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Background
Weismann-Netter-Stuhl syndrome (WNS), first described by Weismann-Netter and Stuhl (France) in 1954, is a rare diaphyseal dysplasia associated with anterior tibial bowing and posterior cortical thickening of the tibia and fibula. 1 With most cases having bilateral involvement, 2-4 short stature and tibialisation of fibula are also commonly seen. 5 6 Majority of the literature on the syndrome exists in the form of case reports and series, 1 2 4 5 with most of them failing to address the appropriate treatment guidelines in correcting the deformity. We present two cases with this syndrome who responded well to fixation using an intramedullary tibial nail after one-level/two-level osteotomy of the tibia and fibula along with bone grafting.
Case presentation
Two siblings, a 152 cm tall 22-year-old man and a 148 cm tall 28-year-old woman presented to us with of bowing of both legs. A detailed family history revealed two more siblings (all 4 of the progeny) and their father being affected in a similar manner with none of their progeny or the previous generation suffering from the same affliction ( figure 1 ). Clinical evaluation of both cases showed anterior bowing of bilateral tibia with deformity in the upper limbs. Neck movements, facial features, spine examination and biochemical studies including serum calcium, phosphate and alkaline phosphatase levels, and screening CT of craniovertebral junction were normal. Psychiatric evaluation showed normal intelligence.
Investigations
Radiographs of lower limbs ( figure 2 ) showed bowing of bilateral tibia and fibula with broadening of fibula and thickening of posterior cortices of the tibia and fibula.
Differential diagnosis
Differentials include persistent physiological varus, vitamin D deficiency rickets, renal osteodystrophy, proximal tibial metaphyseal focal fibrocartilaginous defects, blount disease, type IX Ehlers-Danlos syndrome, fibrous dysplasia, hypophosphatasia and osteogenesis imperfecta. 7
Biochemical markers and routine laboratory investigations, such as serum calcium, serum phosphate and alkaline phosphate levels, are used in children to differentiate from congenital syphillis and rickets which are in addition usually associated with a larger and more generalised involvement of the skeleton. 5 The radiographic findings and clinical history of these diseases are also quite different. While rickets involves mainly the metaphyseal region of...