Upper and Lower Limb Anomalies in Craniofacial Microsomia and Its Relation to the OMENS+ Classification: A Multicenter Study of 688 Patients.

BACKGROUND: Craniofacial microsomia (CFM) is characterized by several malformations related to the first and second pharyngeal arch. Patients typically present with facial asymmetry, but extracraniofacial organ systems might be involved, including limb anomalies. The purpose of this study was to analyze the occurrence of upper and lower limb anomalies in CFM patients. Furthermore, the relation between limb anomalies and the OMENS+ (orbital distortion; mandibular hypoplasia; ear anomaly; nerve involvement; soft-tissue deficiency; and associated extracraniofacial anomalies) classification was examined. METHODS: A retrospective study was conducted including patients with CFM from craniofacial units in three different countries. Patients were included when clinical and/or radiographic images were available. Demographic, radiographic, and clinical information was obtained. RESULTS: A cohort of 688 patients was available and selected for analysis. In total, 18.2% of the patients were diagnosed with at least one upper and/or lower limb anomaly. Upper and lower limb anomalies were seen in, respectively, 13.4% and 7.8% of patients. Patients with other extracraniofacial anomalies had a significantly higher risk for limb anomalies (OR, 27.98; P = 0.005). Laterality of CFM and a higher OMENS score were not associated with limb anomalies. CONCLUSIONS: More than one in six patients with craniofacial microsomia have limb anomalies. Therefore, clinical awareness for these anomalies is warranted. Examination and, if present, follow-up on limb abnormalities in patients with CFM should be implemented in the standard assessment of CFM patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

The unsolved problem of radial longitudinal dysplasia: how can we reliably prevent recurrence, preserve growth and optimize function?

Congenital radial longitudinal dysplasia remains an 'unsolved problem' in hand surgery. The challenges presented by the skeletal deficiency of the distal radius and soft tissue dysplasia of the severe radial longitudinal deficiency have been addressed by a number of techniques that aim to stabilize the position of the hand relative to the forearm and optimize forearm growth and hand function. Analysis of hand function and position in these children is difficult because of the abnormal 'wrist' mechanics, and the published results of the techniques used to date often lack a standardized approach and importantly the perception of function from the patient's perspective. The existing data is reviewed and compared with the results of cohorts from two major congenital upper limb centres. Soft tissue distraction prior to radialization or centralization may offer benefit in ulnar growth and forearm length but there is a need for further research into the long-term functional outcomes of the various techniques available to determine the optimal choice for these children.Level of evidence: V.

Determining the Minimally Important Change of the Michigan Hand outcomes Questionnaire in patients undergoing trigger finger release.

INTRODUCTION: The Michigan Hand outcomes Questionnaire (MHQ) is a widely used instrument to evaluate treatment results for hand conditions. Establishing the Minimally Important Change (MIC) is essential for interpreting change in outcome that is clinically relevant. PURPOSE OF THE STUDY: The purpose of this study was to determine the MIC of the MHQ total and subscale scores in patients undergoing trigger finger release. STUDY DESIGN: This is a prospective cohort study conducted between December 2011 and February 2020. METHODS: Patients completed the MHQ prior to surgery and 3 months postoperatively. The MIC of the MHQ was determined using 5 anchor-based methods (ie, 2 anchor mean change methods and 3 receiver operating characteristic methods). The median MIC value was determined to represent the triangulated MIC. RESULTS: A total of 1814 patients were included. The MIC for the MHQ total score ranged from 7.7 to 10.9, with a triangulated estimate of 9.3. The MIC estimates for 5 of 6 of the MHQ subscales ranged from 7.7 to 20.0. No MICs could be determined for the MHQ subscale "aesthetics" due to low correlations between the anchor questions and MHQ change scores. CONCLUSIONS: These MIC estimates can contribute to the interpretation of clinical outcomes following trigger finger release and for assessment of power in prospective trials.

Variant type and position predict two distinct limb phenotypes in patients with GLI3-mediated polydactyly syndromes.

