Jowral of Bede Orthopedics ESN ESR en Idiopathic or Primary Windswept Deformity: The Etiological Significance of the Radiological Findings Olusola O. A. Oni and H. Keswani Subdepartment of Orthopaedic Surgery. University of Benin and Teaching Hospital, Benin City. Nigeria ‘Summary: The radiographs of 16 knees in eight children who had a valgus deformity of one knee in association with a varus deformity in the other knee ‘were studied. The condition, idiopathic or primary windswept deformity, oc- urs in 1- to 3-year-old healthy children with normal developmental milestones, ‘The onset of radiological and clinical deformity is abrupt. The disease is physeal osteochondrosis, and two varieties of it are identical to Blount’s tibia Vara and to tibia valga, respectively. Key Words: Windswept deformity — Blount’s disease—Tibia vara—Tibia valga—Osteochondrosis, Idiopathic or primary windswept deformity (Fig. 1) refers to the condition in which a valgus defor- mity in one knee is associated with a varus defor- in the other (5). The disease has received little attention in the English literature. It may occur in many situations, but it is the infantile variety of unknown etiology that is discussed here. Itis a dis- ease most commonly described in the black race, and the condition is not uncommonly encountered in Nigeria. The deformity arises spontaneously in the second or third year of life in a previously healthy child with normal developmental mile- stones and is not preceded by any local or systemic illness (10). PATIENTS AND METHODS Eight children seen at the University of B Teaching Hospital satisfied the criteria for the di agnosis of idiopathic or primary windswept defor- mity (10) and form the basis of this report. The chil- dren were all healthy and had no history of local or systemic illness antedating the deformity. They all had normal developmental milestones. Inall cases, the deformity was noted shortly after “This study was presented at the Conference of the West AP rican Assocation of Radiologists. Benin Cty. 1982. ‘Address correspondence and reprint requests to De. Ooi at his present address: I8 Ellenborough Place. Rochampton Close Condon SWIS, England, 293 the child began to walk, at about 9 months to I year on the average. It could not be determined with certainty in all instances whether the varus or the valgus deformity developed first, but most parents found the valgus deformity most alarming in its ra- pidity. ‘None of the patients had any clinical or radiolog~ ical evidence of rickets or other generalized bone disorder. Levels of serum calcium, phosphorus, and acid and alkaline phosphatases were all within normal limits, There was no clinical or hematolog- ical evidence of hemoglobinopathy, anemias, or other disorders in any patient. Three of the patients were males and five were females. The age range was 18 months to 4 years. The length of time from onset to presentation ranged from 6 months to 2 years. All the patients were from the immediate vicinity of Benin City and were referred to the Unit from a variety of sources. The radiographs of the knees were analyzed ac- cording to the type and site of lesions observed. (Case reports Case 1 ‘A 3-year-old male child, weighing 16.8 kg and 92 com tall, the last of six siblings, walked when he was about I year old. Shortly afterward he developed a varus deformity of the left knee followed by a rap- idly developing valgus deformity on the opposite side. There was no family history of the disorder. The child was essentially normal until the knee con-294 0.0. A. ONI AND wor FIG. 1. Photograph of a boy, 4 years old, wth idiopathic oF primary windswept deformity dition developed, and he had no evidence of rickets or other generalized skeletal disease. He was treated with bilateral corrective high tibial osteot- omies Case 5 This 2-year-old female child, weighing 15 kg and 86 cm tall, the last of six children, had normal de- velopmental milestones and walked at 10 months. She gradually developed a valgus deformity of the right knee and by 18 months of age had developed full-blown windswept deformity. There was no ev- idence of rickets or other generalized skeletal dis- ease. She was treated with bilateral serial corrective plaster knee cylinder casts. Case 8 This 18-month-old female child, weighing 12 kg and 80 cm tall, the third of four children, had normal Fig. 2. Radiograph of Type A idiopathic or primary windswept det FIG. 3. Radiograph of Type 8 idiopathic or primary windswept def FIG. 4. Radiograph of Type C idiopathic or primary windswept d reproduced with permission of Archives of Disease in Childhood) J Pediatr Onhop. Vol. 4, No.8, 1984 Tait H. KESWANI developmental milestones and had walked only a few months before sustaining an injury to her lower limbs while playing with other children. Because there were no adults immediately present, the na- ture of the injury and the specific area(s) of the limb injured could not be ascertained. The child recov- ered without medical assistance but shortly after this incident developed the characteristic knee de- formities. She had no clinical, radiological, bio- chemical, or hematological evidence of any ‘bony disorder. She was treated with bilateral serial cor- rective plaster knee cylinder casts. RESULTS The pathological lesions observed radiographi- cally in idiopathic or primary windswept deformity (Figs. 2-4) are specific. There may be a simple wedging of the ossific nucleus, or it may be irreg- ularly ossified with increased density and fragmen- tation. The metaphysis often showed broadening and beaking, and in more severe cases, angulation, depression, and microfractures are observed. The diaphysis immediately below may be thickened, an- sled, or rotated, while the joint invariably showed some degree of widening. Three radiological varieties of idiopathic or pri- mary windswept deformity were identified (Table 1), and their relative incidence in our patient series shown in Table 2. It is suggested that both knees are more likely to be involved in radiological dis- ease than one knee alone. The distribution of radiological lesions in each variety of idiopathic or primary windswept defor- mity is shown in Table 3. Radiological evidence of disease is more likely to be found in both the lower end of the femur and the upper end of the tibia than in either bone alone. formity oF tibia vas ‘associated with simple knockknee. formity or tibia valga assgciated with simple bowleg, Jaformity or combined tibia vara-valga. (Figs. 3 and 4RADIOLOGY OF WINDSWEPT DEFORMITY 295 TABLE 1. Radiological varieties of idiopathic or primary windswept deformity “Type A Unilateral tibia vara or Blount’s disease combined ‘with physiologial knockknee in other knee Type H Unilateral tibia valga combined with physiological bowleg in other knee ‘Type C Bilateral disease: combined iia vara—valga DISCUSSION The clinical and radiological features of this con- dition suggest that idiopathic or primary windswept deformity is an osteochondrosis. The onset of cl ical and radiological alterations is abrupt. The chil- dren are healthy, and the disease arises from a for- merly normal epiphysis. The pathological lesions observed radiographically are identical to those in the osteochondroses (9), and the disease can be classified as a physeal osteochondrosis The radiological features are similar to that of osteochondritis deformans tibiae described by Blount (3) and Golding (6). There is retarded growth on the side of maximum pressure on the upper tibial epiphysis and/or lower femoral epiphysis (4). The pathological lesions may occur in the distal end of the femur, in the upper end of the tibia, or in both simultaneously (Table 3). The medial portion of the epiphysis in genu varum or the lateral portion in enu valgum may become wedge-shaped and of denser consistency while the adjoining metaphysis develops a beaklike projection (2,10). The medial or lateral portions of the epiphysis, the growth plate, and the adjoining metaphysis and diaphysis may all be affected. It follows, therefore. that the etiological consid cations that apply to Blount’s disease and other similar conditions such as tibia valga must also apply to idiopathic or primary windswept defor- mity, Ithas been suggested that this condition could arise from the mechanical pressure effects of a rap- idly occurring deformity at the time of a growth spurt (10) or from susceptibility to stress factors such as intercurrent illness at a period of potential TABLE 2. Relative incidence of each variety of idiopathic or primary windswept deformity in ‘our patient series No. “Type A unilateral tibia vara 2 ‘Type B unilateral tibia vale i ‘Type C tibia vara—valga 5 ‘ Total TABLE 3. Distribution of lesions in each variety of idiopathic or primary windswept deformity according to the affected bone “Tibial Femoral Femorotibiat None Total Wea 2 4 Wes 1 = 7 - oO Typec 4 6 - 0 Teal 4 5 6 epiphyseal instability (7). The sexual, racial, and ‘geographical distribution would seem to suggest a genetic dysplasia, and similar lesions have been considered to be due to developmental abnormali- ties (8). However, these theories remain without proof at the present time, and unilateral disease is more likely to be the result of trauma or infection involving the growth plate. Idiopathic or primary windswept deformity is a heterogeneous condition, and three varieties were identified in this study (Tables | and 2). In Type A. the disease comprised tibia vara associated with a nonpathological valgus deformity (knockknee) in the other knee (Fig. 2). In Type B, tibia valga was associated with a nonpathological varus (bowleg) component (Fig. 3). In Type C, tibia vara was as- sociated with tibia valga, and both knees were in- volved in the discase (Fig. 4). The diseased com- ponents differed from the nonpathological compo- nents in that definite radiological lesions were observed in the epiphysis and metaphysis of dis- eased bone: Type A idiopathic or primary windswept defor- mity (Fig. 2) is similar in all respects to unilateral Blount’s disease except for the presence of a valgus, deformity in the other knee. The radiological fea- tures of the diseased varus component are identical to that in Blount's disease, the age group involved is comparable, and the ethnic distributions overtap. This suggests that Type A idiopathic or primary windswept deformity is really Blount’s disease in association with a valgus deformity in the other knee. This valgus deformity is due to idiopathic ‘genu valgum (simple knockknee), which is common in this age group (7). Further, changes similar to those seen in tibia vara but on the lateral side of the tibia may occur in circumstances similar to that of Blount’s disease (1). This is the so-called reversed Blount’s disease or tibia valga. If it occurs simultaneously with a nonpathological varus deformity of the other knee, ‘a windswept deformity will result (Fig. 3). This is ‘Type B idiopathic or primary windswept deformity, and the varus component is due to idiopathic genu varum (simple bowleg), which is also common in this age group (8). J Pediatr Orthop, Wo 4. No.3, 1986296 0. 0. A. ONI AND H. KESWANI REFERENCES Bateson EM. Nor-rachitc bowleg and knockknee deform ties in young Jamaican children, Br Radia! 196839 92. Bathfield CA. Beighton PH. Blount disease: a review of ae tiological factors in 110 patients. Clin Orthop 1978138229, 3. Blount WP. Tibia vars. J Bone Joint Sung 193731921 4, Brennan JJ. Guarino CA. An unusual ease of metaphyseal growth disturbances. J Bone Join! Surg (Am) 1961:3:88 J Pediatr Orthop, Vol. 4, No.3, 1986 Fulford GE, Brown JK. Postion as a cause of deformity in children with cerebral palsy. Dev Med Child Neurol 19618308. 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