Simple Talectomy is a Beneficial Surgical Procedure for Talipes Equinovarus and Other Severe Neuromuscular Foot Deformities.

Noninvasive techniques are gold standard to redress Severe Neuromuscular Foot Deformity (SNFD). However, simple talectomy may be considered to obtain a stable, plantigrade, pain-free foot. We present a 10-year follow-up accessing radiological correction rates, functional outcomes, complications, and patient satisfaction. This retrospective case series evaluated talectomies in 2012 to 2022. Simple talectomy was combined with Steinman pin fixation of calcaneus to tibia for approximately 6 weeks. Diagnoses primarily included arthrogryposis multiplex congenita and cerebral palsy. Indications were pain, wounds/pressure marks, severe rigidity, and residual/recurrent deformity. The primary outcome was radiological correction. Tibiotalar angle (TiTa) and tibiocalcaneal angle (TiCa) were measured on mediolateral projections. Secondary outcomes were functional scores of pain/deformity graded as good, fair or poor. Furthermore, validated patient-reported outcome measures, that is, EQ-5D-5L and the Scoliosis Research Society-30 Questionnaire (2 items) assessed health-related quality of life and patient satisfaction. Nineteen talectomies in 11 patients were analyzed. Mean follow-up was 62 months (range 9-112 months). Mean TiTa was 137° (95%CI 128;146). TiCa improved significantly: Mean difference -24° (95%CI -44;-5, p = .02). All feet became plantigrade and pain-free with no skin issues. Functional outcomes were graded as 9/19 good, 10/19 fair and 0/19 poor. Parents/primary caregivers were mainly satisfied. Perceived health was 54 (95%CI 34;75) out of 100 on a visual analogue scale, emphasizing complex medical conditions. In conclusion, simple talectomy is a suitable salvage procedure for SNFD.

Spinal Fusion in Patients With Classic Amyoplasia and General Arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita is a group of conditions characterized by joint contractures affecting 2 or more joints. This study describes results of spinal fusion in patients with classic amyoplasia and general arthrogryposis. METHODS: IRB approved retrospective review of patients with a diagnosis of classic amyoplasia and general arthrogryposis who had a primary definitive posterior spinal fusion between 1990 and 2017 at a single pediatric institution. Patients with distal and syndromic arthrogryposis were excluded as well as patients treated with growth-sparing spinal instrumentation. The Modified Clavien-Dindo-sink (MCDS) classification system was used to describe postoperative complications. RESULTS: Over the 28-year period, 342 patients were diagnosed with amyoplasia and general arthrogryposis. Among the 342 patients, 60 (18%) had scoliosis, and 22 (6% of the cohort and 37% of those with scoliosis) were treated surgically. Six patients had growth-sparing techniques, initial fusion elsewhere, or ˂1 year of follow-up, leaving 15 patients. Of the 15, 9 (60%) had a posterior spinal fusion (PSF) and 6 (40%) had a combined anterior spinal fusion (ASF)/PSF. The ASF/PSF group was significantly younger at surgery, had a greater American Society of Anesthesiologists status, longer surgery duration, and lower implant density. The average preoperative major coronal deformity in ASF/PSF patients (108 degrees) was greater than patients treated with PSF alone (88 deg). There were 11 complications in 7 patients, with the most common being deep infection requiring reoperation (5/11, 45%). There was 1 instance (1/11, 9%) of each: prolonged intensive care unit admission (>72 h), superficial wound dehiscence, symptomatic implants requiring removal/revision, coronal plane progression requiring extension of fusion, recurrent pneumothorax requiring return to OR, and pseudoarthrosis leading to implant failure (without revision). Complications occurred in 1/9 (11%) PSF-only patients and 6/6 (100%) ASF/PSF patients with all 6 ASF/PSF patients requiring at least 1 reoperation. The average coronal correction was 48% in the PSF-only group and 28% in the ASF/PSF group. CONCLUSION: Complication rates after spinal fusion for scoliosis in arthrogryposis multiplex congenita patients are high, especially in patients undergoing ASF/PSF, deep infection is common, and major coronal plane curve correction is modest. LEVEL OF EVIDENCE: II Retrospective Study.

