interosseous border gives attachment to the interosseous membrane. The lower end of the tibia is slightly expanded and on its inferior aspect shows a saddle-shaped articular surface for the talus. The lower end is prolonged downward medially to form the medial malleolus. (3,4)
B. Fibula The fibula is the slender lateral bone of the leg. It takes no part in the articulation at the knee joint, but below it forms the lateral malleolus of the ankle joint. It takes no part in the transmission of body weight, but it provides attachment for muscles. The fibula has an expanded upper end, a shaft, and a lower end. The upper end, or head, is surmounted by a styloid process. It possesses an articular surface for articulation with the lateral condyle of the tibia. The shaft of the fibula is long and slender. Typically, it has four borders and four surfaces. The medial or interosseous border gives attachment to the interosseous membrane. The lower end of the fibula forms the triangular lateral malleolus, which is subcutaneous. On the medial surface of the lateral malleolus is a triangular articular facet for articulation with the lateral aspect of the talus. Below and behind the articular facet is a depression called the malleolar fossa. (3,4)
Picture 1: Anatomy of tibia and fibula. (5)
The anterior compartment, or dorsiflexor (extensor) compartment, is located anterior to the interosseous membrane, between the lateral surface of the tibial shaft and the medial surface of the fibular shaft, and anterior to the intermuscular septum that connects them. The anterior compartment is bounded anteriorly by the deep fascia of the leg and skin. The deep fascia of the leg overlying the anterior compartment is dense superiorly, providing part of the proximal attachment of the muscle immediately deep to it. The four muscles in the anterior compartment are the tibialis anterior, extensor digitorum longus, extensor hallucis longus, and fibularis tertius. These muscles pass and insert anterior to the transversely oriented axis of the ankle joint and, therefore, are dorsiflexors of the ankle joint, elevating the forefoot and depressing the heel. The long extensors also pass along and attach to the dorsal aspect of the digits and are thus extensors of the toes. (4)
The lateral compartment is the smallest (narrowest) of the leg compartments, bounded by the lateral surface of the fibula, the anterior and posterior intermuscular septa, and the deep fascia of the leg. The lateral compartment contains the fibularis (peroneus) longus and brevis muscles. (4)
Picture 2: Muscles of the cruris. (5)
The posterior compartment (plantar flexor compartment, is the largest of the three leg compartments. The posterior compartment and the calf muscles within it are divided into superficial and deep subcompartments/muscle groups by the transverse intermuscular septum. The tibial nerve and posterior tibial and fibular vessels supply both parts of the posterior compartment but run in the deep subcompartment deep (anterior) to the transverse intermuscular septum. Muscles of the posterior compartment produce plantarflexion at the ankle, inversion at the subtalar and transverse tarsal joints and flexion of the toes. The superficial group of calf muscles are the gastrocnemius, soleus, and plantaris. Thus, the muscles of the posterior compartment of the leg are popliteus, flexor digitorum longus, flexor hallucis longus, and tibialis posterior. (4)
Picture 3: Muscles of the cruris. (5)
IV) PATHOPHYSIOLOGY There are three types of tibia varum based on the age it begins: infantile (less than three years old), juvenile (occurs between four and 10 years), and adolescent (11 years of age and older). Physiologic tibia varum occurs between the ages of 15 months to three years. There's no need for treatment for this normal stage of development. But it's not always clear at this age if the tibia varum is physiologic (normal variation) or pathologic(Blount's disease).
The general findings have included (1) islands of densely packed cartilage cells displaying greater hypertrophy than expected from their position in the growth plate, (2) islands of nearly acellular fibrocartilage, and (3) exceptionally large clusters of capillary vessels. The physeal cell columns become irregular and disordered in arrangement and normal endochondral ossification is disrupted, both in the medial aspect of the metaphysis and in the corresponding part of the physis. The varus deformity progresses as long as ossification is defective and growth continues laterally. In later stages of the deformity, an actual bony bridge may tether medial growth, and the medial tibial plateau may appear to be deficient posteromedially. However, actual depression of the posteromedial tibial articular surface is probably not present, at least at the outset of the deformity. The deficiency is probably unossified abnormal fibrocartilage whose delay in ossification produces the appearance of a defect and is directly related to the underlying histopathology. Ligamentous laxity on the lateral side of the knee frequently develops in a neglected or recurrent deformity. V) RADIOLOGIC FINDING Radiography is central to establishing the diagnosis of infantile tibia vara. A standing anteroposterior view of the lower extremities from hip to ankle should be obtained. The diagnosis is based on familiar radiographic changes in the proximal end of the tibia: (1) a sharp varus angulation in the metaphysis, (2) a widened and irregular physeal line medially, (3) a medially sloped and irregularly ossified epiphysis, and (4) prominent beaking of the medial metaphysis with lucent cartilage islands within the beak. Unequivocal radiographic changes diagnostic of infantile tibia vara are rarely observed before 18 months of age (the youngest published case wasradiographically diagnosed at 17 months of age).