INTRODUCTION: Pathogenic DNA variants in the GLI-Kruppel family member 3 (GLI3) gene are known to cause multiple syndromes: for example, Greig syndrome, preaxial polydactyly-type 4 (PPD4) and Pallister-Hall syndrome. Out of these, Pallister-Hall is a different entity, but the distinction between Greig syndrome and PPD4 is less evident. Using latent class analysis (LCA), our study aimed to investigate the correlation between reported limb anomalies and the reported GLI3 variants in these GLI3-mediated polydactyly syndromes. We identified two subclasses of limb anomalies that relate to the underlying variant. METHODS: Both local and published cases were included for analysis. The presence of individual limb phenotypes was dichotomised and an exploratory LCA was performed. Distribution of phenotypes and genotypes over the classes were explored and subsequently the key predictors of latent class membership were correlated to the different clustered genotypes. RESULTS: 297 cases were identified with 127 different variants in the GLI3 gene. A two-class model was fitted revealing two subgroups of patients with anterior versus posterior anomalies. Posterior anomalies were observed in cases with truncating variants in the activator domain (postaxial polydactyly; hand, OR: 12.7; foot, OR: 33.9). Multivariate analysis supports these results (Beta: 1.467, p=0.013 and Beta: 2.548, p<0.001, respectively). Corpus callosum agenesis was significantly correlated to these variants (OR: 8.8, p<0.001). CONCLUSION: There are two distinct phenotypes within the GLI3-mediated polydactyly population: anteriorly and posteriorly orientated. Variants that likely produce haploinsufficiency are associated with anterior phenotypes. Posterior phenotypes are associated with truncating variants in the activator domain. Patients with these truncating variants have a greater risk for corpus callosum anomalies.

Lateral Versus Medial Hallux Excision in Preaxial Polydactyly of the Foot.

BACKGROUND: In preaxial polydactyly of the foot, the choice for excision of the lateral or medial hallux is not straightforward, in particular with proximal phalangeal (type IV) and metatarsal (type VI) duplication, because of anatomical characteristics. We evaluated whether medial or lateral hallux excision gives better outcomes in these duplication types, to help clinical decision making. METHODS: Children with type IV or type VI duplication (n=14, age: 4.4-17.2 years), who were operatively treated by excision of the lateral or medial hallux, were assessed for foot function using plantar pressure measurements and clinical examination. Foot aesthetics were scored by the child, an expert, and 10 laypersons, and additional patient-reported outcome questionnaires were obtained. Outcomes were compared between lateral and medial excision, per duplication type. RESULTS: In type IV duplication (n=11), lateral excision showed a better distribution of peak pressure between the hallux and first metatarsal with significantly lower median first metatarsal peak pressure (P = .008). Lateral excision showed more medial hallux deviation (P = .017). Foot aesthetics were not different between excision sides. In type VI duplication (n=12), lateral excision showed a 59% higher hallux peak pressure, larger medial hallux deviation (P = .004), and more reoperations. Foot aesthetics were scored significantly better after lateral excision by experts and laypersons. CONCLUSIONS: Foot function by virtue of plantar pressure was better after lateral hallux excision in type IV and after medial hallux excision in type VI duplication. Surgeons and laypersons perceived the foot as more normal after lateral excision in type VI, whereas children reported no differences. These outcomes can be used in clinical decision making. LEVEL OF EVIDENCE: Level III, therapeutic, comparative study.

Congenital Forearm Pseudarthrosis, a Systematic Review for a Treatment Algorithm on a Rare Condition.

BACKGROUND: A congenital forearm pseudarthrosis is a rare condition and is strongly associated with neurofibromatosis type 1. Several surgical techniques are described in the literature, but the most optimal treatment strategy remains unclear. This systematic review aims to develop a treatment algorithm that may aid in clinical decision making. METHODS: The PROSPERO registration number for this study was CRD42018099602 and adheres to the PRISMA guidelines for systematic reviews. Embase, MEDLINE, Cochrane Central, Web of Science, and Google Scholar databases were searched for published studies reporting on congenital forearm pseudarthrosis not related to other underlying pathologies like bacterial infection or fibrous dysplasia. Results were not restricted by date or study type, only English literature was allowed. Studies were assessed for quality using the critical appraisal checklist for case reports from the Joanna Briggs Institute. Patient characteristics, underlying disease, type of surgery, union rate, and functional outcome were extracted from included studies. RESULTS: Of 829 studies identified, 47 were included in this review (17 case series and 30 case reports, a total of 84 cases). A one-bone forearm procedure showed highest union rates (92%), however, it results in loss of forearm rotation. Free vascularized fibula grafting showed high union rates (87%) and was related to good functional outcome of elbow flexion and forearm rotations. Other procedures showed disappointing outcomes. CONCLUSIONS: Congenital forearm pseudarthrosis is best treated with a free vascularized fibula grafting, a one-bone forearm procedure should be used as a salvage procedure. Evidence extracted from the case reports was sufficient to generate a treatment algorithm to be used in clinical pediatric practice. LEVEL OF EVIDENCE: Level IV-therapeutic.

