Free Vascularized Rectus Abdominis Transfer With Pedicled Intercostal Innervation for Functional Reconstruction of the Elbow in Total Brachial Plexus Palsy.

PURPOSE: Reanimation of palsied upper limbs usually follows an escalating pattern of nerve repair, nerve transfers, and musculotendinous transfers and culminates in free functioning muscle transfers. When there are no other musculotendinous options, we explored the possibility of transferring the rectus abdominus to the biceps by maintaining the nerve pedicle but dividing the vascular pedicle and anastomosing it to the brachial artery. METHODS: We performed anatomical dissection of the nerve and blood supply of 6 rectus abdominis muscles in 3 cadavers. A retrospective analysis of 4 patients in whom a rectus abdominus muscle transfer with a pedicled nerve, but free vascular supply, was then performed. RESULTS: The anatomical feasibility study demonstrated that it was possible to elevate the rectus abdominis on its intercostal nerve supply to the midaxillary line, allowing the muscle to be pedicled on its nerve supply and be transferred to the arm to reconstruct biceps. The vascular supply could be reestablished by anastomosis of inferior epigastric vessels to the brachial artery and veins. In 4 patients, elbow flexion strength of M3 or greater was achieved. Average elbow range of dynamic flexion was 120° (range, 92° to 131°). Shoulder stability and external rotation improved in all patients with resolution of shoulder subluxation. Two patients developed donor site hernias requiring mesh reconstruction. Complications included a hypertrophic recipient site scar in one patient, and recipient site wound dehiscence in another. CONCLUSIONS: Rectus abdominus can be transferred to reconstruct elbow flexion when other musculotendinous transfers are unavailable and as an alternative to free functioning muscle transfer. However, rectus abdominus transfer still requires microsurgical skills for the vessel anastomoses. This is an effective procedure for functional reconstruction of the elbow and adds to the armamentarium in the management of brachial plexus pathology when other transfers are unavailable. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Surgical Techniques for Revascularization in Abdominal Wall Transplantation.

BACKGROUND: Abdominal wall vascularized composite allotransplantation (AW-VCA) can be considered as a technically feasible option for abdominal wall reconstruction in patients whose abdomen cannot be closed using traditional methods. However, successful initial abdominal wall revascularization in the setting of visceral organ transplantation can pose a major challenge as graft ischemia time, operating in a limited surgical field, and variable recipient and donor anatomy must be considered. Several techniques have been reported to accomplish abdominal wall revascularization. METHODS: A literature review was performed using PubMed for articles related to "abdominal wall transplantation (AWT)." The authors of this study sorted through this search for relevant publications that describe abdominal wall transplant anatomy, technical descriptions, and outcomes of various techniques. RESULTS: A total of four distinct revascularization techniques were found in the literature. Each of these techniques was described by the respective authors and reported varying patient outcomes. Levi et al published a landmark article in 2003 that described technical feasibility of AWT with anastomosis between donor external iliac and inferior epigastric vessels with recipient common iliac vessels in end-to-side fashion. Cipriani et al described a microsurgical technique with anastomosis between donor and recipient inferior epigastric vessels in an end-to-end fashion. Giele et al subsequently proposed banking the abdominal wall allograft in the forearm to reduce graft ischemia time. Recently, Erdmann et al described the utilization of an arteriovenous loop for synchronous revascularization of abdominal wall and visceral transplants for reduction of ischemia time, operative time, while eliminating the need for further operations. CONCLUSION: Vascularized composite allotransplantation continues to advance with improving immunotherapy and outcomes in solid organ transplantation. Optimizing surgical techniques remains paramount as the field continues to grow. Refinement of the presented methods will continue as additional evidence and outcomes become available in AW-VCA.

De novo donor-specific HLA antibodies after combined intestinal and vascularized composite allotransplantation - a retrospective study.

