A comparison of piezo surgery osteotomies with conventional internal osteotomies as performed by trainee surgeons: a cadaveric study.

INTRODUCTION: Aesthetic rhinoplasty remains a challenging procedure with high expectations and narrow tolerance for errors. Considerable training is required to achieve controlled and reliable results. Use of the Piezotome is gaining popularity for performing the nasal osteotomies, a key step in rhinoplasty, where it is reported to improve precision and predictability and to keep tissue damage to a minimum. We compare the outcomes of conventional osteotomy techniques to piezosurgery in human cadavers as undertaken by surgical trainees. MATERIALS AND METHODS: Seven human cadavers were used and a total of 14 osteotomies were performed. Conventional osteotomies and piezosurgery were carried out each on one side of the cadaver. A number of fragments and a blinded assessment of the accuracy of the osteotomy compared with the preprocedure skin markings were carried out by two experienced rhinoplasty surgeons. The Mann-Whitney test for statistical analysis was used. RESULTS: The mean number of fragments was 1.57 in the piezosurgery osteotomy and 2.14 using conventional osteotomies. Four of seven piezosurgery osteotomies achieved an accuracy within 1mm. The conventional osteotomies as performed by the trainees showed a significant mismatch of more than 3mm in three of seven of cases. Accuracy within 1mm was achieved in one of seven cases. DISCUSSION: Piezosurgery offers a safe, reliable and precise method of performing lateral nasal osteotomies. This human cadaver study shows a high accuracy of osteotomy and fewer comminuted fractures using this technique compared with conventional osteotomy techniques.

Prospective evaluation of treatment response and disease reversibility of paediatric localized scleroderma (morphoea) to steroids and methotrexate using multi-modal imaging.

BACKGROUND: Paediatric localized scleroderma is a severe inflammatory disorder associated with tissue atrophy, often leading to disability. Assessing disease activity and response to treatment has always been challenging and remains an important difficulty in clinical practice. OBJECTIVES: To investigate prospectively the efficacy of systemic treatment with corticosteroids and methotrexate in children with localized scleroderma and the validity of infrared thermography, laser Doppler flowmetry and high-frequency ultrasound in assessing disease activity. METHODS: Children with localized scleroderma were prospectively treated with corticosteroids (initially pulsed IV methylprednisolone 30 mg/kg/day, maximum 500 mg/day and/or oral prednisolone 0.5-1 mg/kg/day) and methotrexate (15 mg/m2 weekly). Treatment response was evaluated using a clinical activity score. Skin temperature, blood flow, dermal thickness and dermal echogenicity of clinically active skin lesions were determined in relation to the unaffected contralateral site at baseline and after 3, 6, 12 and 18 months. Patient charts were later reviewed for long-term follow-up. RESULTS: Twenty-two patients were included [age 6.0 (0.2-14.4] years; female-to-male ratio 3.4 : 1) All responded well to therapy. Disease reversibility was demonstrated in the majority of children with partial resolution of skin sclerosis and regrowth of hair. Laser Doppler flowmetry and high-frequency ultrasound findings correlated with disease activity at baseline. Thermography had no added value in this cohort. The recurrence rate was 36% in the follow-up period. CONCLUSIONS: Corticosteroids and methotrexate are highly effective as first-line therapy in paediatric localized scleroderma, leading to partial reversal of skin manifestations. However, the recurrence rate is substantial and affected children require long-term follow-up. Laser Doppler flowmetry and high-frequency ultrasound correlate with disease activity in the acute phase and may assist decision-making in these patients.

Influence of different techniques of secondary cleft lip revision surgery on upper lip projection.

