MRI of squamous cell carcinoma of the nail apparatus: report of 6 cases.

Malignant tumors of the nail apparatus are rare and dominated by squamous cell carcinomas (SCC). Routinely, their pre-therapy imaging is limited to radiography. Our purpose is to determine the MRI characteristics in the locoregional assessment of SCC of the nail apparatus through a series of 6 consecutive cases explored by MRI and operated, carried out over a period of 12 years. IRB approval was obtained. Two in situ and 4 invasive squamous cell carcinomas were found, sex ratio was 0.5, and the age was 55 ± 10 years (mean ± SD). Most tumors showed specific signal behavior different from that of the epidermis and dermis with high signal on T2wi (5/6) and complete or partial enhancement (6/6). The mean thickness was 3.4 mm. The deep margin of the tumor with the dermis was always well defined for Bowen's disease (2/2) and blurred for invasive SCC. Localization involved the nail bed epithelium in all cases. Changes of the nail plate were detectable. Extension to lateral and posterior folds, hyponychium, cul-de-sac matrix, deep dermis, and bone was determined. MRI could be proposed as preoperative imaging of squamous cell carcinoma for locoregional assessment and guide biopsy.

Long-Term Outcomes of Atasoy Flap in Children With Distal Finger Trauma.

PURPOSE: Distal finger trauma is one of the most frequent emergencies in children and has the potential for functional and cosmetic damage to the hand. The Atasoy flap (AF) is a vascularized, subcutaneous pedicle V-Y advancement flap used to cover a loss of distal finger substance. Our hypothesis was that the AF is a safe, reliable flap that results in few complications and gives satisfactory functional and cosmetic results in children. METHODS: We retrospectively assessed children with distal finger trauma and AF pulp reconstruction in our pediatric orthopedic department between 2008 and 2017. The lesion zone was classified, and we also evaluated necrosis, infection, the shape of the pulp, pulp sensitivity (Weber test), hyponychial scarring, and the presence of a hook nail deformity. Lastly, we compared patients who developed a hook nail with those who did not. RESULTS: Thirty children were included (mean age at trauma, 6.4 years [range, 1.3-15.7 years]). In 21 cases, the finger damage was located in Ishikawa subzone II. No cases of necrosis or infection were reported. Epicritical tactile sensitivity was good in 20 patients (67%). A hook nail deformity was observed in 15 children (50%) and hyponychial scarring in 22 patients (73%). The pulp had a normal shape in 13 children (43%). The hook nail group displayed more hyponychial scarring, greater nail dystrophy, and lower pulp sensitivity. CONCLUSIONS: The AF yielded contrasting results. High reliability, good coverage, and minimal donor-site morbidity were compromised by suboptimal tip length/shape, nail appearance, and sensitivity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Surgical Treatment of Enchondromas of the Hand During Childhood in Ollier Disease.

PURPOSE: The objective of the present study was to evaluate the outcome of surgical treatment of hand lesions in Ollier disease (OD) carried out during childhood. METHODS: A retrospective review was carried out of 10 pediatric patients with hand involvement of OD, who had undergone surgery for metacarpal or phalangeal enchondromas. The technique comprised curettage and cortical bone reconstruction with corticoplasty, to restore a near-normal phalangeal volume. The range of finger motion (pulp-to-palm distance), the shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire score, cosmetic improvement, radiological findings (according to Tordai's classification), and recurrence were recorded after a mean follow-up of 7.5 years (range, 4-11.3 years). RESULTS: The mean age at surgery was 10.7 years (range, 6-14 years). Curettage was performed on 35 enchondromas, and 9 cavities were filled with a bone graft. The mean pulp-to-palm distance was significantly lower after surgery (from 1.5 cm to 0.25 cm; P < .05). The mean QuickDASH score was 3.84 (range, 0-11.4). A marked cosmetic improvement was noted for 83% of the hands. Three enchondromas recurred in 1 patient, requiring a second curettage. Fifty-seven percent of the cavities were completely filled with bone (Tordai stage 1) at last follow-up. The outcome did not depend on the presence or absence of a bone graft. CONCLUSIONS: Our results suggest that early surgical treatment comprising curettage and corticoplasty leads to good clinical, cosmetic, and radiological outcomes. Early surgical treatment of well-developed and/or symptomatic enchondromas of the hand in OD should be considered. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Recurrence of radial bowing after soft tissue distraction and subsequent radialization for radial longitudinal deficiency.

