Ulnar dimelia - a review of 24 cases.

Ulnar dimelia is a very rare unilateral congenital upper limb anomaly (CULA) affecting the whole extremity. Treatment remains difficult because of the complexity and multi-level involvement. Twenty-four cases with duplicated ulna, absent radius and polydactyly from seven European centres were reviewed according to a structured list of parameters. At first consultation, median age 8 months (1-178), the shoulder movement was good in 17 patients or poor in six, and the median passive elbow range of motion was 20° (0°-90°). The resting wrist position was flexed in 22/24 patients. Following stretching and splinting, elbow surgery included resection of the lateral proximal ulna in 11 patients and muscle transfers in six to improve passive movement and increase active elbow motion, respectively. Tendon transfers were performed in eight wrists and a pollicization or pseudo-pollicization in 23 patients. Overall, patients demonstrate acceptable function postoperatively. Guidelines for treatment of this severe CULA are presented.Level of evidence: IV.

State of the art review: The pathogenesis and management of Madelung deformity.

Madelung deformity remains a fascinating yet unresolved challenge. There is an increasing awareness for early diagnosis by healthcare providers with improvement in diagnostic modalities, however, the exact mechanisms for the development of the deformity have still to be clarified. While some corrective procedures have been described to effectively address an established deformity, the existing literature lacks clear and evidence-based treatment guidelines on how to proceed in daily practice. This review article aims to summarize the current best evidence on this topic, including particular areas of controversy and areas with need for future research.

Satisfactory thumb metacarpophalangeal joint stability after ligament reconstruction with flexor digitorum superficialis in children with radial longitudinal deficiency.

We investigated thumb joint stability and patient-reported and functional outcomes a minimum of 1 year after flexor digitorum superficialis opponensplasty and ligament reconstruction in 23 thumbs of 20 consecutive children with radial longitudinal deficiency. In total, 15 thumbs had preoperative multidirectional instability in the metacarpophalangeal joint. We reconstructed 22 ulnar and 16 radial collateral ligaments. At follow-up, all the metacarpophalangeal joints were stable ulnarly. Seven metacarpophalangeal joints were unstable radially despite ligament reconstruction but had no related complaints. We recommend the flexor digitorum superficialis opponensplasty as a safe and reliable procedure in hypoplastic thumbs to create stability and augment thumb strength.Level of evidence: IV.

Validity and Reliability of the Thumb Grasp and Pinch Assessment for Children After Reconstruction of Congenital Hypoplastic Thumbs.

PURPOSE: The Thumb Grasp and Pinch Assessment (T-GAP) is a new instrument for evaluating thumb use in children with congenital hypoplastic thumbs. The assessors video-record the children while they perform nine specific activities and score their grasp types using T-GAP. A high T-GAP score indicates more mature grasp patterns. The developers reported the instrument's validity and reliability for index finger pollicization. This study investigated T-GAP's validity and reliability in children with reconstructed hypoplastic thumbs. METHODS: Four hand surgeons and two hand therapists from two hospitals rated video clips of 20 Manske type II and IIIa hands twice in 17 patients who performed the T-GAP at least 1 year after opposition transfer and thumb ligament reconstruction. To investigate the validity, we calculated correlation coefficients for T-GAP scores and clinical outcomes, including thumb ROM, grip and pinch strength, and visual analog assessments of thumb function and appearance. To estimate T-GAP's inter- and intrarater reliability, we calculated intraclass correlation coefficients and their 95% confidence intervals (CIs). RESULTS: Thumb Grasp and Pinch Assessment score showed a strong linear correlation (r = 0.815-0.944) and a moderate to strong nonlinear correlation (ρ = 0.527-0.744) with visual analog scale assessments of thumb function and appearance, respectively; a moderate nonlinear correlation (ρ = 0.464) with grip strength; and a moderate nonlinear correlation (ρ = 0.541) with thumb MCP joint range of motion. The intraclass correlation coefficient for the interrater reliability was 0.892 (95% CI, 0.768-0.954) in round 1 and 0.898 (95% CI, 0.754-0.959) in round 2, and for intrarater reliability, the mean was 0.882 (95% CI, 0.785-0.980). CONCLUSIONS: Thumb Grasp and Pinch Assessment score had a moderate to strong construct validity and a moderate concurrent validity. Both inter- and intrarater reliability was strong. CLINICAL RELEVANCE: This study supports the T-GAP instrument's validity and reliability for assessing functional outcomes in congenital hypoplastic thumb reconstruction.

Biopsies from the Distal Radius after Implantation of Calcium Phosphate Cement.

