Management and outcome of acral soft-tissue sarcomas.

AIMS: The aim of this study was to evaluate the surgical management and outcome of patients with an acral soft-tissue sarcoma of the hand or foot. PATIENTS AND METHODS: We identified 63 patients with an acral soft-tissue sarcoma who presented to our tertiary referral sarcoma service between 2000 and 2016. There were 35 men and 28 women with a mean age of 49 years (sd 21). Of the 63 sarcomas, 27 were in the hands and 36 in the feet. The commonest subtypes were epithelioid sarcoma in the hand (n = 8) and synovial sarcoma in the foot (n = 11). RESULTS: In 41 patients (65%), the tumour measured less than 5 cm in its largest dimension (median size 3 cm (2 to 6)); 27 patients (43%) were diagnosed after inadvertent excision prior to their referral to the specialist sarcoma unit. After biopsy and staging, primary surgical intervention at the sarcoma unit was excision and limb salvage in 43 (68%), partial (digit or ray) amputation in 14 (22%), and more proximal amputation in six (10%). At final follow up, local recurrence had been treated by one partial amputation and six amputations, resulting in a partial amputation rate of 24% and a proximal amputation rate of 19%. The five-year survival rate was 82%. Patients who underwent inadvertent excision showed no statistically significant difference in survival or local recurrence, but were more likely to undergo amputation (p = 0.008). Large tumour size (> 5 cm) was associated with lower survival (p = 0.04) and a higher risk of local recurrence (p = 0.009;). CONCLUSION: Most acral soft-tissue sarcomas measure less than 5 cm at presentation, indicating that while size can be a useful prognostic factor, it should not be used as a diagnostic threshold for referral. Increased tumour size is associated with a higher rate of local recurrence and reduced survival. Sarcoma excision with limb preservation does not result in an increased risk of local recurrence. Cite this article: Bone Joint J 2018;100-B:1518-23.

Is there a role for music in reducing anxiety in plastic surgery minor operations?

INTRODUCTION: It is well documented that music plays a role in reducing anxiety levels. Its role in reducing intra-operative anxiety levels in surgical patients while awake is less well known. We report the effects of music on intra-operative patient anxiety in both the elective and trauma plastic surgical setting. METHODS: Two groups of patients undergoing local anaesthetic surgical procedures were identified: those where music was played in the operating theatre (Group 1) and those where it was not (Group 2). Ninety-six patients were included. Subjectively anxiety was evaluated by the patient with a visual analogue scale (VAS) and objectively by the respiratory rate (RR), both pre and post-operatively. The unpaired t-test was used to evaluate the statistical significance of differences between the groups. RESULTS: The mean pre-operative VAS score was similar in both groups (5.7 in Group 1 and 5.8 in Group 2). The mean pre-operative RR was 15 breaths per minute in both groups. Post-operatively, the VAS score and RR were both lower in Group 1 (VAS: 3.5 vs 4.9; p<0.01 and RR: 11 vs 13 breaths per minute; p<0.05). CONCLUSIONS: In the era of the patient centred approach to clinical care, it is crucial to minimise patient anxiety. Music appears to reduce intra-operative anxiety in awake patients in both the elective and trauma plastic surgical setting. Easy listening music and chart classics appear to be suitable genres according to patients. We believe there is a role for a large, multicentre, randomised control study to examine the benefits of music in all local anaesthetic procedures across different specialties.

Successful management of recalcitrant infection related to total hip replacement using pedicled rectus femoris or vastus lateralis muscle flaps.

Deep prosthetic joint infection remains an uncommon but serious complication of total hip replacement. We reviewed 24 patients with recalcitrant hip wounds following infected total hip replacement treated with either pedicled rectus femoris or vastus lateralis muscle flaps between 1998 and 2009. The mean age of the patients was 67.4 years (42 to 86) with ten men and 14 women. There had been a mean of four (1 to 8) previous attempts to close the wound. A total of 20 rectus femoris and five vastus lateralis flaps were used, with one of each type of flap failing and requiring further reconstruction. All patients had positive microbiology. At a mean follow-up of 47 months (9 to 128), 22 patients had a healed wound and two had a persistent sinus. The prosthesis had been retained in five patients. In the remainder it had been removed, and subsequently re-implanted in nine patients. Six patients continued to take antibiotics at final follow-up. This series demonstrates the effectiveness of pedicled muscle flaps in healing these infected wounds. The high number of previous debridements suggests that these flaps could have been used earlier.