Combining vascularized composite allotransplantation (VCA) with intestinal transplantation to achieve primary abdominal closure has become a feasible procedure. Besides facilitating closure, the abdominal wall can be used to monitor intestinal rejection. As the inclusion of a VCA raises the possibility of an enhanced alloimmune response, we investigated the incidence and clinical effect of de novo donor-specific HLA antibodies (dnDSA) in a cohort of patients receiving an intestinal transplant with or without a VCA. The sequential clinical study includes 32 recipients of deceased donor intestinal and VCA transplants performed between 2008 and 2015; eight (25%) modified multivisceral transplants and 24 (75%) isolated small bowel transplants. A VCA was used in 18 (56.3%) cases. There were no episodes of intestinal rejection without VCA rejection. Fourteen patients (14 of 29; 48.3%) developed dnDSA. In the VCA group, fewer patients developed dnDSA; six of 16 (37.5%) VCA vs. eight of 13 (61.5%) non-VCA. There was no statistically significant difference in one- and 3-year overall graft survival stratified for the presence of dnDSA; P = 0.286. In the study, there is no evidence that the addition of a VCA increases the incidence of dnDSA formation compared to transplantation of the intestine alone.

Skype clinics after intestinal transplantation - follow-up beyond post codes.

The follow-up after intestinal transplantation (ITX) is complex and limited to specialized centers. ITX recipients often travel all over the country to be seen in the outpatient clinic of specialized centers which is costly and time-consuming. Videoconferences through Skype have been implemented to eliminate travel time, costs, and to improve patient compliance without jeopardizing safety. Eighteen of 19 patients followed up after ITX or modified multivisceral transplantation (MMVTX) in conventional outpatient clinics in Oxford agreed to attend additional Skype clinics. All patients who were followed up through Skype clinics after ITX/MMVTX received a questionnaire to measure their satisfaction with methods and technical aspects of videoconferencing as well as time/mode of traveling, travel expenses/costs, waiting time in outpatient clinic and patients' satisfaction. Mean travel distance to Oxford was 236 ± 168 miles, mean travel time was 277 ± 175 min, and mean travel cost was 200 ± 56 Great Britain Pounds. A total of 56% had to take time off work and/or find child/family care for the time spent in travel. These patients reported a satisfaction score of 4.38 ± 0.77 of 5 points as opposed to 2.88 ± 0.90 for attending the conventional outpatient clinic. Skype clinics have been proven successful and feasible in highly specialized fields like ITX in eligible patients.

Bartsocas-Papas Syndrome: A Case Report and Review of the Literature.

Bartsocas-Papas syndrome (BPS) is an autosomal recessively inherited form of the popliteal pterygium syndrome characterized by severe growth retardation, midface hypoplasia, popliteal pterygia, and syndactyly. Almost all affected babies die in utero or infancy. We report the difficulties of reconstruction and ongoing plastic surgical management in an 8-year-old child with BPS. With increasingly sophisticated resuscitation and supportive techniques, it is possible that more patients with BPS will survive beyond the neonatal period. This raises new challenges with reconstruction highlighted by this case with a difficult balance between trying to overcome some of the profound effects of the syndrome versus diminishing quality of life for the child by repeated and often unsuccessful surgical procedures.

Current state of abdominal wall transplantation.

PURPOSE OF REVIEW: Primary closure of the abdominal wall remains one of the early challenges of intestinal transplantation. Our aim is to review the role of abdominal wall transplantation in achieving tension-free closure of the abdomen. RECENT FINDINGS: In total, 38 full-thickness vascularized abdominal wall transplants, six partial-thickness vascularized and 17 partial-thickness nonvascularized rectus facia grafts have been reported worldwide. Different techniques have been described. The most popular choice seems to be the full-thickness vascularized abdominal wall allograft, where the anastomosis is performed either in a micro- or macrovascular fashion. Temporary 'remote' revascularisation of the allograft has been performed in some cases onto the recipient's forearm vessels when there is a long anticipated cold ischaemia time (>5 h). Preliminary data suggest that the abdominal wall skin rejection might be an early predictor of intestinal rejection. Vascularized and nonvascularized rectus fascia may be effective when there is inadequate healthy muscle/fascia but sufficient skin cover. SUMMARY: Several centres have already proved the technical and immunologic feasibility of partial or full-thickness abdominal wall transplantation. It is an effective option to achieve primary abdominal closure following intestinal transplantation and in its full-thickness form, it may be useful for monitoring rejection in visceral organs.

The abdominal wall transplant as a sentinel skin graft.