PURPOSE: Centralization and radialization are the most widely reported surgical treatments for Bayne and Klug Type III and IV radial longitudinal deficiency. Prior soft tissue distraction has been introduced to improve reducibility of the deformity without skeletal resection. Satisfying long-term effects have been reported with centralization but are still unclear with radialization. METHODS: This is a retrospective study of 8 consecutive children with Bayne and Klug Type III or IV radial longitudinal deficiency treated with preliminary soft tissue distraction followed by radialization between 2003 and 2008. All children underwent the same surgical protocol. End points of the study were clinical appearance, the hand-forearm angle, and mean angular correction at last follow-up. RESULTS: The mean preoperative hand-forearm angle was 61° (26°-91°). The average duration of distraction was 1.9 month (1-3 mo). The initial postoperative angle averaged 12° (-14°-40°). There were 3 postoperative complications: 2 cases of pin loosening and 1 case of fracture of the base of the small finger metacarpal. Mean follow-up duration was 2.6 years (1-4 y). At last follow-up, 7 of the 8 patients had visible recurrence of the deformity, the hand-forearm angle had deteriorated to 44° (20°-69°), and the mean angular correction was 18° (-43°-59°). CONCLUSIONS: Preoperative distraction allows a gradual realignment of the hand on the forearm without skeletal resection, but the recurrence rate after radialization is high. Tendon transfers and soft tissue tensioning were unable to maintain hand-forearm alignment following soft tissue distraction. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Carpal tunnel syndrome and finger deformities in children with mucopolysaccharidoses and mucolipidoses: a retrospective review of 52 patients.

This single-centre retrospective study reports our management of carpal tunnel syndrome in 52 children (103 hands) with mucopolysaccharidoses and mucolipidoses. All except one were bilateral. The median age at surgery was 4 years (range 1.5 to 12). The diagnosis of carpal tunnel syndrome was confirmed by an electromyogram (EMG) in all patients; 38% of these presented without any clinical signs. Surgical neurolysis was performed in all hands, combined with epineurotomy in 52 hands (50%) and flexor tenosynovectomy in 75 hands (73%). Surgery was bilateral in 98% of children (102 hands). The mean follow-up was 12 years (range 1 to 19) and the EMG was normalized in 78% of hands. Ten patients suffered recurrence, eight of whom required further surgery. Screening for carpal tunnel syndrome is essential for the management of children mucopolysaccharidoses and mucolipidoses. Surgical treatment should be carried out early with follow-up by EMG to detect recurrence.Level of evidence: IV.

Clinical and radiological results of vascularized fibular epiphyseal transfer after bone tumor resection in children.

INTRODUCTION: Vascularized fibular proximal epiphyseal transfer associated to a diaphyseal segment is used to treat childhood epiphyseal defect. The aim of the present study was to analyze surgical technique and long-term clinical and radiological results. MATERIAL AND METHOD: Between 1997 and 2008, 7 patients with a mean age of 5.7 years (range, 2-8 years) were operated on for bone malignancy with vascularized fibular epiphyseal transfer: 5 Ewing sarcomas and 2 osteosarcomas, located in the proximal femur (n=3), proximal humerus (n=3) or distal radius (n=1). Mean transplant size was 13.8cm. Vascularization involved a single artery in 5 cases (3 peroneal, 2 anterior tibial) and both in 2 cases. Internal fixation used intramedullary nailing in 6 cases and screwed plate in 1. All patients underwent pre- and post-operative chemotherapy following French Pediatric Oncology Society (SFOP) protocols. RESULTS: Mean follow-up was 11 years (range, 3 years 11 months to >17 years). All patients were alive and in tumor remission. Reconstructed joint function was satisfactory in 85% of cases. Graft thickening indicated integration in all cases. The transferred cartilage had recovered growth in 4 cases. Complications comprised postoperative infection (n =1), consolidation defects (n = 2), fractures (n = 8), malalignment requiring surgical revision (n = 1), and spontaneously resolving common peroneal nerve palsies (n = 2). DISCUSSION: In young children, vascularized fibular epiphyseal transfer fills bone defect, reconstructs a functional joint and allows continued growth in the resected segment. Growth prostheses, in the authors' experience, always give poor results in this age-group, and fusion fails to address the growth problem. Patients should be informed about the risk of fracture, persisting over the long term. CONCLUSION: Vascularized fibular epiphyseal transfer is a difficult technique, subject to complications, but enables reconstruction of a bone segment involving the epiphysis in young children, conserving function and growth. LEVEL OF EVIDENCE: III, retrospective clinical study.

