[Clubfoot associated with tibial and fibular hemimelia]. / Der Klumpfuß bei tibialer und fibularer Hemimelie.

AIM: Clubfoot is rarely associated with tibial or fibular hemimelia. Treatment is complex and in most of the cases extensive surgery is required. At present experience with Ponseti casting is limited. We describe casting and surgical treatment of 10 clubfeet associated with tibial and fibular hemimelia. MATERIAL AND METHOD: Between 1.1.2004 and 31.12.2009 398 clubfeet were treated with casting in our institution. In the same period 10 clubfeet were associated with fibular or tibial hemimelia. Treatment started in 9 clubfeet with Ponseti manipulation and casting. We used the classification of Weber for tibial hemimelia and the Kalamchi-Achterman classification and Paley classification for fibular hemimelia. Data of all patients were prospectively documented and the result of the foot deformity was evaluated before a first lengthening procedure. Documentation included patient data, associated foot pathologies, surgical procedures, functional results. Functional results were evaluated before the first lengthening procedure started. RESULTS: Three patients had tibial hemimelia, two Weber type 1, one Weber type 2, one Weber type 3. five patients had fibular hemimelia, Paley type IV or Kalamchi-Achterman Type IA. One child had bilateral fibular hemimelia. The prospective leg length discrepancy ranged from 3.2 cm to 14 cm. Four feet had initially a successful treatment with casting. In a type 2 according to Weber we performed an ankle reconstruction procedure to correct tibiofibular diastases. Four feet underwent PMR. We had four relapses. Two equinus relapses were treated with a posterior release. Two severe relapses were finally corrected with resection of the coalition and midfoot osteotomies. In a Weber type 3 case a complex reconstruction was performed using an Ilisarov and a TSF frame. Functional results showed in a mean follow-up of 42.2 months (24-72 months) a dorsiflexion between 5 and 20° (Ø 7.7°) and a plantarflexion between 10 and 40° (Ø 26.1°). CONCLUSION: Treatment of clubfoot associated with tibial or fibular hemimelia with the Ponseti technique is limited because of complex hindfoot deformities including tarsal coalitions. Nevertheless treatment after birth starts with casting. Only mild cases of hemimelia without coalition can be corrected with the Ponseti technique. In a case of tibiofibular diastasis successful casting is possible, but extensive surgery is often necessary. In more severe cases we do not recommend casting. In these cases surgical treatment, including posteromedial release, osteotomies for the hindfoot, resection of coalitions or complex osteotomies with Ilisarov or TSF frame is the treatment of choice.

[Mid-term results of idiopathic clubfeet treated with the Ponseti method]. / Mittelfristige Ergebnisse der Behandlung des idiopathischen Klumpfußes mit der Ponseti-Methode.

AIM: The Ponseti method is accepted worldwide for the treatment of congenital clubfoot. We report about our experience in a 7-year period. The purpose of the study was to evaluate the history of well treated feet between primary correction and the age of 5-6 years with relapse rate and functional results. MATERIAL AND METHOD: Between 1.1.2004 and 31.12.2005 we treated 71 patients with 102 idiopathic clubfeet with the Ponseti method. All patients were prospectively evaluated. We used the Pirani score. The patients' results were documented when the children started to walk and before primary school. The results were compared and statistically evaluated. We used the McKay score and measured the talocalcaneal angle on lateral and a. p. radiographs. RESULTS: 89 % clubfeet were successful treated with the Ponseti method. At walking age plantar flexion was between 30° und 50° (∅ 42°) and dorsiflexion between 5° and 30° (∅ 25°). Before primary school plantar flexion was between 30° and 50° (∅ 37,8°) and dorsiflexion between 0° and 25° (∅ 13,9°). Using the McKay score we had 91 % excellent or good results. 31 % cases had surgical treatment of a relapse. In the relapse group 82 % had an excellent or good result according to the McKay score. CONCLUSION: The Ponseti method is a very effective technique to treat idiopathic clubfeet. In the first 5 to 6 years of age there is a significant loss of range of motion. The relapse rate is comparable to those of other clubfoot treatment concepts. The relapse treatment of the Ponseti technique, with recasting, tibialis anterior tendon transfer and Achilles tendon lengthening leads to good functional results.

[Elastic stable intramedullary nailing as alternative therapy for the management of paediatric humeral shaft fractures]. / Die elastische stabile intramedulläre Nagelung als Therapiealternative in der Versorgung kindlicher Humerusschaftfrakturen.