Outcome of Recurrent Surgery in Dupuytren's Disease: Comparison with Initial Treatment.

BACKGROUND: There are multiple studies about the effectiveness of primary treatment in Dupuytren's disease. However, such studies concerning treatment effectiveness of recurrent disease are scarce. Therefore, the primary aim of this study was to compare treatment effectiveness of initial and repeated surgery in patients with Dupuytren's disease. METHODS: Patients who underwent both initial and repeated treatment were selected from a prospectively maintained database. Outcome measurements consisted of finger goniometry, the Michigan Hand Outcomes Questionnaire, and complications. Treatment effectiveness was defined as improvement in extension deficit and patient-reported hand function. In addition, measurements at intake of both treatments were compared. Subgroup analyses were performed to evaluate influence of type of surgery of initial treatment on outcomes of repeated treatment. RESULTS: One hundred fourteen patients were included in the analyses. Improvement in extension deficit and Michigan Hand Outcomes Questionnaire outcomes was equal for initial and repeated treatments. Extension deficit and Michigan Hand Outcomes Questionnaire scores were worse at intake of repeated treatment compared to these outcomes at intake of initial treatment. In addition, patients who initially underwent needle fasciotomy achieved a better contracture reduction after repeated treatment. CONCLUSIONS: This study demonstrates that treatment of recurrent Dupuytren's disease is as effective as initial treatment, despite larger extension deficit and worse self-assessed hand function before undergoing repeated treatment. Complication rates were similar for initial and repeated treatments. Furthermore, needle fasciotomy for initial treatment results in better outcomes of repeated treatment compared to patients who initially underwent limited fasciectomy. These findings can be used for a more evidence-based preoperative counseling with patients with recurrent Dupuytren's disease. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

A multidisciplinary review of triphalangeal thumb.

Despite being a rare congenital limb anomaly, triphalangeal thumb is a subject of research in various scientific fields, providing new insights in clinical research and evolutionary biology. The findings of triphalangeal thumb can be predictive for other congenital anomalies as part of an underlying syndrome. Furthermore, triphalangeal thumb is still being used as a model in molecular genetics to study gene regulation by long-range regulatory elements. We present a review that summarizes a number of scientifically relevant topics that involve the triphalangeal thumb phenotype. Future initiatives involving multidisciplinary teams collaborating in the field of triphalangeal thumb research can lead to a better understanding of the pathogenesis and molecular mechanisms of this condition as well as other congenital upper limb anomalies.

Triphalangeal thumb: clinical features and treatment.

Triphalangeal thumb is a rare congenital anomaly in which the thumb has three phalanges. Clinical presentation of triphalangeal thumb can vary considerably and can be present in both hands or unilateral. The thumb can be long with a finger-like appearance. The presence of clinodactyly depends on the shape of the extra phalanx varying from wedge-shaped to rectangular. Various joints, ligaments, muscles, and tendons of the first ray can be hypoplastic or absent, with varying degrees of stiffness or instability. The aim of surgical treatment is to reconstruct or correct the anatomic anomalies to obtain greater function and a more acceptable appearance. In our series, operations varied from removal of the delta phalanx with ligament reconstruction to multiple osteotomies and rebalancing of soft tissues. Results in these often complex cases can be rewarding if the surgeon has sufficient knowledge of the underlying anatomic differences. This review summarizes our current concepts of presentation and management of the triphalangeal thumb.

Percutaneous Aponeurotomy and Lipofilling versus Limited Fasciectomy for Dupuytren's Contracture: 5-Year Results from a Randomized Clinical Trial.