Background: Calcium Phosphate (CaP) bone cement is gradually replaced by new bone when used as a gap-filler. Details of the re-modelling process are still unclear. Uncertainty is also present as to the possible release of cement particles during the resorption phase causing local soft tissue reactions. The objective of this study was to perform a comprehensive histological investigation of the injectable CaP bone cement used as a void filler in corrective radius osteotomies and adjacent tissue reactions. Methods: Fourteen patients, median age 56 years (18-72), 4 men/10 women, underwent removal of distal radius plates (11 dorsal/3 volar) due to tenosynovitis-like symptoms. Eleven study patients went through corrective osteotomies with CaP bone cement and three were control patients. Previous surgery in three controls consisted in (1) corrective osteotomy with bone graft (dorsal plate), and (2) plated distal radius fractures (1 dorsal/1 volar plate). Biopsies were taken of bone-cement-bone junctions (11), bone-bone graft-bone junctions (1), bone (2) and juxtaposing soft tissue (14). The interval from corrective CaP cement surgery to biopsy was median 1.1 (0.6-2.3) years. Results: Biopsies of bone-cement junctions showed the different stages of new bone formation from CaP to immature bone and later mature well-organised bone. The cement showed signs of osteoclast-mediated resorption. Cement particles, macrophages, multinucleated giant cells (MNGC) and plasma cells were observed in most soft tissue biopsies. MNGC with internalised particles were seen. Macrophages were found along and/or within tendon sheaths in all patients in both groups, but rarely containing cement particles. Conclusions: Gradual re-modelling of the cement into well-organised bone was observed confirming osteoclast-osteoblast coupling. There was no indication that cement particles were the cause of the tenosynovitis-like symptoms.

Calcium phosphate bone cement and metaphyseal -corrective osteotomies in the upper extremity: long-term follow-up of 10 children.

BACKGROUND AND PURPOSE: The evaluation of metaphyseal angular deformities in children includes indication and timing for corrective osteotomy, and possible need for several operations during growth. Gap-fillers are usually autologous bone grafts, which might cause donor site problems. Calcium phosphate (CaP) bone cement may be a possible alternative. PATIENTS AND METHODS: We performed 15 corrective osteotomies from 2007 to 2013 in 10 children, ages 5 to 18, with Norian SRS bone cement as a gap-filler, in the distal radius (12), proximal radius (1), and proximal humerus (2). Due to growth arrest and gradually increasing malalignments 3/10 children needed 1-3 additional corrections. Locking plates and screws were used except in 1 case at first surgery, aged 5 (K-wires). 2 children needed additional limb lengthening with external fixator. RESULTS: All osteotomies healed. Postoperative radiographs and CT scans showed good alignment and gradual transformation of cement into bone. Remodeling was visible intraoperatively in patients needing multiple surgeries. Return to earlier osteotomy sites was unproblematic. No adverse events from using CaP cement were experienced. INTERPRETATION: CaP cement is an alternative to bone grafts in upper extremity metaphyseal corrective osteotomies in children, and also when greater corrections are necessary or several surgeries indicated during the growth period.

Inter- and intra-rater reliability of the Oberg-Manske-Tonkin classification of congenital upper limb anomalies.

On two occasions, five surgeons classified a cohort of 150 consecutive patients with congenital upper limb anomalies according to the Oberg-Manske-Tonkin classification (2020 update). We estimated reliability for the main anomaly code by means of Cohen's kappa (Κ) for ten rater pairs for five common and easily distinguishable anomalies (Group 1), and for all the other anomalies (Group 2). Inter-rater reliability for all patients (n = 150) was substantial, almost perfect for Group 1 (n = 64), but only moderate for Group 2 (n = 86). Intra-rater reliability was higher for all groups. We suggest simplifications to the Oberg-Manske-Tonkin classification and highlight specific requirements for instructions to increase its reliability.Level of evidence: I.

Outcome After Pollicization for Congenital Thumb Deficiency: A Cohort Study of Cases in a Single Unit, 1987 to 2016.

PURPOSE: To investigate the functional and aesthetic outcomes in a cohort with pollicizations performed due to congenital anomalies in our hospital. METHODS: From 1987 to 2016, we performed pollicizations in 32 hands of children aged 1 to 8 years (median, 2 years). We followed-up on 31 of the hands from 1 to 31 years (median, 10 years) after the procedure. The participants and their caregivers self-assessed their function and appearance with visual analogue scales and patient-reported outcome measures (Patient-Reported Outcomes Measurement Information System Pediatric Upper Extremity; the short version of the Disability of Arm, Shoulder and Hand Outcome Measure; and EQ-5D-3L). We examined the hands with regard to motion, strength, sensitivity, and function. RESULTS: There were 2 complications and 6 reoperations. Participants with mild anomalies (radial longitudinal deficiency Bayne type N/0 to 2) had better subjective and objective hand function than participants with severe anomalies (radial longitudinal deficiency Bayne type 3-4, ulnar dimelia, 5-finger hand). Hands with preoperatively near-normal index fingers had, in most cases, good thumb opposition and pinch, and hands in both groups benefited from the creation of a cylinder grip. Grip and pinch strength were lower than reported in cohort studies where an additional opponensplasty had been performed. CONCLUSIONS: Hands with severe congenital anomalies also benefited from the procedure. We recommend a simplified follow-up program to identify cases where additional surgeries to enhance strength should be considered during growth of the child. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Satisfying Clinical and Functional Results in 12 Apert Children Treated With Soft Tissue Distractor.