PURPOSE OF REVIEW: Abdominal wall transplantation is a technique used to achieve abdominal closure after intestinal and multivisceral transplantation. This review focuses on whether there are additional benefits for the skin component as an immune-monitoring tool. RECENT FINDINGS: The largest series of abdominal wall transplants has recently been published. Alongside the physiological advantage gained in abdominal closure, the authors describe the immunological insight that the skin component can provide and how this contributes to the management of patients. The skin appears to develop a rash with early rejection, which facilitates early systemic treatment before significant visceral rejection occurs. It can also help in cases in which there is diagnostic doubt regarding the cause of bowel dysfunction such as in instances of intestinal infection. Despite the additional immunological burden of donor tissue, there appears to be no requirement for increased immunosuppressive therapy. SUMMARY: The technical and immunological feasibility of abdominal wall transplantation has now been demonstrated by several centres. Skin transplanted as part of the abdominal wall or as a separate vascularized sentinel skin flap may aid in the diagnosis of rejection. This has the potential to improve graft survival and reduce immunosuppressive morbidity.

Surgery for Dupuytren's contracture of the fingers.

BACKGROUND: Dupuytren's disease is a benign fibroproliferative disorder that causes the fingers to be drawn into the palm via formation of new tissue under the glabrous skin of the hand. This disorder causes functional limitations, but it can be treated through a variety of surgical techniques. As a chronic condition, it tends to recur. OBJECTIVES: To assess the benefits and harms of different surgical procedures for treatment of Dupuytren's contracture of the index, middle, ring and little fingers. SEARCH METHODS: We initially searched the following databases on 17 September 2012, then re-searched them on 10 March 2014 and on 20 May 2015: the Cochrane Central Register of Controlled Trials (CENTRAL), The Cochrane Library, the British Nursing Index and Archive (BNI), the Cumulative Index to Nursing and Allied Health Literature (CINAHL), EMBASE, the Latin American Caribbean Health Sciences Literature (LILACS), Ovid MEDLINE, Ovid MEDLINE-In-Process and Other Non-Indexed Citations, ProQuest (ABI/INFORM Global and Dissertations & Theses), the Institute for Scientific Information (ISI) Web of Science and clinicaltrials.gov. We reviewed the reference lists of short-listed articles to identify additional suitable studies. SELECTION CRITERIA: We included randomised clinical trials and controlled clinical trials in which groups received surgical intervention for Dupuytren's disease of the index, middle, ring or little finger versus control, or versus another intervention (surgical or otherwise). We excluded the thumb, as cords form on the radial aspect of the thumb and thus are not readily accessible in terms of angular deformity. Furthermore, thumb disease is rare. DATA COLLECTION AND ANALYSIS: A minimum of two review authors independently reviewed search results to select studies for inclusion by using pre-specified criteria, assessed risk of bias of included studies and extracted data from included studies.We grouped outcomes into the following categories: (1) hand function, (2) other patient-reported outcomes (e.g. satisfaction, pain), (3) early objective outcomes (e.g. correction of angular deformity), (4) late objective outcomes (e.g. recurrence) and (5) adverse effects. MAIN RESULTS: We included 14 articles describing 13 studies, comprising 11 single-centre studies and two multi-centre studies. These studies involved 944 hands of 940 participants; of these, 93 participants were reported twice in separate articles describing early and late outcomes of one trial. Three papers reported the outcomes of two trials comparing different procedures. One trial compared needle fasciotomy versus fasciectomy (125 hands, 121 participants), and the other compared interposition firebreak skin grafting versus z-plasty closure of fasciectomy (79 participants). The other 11 studies reported trials of technical refinements of procedures or rehabilitation adjuncts. Of these, three investigated effects of postoperative splinting on surgical outcomes.Ten studies (11 articles) were randomised controlled trials (RCTs) of varying methodological quality; one was a controlled clinical trial. Trial design was unclear in two studies awaiting classification. All trials had high or unclear risk of at least one type of bias. High risks of performance and detection bias were particularly common. We downgraded the quality of evidence (Grades of Recommendation, Assessment, Development and Evaluation - GRADE) of outcomes to low because of concerns about risk of bias and imprecision.Outcomes measured varied between studies. Five articles assessed recurrence; two defined this as reappearance of palpable disease and two as deterioration in angular deformity; one did not explicitly define recurrence.Hand function on the Disabilities of the Arm, Shoulder and Hand (DASH) Scale (scores between 0 and 100, with higher scores indicating greater impairment) was 5 points lower after needle fasciotomy than after fasciectomy at five weeks. Patient satisfaction was better after fasciotomy at six weeks, but the magnitude of effect was not specified. Fasciectomy improved contractures more effectively in severe disease: Mean percentage reduction in total passive extension deficit at six weeks for Tubiana grades I and II was 11% lower after needle fasciotomy than after fasciectomy, whereas for grades III and IV disease, it was 29% and 32% lower.Paraesthesia (defined as subjective tingling sensation without objective evidence of altered sensation) was more common than needle fasciotomy at one week after fasciectomy (228/1000 vs 67/1000), but reporting of complications was variable.By five years, satisfaction (on a scale from 0 to 10, with higher scores showing greater satisfaction) was 2.1/10 points higher in the fasciectomy group than in the fasciotomy group, and recurrence was greater after fasciotomy (849/1000 vs 209/1000). Firebreak skin grafting did not improve outcomes more than fasciectomy alone, although this procedure took longer to perform.One trial investigated four weeks of day and night splinting followed by two months of night splinting after surgery. The other two trials investigated three months of night splinting after surgery, but participants in 'no splint' groups with early deterioration at one week were issued a splint for use. All three studies demonstrated no benefit from splinting. The two trials investigating postoperative night splinting were suitable for meta-analysis, which demonstrated no benefit from splinting: Mean DASH score in the splint groups was 1.15 points lower (95% confidence interval (CI) -2.32 to 4.62) than in the no splint groups. Mean total active extension in the splint groups was 2.21 degrees greater (95% CI -3.59 to 8.01 degrees) than in the no splint groups. Mean total active flexion in the splint groups was 8.42 degrees less (95% CI 1.78 to 15.07 degrees) than in the no splint groups. AUTHORS' CONCLUSIONS: Currently, insufficient evidence is available to show the relative superiority of different surgical procedures (needle fasciotomy vs fasciectomy, or interposition firebreak skin grafting vs z-plasty closure of fasciectomy). Low-quality evidence suggests that postoperative splinting may not improve outcomes and may impair outcomes by reducing active flexion. Further trials on this topic are urgently required.