BACKGROUND: In the Dupuytren Rotterdam randomized controlled trial, percutaneous aponeurotomy with lipofilling (PALF) was as effective as limited fasciectomy in correcting primary Dupuytren's contracture after 1 year. The authors report the 5-year results of this trial, with a special focus on recurrence of contractures. METHODS: The authors invited all patients who had undergone PALF or limited fasciectomy to participate in a posttrial follow-up assessment. Thirty-one PALF patients and 21 limited fasciectomy patients were assessed by an independent examiner for the degree of contracture and whether patients had undergone a secondary procedure. The primary composite endpoint was recurrence rate, defined as either 20 degrees or greater worsening in contracture (relative to week 3) or as having undergone a secondary procedure for a new or worsening contracture. RESULTS: At 5 years, more joints in the PALF group than in the limited fasciectomy group had a recurrence (74 percent versus 39 percent; p = 0.002). When redefining recurrence as a worsening in total extension deficit of at least 30 degrees for treated digits as often reported, this was 77 percent versus 32 percent (p = 0.001). Total extension deficit was also worse for PALF-reated digits (53 degrees versus 31 degrees; p < 0.010). CONCLUSIONS: Although the authors previously reported that PALF offers a shorter convalescence and fewer long-term complications but a similar degree of contracture correction at 1-year follow-up, at 5 years, the corrections were less durable than those for limited fasciectomy. This again highlights that limited fasciectomy and different types of needle aponeurotomy have specific advantages and disadvantages to weigh by patients and clinicians. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

Documenting Combined Congenital Upper Limb Anomalies Using the Oberg, Manske, and Tonkin Classification: Implications for Epidemiological Research and Outcome Comparisons.

PURPOSE: Congenital upper limb anomalies (CULAs) exhibit a wide spectrum of phenotypic manifestations. To help the clinician evaluating this variety of CULAs, the Oberg, Manske, and Tonkin (OMT) classification was recently introduced. The OMT classification allows for documentation of combined hand anomalies. However, subsequent epidemiological and validation studies using the OMT scheme commonly registered only the main anomaly per arm. This study illustrates both the deficits of single diagnosis documentation as well as the merits of registering every anomaly for epidemiological research, outcome comparison, and overall applicability of the classification. METHODS: We retrospectively reviewed patients visiting the Erasmus MC - Sophia Children's Hospital between 2012 and 2014. All congenital anomalies of both limbs were classified according to the OMT scheme. The frequency of combined diagnoses as well as recurrent combinations were analyzed. The relation to the coregistered syndromes was studied. RESULTS: We included 746 patients, 79.5% of whom could be documented with a single OMT diagnosis. In 20.5%, a combination of OMT diagnoses was documented. We documented 149 different combinations: 102 were documented once, 47 were documented repeatedly (n = 196); for example, in patients with Greig syndrome. The prevalence of this syndrome was significantly higher in patients with a combination of radial polydactyly, ulnar polydactyly, and/or syndactyly (2.9% vs 33.3% and 60% in patients with 1 vs 2 and 3 diagnoses). CONCLUSIONS: Documentation of combined OMT diagnoses is required in a fifth of the patients. Not doing so will cause loss of phenotypic information and can hamper outcome comparison and epidemiological research. Documentation of combined OMT diagnoses can help to identify subgroups within a population, for example, patients with an underlying syndrome. Last, combined documentation of diagnoses improves flexibility of the classification and thereby better allows universal application. CLINICAL RELEVANCE: Consensus on the application of the OMT classification is critical to achieving the universal adoption of the system by hand surgeons and other medical professionals.

Controversies in Poland Syndrome: Alternative Diagnoses in Patients With Congenital Pectoral Muscle Deficiency.

PURPOSE: Poland syndrome was first described as a deficiency of the pectoral muscle with ipsilateral symbrachydactyly. Currently, numerous case reports describe variations of Poland syndrome in which pectoral muscle deficiency is often used as the only defining criterion. However, more syndromes can present with pectoral muscle deficiency. The aim of this review is to illustrate the diversity of the phenotypic spectrum of Poland syndrome and to create more awareness for alternative diagnoses in pectoral muscle deficiency. METHODS: A systematic literature search was performed. Articles containing phenotypical descriptions of Poland syndrome were included. Data extraction included number of patients, sex, familial occurrence, and the definition of Poland syndrome used. In addition, hand deformities, thoracic deformities, and other deformities in each patient were recorded. Alternative syndrome diagnoses were identified in patients with a combination of hand, thorax, and other deformities. RESULTS: One hundred-and-thirty-six articles were included, describing 627 patients. Ten different definitions of Poland syndrome were utilized. In 58% of the cases, an upper extremity deformity was found and 43% of the cases had an associated deformity. Classic Poland syndrome was seen in 29%. Fifty-seven percent of the patients with a pectoral malformation, a hand malformation, and another deformity had at least 1feature that matched an alternative syndrome. CONCLUSIONS: Pectoral muscle hypoplasia is not distinctive for Poland syndrome alone but is also present in syndromes with other associated anomalies with a recognized genetic cause. Therefore, in patients with an atypical phenotype, we recommend considering other diagnoses and/or syndromes before diagnosing a patient with Poland syndrome. This can prevent diagnostic and prognostic errors. CLINICAL RELEVANCE: Differentiating Poland syndrome from the alternative diagnoses has serious consequences for the patient and their family in terms of inheritance and possible related anomalies.