Non-vascularized toe phalanx transfer for correction of severe clinodactyly of the thumb in Rubinstein-Taybi syndrome.

In Rubinstein-Taybi syndrome, patients may have a particularly severe clinodactyly of the thumb. We evaluated a new method for correction of these severe clinodactylies using non-vascularized toe phalanx transfer as a replacement for the abnormal delta phalanx. Results of the new technique are presented, together with those of an osteotomy technique. We retrospectively recorded the angle of the clinodactyly before and after surgery and at long-term follow-up of 11 osteotomies and five transfers in nine patients from 1990 to 2017. The pre-operative angle of clinodactyli was similar between the two groups with a mean of 59°. After surgery, the correction was equivalent (7° and 11°). At the last follow-up (7 and 18 years), the relapse of clinodactyly was 17° for osteotomies and 1° for phalanx transfers. We noticed growth of the transferred phalanx, resulting in an excellent thumb length. We conclude that non-vascularized toe transfer can be an effective correction of severe clinodactyly and may be more stable than osteotomy in the long-term.Level of evidence: IV.

One-stage circumferential limb ring constriction release and direct circular skin closure in amniotic band syndrome: a 14-case series.

The aim of the present study was to report results of direct circular suture after 1-stage circumferential resection of limb ring constriction in amniotic band syndrome. A multicentre retrospective study included 14 patients with amniotic band syndrome (mean age, 13.3 months) operated on between 2004 and 2019 by circumferential release of ≥1 ring constriction. Assessment was based on limb function and clinical scar aspect on the POSAS and Vancouver scales. Mean follow-up was 3.9 years. There were no scar-related, vascular or neurologic complications, postoperatively or at last follow-up. POSAS and Vancouver scores were satisfactory. One-stage circumferential release with direct closure is a simple technique that provides satisfactory functional and esthetic results.

Diagnosis and Management of Carpal Tunnel Syndrome in Children with Mucopolysaccharidosis: A 10 Year Experience.

INTRODUCTION: Mucopolysaccharidoses (MPS) are rare and clinically heterogeneous lysosomal storage disorders. Carpal tunnel syndrome (CTS) is a frequent complication in MPS types I, II, VI, and VII. CTS symptoms are difficult to recognize in these children, and often there is a lack of appropriate investigations. PATIENTS AND METHODS: In this retrospective study, all MPS patients were referred to the electrodiagnostic (EDX) laboratory of a single academic center during a 10-year period. Forty-eight children underwent serial EDX studies for CTS diagnosis and follow-up after surgery. Forty-two patients were diagnosed with CTS. Sensory nerve conduction velocity (SNCV), distal motor latency (DML), and motor nerve conduction velocity through the wrist (MNCV-W) of the median nerve were reviewed and analyzed. RESULTS: One-hundred-three EDX examinations were performed on 48 patients. The median age at disease diagnosis was 2.1 years versus 4.9 years for CTS diagnosis. Analysis of the series revealed that electrophysiological abnormalities of CTS could have started much earlier (before the age of 2 years or at diagnosis of MPS). Diagnosis was based on SNCV and DML results, and MNCV-W was taken into consideration. Bilateral CTS was frequent (88%) in the types of MPS studied in our population and was observed from the first year of life, and may not have be associated with obvious clinical symptoms. EDX studies also helped in the follow-up and detection of CTS relapses, thus leading to an early intervention allowing a better recovery. CONCLUSION: EDX studies should be performed promptly and regularly in these patients. Prospective studies are required in order to understand the effect of disease-specific therapies in preventing the development of CTS in these patients. SYNOPSIS: EDX studies should be performed in MPS patients soon after diagnosis and during routine follow-up, before and after surgical decompression.

Post-traumatic distal interphalangeal finger joint reconstruction using a free hemi-joint transfer from the fifth toe middle phalanx.

We present three cases of finger distal interphalangeal joint reconstruction in children using a new donor site, the middle phalanx of the fifth toe, transferred as a non-vascularized graft. The patients were followed a minimum of 36 months with serial radiographs, magnetic resonance imaging and photographs. Alignment was improved and persisting growth was demonstrated in all cases. Postoperative magnetic resonance imaging of the grafted phalanx and control toe showed comparable signals. The absence of a proximal epiphysis of these toe phalanges may have an important role in their survival after free transplantation, and may promote continued growth.