Genetic screening of 202 individuals with congenital limb malformations and requiring reconstructive surgery.

BACKGROUND: Congenital limb malformations (CLMs) are common and present to a variety of specialties, notably plastic and orthopaedic surgeons, and clinical geneticists. The authors aimed to characterise causative mutations in an unselected cohort of patients with CLMs requiring reconstructive surgery. METHODS: 202 patients presenting with CLM were recruited. The authors obtained G-banded karyotypes and screened EN1, GLI3, HAND2, HOXD13, ROR2, SALL1, SALL4, ZRS of SHH, SPRY4, TBX5, TWIST1 and WNT7A for point mutations using denaturing high performance liquid chromatography (DHPLC) and direct sequencing. Multiplex ligation dependent probe amplification (MLPA) kits were developed and used to measure copy number in GLI3, HOXD13, ROR2, SALL1, SALL4, TBX5 and the ZRS of SHH. RESULTS: Within the cohort, causative genetic alterations were identified in 23 patients (11%): mutations in GLI3 (n = 5), HOXD13 (n = 5), the ZRS of SHH (n = 4), and chromosome abnormalities (n = 4) were the most common lesions found. Clinical features that predicted the discovery of a genetic cause included a bilateral malformation, positive family history, and having increasing numbers of limbs affected (all p<0.01). Additionally, specific patterns of malformation predicted mutations in specific genes. CONCLUSIONS: Based on higher mutation prevalence the authors propose that GLI3, HOXD13 and the ZRS of SHH should be prioritised for introduction into molecular genetic testing programmes for CLM. The authors have developed simple criteria that can refine the selection of patients by surgeons for referral to clinical geneticists. The cohort also represents an excellent resource to test for mutations in novel candidate genes.

Necrotising fasciitis of the thigh secondary to a perforated rectal cancer.

SUMMARY: We report a rare case of rectal cancer presenting with necrotising fasciitis of the posterior thigh. Perforation of a rectal cancer adjacent to the piriformis muscle provided a route for bowel contents to travel into, and contaminate, the posterior compartment of the thigh. This case highlights the need for prompt diagnosis, urgent aggressive surgical debridement and consideration of a rare underlying cause in the management of necrotising fasciitis.

Functional and oncological outcomes after limb-salvage surgery for primary sarcomas of the upper limb.

The surgical treatment of upper limb sarcoma poses an oncological and reconstructive challenge. Limb-salvage surgery aims to balance adequate excision margins for disease control and preservation of all important structures to retain maximum function. Reported here is an assessment of the functional and oncological outcomes of limb salvage surgery for primary sarcoma of the upper limb and limb girdle in 72 patients referred to a specialist musculoskeletal tumour unit over 9 years. All patients underwent excision of the sarcoma with reconstruction and adjuvant treatment as needed. Functional outcome was assessed using the Toronto Extremity Salvage Score (TESS) after discharge from hospital. The upper limb sarcomas treated by limb-salvage surgery achieved planned margins of excision in 85% of cases with primary surgery. This increased to 100% with re-excision, resulting in local recurrence in 15% and survival of 75% among those at 5 years or more after surgery, while retaining good to excellent function (TESS mean of 87 out of 100). A total of 38 patients completed TESS questionnaires and, as a single group, had a mean TESS of 87. Patient age, anatomical site of tumour and adjuvant treatment made no significant difference to TESS. Liposarcomas had a significantly better TESS than leiomyosarcomas, chondrosarcomas and malignant peripheral nerve sheath tumours. Oncological outcome was assessed in terms of excision margins achieved, local recurrence, re-excision and disease-free survival. Eight patients died of disease, all with high-grade primary tumours. Fourteen had local recurrence, four low-grade disease and the remaining high-grade disease. All four low-grade recurrences were successfully re-excised, as were five of the high-grade recurrences. The remaining five died of disease, with or without further recurrences. Consistently good functional results as measured by TESS were reported by responders, but high-grade disease and early recurrence were identified as predictors of recurrence and death from disease, respectively.

Chronic sternal osteomyelitis complicating primary manubriosternal septic arthritis.

We present a 40-year-old woman complaining of worsening chest pain. She was previously treated with a prolonged course of antibiotics for suspected sternal osteomyelitis. Radiological investigations were suggestive of ongoing inflammation within the manubriosternal joint. Formal surgical debridement yielded evidence corroborating the diagnosis although microbiological samples were negative. Prolonged empirical treatment with intravenous antibiotics leads to a complete resolution of symptoms. Primary culture-negative manubriosternal septic arthritis is rare, and suboptimal treatment, particularly if confused with other conditions such as synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome, compounds its considerable morbidity.

