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Purpose Recurrent genu valgum deformity complicates treatment of congenital femoral deficiencies (CFD) and fibular hemimelia (FH). We analysed factors influencing recurrence. Methods Patients who underwent limb lengthening or deformity correction for CFD and/or FH were reviewed. Radiographs after surgery and after a minimum of a further six months were analysed. Change in parameters of mechanical axis deviation per month (Δ MAD/month) and of angle per month were calculated. These parameters were tested against cofactors patient age, baseline MAD, type of CFD and FH, severity of ball-and-socket joints, ankle-joint stiffness, absence of cruciate ligaments and resection of the fibular anlage. Results Recurrent valgus deformity was found in 23 of the 42 limbs included with a mean change of MAD of 23.4 mm (5-60 mm). There was no significant difference between patients with Δ MAD/month <0.5 mm versus >1 mm regarding MAD in the first radiograph and patient age. CFD cases Pappas types VII and VIII showed a Δ MAD/month of 1.6 mm, whereas milder cases of Pappas IX showed a Δ MAD/month of 0.8. Mild FH (type Ia) showed a mean Δ MAD/month of 0.39 mm, whereas mean Δ MAD/month for FH type Ib/II was 0.72 mm. In FH type II cases, mean Δ MAD/month was 0.79 mm after resection of the fibular anlage compared with 1.98 mm in those without resection.Conclusions Recurrence in FH and CFD was not dependent on patient age but partly on FH and CFD type. Limbs with more severe ball-and-socket knee joints showed more recurrence. Overcorrection depending deformity type should be performed.

We describe one case of forearm deformity in a patient affected by multiple cartilaginous exostoses - also known as the forearm 'candy stick deformity'. Surgical treatment usually focuses on the correction of the wrist deformity without correcting the forearm shortening, the latter not being given the same consideration as lower limb shortening. In the presented case, radius and ulna corticotomies were performed and distal forearm deformity and shortening were corrected by two independent monoaxial external fixators, with full pronosupination. It is our belief that simultaneous treatment of forearm shortening and deformity not only results in an improved clinical and functional result but also provides significant psychological benefit. We recommend a long-term follow-up.

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Posttraumatic radioulnar synostosis (RUS) is a rare event following forearm fractures. Consequences are disabling for patients who suffer from functional limitation in forearm pronosupination. Distal RUS are even more rare and more difficult to treat because of high recurrence rates. The patient we describe in this paper came to our attention with a double distal RUS recurrence and a Darrach procedure already performed. We performed a radical excision of RUS and interposition with a vascularized dorsoulnar artery (DUA) adipofascial perforator flap. Four years after surgery, the patient shows the same complete range of motion in pronosupination, and MRI confirms that the flap is still in place with signs of vascularization. Simple synostosis excision has been proven ineffective in many cases. Interposition is recommended after excision, and biological material interposition seems to be more effective than foreign material. Surgeons are increasingly performing vascularized interposition, and the results are very encouraging.

We describe a unique case of a bilateral osteochondrosis of the femoral heads, similar to Perthes disease, in a boy affected by Alagille syndrome. This is a rare genetic syndrome, caused by vascular anomalies, and characterized by five main features: hepatic, cardiovascular, ophthalmological, skeletal malformations, and characteristic facial appearance. The most frequent skeletal finding is the 'butterfly vertebra'. We have followed the patient from the age of 5 years to the age of 20 years. We performed two bilateral valgus osteotomies when he was 10 years old to limit the progression of the deformity. We believe that the association of a bilateral osteochondrosis of the femoral heads with Alagille syndrome, a disease characterized by a vascular etiology, supports the hypothesis of angiogenic pathogenesis of Perthes disease.

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