Wnt signaling and Dupuytren's disease.

BACKGROUND: Dupuytren's disease is a benign fibromatosis of the hands and fingers that leads to flexion contractures. We hypothesized that multiple genetic and environmental factors influence susceptibility to this disease and sought to identify susceptibility genes to better understand its pathogenesis. METHODS: We conducted a genomewide association study of 960 Dutch persons with Dupuytren's disease and 3117 controls (the discovery set) to test for association between the disease and genetic markers. We tested the 35 single-nucleotide polymorphisms (SNPs) most strongly associated with Dupuytren's disease (P<1×10(-4)) in the discovery set in three additional, independent case series comprising a total of 1365 affected persons and 8445 controls from Germany, the United Kingdom, and The Netherlands. RESULTS: Initially, we observed a significant genomewide association between Dupuytren's disease and 8 SNPs at three loci. Tests of replication and joint analysis of all data from 2325 patients with Dupuytren's disease and 11,562 controls yielded an association with 11 SNPs from nine different loci (P<5.0×10(-8)). Six of these loci contain genes known to be involved in the Wnt-signaling pathway: WNT4 (rs7524102) (P=2.8×10(-9); odds ratio, 1.28), SFRP4 (rs16879765) (P=5.6×10(-39); odds ratio, 1.98), WNT2 (rs4730775) (P=3.0×10(-8); odds ratio, 0.83), RSPO2 (rs611744) (P=7.9×10(-15); odds ratio, 0.75), SULF1 (rs2912522) (P=2.0×10(-13); odds ratio, 0.72), and WNT7B (rs6519955) (P=3.2×10(-33); odds ratio, 1.54). CONCLUSIONS: This study implicates nine different loci involved in genetic susceptibility to Dupuytren's disease. The fact that six of these nine loci harbor genes encoding proteins in the Wnt-signaling pathway suggests that aberrations in this pathway are key to the process of fibromatosis in Dupuytren's disease.

Interventions for treating fingertip entrapment injuries in children.