Congenital vertical talus deformity in children with distal arthrogryposis: good clinical outcomes despite high rate of residual radiographic deformity.

Patients with a diagnosis of arthrogryposis often present with various orthopedic conditions, one of which is congenital vertical talus (CVT). This is the first study of this specific subset of syndromic patients to evaluate the medium-term outcomes of CVT correction using the minimally invasive Dobbs method. All patients with vertical talus and distal arthrogryposis who received treatment at our institution between January 2006 and June 2021 were identified. Radiographs, clinical notes and Patient-Reported Outcome Measurement Information System (PROMIS) scores (when available) were retrospectively reviewed. An alpha of 0.05 was used for all statistical analyses. In total 12 patients (19 feet) met all inclusion criteria and were included in the final analysis. By the time of the most recent visit, the average lateral Talar-Axis First Metatarsal Base Angle of the entire cohort increased from 13.73 ± 9.75 degrees 2 weeks postoperatively to 28.75 ± 23.73 degrees ( P = 0.0076). Radiographic recurrence of the talonavicular deformity was seen in nine feet (47.4%), 4 (21.1%) of which required additional unplanned surgery, The average PROMIS scores of the entire cohort in the pain interference, mobility and peer relationship domains were 48.97 ± 9.56, 47.9 ± 11.60 and 52.87 ± 8.31, respectively. Despite a higher radiographic recurrence rate of talonavicular deformity in this specific subset of syndromic patients, these patients still report PROMIS scores near the population average in the pain interference, mobility and peer relationships domains. We believe that the minimally invasive Dobbs method should be recommended as the first-line treatment method for these patients. Level of evidence: Level III.

Talectomy for arthrogrypotic foot deformities: A systematic review.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is one of the most common congenital joint contracture syndromes. Talectomy has been proposed for severe foot deformities in AMC, but the literature is limited. The purpose of this systematic review is to evaluate the indications, outcomes and complications of talectomy in arthrogrypotic foot deformities. METHODS: The articles were found through Embase and Medline. Screening was conducted by two independent investigators with disagreements resolved by a third reviewer. Relevant data regarding demographics, outcomes and complications were collected. RESULTS: Of 232 feet, 71.98 % and 62.22 % had clinical and radiographic improvements respectively. Amongst 122 patients, 92.62 % could ambulate following surgery. Recurrent deformities and revision surgery were seen in 16.81 % and 13.36 % of cases respectively. CONCLUSIONS: Talectomy is a valid surgical option for severe arthrogrypotic foot deformities with favorable post-operative outcomes and low complication rate.

Open Reduction at 15 Months of Left Hip Dislocation in a Male Infant Diagnosed with Arthrogryposis.

BACKGROUND Arthrogryposis is a congenital condition of multiple contractures of joints associated with hip dislocation. The outcome of open reduction of hip dislocation in arthrogryposis patients is debatable. Open reduction of arthrogryposis is challenging for shallow acetabulum and extensive adhesions and fibrosis. For this reason, a careful extensive release must be carried out to achieve the open reduction of the hip in arthrogryposis patients. The literature lacks surgical recommendations for open reduction of the hip in arthrogryposis patients and how to deal with cases of the extruded bone segment during open reduction. CASE REPORT The patient presented in the first few weeks of life with bilateral clubfoot and left hip dislocation. Clinical diagnosis of arthrogryposis was made after referral to a genetics specialist. The hip was clinically irreducible. The patient underwent open reduction and femoral shortening using the Smith Peterson approach at the age of 15 months, with accidental extrusion of the proximal femur, which was retained immediately. The clinical outcome showed a painless, good range of motion. Radiographically, features of avascular necrosis and healed osteotomy site were evident. CONCLUSIONS A difficult hip reduction was expected in this arthrogryposis patient, which required careful dissection of surrounding fibrosis and appropriate femoral shortening. Careful dissection should be carried out during open reduction to avoid jeopardization of femoral head vascularity or even complete devitalization of the proximal femur.