Identification of Associated Genes and Diseases in Patients With Congenital Upper-Limb Anomalies: A Novel Application of the OMT Classification.

PURPOSE: Congenital upper-limb anomalies (CULA) can present as a part of a syndrome or association. There is a wide spectrum of CULA, each of which might be related to different diseases. The structure provided by the Oberg, Manske, and Tonkin (OMT) classification could aid in differential diagnosis formulation in patients with CULA. The aims of this study were to review the Human Phenotype Ontology (HPO) project database for diseases and causative genes related to the CULA described in the OMT classification and to develop a methodology for differential diagnosis formulation based on the observed congenital anomalies, CulaPhen. METHODS: We reviewed the HPO database for all diseases, including causative genes related to CULA. All CULA were classified according to the OMT classification; associated non-hand phenotypes were classified into 12 anatomical groups. We analyzed the contribution of each anatomical group to a given disease and developed a tool for differential diagnosis formulation based on these contributions. We compared our results with cases from the literature and with a current HPO tool, Phenomizer. RESULTS: In total, 514 hand phenotypes were obtained, 384 of which could be classified in the OMT classification. A total of 1,403 diseases could be related to those CULA. A comparison with 10 recently published cases with CULA revealed that the presented phenotype matched the descriptions in our dataset. The differential diagnosis produced using our methodology was more accurate than Phenomizer in 4 of 5 examples. CONCLUSIONS: The OMT classification can be used to describe hand anomalies that may present in over 1,400 diseases. CulaPhen was developed to provide a (hand) phenotype-based differential diagnosis. Differential diagnosis formulation based on the proposed system outperforms the system in current use. CLINICAL RELEVANCE: This study illustrates that the OMT diagnoses, either individually or combined, can be cross-referenced with different diseases and syndromes. Therefore, use of the OMT classification can aid differential diagnosis formulation for CULA patients.

Recurrence of Dupuytren's contracture: A consensus-based definition.

PURPOSE: One of the major determinants of Dupyutren disease (DD) treatment efficacy is recurrence of the contracture. Unfortunately, lack of agreement in the literature on what constitutes recurrence makes it nearly impossible to compare the multiple treatments alternatives available today. The aim of this study is to bring an unbiased pool of experts to agree upon what would be considered a recurrence of DD after treatment; and from that consensus establish a much-needed definition for DD recurrence. METHODS: To reach an expert consensus on the definition of recurrence we used the Delphi method and invited 43 Dupuytren's research and treatment experts from 10 countries to participate by answering a series of questionnaire rounds. After each round the answers were analyzed and the experts received a feedback report with another questionnaire round to further hone in of the definition. We defined consensus when at least 70% of the experts agreed on a topic. RESULTS: Twenty-one experts agreed to participate in this study. After four consensus rounds, we agreed that DD recurrence should be defined as "more than 20 degrees of contracture recurrence in any treated joint at one year post-treatment compared to six weeks post-treatment". In addition, "recurrence should be reported individually for every treated joint" and afterwards measurements should be repeated and reported yearly. CONCLUSION: This study provides the most comprehensive to date definition of what should be considered recurrence of DD. These standardized criteria should allow us to better evaluate the many treatment alternatives.

The Rotterdam Foot Classification: A Classification System for Medial Polydactyly of the Foot.