Patient dissatisfaction with labial appearance in the adult cleft lip is frequently linked to poor upper lip projection. Other areas of concern include asymmetry and impaired upper lip height. Different surgical techniques are available to address volumetric deficiencies, according to extent and localization. However, data comparing outcomes in these different areas are limited. The main aim of this study was to assess the relative gains in upper lip projection. An evaluation of upper vermilion height and symmetry was also performed. Thirty-seven consecutive patients treated by a single surgeon had their pre- and postoperative results measured using standardized photographs; these were analysed using subjective and objective outcome measures. Seven examiners evaluated anonymized pre- and postoperative side and front views for subjective evaluation. The objective analysis was performed using Adobe Photoshop. Fifteen lip revisions, four Abbe flaps, 12 dermal grafts, and six PermaLip implants were performed. In bilateral cleft lip and palate patients, Abbe flaps showed the most significant improvement in labial projection, followed by PermaLip implants and dermal grafts. In unilateral cleft lip and palate patients, PermaLip implants best addressed impaired lip projection, followed by dermal grafts. Overall, functional lip revisions showed excellent outcomes for upper lip symmetry; however, only minor changes in labial projection were found.

The use of Medpor implants for midface contouring in cleft patients.

Three-dimensional midfacial deficiency in cleft patients is common and is frequently connected to impairment of the aesthetic facial appearance. Different approaches to augment relevant facial regions are available. Alloplastic facial implants have been established as a viable alternative to autologous tissue augmentation in various circumstances. However, in cleft patients, the application of facial implants has rarely been reported. This retrospective study aimed to evaluate the use of Medpor implants for midfacial contouring in cleft patients. Fifty-one patients with orofacial clefts were assessed with regard to defined parameters. A range of Paranasal, Malar and Nasal Dorsum Medpor implants had been used. Unilateral cleft lip and palate (UCLP) represented the most common indication, followed by bilateral cleft lip and palate (BCLP). Bilateral implant insertion was performed as a general rule with few exceptions. Insertion of implants was frequently combined with other cleft-related surgical procedures. Even after orthognathic surgery, midfacial augmentation was implemented to specifically address residual volume deficiency, particularly in the malar region. The complication rate amounted to 4.9% (6/122 implants). Based on our findings, Medpor implants are reliable and long-term stable materials to successfully augment paranasal, subnasal and malar areas as well as a solid nasal dorsum material with few complications in cleft patients.

Classification of neurological abnormalities in children with congenital melanocytic naevus syndrome identifies magnetic resonance imaging as the best predictor of clinical outcome.

BACKGROUND: The spectrum of central nervous system (CNS) abnormalities described in association with congenital melanocytic naevi (CMN) includes congenital, acquired, melanotic and nonmelanotic pathology. Historically, symptomatic CNS abnormalities were considered to carry a poor prognosis, although studies from large centres have suggested a much wider variation in outcome. OBJECTIVES: To establish whether routine MRI of the CNS is a clinically relevant investigation in children with multiple CMN (more than one at birth), and to subclassify radiological abnormalities. METHODS: Of 376 patients seen between 1991 and 2013, 289 fulfilled our criterion for a single screening CNS MRI, which since 2008 has been more than one CMN at birth, independent of size and site of the largest naevus. Cutaneous phenotyping and radiological variables were combined in a multiple regression model of long-term outcome measures (abnormal neurodevelopment, seizures, requirement for neurosurgery). RESULTS: Twenty-one per cent of children with multiple CMN had an abnormal MRI. Abnormal MRI was the most significant predictor of all outcome measures. Abnormalities were subclassified into group 1 'intraparenchymal melanosis alone' (n = 28) and group 2 'all other pathology' (n = 18). Group 1 was not associated with malignancy or death during the study period, even when symptomatic with seizures or developmental delay, whereas group 2 showed a much more complex picture, requiring individual assessment. CONCLUSIONS: For screening for congenital neurological lesions a single MRI in multiple CMN is a clinically relevant strategy. Any child with a stepwise change in neurological/developmental symptoms or signs should have an MRI with contrast of the brain and spine to look for new CNS melanoma.

Midfacial augmentation in teenage cleft patients using malar and paranasal Medpor implants.

