High Above Knee Amputations for Femoral Osteosarcomas Treated with Free Microsurgical Tibial Reconstruction in the Pediatric Population.

SUMMARY: Improvements in the management of pediatric sarcoma including imaging, neo- and adjuvant therapy, and surgical technique has enhanced long-term survival of patients. Pediatric patients diagnosed with a femoral osteosarcoma undergoing oncologic resection who are ineligible for limb preservation reconstruction or rotationplasty are offered an above knee amputation (AKA). Limb amputations in the skeletally immature patient poses particular problems specific to endosteal bone overgrowth and spiking. Approximately half of these patients undergo revision of their amputation site. Furthermore, a high AKA often requires a hip-based prosthesis, which can be uncomfortable leading to poorer outcomes and higher energy expenditures.We have completed four 'spare parts' microvascular free tibial transfers for pediatric patients diagnosed with femoral osteosarcoma who were treated with an AKA. Two of these patients are currently ambulatory with their prosthetic and have not required long-term revision of their amputation site. One patient demonstrated radiographic evidence of bony union four months post-operatively, but subsequently succumbed to chemotherapy-resistant metastases. The fourth patient is in the process of prosthetic fitting and rehabilitation.This is a novel technique and the first series describing both proximal and distal free microvascular tibial bone transfers as a reconstructive option for pediatric patients with femoral osteosarcomas. It is a reliable flap with possible variations in vascular anatomy. Furthermore, it offers multiple benefits including the prevention of bony spiking, AKA stump augmentation, and facilitation of appropriate prosthetic fitting improving functional outcomes and decreased energy expenditure. However, the soft tissue coverage must be well planned.

Bilateral Dorsal Nail Curvature of the Thumb Distal Phalanx - A Case Report.

Congenital dorsal curvature of the distal phalanx has been previously described as 'reverse Kirner' or 'ski-jump' deformity. This report describes bilateral occurrence in the thumbs. A 13-year-old male presented with difficulty caring for his thumbnails and in picking up small objects. Examination showed dorsal curvature of the distal phalanges of both thumbs, with greater curvature of the right side. Radiographs showed wedge-shaped epiphyses and dorsal curvature without coronal plane deviation of the distal phalanges. There was objective and subjective decrease in function associated with lateral pinch and tripod grasp. The reported aetiopathogenesis for Kirner deformity cannot explain the observed dorsal curvature. The bilateral nature makes a secondary physeal cause unlikely and suggests an embryologic basis. Due to the noticeable deficits in function, operative intervention may be warranted. Level of Evidence: Level V (Therapeutic).

Intramedullary Fixation of Double-Barrel Vascularised Fibula Grafts With Subsequent Lengthening for Reconstruction of the Distal Femur in Patients With Osteosarcoma.

BACKGROUND: The use of vascularised fibula grafts is an accepted method for reconstructing the distal femur following resection of malignant childhood tumors. Limitations relate to the mismatch of the cross-sectional area of the transplanted fibula graft and the local bone, instability of the construct and union difficulties. We present midterm results of a unique staged technique-an immediate defect reconstruction using a double-barrel vascularised fibula graft set in in A-frame configuration and a subsequent intramedullary femoral lengthening. METHODS: We retrospectively included 10 patients (mean age 10 y) with an osteosarcoma of the distal femur, who were treated according to the above-mentioned surgical technique. All patients were evaluated with regards to consolidation of the transplanted grafts, hypertrophy at the graft-host junctions, leg length discrepancies, lengthening indices, complications as well as functional outcome. RESULTS: The mean defect size after tumor resection was 14.5 cm, the mean length of the harvested fibula graft 22 cm, resulting in a mean (acute) shortening of 4.7 cm (in 8 patients). Consolidation was achieved in all cases, 4 patients required supplementary bone grafting. Hypertrophy at the graft-host junctions was observed in 78% of the evaluable junctions. In total 11 intramedullary lengthening procedures in 9 patients had been performed at the last follow up. The mean Muskuloskeletal Society Rating Scale (MSTS) score of the evaluable 9 patients was 85% (57% to 100%) with good or excellent results in 7 patients. CONCLUSIONS: A-frame vascularised fibula reconstructions showed encouraging results with respect to defect reconstruction, length as well as function and should therefore be considered a valuable option for reconstruction of the distal femur after osteosarcoma resection. The surgical implementation is demanding though, which is emphasized by the considerable high number of complications requiring surgical intervention, even though most were not serious. LEVEL OF EVIDENCE: Level IV-case series.

Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor of dermal origin. Six percent of all cases present in children, with a childhood incidence of 1 per million. METHODS: This is a retrospective review of all cases of pediatric DFSP managed at a single institution over a 23-year period. RESULTS: Seventeen patients (10 male; mean age, 9.9 years) were managed during the study period. The median follow-up was 29 months. All patients had surgical excision. Three patients required further excision to achieve uninvolved final margins. There were no recurrences observed. CONCLUSIONS: Pediatric DFSP should be managed by a soft tissue tumor multidisciplinary team, with experienced pathologists and reconstructive surgeons. Where R0 resections are obtained, patients can experience recurrence-free survival.

Pedicled fibular transfer for biologic knee extensor tendon reinsertion following proximal tibial resection in pediatric osteosarcoma: Long-term outcomes.

INTRODUCTION: Proximal tibial sarcoma resections result in a reconstructive challenge, necessitating joint and extensor mechanism reconstruction. The gait and functional outcomes for children reconstructed with a combination of megaprosthesis and pedicled fibular flap for extensor mechanism reconstruction, are presented. METHODS: Four patients, aged 11-18 years old, were available for comprehensive analysis. The proximal tibial osteosarcoma was resected, and the reconstructive technique involved a megaprosthesis for the knee joint, used in combination with a pedicled fibula flap as a biologic structure for reinsertion of the knee extensor mechanism. Outcomes were measured with three-dimensional gait analysis and patient questionnaires. RESULTS: Minor postoperative wound issues occurred in some patients, requiring debridement with skin grafting. One patient fractured their transferred fibula, requiring fixation. The follow up period ranged from 1.7 to 24 years postoperatively. The longevity and quality of reconstructions were strong, measured by both objective and patient-reported outcomes. All patients reported independent walking >500 m in the Functional Mobility Scale and rated their walking as a nine or 10 (out of 10) on the Functional Assessment Questionnaire. Knee society scoring revealed overall satisfaction rate of 75-80%. No patients required gait aids. The gait profile analysis revealed effective gait patterns, with patterns deviating 5.4-7° from "typical gait." Deviations >6.5° are considered abnormal. CONCLUSION: The long-term results of combining a megaprosthesis with a pedicled fibula flap for extensor reinsertion, revealed a high level of independent function. The patients performed well, without the need for aids, and gait study evidence of minimal gait deviations.

A Systematic Review of Intra-abdominal Tissue Expansion for the Treatment of Exomphalos Major and a Case Report Describing a Refinement of the Technique.

ABSTRACT: The management of complex exomphalos major is difficult, and traditional techniques fail to address the visceroabdominal disproportion in the most severe cases. Intra-abdominal tissue expansion is a novel technique and has been used in a small number of patients to safely increase the intra-abdominal volume and allow the reduction of viscera and subsequent closure of the abdominal domain. We review 7 published reports of this technique and add a case report describing our refinement of the technique. We propose that the use of multiple expanders placed in the intra-abdominal preperitoneal space, when expanded slowly, can allow safe reduction of viscera and immediate direct closure of the musculofascial layer of the abdomen.

The psychosocial impact of congenital hand and upper limb differences on children: a qualitative study.

This qualitative study explored the psychosocial concerns of children born with congenital hand and upper limb differences (CHULDs) from the point of view of these children and their parents. Qualitative, in-depth, semi-structured interviews were conducted face-to-face with eight parent-child dyads. Open format questions allowed spontaneous emergence of relevant themes, followed by guided questioning. Thematic analysis of audio-recorded and transcribed interviews found that children as young as 5 years old had unique and meaningful opinions about their CHULD, of which parents were sometimes unaware. Children reported that unsolicited questions from peers caused significant stress, and this increased around the time of surgery. All children used planned responses to peers as an effective coping mechanism. Children also identified positive aspects of their difference, including an increased sense of determination and an appreciation for being unique. Strategies that may improve psychosocial outcomes for children with CHULDs are discussed.Level of evidence: IV.

An International Collaborative Standardizing Patient-Centered Outcome Measures in Pediatric Facial Palsy.