BACKGROUND: Polydactyly at the medial side of the foot ("medial polydactyly" of the foot) is a rare and diverse congenital anomaly. In order to plan and evaluate surgical treatment, the classification of medial polydactyly is useful. The aim of our study was to develop a reliable and valid classification system for medial polydactyly of the foot that is more useful than previous systems for preoperative evaluation and surgical planning. METHODS: A review of the literature and the clinical experience of a single experienced surgeon were used to determine classification categories. We identified all patients with medial polydactyly who had preoperative radiographs and clinical photographs and were treated at our hospital between 1993 and 2014. All affected feet were assessed according to our proposed classification system, the Rotterdam foot classification. The intrarater and interrater reliability among 5 observers who evaluated 30 feet were assessed with use of the Cohen kappa (κ) statistic. RESULTS: We developed a classification system that describes duplication type, syndactyly, the presence of a hypoplastic ray, and deviation of the hallux. Seventy-three feet were classified according to the system. Seven duplication types were distinguished. Complete metatarsal duplication was most frequently seen (in 29%). Twelve feet showed a broad hallux without external expression of duplication. Syndactyly between medial and lateral (duplicate) halluces was present in 30 feet; between the lateral hallux and second toe, in 13 feet; and between both duplicated halluces and the lateral hallux and second toe, in 21 feet. A hypoplastic ray was seen in 75% of the feet. Intrarater agreement for duplication, hypoplastic rays, syndactyly, and deviation were, respectively, κ = 0.79, 0.75, 0.59, and 0.78. Interrater agreement for duplication, hypoplastic rays, syndactyly, and deviation were, respectively, κ = 0.72, 0.54, 0.48, and 0.64. CONCLUSIONS: The proposed classification system contains 4 categories of anatomic features of the foot. Classification of all categories shows moderate to good reliability. Use of the Rotterdam classification in evaluating medial polydactyly improves type-specific description, which may, in the future, enhance the evaluation of surgical treatment. CLINICAL RELEVANCE: The Rotterdam foot classification system is a reliable and easy-to-use system that we believe will improve communication between clinicians and researchers and facilitate the evaluation of treatment results in medial polydactyly of the foot.

Percutaneous Aponeurotomy and Lipofilling (PALF) versus Limited Fasciectomy in Patients with Primary Dupuytren's Contracture: A Prospective, Randomized, Controlled Trial.

BACKGROUND: As an alternative to needle aponeurotomy release and limited fasciectomy treatment of Dupuytren's contracture, the authors introduced an extensive percutaneous aponeurotomy and lipofilling (PALF) procedure. In their previous study, the authors reported that contractures significantly improved and most patients returned to normal use of the hand within 2 to 4 weeks. To establish the safety and efficacy of PALF, the authors compared it to the standard limited fasciectomy in a single-blind, multicenter, prospective, randomized, controlled trial. METHODS: Patients with a primary Dupuytren's contracture were assigned randomly to the limited fasciectomy group or the PALF group. Patients were measured at baseline and at 2 weeks, 3 weeks, 6 months, and 1 year postoperatively. Primary outcome of the trial was contracture correction and convalescence time. Groups were compared using a mixed models approach. RESULTS: Eighty patients were randomized to PALF or limited fasciectomy. In both groups, almost full metacarpophalangeal joint contracture correction was obtained, whereas for the proximal interphalangeal joint, some residual contracture remained. Patients in the PALF group returned significantly earlier to their normal daily activity. At 1 year after surgery, no significant differences in recurrence rate or hand function were present. However, limited fasciectomy seems to have a higher incidence of permanent complications. CONCLUSIONS: PALF demonstrates a significantly shorter convalescence, similar operative contracture correction, lower incidence of long-term complications, and no significant difference regarding 1-year postoperative results compared with limited fasciectomy. It is therefore a valuable, minimally invasive alternative to limited fasciectomy in the treatment of Dupuytren's disease. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

A Matched Comparative Study of the Bilhaut Procedure Versus Resection and Reconstruction for Treatment of Radial Polydactyly Types II and IV.