Malar and paranasal implants offer a way to augment and reconstruct midfacial deformities, and can be used across a broad spectrum of craniofacial deformities. Three patients aged between 13 and 15 years underwent such a procedure. Access was achieved via a labial mucosal approach; 'super petite' and 'petite' malar and paranasal Medpor implants were inserted in a subperiosteal plane and secured with titanium screws. All patients underwent an uneventful postoperative recovery and have remained pleased with their reconstruction at follow-up. In carefully selected patients, Medpor implants may have a role in teenage cleft patients with subtle midfacial hypoplasia, where formal osteotomies are not felt to be required, or where the lengthy preparation period might not be tolerated. In the cases described, they were used as a bridging or temporizing measure as there was not yet the skeletal maturity for definitive osteotomies. These patients may otherwise face a lengthy wait during their teenage years, in which they may struggle to manage what they may perceive as a significant facial deformity.

Misdiagnosis and delay in referral of children with localized scleroderma.

BACKGROUND: Localized scleroderma (LS) usually begins in childhood with a broad clinical spectrum and the diagnosis is often delayed. OBJECTIVES: To investigate the diagnostic pathway in a large cohort of paediatric patients with LS, to identify the duration until correct diagnosis and to characterize clinical clues for early diagnosis. METHODS: A retrospective case note review of 50 children with LS. RESULTS: The median (range) age at disease onset was 5·2 (0·1-14·4) years and disease duration until diagnosis 11·1 (1·8-79) months. The patients were first seen by a general practitioner (or paediatrician) after 1·2 (0·2-48·7) months and in none of the cases was the condition recognized at presentation according to a parental questionnaire (no diagnosis in 44%, misdiagnosis of atopic eczema 20%, melanocytic naevus 8%, fungal infection 6%, bruise 4%, varicose vein 4%, bacterial infection 4% and others). The patients were referred to a local specialist (dermatologist in 72%) after a disease duration of 7·5 (1·0-70·9) months and in 64% the correct diagnosis was established. In 20% the diagnosis remained unknown, 8% were misdiagnosed as port-wine stains and others as atopic eczema and melanocytic naevus. The correct diagnosis was eventually identified by the referring dermatologists, the paediatric dermatologists at our hospital, external maxillofacial surgeons and a paediatrician in 29 (58%), 17 (34%), 3 (6%) and 1 (2%), respectively. Histology was performed in 15 (30%). The patients were commenced on appropriate treatment after a disease duration of 16·6 (1·8-113·4) months. The main clinical diagnostic clues were: Blaschko-linear distribution 76%, atrophic changes 68%, skin fibrosis 40% and loss of scalp hair or eyelashes 36%. CONCLUSIONS: Physicians involved in the care of these children need to be aware of the characteristic clinical appearance of LS for early recognition and prompt initiation of treatment.

The economic viability of breast reconstruction in the UK: comparison of a single surgeon's experience of implant; LD; TRAM and DIEP based reconstructions in 274 patients.

A retrospective audit was performed of patients undergoing breast reconstruction under the care of the senior author from 2000 to 2007. We documented reconstruction type, length of stay and total number of revisions. Income to the trust based on the 2008/9 HRG codes along with any "top ups" was also recorded. This was compared to calculations of cost to the trust of performing each reconstruction. 274 patients had 278 primary reconstructions and a further 366 revisions. Only patients with a minimum one-year's follow-up were included (mean 3 years). This included 68 DIEPs'; 39 TRAMs'; 98 LDs'; and 73 implant reconstructions. The median length of stay for implant based reconstruction was 4 days; 9 for LD flaps; 11 for TRAMs' and 8 for DIEPs'. This was significantly shorter for the implant group compared to other reconstructions (P<0.001). The mean number of surgical revisions was 1.5 for implant reconstructions; 1.6 for LDs; 0.9 for TRAMs' and 0.8 for DIEPs'. There were significantly more revisions of implant reconstructions than DIEPs (P=0.037) and significantly more revisions of LDs compared to TRAM and DIEPs' (P=0.012 and 0.0023). In our study, the cost of an LD, TRAM or DIEP reconstruction including both primary surgery and any revisions was similar, and while at an average of three years, the implant reconstruction remains cheaper, that patient will still require more revisions, and if followed up enough will lose this small financial benefit. Furthermore, the difference is small (£8034 for implants vs. £10910 for DIEPs), and it could be argued this is justified by the increased patient satisfaction and cosmetic outcome. Finally we highlight several areas of financial inequality, including insufficient remuneration for providing individual operations, the lack of payment for performing more than one procedure at the same time and lack of payment for bilateral procedures.

