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.

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.

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.

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.

The use of V-Y advancement flaps for closure of pretibial skin defects after excision of cutaneous lesions.

METHODS: We retrospectively analyzed 88 consecutive V-Y flap closures of pretibial surgical defects of up to 45 mm in diameter. RESULTS: Eight percent of patients developed wound infection, which showed a significant association with antihypertensive use. No bleeding complications were experienced in any of the patients, including 40 patients on antiplatelet or anticoagulation therapy. Health care costs were calculated as far less than those for patients treated with split skin grafting because of shorter hospital stay and fewer dressing visits. CONCLUSION: V-Y flap closure is a safe and reliable technique and is a superior alternative to split skin grafting in appropriate defects in the pretibial area.

The Manta Ray flap: a technique for first web space release.

Release of the first web space adduction contracture is important for normal hand function, as it allows improved grasp. Many methods of achieving this have been described. We describe a local flap technique that utilizes first web space skin and introduces available skin from the sides of the adjacent digits for resurfacing the released first web contracture thus providing an excellent coverage with well-vascularized, pliable, and sensate skin for the moderate first web space contractures.

Lipoblastoma: An important differential diagnosis of tumours of the hand in children.

Lipoblastoma is a rare benign neoplasm of infancy and childhood with the potential for locally invasive, rapid growth. It has an excellent prognosis, and does not metastasise. An important differential diagnosis for these rapidly-enlarging tumours is liposarcoma. Ultrasound, magnetic resonance imaging, fine needle aspiration, and cytogenetics are important diagnostic tools for this rare tumour. We present two cases of lipoblastoma of the hand that were excised within a three month period with no evidence of recurrence at 14, and 12, months respectively.

Intraoperative nerve stimulation: a simple, effective, and inexpensive alternative to standard devices.

Many surgical procedures require intraoperative stimulation of peripheral nerves. Using a pair of conventional bipolar forceps and an anesthetic impulse generator (Stimlocator, Model SL3, B. Braun Australia Pty Ltd, Australia), we have developed a simple, effective, and inexpensive alternative to standard nerve stimulation devices that enables the accurate localization of motor fascicles intraoperatively. Bipolar forceps provide better control than a monopolar electrode, as the current generated across the nerve fibers generates an action potential that is then propagated down the axon. This inexpensive and reusable device is routinely used at Royal Children's Hospital, Melbourne, and its efficacy and ease of use has been demonstrated over a long period.

Reduction otoplasty: correction of the large or asymmetric ear.

Reduction otoplasty is an uncommon procedure performed for macrotia and ear asymmetry. Techniques described in the literature for this procedure are few. The authors present their ear reduction approach that not only achieves the desired reduction effectively and accurately, but also addresses and creates the natural anatomic proportions of the ear, leaving a scar well hidden within the fold of the helix.

Brachial artery thrombosis in infants: an algorithm for limb salvage.

BACKGROUND: Arterial occlusion in infants, although uncommon, is usually an iatrogenic event associated with arterial vascular access. Most common in the upper limb, the consequences of iatrogenic arterial occlusion may be gangrene and limb loss. Even if there is adequate collateral flow and limb loss is avoided, long-term growth disturbances may be seen. There are few published data to guide the management of arterial occlusion in premature or sick infants. In general, there is agreement regarding the importance of early diagnosis and the reestablishment of limb perfusion with the fewest risks, but the optimal choice and timing of treatment modalities remain unknown. METHODS: This article examines the authors' experiences at the Royal Children's Hospital, Melbourne, and provides their algorithm for the management of this complex iatrogenic disease. RESULTS: The management algorithm has successfully treated 11 limbs in 11 patients with arterial vascular access-associated thrombosis over the period 1995 to 2003, with no instances of limb loss. Five of these patients required surgical intervention. CONCLUSIONS: The authors recommend a multidisciplinary approach involving plastic surgeons and hematologists for all cases of suspected or confirmed arterial thrombosis. A consensus algorithm that determines the role of heparin, thrombolysis, and acute surgical interventions, and the sequence of such interventions, is useful in providing the framework of therapy. The early recognition of the limb at risk is a key factor in obtaining a successful outcome.

Method of quadriceps attachment following upper tibial resection.

Limb salvage procedures for osteosarcomas of the upper tibia present combined problems of knee-joint and tibial reconstruction. Many methods of overcoming this have been described. We describe a new technique of prosthetic knee-joint and upper-tibial reconstruction, combined with a vascularized fibular bone flap for reattachment of the quadriceps muscle and tendon unit, resulting in superior long-term function.

Toe-to-hand transfer in symbrachydactyly.

Symbrachydactyly, or central atypical cleft, is classified as a failure of formation. For the adactylous or monodactylous forms, free toe transfer is the treatment of choice. We present 18 free toe transfers in 13 patients for symbrachydactyly. Despite abnormal anatomy, the functional building blocks have always been available and transfer technically possible. The result has been improved function and high levels of parental satisfaction.

Functional and cosmetic outcome of surgery for cerebral palsy in the upper limb.

Function, cosmesis, hygiene, and ability to dress can be improved by appropriate surgery. This frequently involves multiple simultaneous procedures and addresses the muscles, their tendons, and the joints they pass over. The improvement in cosmesis is most profound in hemiplegic patients. Overall, patients are satisfied with the results of surgery.