PURPOSE: To compare outcomes of the Bilhaut procedure with outcomes of conventional resection and reconstruction in radial polydactyly types II and IV. METHODS: Patients treated with the Bilhaut procedure were radiologically matched with patients treated with reconstruction. Evaluated outcomes included the Rotterdam assessment system, pinch strength, and thumb size measurements. To determine objectively which aesthetic outcome scores truly depended on surgical technique rather than observer opinion, we analyzed evaluations by a panel of 22 individuals with varying clinical experience who were blinded to the study protocol, using a linear mixed regression model. RESULTS: The Bilhaut procedure reduced the risk of suboptimal outcome for metacarpophalangeal joint instability in type IV radial polydactyly. Conversely, the Bilhaut procedure increased the risk of suboptimal scar appearance, residual prominence at amputation site, thumb size, and nail appearance. Tip pinch strength was more significantly reduced after the Bilhaut compared with reconstruction, whereas pulp circumference and nail width exceeded 100% of the unaffected contralateral hand after the Bilhaut reconstruction. There was no significant difference in active range of motion between procedures. Nail appearance proved the only aesthetic drawback of the Bilhaut procedure after adjustment for clinical experience. CONCLUSIONS: There was superior metacarpophalangeal joint stability after the Bilhaut procedure for radial polydactyly type IV, but this did not result in the presumed benefit to thumb strength. For experienced surgeons, both procedures resulted in comparable thumb active range of motion. However, aesthetic results were more likely perceived as pleasing after conventional reconstruction, even after adjusting for observer experience with regard to nail appearance. Despite possible benefits of modified Bilhaut procedures that preserve the nail, conventional reconstruction is the preferred procedure until otherwise proven. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.

Patients' Preferences for Treatment for Dupuytren's Disease: A Discrete Choice Experiment.

BACKGROUND: Although in modern medicine, patients' preferences are important, these have never been defined for characteristics of Dupuytren treatment. This study determines these patients' preferences using a discrete choice experiment. METHODS: A multicenter discrete choice experiment study was conducted among patients with Dupuytren's disease who had been treated previously. Patients were asked about their preferences for attributes of Dupuytren treatments using scenarios based on treatment method, major and minor complication rates, recurrence rates, convalescence, residual extension deficit after treatment, and aesthetic results. The relative importance of these attributes and the tradeoffs patients were willing to make between them were analyzed using a panel latent class logit model. RESULTS: Five-hundred six patients completed the questionnaire. All above-mentioned attributes proved to influence patients' preferences for Dupuytren treatment (p < 0.05). Preference heterogeneity was substantial. Men who stated they performed heavy labor made different tradeoffs than women or men who did not perform heavy labor. In general, recurrence rate (36 percent) and extension deficit (28 percent) were the most important attributes in making treatment choices, followed by minor complication rate (13 percent). Patients accepted an increase in recurrent disease of 11 percent if they could receive needle aponeurotomy treatment instead of limited fasciectomy. CONCLUSIONS: This study confirms the importance of low recurrence rates and complete contracture corrections, but also emphasizes the significance of low complication rates. Convalescence was not an attribute, which scored high. The preference heterogeneity shows that patient consultations need to be targeted differently, which may result in different treatment decisions, depending on patient characteristics and preferences.

Collagenase Clostridium Histolyticum versus Limited Fasciectomy for Dupuytren's Contracture: Outcomes from a Multicenter Propensity Score Matched Study.

BACKGROUND: Controversy exists about the relative effectiveness of injectable collagenase (collagenase clostridium histolyticum) and limited fasciectomy in the treatment of Dupuytren's contracture. The authors compared the effectiveness of both techniques in actual clinical practice. METHODS: This study evaluated all subjects treated with collagenase clostridium histolyticum or limited fasciectomy for metacarpophalangeal and/or proximal interphalangeal joint contractures between 2011 and 2014 at seven practice sites. The authors compared the degree of residual contracture (active extension deficit), Michigan Hand Outcomes Questionnaire scores, and adverse events at follow-up visits occurring between 6 and 12 weeks after surgery or the last injection with the use of propensity score matching. RESULTS: In 132 matched subjects who were treated with collagenase (n = 66) or fasciectomy (n = 66), the degree of residual contracture at follow-up for affected metacarpophalangeal joints was not significantly different (13 degrees versus 6 degrees; p = 0.095) and affected proximal interphalangeal joints had significantly worse residual contracture in the collagenase group compared with those in the fasciectomy group (25 degrees versus 15 degrees; p = 0.010). Collagenase subjects experienced fewer serious adverse events than did fasciectomy subjects and reported larger improvements in the Michigan Hand Outcomes Questionnaire subscores evaluating satisfaction with hand function, activities of daily living, and work performance. CONCLUSIONS: This propensity score-matched study showed that collagenase clostridium histolyticum was not significantly different from limited fasciectomy in reducing metacarpophalangeal joint contractures, whereas proximal interphalangeal joint contractures showed slightly better reduction following limited fasciectomy. Collagenase provided a more rapid recovery of hand function than did fasciectomy and was associated with fewer serious adverse events. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.