Congenital tufted angioma with persistent low-grade coagulopathy.

A female infant, born with a tufted angioma, developed a coagulopathy with prolonged bleeding time, with the risk of progression to Kasabach-Merritt phenomenon. The difficulty in this case was judging the degree of this risk and therefore the most appropriate management. We opted for a conservative approach of observation, which was vindicated by the outcome.

Measurement of nitric oxide and 8-isoprostane in exhaled breath of children with atopic eczema.

BACKGROUND: Children with atopic eczema (AE) are at risk of developing asthma. Airway inflammation has been shown to be present before the onset of clinical asthma. Increased exhalation (forced expiration; FE) of nitric oxide (FE(NO)) and 8-isoprostane seems to be a feature of bronchial inflammation in people with asthma. AIM: To determine whether the exhalation of these two molecules is increased in children with eczema, even in the absence of overt asthma. METHODS: In total, 21 children with AE were recruited and compared with healthy controls. A questionnaire was completed to identify respiratory symptoms compatible with asthma. The severity of AE was graded clinically. Spirometry, FE(NO) measurements and exhaled breath condensate collection for 8-isoprostane were performed. RESULTS: The mean level of 8-isoprostane was similar for children with AE (2.33 +/- 4.76 pg/mL) and controls (3.37 +/- 3.43). FE(NO) was increased in children with AE (mean 64.97 parts per billion) compared with the normal range, even in the absence of respiratory symptoms and in the presence of normal lung function. CONCLUSIONS: FE(NO) but not 8-isoprostane levels in exhaled breath condensate are higher in children with AE without asthma. Our finding may indicate a predictive role for FE(NO) for the development of asthma.

Treatment with carbamazepine and gabapentin of a patient with primary erythermalgia (erythromelalgia) identified to have a mutation in the SCN9A gene, encoding a voltage-gated sodium channel.

Primary erythermalgia (erythromelalgia) is a rare autosomal dominant condition characterized by intermittent attacks of erythema, increased skin temperature and severe burning pain in the extremities, in a bilateral symmetrical distribution. Mutations in the SCN9A gene, which encodes a voltage-gated sodium channel have been shown to cause this disease. We report a family identified to have a mutation in the SCN9A gene, in which one severely affected family member has responded to the therapeutic combination of gabapentin and carbamazepine treatment.

Self removal of unwanted tattoos.

Tattooing has been around since early civilisation, with origins that can be traced back to Otzi the ice man dating from the fourth to fifth millennium bc (Renaut, 2004). Various methods of tattoo removal are described in the literature ranging from salabrasion as described by Aetius, a Greek physician, in 543ad (Scutt, 1972) to laser treatment in the late 20th century (Goldman et al, 1967).

Great Ormond Street Hospital for Children Registry for congenital melanocytic naevi: prospective study 1988-2007. Part 1-epidemiology, phenotype and outcomes.