BACKGROUND: Fingertip entrapment injuries, which involve lacerations to the pulp and nail and often a fracture of the underlying bone, commonly occur in children, usually as the result of a crushing injury. Treatment is either conservative (wound cleaning and fingertip dressing)or surgical (repair of lacerations, reduction and stabilisation of fractures); however, no consensus currently exists regarding the most appropriate treatment modality. OBJECTIVES: To assess the effects (benefits and harms) of surgical and conservative interventions for fingertip entrapment injuries in children. We aimed to compare: different methods of conservative treatment; surgical versus conservative treatment; different methods of surgical treatment; and different methods of management after initial conservative or surgical treatment. SEARCH METHODS: We searched the Cochrane Bone, Joint and Muscle Trauma Group Specialised Register, the Cochrane Central Register of Controlled Trials (The Cochrane Library), MEDLINE, EMBASE, the World Health Organization Clinical Trials Registry Platform and reference lists of articles up to 30 April 2013. We did not apply any restrictions based on language or publication status. SELECTION CRITERIA: Randomised controlled trials (RCTs) and quasi-RCTs comparing interventions for treating fingertip entrapment injuries in children.The primary outcomes were fingertip function, nail growth, nail deformity and adverse events such as infection. DATA COLLECTION AND ANALYSIS: Two authors independently screened studies for inclusion, assessed the risk of bias in each included trial and extracted data.We resolved disagreements through discussion. MAIN RESULTS: We included two RCTs examining a total of 191 young children, 180 of whom were included in the analyses. The two trials tested different comparisons. Both trials were at high risk of bias, particularly from lack of blinding of participants and personnel, and of outcome assessment. The trials did not record fingertip function, nail growth or nail deformity. The quality of the evidence for the reported outcomes was judged to be 'low' using the GRADE approach (i.e. further research is very likely to have an important impact on our confidence in the estimate of the effect and is likely to change the estimate).One trial compared a seven-day course of antibiotics with no antibiotics after formal surgical repair of fingertip entrapment injuries.One child in each group had an infection at day seven (1/66 antibiotic group versus 1/69 no antibiotic group; RR 1.05, 95% CI 0.07 to 16.37). Both participants with infections had a more severe injury (partial fingertip amputation).The other trial compared two different dressings (silicone net and paraffin gauze) for use after either surgical or initial conservative management of fingertip entrapment injuries. It reported that two of 20 children in the silicone group versus one of 25 in the paraffin group had complications of wound infection (RR 2.50, 95% CI 0.24 to 25.63) and that one of 20 children in the silicone group versus two of 25 in the paraffin group had skin necrosis (RR 0.63, 95% CI 0.06 to 6.41). All complications healed with conservative treatment. The results for mean healing times and mean number of dressing changes were similar between groups but benefits of either silicone or paraffin dressings could not be excluded (silicone mean 4.1 weeks versus paraffin mean 4.0 weeks;MD 0.10 weeks, 95% CI-0.61 to 0.81); (silicone mean 4.3 dressing changes versus paraffin mean 4.2 dressing changes; MD 0.10, 95% CI -0.57 to 0.77). The trial found that a silicone dressing was less likely to adhere to the wound or cause distress for the child at the one-week dressing change. AUTHORS' CONCLUSIONS: There is a lack of evidence from RCTs to inform all key treatment decisions for the management of fingertip entrapment injuries in children.Given that the quality of evidence is low from one trial, we do not have conclusive evidence that prophylactic use of antibiotics after surgical repair fails to reduce risk of infection. The two children who experienced infection had more severe wounds.Similarly, the low quality evidence from one trial has not enabled us to draw firm conclusions regarding the effect on healing time or complications (infection, skin necrosis) at four-week follow-up between a silicone net dressing and a paraffin gauze dressing when applied post-surgery or after simple wound irrigation; however, the silicone net dressing may be easier to remove in the first week.Further RCTs are required in this area, preferably comparing surgical with conservative methods of managing fingertip entrapment injuries. Outcome assessment should include fingertip function, nail growth and nail deformity for a minimum of three months posttreatment.

Second toe metatarsophalangeal joint transfer for sternoclavicular joint reconstruction.