However, a normal knee radiograph in a toddler does not rule out infantile tibia vara. As an aid to early identification of toddlers who are at risk for the development of infantile tibia vara but who have no physeal or metaphyseal changes, Levine and Drennan measured the tibial metaphyseal diaphyseal angle (MDA), the angle created by the intersection of a line connecting the most prominent medial portion of the proximal tibial metaphysis (the beak) and the most prominent lateral point of the metaphysis with a line drawn perpendicular to the long axis of the tibial diaphysis . VI) CLASSIFICATION In 1952, Langenskild classified infantile tibia vara according to the degree of metaphysealepiphyseal changes seen on radiographs, with six stages varying with advancing age. General prognostic guidelines were also provided. Restoration to normal was common in stages I and II disease and possible in stages III and IV disease, whereas stages V and VI disease were associated with recurrence and permanent sequelae after treatment by mechanical realignment (osteotomy). Although Langenskild's classification was primarily intended to be a radiographic description of infantile tibia vara, prognostic implications have gradually been derived from later studie. In 1964, Langenskild and Riska reported that a simple osteotomy could cure the deformity in patients 8 years old or younger.In the few cases in which simple osteotomy failed, inadequate surgical correction was implicated. Radiographic stage progression of the deformity was thought to be a consequence of skeletal maturation rather than an indication of progressive inhibition of medial physeal growth and worsening of the condition. The premise that 8 years is the critical age up to which the condition is surgically curable has undoubtedly resulted in a certain complacency in treating young children, particularly those with demonstrable stage progression. A number of investigators have reported difficulty applying the Langenskild classification to predict outcome in their own patients.
1. Infantile Blounts Disease Abnormal tibia vara More common and usually affects both extremities Classic presentation is in a child who is overweight and who begins walking before 1 year of age; disease is associated with internal tibial torsion. 2. Adolescent Blounts Disease Less severe than infantile forms and more often unilateral The epiphysis appears relatively normal and does not have the beaking seen in infantile forms. The most characteristic radiographic finding is widening of the proximal medial physeal plate.
VI) CLINICAL MANIFESTATION Genu varum (bowed legs) normal in children less than 2 years old. Blounts disease (tibia varum) is best divided into two distinct entities: invantile (0-4 years of age) and adolescent (over 10 years of age). Infantile Blounts disease: more common and usually affects both of extremities. It occurs more often in the overweight child who begins walking at less than 1 year of age and is associated with internal tibial torsion. Adolescent Blounts disease: less severe and more often unilateral. The child walks with an outward thrust of the knee; in the worst cases there may be lateral subluxation of the tibia.
VII) TREATMENT Untreated infantile tibia vara generally results in a nonresolving and sometimes progressive varus deformity that produces joint deformity and growth retardation, which can then be corrected only with complex surgical procedures. Even when such surgery is performed, substantial articular disruption of both compartments of the knee may have already occurred.Thus, once the radiographic diagnosis of infantile tibia vara is certain, the orthopaedist should recommend treatment immediately because patients treated in the early stages of the disease have a better prognosis. There is no justification for simply observing a patient with an unequivocal diagnosis. Treatment choices and prognosis depend greatly on the age of the patient at the time of diagnosis, which should be the same age at which treatment is recommended. Orthoses. If the child is younger than 3 years of age and the lesion is no greater than Langenskild stage II, orthotic treatment is recommended because 50% or more of these patients can be successfully treated with braces, especially if they have only unilateral involvement. There may be an inclination to brace patients before a true Blount lesion is visible on radiographs, particularly when the MDA is suggestive of varus progression. Thus, when evaluating the reported good outcomes from brace treatment, one must realize that some patients probably had physiologic genu varum rather than true infantile tibia vara. Nevertheless, orthotic treatment appears to affect the natural history favorably The type of orthosis prescribed and the length of time that the orthosis is worn during a 24-hour period vary. Raney and associates used a knee-ankle-foot orthosis (KAFO) that produced a valgus force by three-point pressure in 60 tibiae (38 patients), with lesions in 54 tibiae (90%) resolving without surgery. Significant risks for failure included ligamentous instability, patient weight above the 90th percentile, and late initiation of bracing. Of the 54 tibial lesions that resolved, 27 were treated by full-time orthotic use, 23 by nighttime use only, and 4 by daytime use. Three of the six tibiae requiring surgery had been treated with full-time orthotic use and three with nighttime