Abductor pollicis brevis rerouting and first web deepening for clasped thumb deformity in arthrogryposis multiplex congenita.

LEVEL OF EVIDENCE: III.

The Hand in Distal Arthrogryposis.

Distal arthrogryposis is the second most common type of arthrogryposis after amyoplasia and is defined as arthrogryposis that affects hands and feet; it is mostly inherited in an autosomal-dominant fashion. This review discusses up-to-date background information, clinical features, and treatment of distal arthrogryposis in hands concentrating on camptodactyly, thumb-in-palm deformity, and windblown hand deformity, which are the most common and functionally limiting deformities. Treating these deformities should be individualized and follow a multidisciplinary approach. Most deformities can be initially treated nonoperatively, and if not responsive, operative treatment may be pursued to improve function. Surgery primarily aims to release soft-tissue contractures, rebalance muscle forces, and may need bony correction based on the deficits of each case. Current literature suggests that early treatment leads to better outcomes. However, reported cases are scarce, and no consensus or gold standard for treatment exists. Therefore, long-term (multicenter) studies are needed to assess outcomes and standardize the treatment of such deformities whenever possible.

Total joint replacement of the hip and knee in patients with arthrogryposis multiplex congenita: a report of six joints.

INTRODUCTION: Arthrogryposis multiplex congenita (AMC) is a rare congenital condition that leads to severe joint contractures and deformities. As painful joint dysplasia and degeneration might develop over time, total joint replacement (TJR) can be a potential treatment option for these patients. The aim of this study is to investigate functional results, implant survivorship and potential complications in patients with AMC who undergo hip or knee arthroplasty. MATERIALS AND METHODS: We retrospectively identified six TJR in three patients at a single centre performed between 2006 and 2019. The median patient age at surgery was 23 years and the median follow-up period was 69 (IQR 55-99) months. We analysed surgical technique, implant survivorship and complications as well as functional outcome determined by pain reported on the Numerical Rating Scale (NRS), patient-reported outcome scores [Oxford Hip Score (OHS), Harris Hip score (HHS), Oxford Knee Score (OKS)], range of motion and ambulatory status. Depending on data distribution means with ranges and median with interquartile range were compared with the Wilcoxon signed rank test or Student's t test. The level of significance was defined at < 0.05. RESULTS: In hips, the mean range of motion in flexion/extension (52° vs. 85°, p = 0.014) and in rotation (28° vs. 68°, p = 0.02) as well as mean pain score on the NRS (8.5 vs. 0, p = 0.001), OHS (9 vs. 26, p = 0.031) and HHS (17 vs. 52, p = 0.007) significantly improved. In knees, mean range of motion (55° vs. 93°, p = 0.403), mean pain score on the NRS (0 vs. 7) and the OKS (2 vs. 21) also improved. While the ambulatory status did not change, the patients who were wheelchair dependent reported less problems with transfers to a bed or chair and the patient who ambulated reported an improved walking distance. One total knee arthroplasty (TKA) underwent revision for an acute, late infection 155 months following the initial surgery. CONCLUSIONS: TJR is a safe procedure in patients with AMC that effectively improves function and reduces pain irrespective of preoperative ambulatory status.

Improvement of Pulmonary Function in Arthrogryposis Multiplex Congenita Patients Undergoing Posterior Spinal Fusion Surgery for Concomitant Scoliosis: A Minimum of 3-Year Follow-up.