BACKGROUND: The aetiology of congenital melanocytic naevi (CMNs) is unknown. OBJECTIVES: To identify potential aetiological factors in families of children with CMNs, and to relate these to long-term outcome measures. METHODS: Three hundred and forty-nine CMN families completed questionnaires about pregnancy and parental factors, and yearly questionnaires on the health of their child and details of the CMN. Seventy-nine control families completed one set of questionnaires, excluding CMN details. RESULTS: The mean prospective follow-up of 301 CMN families was 9.2 years, median 8.9 years, total 2679 years. Forty per cent of patients had CMNs > 20 cm projected adult size (PAS) or multiple CMNs. Twenty per cent of patients had abnormal neurodevelopment and although this was positively associated with PAS it was seen across all size categories. The rate of malignant melanoma was 1.4%. This was strongly associated with PAS with all five cases in patients with CMNs > 60 cm PAS/multiple CMNs (rate in that group 14%). Twenty-five per cent of CMN patients had a positive family history of a CMN in a second-degree relative (FHCMN). This group had a significantly different gender ratio, suggesting a different underlying mutation. Maternal FHCMN was negatively associated with PAS and satellites at birth, and maternal freckling was negatively associated with PAS. Other factors found to be significantly increased in CMN families compared with controls were maternal smoking and ill health during pregnancy. Maternal smoking was positively associated with PAS. CONCLUSIONS: This study relies on data from families after they have had a child with a CMN, and therefore may be subject to recall bias. Despite this, it contributes significantly to the knowledge of epidemiology of CMNs, and provides some important clues to the genetic basis of the condition.

Great Ormond Street Hospital for Children Registry for Congenital Melanocytic Naevi: prospective study 1988-2007. Part 2--Evaluation of treatments.

BACKGROUND: The treatment of congenital melanocytic naevi (CMNs) has become controversial as better data on complications have been published. OBJECTIVES: To determine the longer-term risks and benefits of surgery in treatment of CMNs. METHODS: In this 19-year prospective study, 301 families completed yearly questionnaires about treatments and CMN changes. Forty per cent of CMNs were > 20 cm projected adult size (PAS) or multiple CMNs. RESULTS: Girls were more likely to have had surgical treatments. There were no significant effects of treatment on the incidence of adverse clinical outcomes, although the numbers for melanoma were small. The majority of untreated CMNs lightened spontaneously during the follow-up period. Surgical treatment and satellites at birth were independently significantly associated with reported darkening of the CMN over the follow-up period. However there was no absolute measurement of final colour. Surgical treatment was associated with decreasing hairiness of the CMN over the follow-up period. PAS was associated with increasing hairiness. Excision with tissue expanders and PAS were significantly associated with an increased incidence of new satellite lesions. A proportion of patients reported new pigmentation in previously unaffected skin at the edge of a treated area, the majority after complete excision. There was a high level of satisfaction with surgery in the < 20 cm group and in those with facial CMNs. This was significantly reduced with increasing PAS. CONCLUSIONS: There is no evidence here that surgery reduces the incidence of adverse clinical outcomes in childhood. The natural history of the majority of untreated CMNs is to lighten spontaneously, whereas some treatments may cause adverse effects.

Complications of congenital melanocytic naevi in children: analysis of 16 years' experience and clinical practice.

BACKGROUND: Congenital melanocytic naevi (CMNs) can be associated with abnormalities of the cental nervous system (CNS) and/or with melanoma. Quoted incidences for these complications vary in the literature, as do recommendations for investigations and follow-up. OBJECTIVES: To determine the incidence of complications, and to identify phenotypic features associated with a higher risk of complications. METHODS: We reviewed records of 224 patients with CMNs seen in Dermatology clinic between 1991 and 2007. Patients were excluded if they had a complication at the time of referral. Magnetic resonance imaging (MRI) of the CNS was offered on the basis of CMN phenotype. Follow up was in clinic and/or by postal questionnaires. RESULTS: One hundred and twenty patients (54 boys and 66 girls) who had MRI of the CNS were included in the analysis. Mean age at MRI was 2.46 years (median 1.20). Mean follow up was 8.35 years (median 7.86). Sixty-five per cent had naevi > 20 cm projected adult size or multiple CMNs (40% > 40 cm), and 83% had satellite lesions at birth. Outcome measures were MRI abnormality, clinical neurological abnormality, any tumour, malignant melanoma, and death. No complications were seen in the 16 patients with no satellite lesions at birth. MRI and/or clinical neurological abnormalities were found in 22 patients (18%) and were significantly associated with projected adult size of the CMN (particularly > 40 cm), and independently with male gender. Tumours occurred in five patients, two of which were malignant melanoma (1.7%). Due to small numbers there was no significant association between phenotype and occurrence of tumours. Three patients (2.5%) died (one from neuromelanosis and two from melanoma in patients with normal MRI scans). Death was significantly associated with CMN size > 40 cm. Importantly, there was no significant association between CMN distribution (including posterior axial location) and adverse outcomes. CONCLUSIONS: This is the largest study of CNS imaging in patients with CMNs. We report a newly recognized association between male gender and neurological complications, dispute the previously reported association between CMN site and neurological complications, and quantify the associations between CMN size, satellite lesions and neurological complications. We make recommendations for the management of these patients.