Duplicate publication in the Journal of Hand Surgery.

This study evaluates the extent of duplicate publication in the Hand Surgery literature. A retrospective review of original articles published in the American and the British & European editions of Journal of Hand Surgery during the years 1999 and 2000 was performed using MEDLINE (PUBMED) search engine. Index articles suspected of dual publication were identified by using key words in the title and the names of the first, second and last authors. The full initial text was carefully studied and suspected duplicate articles were classified as dual, potentially dual or fragmented. Six hundred articles were evaluated, of which 25 (4%) index articles were identified with 33 "suspects". Eleven "index" and 15 "suspected" articles were cleared on closer scrutiny. Thus 14 "index" articles (2%) were found to be associated with 18 duplicated articles. Of these, four were classified as dual, five as potentially dual and nine as fragmented. We conclude that although duplicate publication of articles in the Journals of Hand Surgery (American and British/European Volumes) does occur, the incidence in the sample studied is lower than some other surgical journals.

Carbohydrate-protein interactions at interfaces: comparison of the binding of Ricinus communis lectin to two series of synthetic glycolipids using surface plasmon resonance studies.

Two C-lactosyl lipids and the related C-galactosyl lipids have been synthesised and their binding to RCA120 plant lectin was compared with a second series of thiolactosylethoxyalkanes. The interactions were measured quantitatively in real time by surface plasmon resonance (BIAcore) at a range of concentrations and temperatures from 5 to 30 degrees C. The C-galactosyl lipid (1,3-dimethyl-5-[beta-D-galactopyranosyl]-5-(4-octadecyloxybenzyl)pyrimidine-2,4,6-trione) bound much more weakly with a K(A) = 8.86 x 10(5) than the corresponding C-lactosyl lipid (1,3-dimethyl-5-[beta-D-galactopyranosyl-(1 --> 4)-beta-D-glucopyranosyl]-5-(4-octadecyloxybenzyl)pyrimidine-2,4,6-trione) (K(A) = 2.31 x 10(7)). The influence of the linker region of the two different series of lactosyl lipids was clearly demonstrated by the differences in the binding to RCA120 lectin. The changes in kinetic values and in the enthalpic and entropic contribution to the free energy of binding reflected the importance of the linker and the hydrocarbon anchor holding the synthetic glycolipids in the neomembrane.

Spinocerebellar ataxia type 3 phenotypically resembling parkinson disease in a black family.

BACKGROUND: Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), can present with parkinsonism. However, classically, atypical features, including pyramidal and cerebellar signs, peripheral neuropathy, and/or anterior horn cell dysfunction, are also seen. Levodopa responsiveness is unusual in this disorder. OBJECTIVE: To determine the cause of apparent parkinsonism suggestive of Parkinson disease (PD) in a large family of African origin. METHODS: We studied a large family in which apparent autosomal dominant parkinsonism suggestive of PD occurs in order to find the causal genetic mutation. Affected and unaffected family members were screened for the presence of a pathogenic expansion at the MJD/SCA3 locus using a polymerase chain reaction polyacrylamide gel electrophoresis-based assay. RESULTS: Three of the 4 individuals who were examined have a phenotype reminiscent of PD. Specifically, they have at least 2 of the cardinal features, are levodopa responsive, and have no atypical features. All affected family members were shown to possess pathogenic expansions in the MJD/SCA3 gene. CONCLUSIONS: Parkinsonism suggestive of PD due to MJD/SCA3 has not been previously reported, to our knowledge. However, atypical, though also levodopa-responsive, parkinsonism has been previously reported to occur in African American families, suggesting that that this phenotype is associated with African ancestry. In this regard, it is perhaps significant that all the individuals with parkinsonism have relatively low numbers of repeats (normal, 16-34; pathologic, 60-84). In families in which linkage analysis is being performed to determine a locus for autosomal dominant parkinsonism suggestive of PD, evaluation for the MJD/SCA3 mutation is indicated.

Lower limb amputation and grade of surgeon.