OBJECTIVE: We sought to investigate the long-term outcome of pulmonary function for arthrogryposis multiplex congenita (AMC) patients undergoing posterior spinal fusion (PSF) and to further determine influential factors. METHODS: Eighteen AMC patients with a minimum of 3-year follow-up after PSF were prospectively collected. All the patients underwent a pulmonary function test before surgery and at the final follow-up. The percentage predicted values of vital capacity (VC%) and forced vital capacity (FVC%) were recorded. The following radiographic parameters were collected including Cobb angle and thoracic kyphosis. The total lung volumes (TLV) were measured on the image of 3-dimensional computed tomography scan by the reconstruction software. RESULTS: There were 10 males and 8 females with a mean age of 13.8 ± 6.1 years. The mean preoperative VC% and FVC% were 40.5% ± 7.6% and 39.5% ± 4.7%, which were significantly increased to 52.0% ± 7.5% and 51.2% ± 6.8% at the final follow-up (P < 0.001). Besides, there was remarkable improvement in terms of TLV (1.57 ± 0.2 L vs. 2.39 ± 0.6 L, P < 0.001). Remarkable correlations were observed between TLV and pulmonary function tests (r = 0.79, P < 0.001 for VC%; r = 0.78, P < 0.001 for FVC%). Multiple regression analysis showed that 2 variables including Δ thoracic kyphosis and Δ Cobb angle were independently associated with the improvement of pulmonary function. CONCLUSIONS: The pulmonary function of AMC patients can be well improved through PSF surgery. It was remarkably associated with the correction of curve magnitude and restoration of thoracic kyphosis.

Functional Outcomes of Talectomy in Pediatric Feet.

BACKGROUND: Historically, talectomy has been predominantly performed to operatively treat severely rigid equinovarus feet. A limited number of investigators have studied functional outcomes in pediatric patients posttalectomy. We aimed to assess the outcomes of pediatric patients undergoing talectomy using the American Orthopaedic Foot & Ankle Society (AOFAS) score and a subjective survey of patients' and their caregivers' satisfaction. METHODS: We performed a retrospective cohort study that included 31 patients with nonidiopathic severely rigid talipes equinovarus, in a single center, using consecutive sampling. All medical records of those patients were reviewed, and relative data were extracted. The AOFAS score was used to measure the outcomes during the last visit (April 2020). Satisfaction was evaluated in a binary manner by questioning the patients and their caregivers if they would undergo the same surgery again for the same result. RESULTS: Thirty-one patients were included. Myelomeningocele was the primary diagnosis in 13 patients (41.9%), and arthrogryposis was diagnosed in 11 patients (35.5%). Twenty-two patients had bilateral procedures. The mean age at the time of surgery was 6.0 ± 3.0 years, and the mean follow-up was 6.0 ± 1.0 years. Plantigrade feet following the primary surgery were achieved in 88.5% of cases. Postoperatively, braces were well tolerated in 86.5% of patients. Deformity recurrence was observed in 21.2% of patients, and 17.3% of patients required subsequent surgeries. Patients with arthrogryposis had significantly higher AOFAS scores than those with myelomeningocele and other diagnoses (P = .017). Further, patients who tolerated braces had higher AOFAS scores than those who did not tolerate braces (P = .006). However, patients who developed hindfoot varus and dorsal bunion postoperatively had lower AOFAS scores (P = .054 and P = .006, respectively). Patients who had recurrent deformities or required further surgeries also had lower AOFAS scores (P = .025 and P = .015, respectively). Although 17.3% of patients were not able to comment about their satisfaction due to their general medical condition, 63.5% of patients reported that they were satisfied. Furthermore, 75.0% of caregivers were satisfied with the outcomes and their children's functional status posttalectomy. CONCLUSION: The observed outcomes of primary and salvage talectomies demonstrate the general overall effectiveness of this operative intervention as an end-stage treatment for pediatric patients with severely rigid talipes equinovarus. LEVEL OF EVIDENCE: Level III; retrospective cohort study.

Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes. METHODS: Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression. RESULTS: Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS. CONCLUSIONS: Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Nerve wrapping for recurrent compression neuropathy: A systematic review.