PURPOSE: We describe the anatomical basis for microsurgical reconstruction of the sternoclavicular joint using a vascularized, innervated second metatarsophalangeal joint, the surgical technique, and outcomes in 2 patients. METHODS: We harvested the second metatarsophalangeal joint along with the joint capsule, portions of the metatarsal and phalanx, the flexor sheath and flexor tendon, the extensor sheath and tendon, the first metatarsal artery, and the deep peroneal nerve. This composite tissue was used for reconstruction of an excised sternoclavicular joint following infection or chondrosarcoma. The proximal phalanx was dowel-jointed into the manubrium and fixed with 1 or 2 screws; the metatarsal was plated to the remaining clavicle. The joint was oriented to allow maximal elevation and restricted depression, and the normal mediolateral laxity allowed anterior and posterior movement. Vascular anastomoses were performed to branches of the thoracoacromial axis vessels, and digital nerves were connected to a supraclavicular nerve. RESULTS: Two patients had their excised sternoclavicular joints reconstructed using this technique. Both achieved union at the clavicular and sternal junctions. Both obtained restoration of movement of the sternoclavicular joint and upper limb. One patient developed joint subluxation and pain requiring tendon graft reconstruction of the costoclavicular ligament. CONCLUSIONS: In these 2 cases, the vascularized second toe metatarsophalangeal joint satisfactorily reconstructed the widely excised sternoclavicular joint and costoclavicular ligament and restored function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

State of the art review. Upper extremity revision nerve compression surgery.

Although surgical release of upper extremity nerve compression syndromes is highly effective, persistence or recurrence of symptoms and signs may occur. Thorough investigation is necessary in this situation before treatment is recommended. If the symptoms cannot be explained by other pathology than compression of the affected nerve and if conservative management has not provided improvement, reoperation may be considered. This review provides an overview of the diagnostic and surgical considerations in the revision of carpal tunnel syndrome, cubital tunnel syndrome and thoracic outlet syndrome.Level of evidence: V.

Clinical Anatomy of the Sacral Nerve Roots and Its Relevance to Their Reconstruction After Sacrectomy.

BACKGROUND AND OBJECTIVES: En bloc sacrectomy is associated with sacral root transection causing loss of urinary bladder, rectum, and sexual function. The aim of the study was to determine the position of the pudendal branches (sensorimotor) and pelvic splanchnic nerves (parasympathetic) on the sacral roots relative to the sacrum, and the minimal and maximal defects in the sacral roots that can be reconstructed by grafting after various types of sacrectomy. METHODS: Five cadaveric pelves were dissected bilaterally. The lengths and widths of the S1-S4 roots and their branches were measured. Then, the minimal and maximal defects between the proximal and distal stumps of the sacrificed roots were measured following 3 models of sacrectomy (below S2, below S1, and total sacrectomy). RESULTS: The mean distance of the splanchnic nerves from the S2 and S3 anterior sacral foramina was 17.7 ± 7.3 and 23.6 ± 11.1 mm, respectively, and the mean distance of the pudendal S2 and S3 branches was 36.8 ± 13.7 and 30.2 ± 10.8 mm, respectively. The mean widths of the S2 and S3 roots were 9.3 ± 1.9 and 5.4 ± 1.2 mm, respectively. The mean maximal defects in S2 and S3 roots after various types of sacrectomies were between 61.8 ± 16.3 and 100.7 ± 14.3 mm and between 62.7 ± 20.2 and 84.7 ± 25.1 mm, respectively. There were no statistically significant differences between sides or sexes for all obtained measurements. CONCLUSION: The reconstruction of the S2-S3 roots is anatomically feasible after partial or total sacrectomies in which the resection of the soft tissue does not extend further than approximately 1.5 to 2 cm ventrally from the sacrum.

Toxin for Treating Raynaud Conditions in Hands (The TORCH Study): A Systematic Review and Meta-analysis.

Background: Raynaud disease of the hands is a complex disorder resulting in inappropriate constriction and/or insufficient dilation in microcirculation. There is an emerging role for botulinum toxin type A (BTX-A) in the treatment armamentarium for refractory Raynaud disease. The aim of this systematic review was to critically evaluate the management of primary and secondary Raynaud disease treated with BTX-A intervention. Methods: We performed a Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant systematic review of clinical studies assessing treatment of primary or secondary Raynaud disease with BTX-A by searching Ovid MEDLINE and Embase databases from inception to first August 2023. The review protocol was prospectively registered on the PROSPERO database (CRD42022312253). Results: Our search strategy identified 288 research articles, of which 18 studies [four randomized controlled trials (RCTs), two non-RCTs, five case series, and seven retrospective cohort studies] were eligible for analysis. Meta-analysis demonstrated that the probability of pain visual analog scale score improvement with BTX-A intervention was 81.95% [95% confidence interval (74.12-87.81) P = 0.19, heterogeneity I 2 = 26%] and probability of digital ulcer healing was 79.37% [95% confidence interval (62.45-89.9) P = 0.02, heterogeneity I 2 = 56%]. Conclusions: Delivery of BTX-A to digital vessels in the hand may be an effective management strategy for primary and secondary Raynaud disease. A definitive, appropriately-powered RCT with objective functional and patient-reported outcome measures is required to accurately assess and quantify the efficacy of BTX-A in Raynaud disease of the hands.