A randomised controlled trial of a double layer of Allevyn compared to Jellonet and proflavin as a tie-over dressing for small skin grafts.

INTRODUCTION: The tie-over dressing is used to encourage skin graft take by minimising dead space, reducing seroma and haematoma formation and by graft immobilisation. Various materials have been proposed, however we have compared one of the most popular, Jellonet with a bolster of proflavin-soaked cotton wool, to a newer dressing, Allevyn foam. PATIENTS AND METHODS: Sixty patients were recruited and randomised into either group. Any patient requiring surgery involving a split or full thickness graft due to be carried out in the outpatient department on any site was invited to participate. Outcome measures included percentage take, pain on dressing removal (visual analogue scale) and infection. Ethical approval was obtained from the North and Mid Essex Local Research Ethics Committee. RESULTS: There was no statistical difference in graft take between the two groups at day 5 (P=0.963). The Allevyn dressing was statistically more comfortable (P=0.0182). DISCUSSION: We propose that Allevyn foam provides an effective and comfortable method for securing small split and full thickness skin grafts. While offering equal levels of graft take, improved levels of comfort may lend itself to use in sensitive areas such as the nose, ear and around the eye.

Hutchinson-Gilford progeria syndrome: clinical findings in three patients carrying the G608G mutation in LMNA and review of the literature.

BACKGROUND: Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature ageing disorder that belongs to a group of conditions called laminopathies which affect nuclear lamins. Classical and atypical forms of HGPS have been reported and there are clinical overlaps with mandibulo-acral dysplasia and restrictive dermopathy. To date, mutations in two genes, LMNA and ZMPSTE24, have been found in patients with HGPS. The p.G608G LMNA mutation is the most commonly reported mutation. Correlations between genotype and phenotype in children with progeroid syndromes are beginning to emerge. OBJECTIVES: To establish whether the LMNA p.G608G mutation is associated with a particular phenotype of HGPS. METHODS: We reviewed the clinical features and skin histology of three children with HGPS associated with the p.G608G LMNA mutation, and compared our findings with those reported in the literature. RESULTS: Our patients shared a very similar presentation and clinical course. Skin changes were the earliest finding in all three. Skin histology showed nonspecific changes only. CONCLUSIONS: The LMNA p.G608G mutation results in a uniform phenotype through early to mid-childhood, in keeping with that described in classical HGPS. Skin changes are the earliest distinctive clinical finding and should prompt careful physical and radiological examination for other features of HGPS. Skin biopsy for histology is not a useful investigation when a diagnosis of HGPS is suspected.

Relocation of neuromas of the lateral antebrachial cutaneous nerve of the forearm into the brachialis muscle.

Painful neuromas following injury to the radial side of the wrist can be treated by relocation away from the zone of injury and implantation into muscle. Relocation to the brachialis muscle is useful for isolated neuromas of the lateral antebrachial cutaneous nerve and involves a shorter dissection than relocation to the brachioradialis. It is also useful in patients undergoing multiple procedures to avoid disturbing previous relocations to the brachioradialis. This paper describes the successful relocation of painful neuromas of the lateral antebrachial cutaneous nerve to the brachialis muscle in seven patients.