BACKGROUND: The recurrence of symptoms following primary nerve compression surgery can occur in up to 25% of cases. Nerve wrapping can be utilised for revision surgery. An ideal barrier should minimise the chance of fibrosis, scarring and allow for adequate nerve gliding. This review evaluates the use of autologous or commercially available allograft and xenograft options as barriers against nerve scarring following revision surgery. METHODS: PubMed, Ovid Embase and Cochrane databases were searched using the All Fields Index. Nine hundred titles underwent title screening with 11 studies being included in the final analysis. The risk of bias was assessed using the Methodological Index for Non-Randomised Studies (MINORS) tool. PRISMA guidelines were followed at all stages and the review was registered with PROSPERO (CRD 42020182818). RESULTS: The 11 studies comprised of all case series. In total, 114 patients were included, with ages ranging from 28 to 90. Previously, the number of revision surgeries ranged from 0 to 5. Autologous veins were used in 6 studies, collagen in 3 studies and human amniotic membrane in 2 studies. Improvements in subjective and objective outcomes were seen with all wrap types. Pain was the most common residual symptom (46% of patients). The most common complication was pain at the donor site following vein harvest (19% of patients). CONCLUSION: This is the first systematic review to summarise the outcomes of nerve wraps for revision compression neuropathy. While improvements in outcomes were reported, further comparative studies are needed to determine the best nerve wrap.

Talectomy by Medial Surgical Approach for Congenital Vertical Talus in Arthrogryposis Multiplex Congenita.

Arthrogryposis multiplex congenita involves stiff contracture of joints and weak atrophic muscles presenting at birth. The two most common forms are amyoplasia and distal arthrogryposis. Amyoplasia affects all 4 extremities: internally rotated shoulders, extended fixed elbows, flexed fixed wrists, extended fixed knees, clubfeet, and decreased muscle volume. Distal arthrogryposis is a group of syndromes with a genetic basis. The distal joints are contracted. Clubfeet and congenital vertical talus are the most common foot deformities. A 10-year-old boy presented with distal arthrogryposis with bilateral congenital tali. He reported having deformed and painful feet and difficulty wearing shoes. His rocker-bottom foot deformities caused him to walk with a heel to heel gait. He also had stiff extended knees. His previous foot surgeries included failed open reduction and pin fixation of the talonavicular joints with Achilles tendon lengthening and capsulotomies. The boy underwent bilateral talectomies and releases of contracted joint capsules and lengthening of multiple extrinsic tendons through separate incisions. The talectomy of each foot was performed via a novel medial surgical approach. At 2-year follow-up, he had normal-appearing plantar grade feet. He had a painless gait, could ambulate independently, and was considered to have an excellent result. This is the first detailed report of performing a talectomy via a medial approach for bilateral congenital tali in a patient with arthrogryposis multiplex congenita. [Orthopedics. 2020; 43(6):e623-e626.].

Bipolar Latissimus Dorsi Transfer for Arthrogryposis Multiplex Congenita: Minimum 10-Month Follow-Up.