BACKGROUND: The aim of this study was to identify clinical and operative risk factors that might influence the rate of independent ambulation following major limb amputation. METHODS: Between 1989 and 1993, 172 patients had 193 lower limb amputations for peripheral vascular disease: 98 below-knee, 86 above-knee and nine through-knee. RESULTS: The overall revision rate was 13.5 per cent. Revision was significantly more frequent in those patients who had a previous vascular reconstruction. Only 26 per cent of patients were able to ambulate independently after rehabilitation and limb-fitting. Univariate risk factor analysis suggested that independent ambulation was not affected by age, mode of presentation, the presence of ischaemic heart disease, diabetes, smoking status, previous vascular reconstruction or the level of amputation. However, fewer patients whose amputation was performed by a junior trainee could walk with a prosthetic limb (P = 0.03). CONCLUSION: Patients considered suitable for walking training should have amputation performed by a senior trainee or consultant to optimize independent ambulation with a prosthetic limb.

Trisaccharide synthesis by glycosyl transfer from p-nitrophenyl beta-D-N-acetylgalactosaminide on to disaccharide acceptors catalysed by the beta-N-acetylhexosaminidase from Aspergillus oryzae.

The beta-N-acetylhexosaminidase from Aspergillus oryzae catalysed the transfer of beta-D-N-acetylgalactosaminyl residues from p-nitrophenyl beta-D-N-acetylglucosaminide on to disaccharide acceptors consisting of thioethyl glycosides of alpha-D-Glc-(1-->4)-beta-D-Glc, beta-D-Glc-(1-->4)-beta-D-Glc and beta-D-Glc-(1-->6)-beta-D-Glc. The principle of 'anomeric control' was exemplified by the results which showed that an alpha-linkage between the units of the acceptor favoured exclusively the formation of a new (1-->4)-linkage, whereas the beta-configuration in the acceptor led to a mixture of (1-->4)- and (1-->3)-linked products, as observed for simple glycosides of monosaccharide acceptors. With the thioethyl beta-lactoside as acceptor, beta-D-Gal-(1-->6)-beta-D-Gal-(1-->4)-beta-D-GlcSEt was formed, owing to the action of residual beta-D-galactosidase activity in the N-acetylhexosaminidase on the thioethyl beta-lactoside acting as both donor and acceptor.

Glycosidase-catalysed oligosaccharide synthesis: preparation of N-acetylchitooligosaccharides using the beta-N-acetylhexosaminidase of Aspergillus oryzae.

The beta-N-acetylhexosaminidase of Aspergillus oryzae catalyses the formation of 2-acetamido-4-O-(2-acetamido-2-deoxy-beta-D-glucopyranosyl)-2-deoxy-D- glucopyranose (di-N-acetylchitobiose) and 2-acetamido-6-O-(2-acetamido-2-deoxy-beta-D-glucopyranosyl)-2-deoxy-D- glucopyranose from p-nitrophenyl 2-acetamido-2-deoxy-beta-D-glucopyranoside and 2-acetamido-2-deoxy-D-glucopyranose. The ratio of the two disaccharides is time-dependent. The ratio of (1-->4)- to (1-->6)-isomers is a maximum (approximately 9:1) at the point of disappearance of the glycosyl donor. If left to evolve, the ratio changes to 92:8 in favour of the (1-->6)-isomer. Either the (1-->4)- or the (1-->6)-isomer can be isolated by treating the appropriately enriched dissaccharide mixture with the beta-N-acetylhexosaminidase of Jack bean (Canavalia ensiformis) or the beta-N-acetylhexosaminidase of A. oryzae, respectively. Di-N-acetylchitobiose [GlcNAc(beta 1-4)GlcNAc] is an efficient donor of 2-acetamido-2-deoxy-D-glucopyranosyl units in reactions catalysed by the N-acetylhexosaminidase of A. oryzae. Di-N-acetylchitobiose itself acts as acceptor to give tri-N-acetylchitotriose [GlcNAc(beta 1-4)GlcNAc(beta 1-4)GlcNAc]. As the trisaccharide accumulates it, in turn, acts as acceptor giving tetra-N-acetylchitotetraose [GlcNAc(beta 1-4)GlcNAc(beta 1-4)GlcNAc(beta 1-4)GlcNAc]. The product mixture consisting of mono-, di-, tri-, and tetrasaccharides is conveniently separated by charcoal-Celite chromatography.

Adult congenital lobar emphysema in pregnancy.

A young woman presented with left sided chest pain. Chest radiography revealed a hyperexpanded left upper lobe and the rare diagnosis of congenital lobar emphysema was made. She was then found to be pregnant. Thoracotomy and left upper lobectomy were performed during the pregnancy without adverse effects to the mother or fetus. The implications of pregnancy on the surgical management of this rare condition are discussed.