use only. Based on these findings, the authors conjectured that nighttime-only bracing might be as efficacious as full-time bracing, although they acknowledged that inherently one would expect daytime use (i.e., during weight bearing) to be the most important factor in successful orthotic treatment. On the other hand, Zionts and Shean reported daytime ambulatory bracing to be successful in altering the natural history of tibia vara in patients younger than 3 years with Langenskild stage I or II disease. We have used conventional KAFOs, conventional hip-knee-ankle-foot orthoses (HKAFOs), and elastic KAFOs in the treatment of infantile Blount's disease. Since 1987, the elastic Blount brace, a medial upright design that uses a wide elastic band just distal to the knee joint, has been used almost exclusively because of its ease of fabrication and smaller profile. With this orthosis, 65% of tibiae had successful outcomes at an average follow-up of 5.9 years. However, corrective osteotomies for one or both extremities were eventually required in 70% of patients with bilateral involvement, as opposed to only 6% of patients with unilateral involvement. All of the patients were instructed to use the brace during the day (i.e., during weight bearing). Depending on the patient's physician, some patients were encouraged to use the brace for 20 to 24 hours per day. Treatment of Langenskild Stage II Lesions. Surgical treatment in the early stages of the disease (stage II) is crucial to achieve permanent and lasting correction and to avoid the sequelae of joint incongruity, limb shortening, and persistent angulation. Patients with stage I or II disease have a significantly lower incidence of repeat osteotomy than do those with stage III disease.Surgical overcorrection of the mechanical axis to at least 5 degrees of valgus by 4 years of age, along with lateral translation of the distal osteotomy fragment, is believed to be optimal. Such overcorrection ensures that the supine correction attained at surgery will be sufficient to translate the mechanical axis into the lateral compartment of the knee once the patient begins bearing weight. Overcorrection of the mechanical axis offsets the tendency of the knee to go back into varus as a result of any sloping of the medial epiphyseal surface and relaxation of the lateral ligaments. Although Schoenecker and colleagues reported that correction to within 5 degrees of neutral alignment would prove adequate, most authors recommend physiologic valgus or overcorrection. Based on the physeal inhibition phenomenon proposed by Cook and associates, overcorrection to absolute valgus alignment is required to reverse the excessive compressive forces medially and allow a Langenskild II or III physis not already irreversibly damaged to respond to such mechanical unloading. Treatment of Langenskild Stage III Lesions. Stage III lesions can respond to corrective osteotomy alone in patients older than 4 years. However, the longer the delay in surgery after 4 years of age, the greater the risk for recurrence, which is not uncommon with stage III lesions. Thus, because of the worsening prognosis, neither observation nor orthotic treatment is recommended beyond this age, especially if the deformity exceeds 10 degrees of femorotibial varus. Treatment of Langenskild Stages IV/V Lesions. Lesions greater than stage III cannot be definitively corrected by simple mechanical realignment because physiologic physeal arrest has already occurred by stage IV.Even though no bony bridge can be visualized by tomographic methods in stage IV or V lesions, physeal damage has progressed to the point where stages IV and V lesions effectively act as medial physeal arrests. Treatment of Langenskild Stage VI Lesions. Treatment of stage VI lesions with established bony bridges must also be individualized. Factors to be considered are patient age, the amount of skeletal growth remaining, and the degree of deformity of the joint surface. If the patient has less than 2 years of growth remaining and a relatively normal joint surface, corrective osteotomy with complete physeal closure is a practical means of obtaining and maintaining correction. The osteotomy can performed through the physis so that the mechanical correction is placed as close to the joint as possible and permanent physeal closure occurs. As previously mentioned, resection of the bony bridge with placement of interposition material is appropriate in patients younger than 7 years. Unfortunately, a patient with a stage VI lesion will probably be older than this age limit, when epiphysiolysis is less predictable. Treatment options in patients with more than 2 years' growth remaining include completion of the lateral tibial epiphysiodesis, angular correction, and lengthening, if indicated, usually during the same treatment session.In patients requiring limb length equalization with or without correction of deformity, correction by external fixation and distraction osteogenesis is an effective and invaluable method for salvaging a potentially unsatisfactory extremity. Breaking of a physeal bridge by asymmetric physeal distraction has been described as an alternative approach to resection of the bony bridge in children near skeletal maturity.
References : - Solomon, L, Warwick D.L, Nayagam,S. Apleys system of orthopaedics and fractures. 9 th editions. 2010. - Miller M. D, Review of Orthopaedics . 5th edition. 2008.