IMPORTANCE: Standardization of outcome measurement using a patient-centered approach in pediatric facial palsy may help aid the advancement of clinical care in this population. OBJECTIVE: To develop a standardized outcome measurement set for pediatric patients with facial palsy through an international multidisciplinary group of health care professionals, researchers, and patients and patient representatives. DESIGN, SETTING, AND PARTICIPANTS: A working group of health care experts and patient representatives (n = 21), along with external reviewers, participated in the study. Seven teleconferences were conducted over a 9-month period between December 3, 2016, and September 23, 2017, under the guidance of the International Consortium for Health Outcomes Measurement, each followed with a 2-round Delphi process to develop consensus. This process defined the scope, outcome domains, measurement tools, time points for measurements, and case-mix variables deemed essential to a standardized outcome measurement set. Each teleconference was informed by a comprehensive review of literature and through communication with patient advisory groups. Literature review of PubMed was conducted for research published between January 1, 1981, and November 30, 2016. MAIN OUTCOMES AND MEASURES: The study aim was to develop the outcomes and measures relevant to children with facial palsy as opposed to studying the effect of a particular intervention. RESULTS: The 21 members of the working group included pediatric facial palsy experts from 9 countries. The literature review identified 1628 papers, of which 395 (24.3%) were screened and 83 (5.1%) were included for qualitative evaluation. A standard set of outcome measurements was designed by the working group to allow the recording of outcomes after all forms of surgical and nonsurgical facial palsy treatments among pediatric patients of all ages. Unilateral or bilateral, congenital or acquired, permanent or temporary, and single-territory or multiterritory facial palsy can be evaluated using this standard set. Functional, appearance, psychosocial, and administrative outcomes were selected for inclusion. Clinimetric and psychometric outcome measurement tools (clinician-, patient-, and patient proxy-reported) and time points for measuring patient outcomes were established. Eighty-six independent reviews of the standard set were completed, and 34 (85%) of the 40 patients and patient representatives and 44 (96%) of the 46 health care professionals who participated in the reviews agreed that the standard set would capture the outcomes that matter most to children with facial palsy. CONCLUSIONS AND RELEVANCE: This international collaborative study produced a free standardized set of outcome measures for evaluating the quality of care provided to pediatric patients with facial palsy, allowing benchmarking of clinicians, comparison of treatment pathways, and introduction of value-based reimbursement strategies in the field of pediatric facial palsy. LEVEL OF EVIDENCE: NA.

End-to-side Somatic-to-autonomic Nerve Grafting to Restore Erectile Function and Improve Quality of Life After Radical Prostatectomy.

BACKGROUND: Radical prostatectomy (RP) is recommended for the treatment of men with clinically localised prostate cancer. However, RP is associated with a high incidence of erectile dysfunction (ED), which can impact the quality of life (QoL) significantly. OBJECTIVE: To evaluate the effectiveness of end-to-side nerve grafting surgery to restore erectile function and improve sexual QoL in men with ED after RP. DESIGN, SETTING, AND PARTICIPANTS: A retrospective review of a single-centre experience of nerve grafting in men with ED following RP was performed. Seventeen men had surgery between March 2015 and October 2017 in Melbourne, Australia, which fulfilled study inclusion and exclusion criteria. INTERVENTION: Microsurgical bilateral end-to-side nerve grafts from a selective fascicular neurotomy of the femoral nerve to the penile corpora cavernosa. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: Results were serially measured utilising the International Index of Erectile Function (IIEF-5) and the sexual domain of Expanded Prostate Cancer Index Composite (EPIC-26). The proportion and 95% confidence interval (CI) of men recovering sexual function following nerve grafting were determined. RESULTS AND LIMITATIONS: All patients had ED following their RP. Median age at nerve grafting was 64yr (interquartile range [IQR] 60-66yr). Median time between nerve- and non-nerve-sparing RP, and nerve grafting was 2.4 (IQR 2.1-3.1) and 2.2 (IQR 1.7-5.1)yr, respectively. Median follow-up was 18 (IQR 15-24) mo. At 12mo after nerve grafting, 71% (95% CI 44-90%) of patients had erectile function recovery sufficient for satisfactory sexual intercourse, and 94% (95% CI 71-99%) and 82% (95% CI 57-96%) had clinically significant improvements in sexual function and reduced bother, respectively. There were two minor wound infections. Limitations include the retrospective study design. CONCLUSIONS: End-to-side nerve grafting restored erectile function in 71% of men with ED following RP, supporting previous findings. Of the men, 94% had clinically relevant improvements in sexual QoL. We recommend multicentre implementation of post-RP nerve grafting into clinical practice with appropriate data collection to confirm its efficacy and feasibility. PATIENT SUMMARY: We provide confirmatory evidence that end-to-side nerve grafting surgery restored erectile function and improved sexual quality of life in, respectively, 71% and 94% of men with erectile dysfunction following radical prostatectomy.