PURPOSE: To examine the outcomes of patients who have undergone bipolar latissimus dorsi transfer for loss of elbow flexion in arthrogryposis multiplex congenita (AMC). METHODS: This study retrospectively evaluated 6 cases (5 patients) of bipolar latissimus dorsi transfer performed to restore active elbow flexion in pediatric patients with AMC. Elbow range of motion and strength were evaluated before and after surgery. Functional outcomes were evaluated by the patients' ability to perform activities of daily living. Complications and patient satisfaction were also evaluated at final follow-up. RESULTS: The patients were a mean age of 7.8 ± 3 years. The mean follow-up was 30.2 months (range, 10-44 months). At most recent follow-up, all cases reported improved function of the surgical extremity when performing activities of daily living and overall satisfaction. The postoperative active range of motion was 76° ± 14°. All cases had active elbow flexion against gravity. One patient was noted to have decreased muscle activation of the transfer 6 months after surgery, but strength improved by the 10-month follow-up. No other complications were noted. CONCLUSIONS: We recommend bipolar latissimus dorsi transfer as a reliable option to restore functional elbow flexion in patients with AMC. Meticulous pedicle handling and assessment of the latissimus dorsi viability is paramount. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Early open reduction of dislocated hips using a modified Smith-Petersen approach in arthrogyposis multiplex congenita.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a rare syndrome with multiple joint contractures. Within the medical community, there is controversy surrounding AMC in terms of the ideal surgical approach and age for performing a reduction of dislocated hips. The purpose of this retrospective study was to evaluate the clinical outcomes of early open reduction of infant hip dislocation with arthrogryposis multiplex congenita following a modified Smith-Petersen approach that preserves the rectus femoris. METHODS: From 2010 to 2017, we performed this procedure on 28 dislocated hips in 20 infants under 12 months of age with AMC. The clinical and radiology data were reviewed retrospectively. The mean age at surgery was 6.9 ± 5.1 months, with a mean follow-up of 42.4 ± 41.1 months. RESULTS: After open reduction, the average hip acetabular index (AI), the international hip dysplasia institute classification (IHDI), and the hip range of motion significantly improved (all P < 0.001). After the surgery, 16 patients were community walkers, and four patients were home walkers. Three hips in two patients required secondary revision surgery for residual acetabular dysplasia with combined pelvic osteotomy and femoral osteotomy. Seven of the hips that had been operated on showed signs of avascular necrosis (AVN). Among them, four were degree II, two were degree III, and one was degree IV. Multiple linear regression analysis demonstrated that greater age (in months) heightened the risk for secondary revision surgery (P = 0.032). CONCLUSIONS: The modified Smith-Petersen approach preserving the rectus femoris is an encouraging and safe option for treating hip dislocation in young AMC patients (before 12 months). If surgery takes place at less than 12 months of age for patients with AMC, this earlier open reduction for hip dislocation may reduce the chances of secondary revision surgery. LEVEL OF EVIDENCE: IV, retrospective non-randomized study.

Findings, Phenotypes, Diagnostic Accuracy, and Treatment in Freeman-Burian Syndrome.

Freeman-Burian syndrome (FBS) is a rare congenital myopathic craniofacial syndrome. Since publication of the genotype-correlated clinical diagnostic criteria, no complete survey of the literature has been accomplished. As part of the clinical practice guideline development, we evaluate diagnostic accuracy for FBS from 1938 to 2019 and range of findings, complications, treatments, and outcomes. Published manuscripts in PubMed, Google Scholar, and OMIM describing cases with a reported diagnosis of FBS, Sheldon-Hall syndrome, and distal arthrogryposes type 1 and 3 are initially included. Articles with sufficient case-level data for diagnosis verification are analyzed further. Of 724 unique papers considered, 188 papers describing 304 unique patients are included; 101 papers and 119 patients reflect an FBS diagnosis, with 80 patients meeting the full diagnostic criteria. Most cases are re-screened as distal arthrogryposis type 1. Among all cases re-screened as FBS, the presence of FBS pathognomonic craniofacial findings is not correlated with other physical findings. There are no significant differences between patients meeting the full diagnostic criteria and those not, but both are distinct from other diagnoses. Plastic surgery demonstrates the highest cumulative diagnostic accuracy for FBS overall (86.66%), while orthopedic surgery shows the lowest (44.83%). No statistically usable treatment-related or psychosocial data are available. Quality of case reports and patient data vary widely, reducing the statistical strength and significance. Major knowledge gaps exist in treatment, psychosocial, and longitudinal outcomes. At this point, it is impossible to derive clinical practice guidelines exclusively from the literature.

Elbow Release and Tricepsplasty in Arthrogrypotic Patients: A Long-Term Follow-Up Study.