BACKGROUND: The complex syndactyly in Apert syndrome hands is challenging to operate. The synostosis and tightness of skin between third and fourth digits lead to severe coverage problems during ray release. A soft tissue distractor can simplify the release with the aim to keep all 10 fingers. METHODS: A retrospective follow-up of 12 patients/24 hands, median age 8 years (6 to 17 y), 6 boys and 6 girls, operated between 2000 and 2013 was done from 2015 to 2016. The surgical management started with syndactyly release of the first and fourth web, and later of the second. The third stage was placing a soft tissue distractor on the third and fourth finger after osteotomy on the synostosis between them. Four weeks of distraction and 2 weeks of rest resulted in regenerated skin between the digits giving much better coverage of the released digits at time of separation 6 weeks later. Assessment of hand function, grip strength and completion of the Patient Reported Outcome Measure CHEQ was performed. RESULTS: Soft tissue coverage at the time of digit separation was considerably facilitated. We experienced 2 infections in 2 hands. In 18/24 hands median 2 (1 to 3) small full thickness skin grafts were needed, usually for coverage of the base of the digits. All wounds healed well. The children managed different practical tasks well, alternating between best functioning grip depending on the activity. According to CHEQ, the children did median 19 (13 to 27) activities independently and median 8 (2 to 15) nonindependently, of a total of 29. Peak strength values for 10/12 children were for the right hand median 17.8% (9.6% to 40.6%) of normative data and for left hand median 13.6% (2.4% to 20.5%) of normative data. CONCLUSION: Soft tissue distraction facilitates the treatment of acrocephalosyndactyly hands, giving 5-fingered hands. Apert children manage many activities independently but struggled with fine motor skills demanding strength. LEVEL OF EVIDENCE: Level IV evidence.

CaP cement is equivalent to iliac bone graft in filling of large metaphyseal defects: 2 year prospective randomised study on distal radius osteotomies.

The purpose of this prospective randomised study was to compare the clinical and radiological outcomes of injectable CaP bone cement with corticocancellous bone graft used to fill voids after corrective opening wedge osteotomies in the distal radius. 17 women/3 men, median age 56 (51.3; 61.0), underwent an open-wedge osteotomy of a dorsal malunion in the distal radius randomised to filling the defect either with bone graft (10) or CaP bone cement (10). Dorsal titanium locking plates were used and the wrist was plastered for 8 weeks. Follow-ups for 24 months included X-rays, CT scans, VAS on wrist and iliac crest, grip strength, ROM, Quick-DASH and Gartland & Werley scores. No difference was found between the 2 groups as to clinical outcome or radiological results with no loss of reduction. One bone graft patient developed a pseudarthrosis and one CaP patient suffered a plate fracture 6 months post-operatively. CaP bone cement is a good alternative to bone graft as a void filler in open-wedge osteotomies of the distal radius. The procedure is shorter, easier with the post-operative advantage of no donor site pain. Level of Evidence Randomised controlled trial. Level I evidence.

Effect of soft-tissue attachment on tibial fracture healing in rats.

PURPOSE: To compare tibial fracture healing in rats with or without soft-tissue attachment. METHODS: The left tibias of 30 Wistar rats were osteotomised and equally randomised into 3 groups. In the avascular segmental fracture group, an 8-mm bone segment were first removed and then immediately put back. In the vascular segmental fracture group, the 8-mm bone segment was not displaced, with periosteal and muscular attachments. In the simple fracture group, a simple fracture in the middle tibia was made. All tibias were then stabilised with an intramedullary nail through the patellar tendon, and the wound was closed with sutures. After 8 weeks, all left tibias and 9 of the intact right tibias were harvested. Bone mineral content and density of the calluses were assessed using dual energy X-ray absorptiometry scanning. The maximum torsional strength, rigidity, and energy to failure of the tibias were measured. RESULTS: All tibias healed (callus formation), without mal-union or pin migration. The 3 groups did not differ significantly in terms of torsional strength, rigidity, energy to failure, bone mineral content, and bone mineral density. The mean torsional strength of the 9 intact tibias was significantly higher than the healed tibias (14.9 vs. 10.6 Nmm, p=0.021). CONCLUSION: Soft-tissue detachment from bone segments did not impair bone healing in rats.

Calcium phosphate bone cement: a possible alternative to autologous bone graft. A radiological and biomechanical comparison in rat tibial bone.

BACKGROUND: Donor site morbidity is a problem after autologous bone transplantation. An injectable CaP bone cement indicates properties similar to bone. MATERIALS AND METHODS: Double osteotomies on rat tibias were performed. The intercalated segments were avascular (10), vascular (10), or avascular with Norian SRS(®) CaP bone cement replacing cancellous bone (10). Controls were non-operated contralateral tibias (15). All osteotomies were stabilised with an intra-medullary nail. After 8 weeks, all rats were killed. The harvested tibias were compared using X-ray, DEXA scanning, microCT scans and a biomechanical torsional test. RESULTS: No difference in healing processes or biomechanical results has been found between the avascular bone graft, vascular bone graft and CaP bone cement groups. CONCLUSION: The injectable CaP bone cement confirms its similarities to bone, implying that it can be used as an adjunct to secure bone fragments and as a possible alternative to autologous bone transplantation in clinical practice.