Carpal Tunnel Syndrome in Aberrant Muscle Syndrome: A Case Report and Review of the Literature.

Aberrant Muscle Syndrome (AMS) is a rare congenital hand difference that is characterised by unilateral non-progressive muscular hyperplasia. The aetiology of aberrant muscle syndrome is not known, but a recently published case has shown a somatic PIK3CA activating mutation in a patient with AMS. Carpal tunnel syndrome (CTS) in children is rare. The most common causes are the mucopolysaccaridoses but space-occupying lesions have also been reported to cause CTS in children. We report the first case of CTS in a child with AMS successfully treated with open carpal tunnel release and excision of aberrant muscles.

Investigation of the activation of the temporalis and masseter muscles in voluntary and spontaneous smile production.

INTRODUCTION: Masticatory muscles or their nerve supply are options for facial reanimation surgery, but their ability to create spontaneous smile has been questioned. This study assessed the percentage of healthy adults who activate the temporalis and masseter muscles during voluntary and spontaneous smile. METHODS: Healthy volunteer adults underwent electromyography (EMG) studies of the temporalis and masseter muscles during voluntary and spontaneous smile. Responses were repeated three times and recorded as negative, weakly positive, or strongly positive according to the activity observed. The best response was used for analysis. RESULTS: Thirty healthy adults (median age: 34 years, range: 25-69 years) participated. Overall, 92% of the masseter muscles were activated during voluntary smile (22% strong, 70% weak). Seventy-seven percent of the masseter muscles were activated in spontaneous smile (12% strong, 65% weak). The temporalis muscle was activated in 62% of responses in voluntary smile (15% strong, 47% weak) and in 45% of responses in spontaneous smile (13% strong, 32% weak). No significant difference was found for males vs females or closed vs open mouth smiles. There was no significant difference in responses between voluntary and spontaneous smiles for the temporalis and masseter muscles, and their use in voluntary smile did not predict activity in spontaneous smile. CONCLUSIONS: Our study has shown that masseter and temporalis are active in a high proportion of healthy adults during voluntary and spontaneous smiles. Further work is required to determine the relationship between preoperative donor muscle activation and postoperative spontaneous smile, and whether masticatory muscle activity can be upregulated with appropriate training.

A-Frame free Vascularized Fibular Graft and Femoral Lengthening for Osteosarcoma Pediatric Patients.

BACKGROUND: Pediatric limb reconstruction after resection of a malignant tumor presents specific challenges. Multiple surgical techniques have been used to treat these patients. This paper describes a staged surgical technique for the reconstruction of large distal femoral defects due to tumor resection in skeletally immature patients. METHODS: Three pediatric patients with osteosarcoma of the distal femur underwent staged reconstruction. Neoadjuvant chemotherapy was followed by en bloc tumor resection and immediate reconstruction of the distal femoral defect with a vascularized free fibular autograft utilizing a unique A-frame construct combined with intramedullary nail fixation. The second stage was a planned gradual lengthening of the healed construct, over a custom-made magnetically driven expandable intramedullary nail. RESULTS: All patients achieved bony union and satisfactory length with minimal complications. The patients all returned to full, unlimited physical activities. CONCLUSIONS: The early results confirm that the described technique is a safe and reliable procedure for the reconstruction of large femoral defects in pediatric patients with osteosarcoma. LEVEL OF EVIDENCE: Level IV-therapeutic.

Worldwide Testing of the eFACE Facial Nerve Clinician-Graded Scale.