Alternative recruitment strategies influence saliva sample return rates in community-based genetic association studies.

Collection of saliva for DNA extraction has created new opportunities to recruit participants from the community for genetic association studies. However, sample return rates are variable. No prior study has specifically addressed how study design impacts sample return. Using data from three large-scale genetic association studies we compared recruitment strategy and sample return rates. We found highly significant differences in sample return rates between the studies. In studies that recruited retrospectively, overall returns were much lower from families with a self-limiting condition who provided samples at a research centre or home visit, than adult elderly individuals with a chronic disease who provided samples by post (59% vs. 84%). Prospective recruitment was associated with high agreement to participate (72%), but subsequent low return of actual saliva samples (42%). A telephone call had marginal effect on recruitment in a retrospective family study, but significantly improved returns in a prospective family study. We found no effect upon DNA yield comparing observed versus unobserved sample collection, or between male and female adult participants. Overall, study design significantly impacts upon response rates for genetic association studies recruiting from the community. Our findings will help researchers in constructing and costing a recruitment protocol.

Macrodactyly in the setting of a plexiform schwannoma in neurofibromatosis type 2: case report.

Macrodactyly is a rare anomaly. Little is understood about the etiology and underlying mechanisms. To our knowledge, macrodactyly has not previously been associated with neurofibromatosis type 2. We present a case of macrodactyly of the small finger associated with a digital nerve plexiform schwannoma in a patient with neurofibromatosis type 2.

Systematic Review of Thumb Carpometacarpal Joint Hemiresection Interposition Arthroplasty Materials.

BACKGROUND: Osteoarthritis of the first carpometacarpal joint is a common condition. Various management options and surgical procedures have been described to treat symptomatic cases. Many systematic reviews examine aspects of thumb carpometacarpal joint osteoarthritis treatment, although none solely examines the outcomes of trapezial partial resection and interposition arthroplasty in stage II to III patients in detail, yet this technique is of growing interest as surgeons seek more nuanced, tailored approaches for osteoarthritis of the first carpometacarpal joint. METHODS: A systematic review of the thumb carpometacarpal joint hemiresection and interposition arthroplasty was performed with pain assessment as a primary outcome measure and patient-reported outcome measures (PROMs) and reoperation rate as secondary outcome measures. A search was performed between 2004 and 2019 using MEDLINE, Embase, and PubMed. Preferred Reporting Items and Systematic Reviews and Meta-Analyses guidelines were used. RESULTS: Twenty-nine articles met the inclusion criteria and were included in the final review. Pain relief and improved PROMs were described in all the articles using this technique with all the interposition materials. Materials such as polyurethane urea matrix and poly-l/d-lactide scaffold had higher complication rates. Revision rates varied and were highest with the polyurethane urea matrix. CONCLUSIONS: This review shows that hemiresection interposition arthroplasty is a useful technique and provides symptomatic benefit in patients with Eaton-Littler stage II and III osteoarthritis. Revision surgery rates due to persistent pain and instability were higher with the use of implants. Larger and long-term studies of this technique using autologous or more bioinert materials and implants are required to assess duration of symptomatic benefit.

Population Study of Hand and Wrist Manifestations of Congenital Insensitivity to Pain.