AIM: We report about our experiences with the elastic stable intramedullary nailing (ESIN) of traumatic humeral shaft fractures in children and adolescents. METHOD: Children with traumatic humerus shaft fractures who were treated by ESIN were included. All patients underwent clinical follow-up examinations. RESULTS: 31 children (average age 11.4 years) with traumatic humeral shaft fractures could be included. There were 14 oblique, 12 transverse and 5 wedge fractures. In 5 cases the fracture was located in the proximal third, in 22 cases in the middle third and in 4 cases in the distal third. After an average period of 32 months a follow-up-examination with the Constant-Murley score was performed. All children attained 100 points and all patients could take part in sports activities like before the accident. 30 children and their parents were very satisfied with the treatment success and 1 patient was satisfied. The following complications were seen: 1 postoperative damage of the radial nerve in a patient with secondary fracture dislocation (complete remission), 1 skin irritation, 1 fracture dislocation with axial deviation, 1 secondary dislocation of the nails after a second accident and 1 secondary axial deviation. CONCLUSIONS: Elastic stable intramedullary nailing (ESIN) of humerus shaft fractures has a low complication rate if attention is paid to biomechanical principles. The observed complications are based on mistakes concerning the indication or technical errors. The ESIN shows very good functional and cosmetic results. It allows an early functional and cast-free follow-up with a quick pain reduction. The ESIN of humeral shaft fractures is a minimally invasive, simple and well reproducible technique with a steep learning curve. Because of the excellent objective and subjective results, the operative stabilization of humerus shaft fractures with ESIN should be recommended to the patients and their parents.

[Treatment of congenital clubfoot with the Ponseti method]. / Die Behandlung des kongenitalen Klumpfusses mit der Ponseti-Methode.

AIM: The primary therapy for congenital clubfoot is non-surgical involving manipulation and serial casting. With traditional casting, relatively large numbers of feet require extensive surgery to achieve full correction. The purpose of this study was to evaluate the efficacy of the Ponseti method. METHODS: Between 1.1.2004 und 31.12.2005, 29 patients with 41 clubfeet were treated with the Ponseti method. Only patients without any prior treatment were included. Classification followed Pirani's score. The number of casts to full correction, tenotomies, number of posteromedial releases, dorsi-, plantarflexion and hindfoot position were documented. The follow-up time was 1-9 months, the average follow-up time was 9.1 months. RESULTS: 39 clubfeet were successfully treated with the Ponseti method. The average Pirani score was 4.9. Percutaneous tenotomies were necessary in 34 of the clubfeet. Average dorsiflexion was 19 degrees and plantarflexion 42 degrees . After failed Ponseti treatment 2 feet were treated with a posteromedial release. CONCLUSION: With the Ponseti method the need for extensive corrective surgery is greatly reduced. We recommend the Ponseti method as standard therapy in clubfoot management.

Early age of onset in fatal familial insomnia. Two novel cases and review of the literature.

Fatal familial insomnia (FFI) is a prion disease exhibiting the PRNP D178N/129M genotype. Features of this autosomal dominant illness are progressive insomnia, dysautonomia, myoclonus, cognitive decline and motor signs associated with thalamic nerve cell loss and gliosis. In contrast to the new variant of Creutzfeldt-Jakob disease (vCJD) the onset of FFI is in middle to late adulthood. We report two male patients who belong to a large German FFI kindred. They were examined clinically, and postmortem neuropathological examination was carried out in collaboration with the German reference centre for prion disease. Additionally, the prion protein gene (PRNP) was analysed. To identify further patients with disease onset under 30 years of age a comprehensive literature review was carried out. Two male patients presented with typical symptoms of FFI at the age of 23 and 24 years. In their kindred, the age of onset has never before been under 44 years of age. Our literature review identified five additional early onset cases who died at age 21 to 25 years. In all 22 reviewed FFI families the median manifestation age was 49.5 years. Although phenotypic variability of FFI is common, age of onset under 30 years has been considered to be a hallmark of vCJD with a mean manifestation at 27 years of age. Our findings underline that in addition to vCJD, FFI must be considered in cases of young-onset prion disease. This has considerable impact on clinical management and genetic counselling.

Novel polymer-bound chiral selenium electrophiles.

[reaction: see text]. Polymer-bound chiral electrophilic selenium reagents have been developed and applied to stereoselective selenenylation reactions of various alkenes. Different cleavage protocols allow further functionalization of the addition products leading to improvements in selenium-based solid-phase chemistry.