BACKGROUND: The electronic, clinician-graded facial function scale (eFACE) is a potentially useful tool for assessing facial function. Beneficial features include its digital nature, use of visual analogue scales, and provision of graphic outputs and scores. The authors introduced the instrument to experienced facial nerve clinicians for feedback, and examined the effect of viewing a video tutorial on score agreement. METHODS: Videos of 30 patients with facial palsy were embedded in an Apple eFACE application. Facial nerve clinicians were invited to perform eFACE video rating and tutorial observation. Participants downloaded the application, viewed the clips, and applied the scoring. They then viewed the tutorial and rescored the clips. Analysis of mean, standard deviation, and confidence interval were performed. Values were compared before and after tutorial viewing, and against scores obtained by an experienced eFACE user. RESULTS: eFACE feedback was positive; participants reported eagerness to apply the instrument in clinical practice. Standard deviation decreased significantly in only two of the 16 categories after tutorial viewing. Subscores for static, dynamic, and synkinesis all demonstrated stable standard deviations, suggesting that the instrument is intuitive. Participants achieved posttutorial scores closer to the experienced eFACE user in 14 of 16 scores, although only a single score, nasolabial fold orientation with smiling, achieved statistically significant improvement. CONCLUSIONS: The eFACE may be a suitable, cross-platform, digital instrument for facial function assessment, and was well received by facial nerve experts. Tutorial viewing does not appear to be necessary to achieve agreement, although it does mildly improve agreement between occasional and frequent eFACE users.

The "Trigger" Thumb Locked in Extension - An Unusual Presentation of a Common Paediatric Condition.

BACKGROUND: We report a variant of paediatric trigger thumb which is locked in extension rather than flexion. METHODS: Eleven children with 14 trigger thumbs (three bilateral) locked in extension were reviewed retrospectively over a 12-year period. The number of flexed trigger thumbs encountered over this period was established from the operating room database. RESULTS: All children were treated with release of the A1 pulley. Nine children achieved a full range of motion at the interphalangeal joint. One child with bilateral extended trigger thumbs required bilateral dorsal capsulotomy and another child developed temporary mild triggering in flexion. CONCLUSIONS: Approximately 1% of trigger thumbs treated operatively at this institution presented as the extended variant. Trigger thumb locked in extension should be considered in a child presenting with inability to flex the thumb.

Spinal accessory nerve transfer outperforms cervical root grafting for suprascapular nerve reconstruction in neonatal brachial plexus palsy.

BACKGROUND: The authors evaluated long-term shoulder function in patients with neonatal brachial plexus palsy undergoing suprascapular nerve reconstruction with cervical root grafting or spinal accessory nerve transfer. METHODS: A retrospective review was performed on all infants presenting with neonatal brachial plexus palsy between 1994 and 2010. Functional outcomes were compared by type of suprascapular nerve reconstruction. RESULTS: Seventy-four patients met the inclusion criteria (46 transfers, 28 grafts). Both groups presented with an active movement scale score of 2.0 for shoulder abduction and 0.0 for external rotation. Postoperative follow-up was 9.0 years for the graft group and 6.7 years for the transfer group. Both groups achieved an active movement scale score of 5.0 for shoulder abduction at 12, 24, and 36 months postoperatively. Active movement scale scores for shoulder external rotation were 1.0, 2.0, and 2.5 for the graft group versus 2.0, 2.0, and 3.0 for the transfer group at 12, 24, and 36 months postoperatively. None of these differences reached statistical significance. Composite Mallet scores were 13.0 for the graft group versus 15.0 for the transfer group at 3 years (p = 0.06) and 13.0 for the graft group versus 16.0 for the transfer group at 5 years postoperatively (p = 0.07). Secondary shoulder surgery was performed on 57.1 percent (16 of 28) of patients with grafts compared with 26.1 percent (12 of 46) of patients with transfers (OR, 3.17; p = 0.02). CONCLUSION: Suprascapular nerve reconstruction by cervical root grafting results in poorer shoulder function and a two-fold increase in secondary shoulder surgery compared with spinal accessory nerve transfer. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Tragal reconstruction after tumor excision.

The tragus forms a distinct and important landmark in the overall artistic and anatomical landscape of the ear. Despite its small size, it functions to cover and prevent direct access to the ear canal. Aesthetically, it has an important role in defining the morphology of the auricle in 3 dimensions, not only covering the meatus but also casting a shadow into the conchal bowl that gives the impression of depth. Most of the techniques for tragal reconstruction are associated with creation of a tragus in patients with microtia. We present here a new technique for tragal reconstruction in the setting of tumor ablation.