Background: Congenital insensitivity to pain is a rare autosomal recessive condition characterized by insensitivity to painful stimuli due to absence of sensory and sympathetic post ganglionic neurons in the skin and skeletal system leading to lack of protective sensation and altered joint propioception. This study was performed to assess hand and wrist manifestations of patients with congenital insensitivity to pain in the Maltese Islands. Methods: Records of public and private hospitals were reviewed to identify patients suffering from this condition. A review of notes, patients, and imaging was performed. A Disabilities of the Arm, Shoulder, and Hand score was obtained to assess level of function. Results: Nine patients were identified. Mean age of diagnosis was 8.9 years. Interphalangeal joints were most commonly affected. Multiple spontaneous or posttraumatic fingertip ulceration occurred in 5 patients. Anhidrosis resulted in more protracted ulcers and infections, requiring amputation of distal and middle phalanges due to osteomyelitis. The wrist joint was less commonly involved and showed more complex joint involvement. Conclusion: The hand and wrist are involved in different ways, with fingertip ulceration leading to potential infection and osteomyelitis in the hand, whereas the wrist joint is involved in cases of increased axial loading and load transfer, such as following prolonged use of walking and mobility aids. The latter should be borne in mind during management of lower limb conditions. Hand care and hygiene is important in all patients, especially in cases of anhidrosis due to the increased rate of ulceration and osteomyelitis requiring surgical intervention. Despite the severity of the condition, patients report good overall function.

Variants in ALDH1A2 reveal an anti-inflammatory role for retinoic acid and a new class of disease-modifying drugs in osteoarthritis.

More than 40% of individuals will develop osteoarthritis (OA) during their lifetime, yet there are currently no licensed disease-modifying treatments for this disabling condition. Common polymorphic variants in ALDH1A2, which encodes the key enzyme for synthesis of all-trans retinoic acid (atRA), are associated with severe hand OA. Here, we sought to elucidate the biological significance of this association. We first confirmed that ALDH1A2 risk variants were associated with hand OA in the U.K. Biobank. Articular cartilage was acquired from 33 individuals with hand OA at the time of routine hand OA surgery. After stratification by genotype, RNA sequencing was performed. A reciprocal relationship between ALDH1A2 mRNA and inflammatory genes was observed. Articular cartilage injury up-regulated similar inflammatory genes by a process that we have previously termed mechanoflammation, which we believe is a primary driver of OA. Cartilage injury was also associated with a concomitant drop in atRA-inducible genes, which were used as a surrogate measure of cellular atRA concentration. Both responses to injury were reversed using talarozole, a retinoic acid metabolism blocking agent (RAMBA). Suppression of mechanoflammation by talarozole was mediated by a peroxisome proliferator-activated receptor gamma (PPARγ)-dependent mechanism. Talarozole was able to suppress mechano-inflammatory genes in articular cartilage in vivo 6 hours after mouse knee joint destabilization and reduced cartilage degradation and osteophyte formation after 26 days. These data show that boosting atRA suppresses mechanoflammation in the articular cartilage in vitro and in vivo and identifies RAMBAs as potential disease-modifying drugs for OA.

Functional and oncological outcome following marginal excision of well-differentiated forearm liposarcoma with nerve involvement.

PURPOSE: Liposarcoma is one of the most common soft tissue sarcomas in adults. It is often low-grade and can occasionally involve neurovascular structures. We present the functional and oncological outcome resulting from planned marginal excision of a series of forearm low-grade liposarcomas with nerve involvement. METHODS: The Oxford tumor registry was used to identify cases of histologically proven, well-differentiated liposarcoma of the forearm, with nerve involvement, treated surgically between 1997 and 2006. Nerve involvement was identified clinically with symptoms or signs of nerve compression, or by images showing direct contact of the tumor with a nerve on magnetic resonance imaging. This was then further defined at the time of surgery as tumor abutting (capsular involvement) or encasing a peripheral nerve. Demographic and clinical data were collected and oncological outcome was assessed by noting local and distant recurrence during follow-up. Postoperative functional outcome was assessed using the Toronto Extremity Salvage Scores. RESULTS: Eight cases were identified, 6 with preoperative neurological symptoms. The total group comprised 6 men and 2 women with a mean age of 61 (range, 30-71) years. At surgery, all had their tumors successfully excised, with preservation of the involved nerves. In those with preoperative neurological symptoms, complete recovery occurred by 18 months after surgery. The average follow-up was 5 years (range, 3-9 y). There were no cases of either local or distant recurrence of disease, with a mean Toronto Extremity Salvage Score of 99%. CONCLUSIONS: Planned marginal excision of a well-differentiated liposarcoma, arising in the forearm and involving nerve, can result in excellent functional and oncological outcome. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.