PURPOSE: Obtaining elbow flexion to improve hand-to-mouth reach capability is an essential component of achieving functional independence in pediatric patients with arthrogryposis. This study analyzed the long-term outcomes of elbow release and tricepsplasty in a series of children with arthrogryposis at a tertiary institution. METHODS: Medical records of patients with arthrogryposis who underwent elbow release and tricepsplasty from 1993 to 2015, with at least 2 years of follow-up, were reviewed. Collected measures included preoperative elbow passive range of motion (ROM), postoperative elbow passive and active ROM, shoulder passive and active ROM, and Pediatric Outcomes Data Collection Instrument (PODCI) scores. Our analysis compared pre- and postoperative follow-up of elbow passive ROM and reviewed PODCI scores with age-adjusted normative values. RESULTS: Seventeen patients (4 female and 13 male) with 24 affected upper extremities (10 left elbow and 14 right elbow) were included in final analysis. Age at final follow-up averaged 11 years (range, 4-20 years), mean age at surgery was 2.7 years (range, 9.6 months-9.3 years) with mean follow-up by extremity at 8.3 years (range, 2-18 years). Differences in pre- and post-operative passive elbow ROM were significant for extension, flexion, and total arc of motion. Most parent and self-reported PODCI scores were less than the age-adjusted normal population, except in the domains of Comfort and Pain and Happiness. CONCLUSION: Long-term follow-up of elbow release and tricepsplasty in patients with arthrogryposis indicates both increased and sustained elbow flexion and arc of motion. Although PODCI scores were lower compared with the age-adjusted norm, pediatric patients with arthrogryposis were just as happy and had no more discomfort than their unaffected age-adjusted norms. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Comparison of plate and screw constructs versus screws only for anterior distal femoral hemiepiphysiodesis in children.

The study aim was to compare methods of anterior distal femoral hemiepiphysiodesis (ADFH) for treatment of fixed knee flexion deformities in ambulatory children with neuromuscular conditions and flexed knee gait. This is a retrospective review of 47 children (14 female, 33 male, age at surgery: 12.1 ± 2.7 years) who underwent ADFH between 2009 and 2016. Subjects were grouped by ADFH construct: one transphyseal screw (N = 11), two transphyseal screws (N = 28) or plates and screws (P/S group, N = 8). Clinical/radiographic variables were analyzed using paired t tests, χ tests, multiple regression and analysis of covariance. Participants experienced significant reduction in knee flexion contractures (Δ12°, P < 0.006), with no difference among groups (P = 0.43). Postoperative knee pain was significantly more prevalent in the P/S group (5/8, 63%) than the 1-SCR group (0/11, 0%) and the 2-SCR group (2/28, 7%) (P = 0.002). ADFH results in significant reduction of knee flexion deformity and improved knee extension during gait. Plate and screw constructs, the 1 and 2 transphyseal screw techniques are equally effective, but plate and screw constructs may be associated with a higher risk of persistent postoperative knee pain.

Tension band plate-guided growth of knee-flexion deformity in arthrogryposis multiplex congenita in which metaphyseal funnelization induced screw encroachment upon the neurovascular bundle.

Knee-flexion deformity in arthrogryposis multiplex congenita is treated by serial casting into extension, distal femoral osteotomies, distal femoral-guided growth, hemiepiphysiodesis, external fixation, capsulotomy, and soft-tissue releases. We are aware of four cases treated by distal anterior femoral-guided growth with tension band plates in which an unreported complication occurred: the screws of the tension band plates penetrated the posterior cortex of the femur during remodeling with metaphyseal funnelization risking the neurovascular bundle. Inclusion criteria were cases with arthrogryposis multiplex congenita and knee-flexion deformity, treatment at our institution by distal anterior femoral-guided growth with tension band plates, and radiographic evidence of posterior cortex screw penetration during remodeling from growth. Six knees (four cases) met the inclusion criteria. The average age at the distal anterior femoral-guided growth with tension band plate operation was 5.8 years. Radiographs after 6.6 years of follow-up showed that the screws of the tension band plates, which at surgery were intrametaphyseal, had penetrated the posterior cortex of the femur. Four knees (two cases) had diffuse pain around the knee to lower leg area, and instrumentation removal alleviated the symptoms. During distal anterior femoral-guided growth with tension band plate operation for knee-flexion deformity in arthrogryposis multiplex congenita, we found that the screws of the tension band plates, which were initially located inside the metaphysis, may protrude through the posterior bone cortex during metaphyseal funnelization with growth, and may encroach upon the neurovascular tissues. Level of evidence: Level IV - case series.