Neil Bulstrode

Great Ormond Street Hospital for Children NHS Foundation Trust, Londinium, England, United Kingdom

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Publications (15)36.15 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: The incidence of midline frontonasal dermoids cysts is 1 in 20000-40000. These lesions may have intracranial extension correlating with the anatomy and embryology of nasofrontal development. Skin involvement may be extensive, with puncta and hair-bearing sinus tracks in the skin of the dorsum of the nose. Incomplete excision frequently leads to recurrence.We report our experience and pathway for management of midline dermoids.
    Plastic &amp Reconstructive Surgery 10/2014; · 3.33 Impact Factor
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    ABSTRACT: Treacher Collins syndrome (TCS), mandibulofacial dysostosis, or Franceschetti–Zwahlen–Klein syndrome, is a rare genetic disorder characterised by dysgenesis of the hard and soft tissues of the first and second branchial arches. Early operations focus on maintaining the airway, protecting the eyes, and supporting auditory neurological development. Later operations include staged reconstruction of the mouth, face, and external ear. Bimaxillary surgery can improve the maxillomandibular facial projection, but correction of malar, orbital rim, and temporal defects may be more difficult. We present a clinical review of the syndrome with a chronological approach to the operations.
    British Journal of Oral and Maxillofacial Surgery 09/2014; · 1.13 Impact Factor
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    ABSTRACT: Jeune syndrome (asphyxiating thoracic dystrophy) is a rare disorder characterized by skeletal dysplasia, reduced diameter of the thoracic cage and extrathoracic organ involvement. Fatal, early respiratory insufficiency may occur. Two-stage lateral thoracic expansion has been reported, addressing each side sequentially over 3-12 months. While staged repair theoretically provides less invasive surgery in a small child with respiratory distress, we utilized a single stage, bilateral procedure aiming to rapidly maximize lung development. Combined bilateral surgery also offered the chance of rapid recovery, and reduced hospital stay. We present our early experience of this modification of existing surgical treatment for an extremely rare condition, thought to be generally fatal in early childhood. Nine children (6 males, 3 females; median age 30 months [3.5-75]) underwent thoracic expansion for Jeune syndrome in our centre. All patients required preoperative respiratory support (5 with tracheostomy, 8 requiring positive pressure ventilation regularly within each day/night cycle). Two children underwent sequential unilateral (2-month interval between stages) and 7 children bilateral thoracic expansion by means of staggered osteotomies of third to eighth ribs and plate fixation of fourth to fifth rib and sixth to seventh rib, leaving the remaining ribs floating. There was no operative mortality. There were 2 deaths within 3 months of surgery, due to pulmonary hypertension (1 following two-stage and 1 following single-stage thoracic expansion). At the median follow-up of 11 months (1-15), 3 children have been discharged home from their referring unit and 2 have significantly reduced respiratory support. One child remains on non-invasive ventilation and another is still ventilated with a high oxygen requirement. Jeune syndrome is a difficult condition to manage, but bilateral thoracic expansion offers an effective reduction in ventilator requirements in these children. While two-stage repair has been described previously, this is the first report of single-stage bilateral thoracic expansion. Single-stage repair is feasible and may offer better resource management and significant cost savings by potentially reducing theatre usage and overall length of stay (intensive care and hospital) without compromising clinical outcomes.
    European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 03/2014; · 2.40 Impact Factor
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    ABSTRACT: Postpneumonectomy syndrome is a rare complication following pneumonectomy with its related change in mediastinal configuration. Symptoms range from airway obstruction to esophageal symptoms, leading at times to a persistent requirement for respiratory support. Surgery is often beneficial, which is in the form of placement of a prosthesis, with variable results. We report 2 cases of postpneumonectomy syndrome: one with successful relief, and the other in which the saline-filled prosthesis failed to achieve the desired result.
    Asian cardiovascular & thoracic annals 12/2013;
  • S Cugno, R D Farhadieh, N W Bulstrode
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    ABSTRACT: Autologous microtia reconstruction is generally performed in two stages. The second stage presents a unique opportunity to carry out other complementary procedures. The present study describes our approach to microtia reconstruction, wherein the second stage of reconstruction is combined with final refinements to the ear construct and/or additional procedures to enhance facial contour and symmetry. Retrospective analysis of patients who underwent two-stage microtia reconstruction by a single surgeon (NWB) was conducted in order to ascertain those that had ancillary procedures at the time of the second stage. Patient and operative details were collected. Thirty-four patients (male, 15, median age and age range at second stage, 11 and 10-18 years, respectively) who had complementary procedures executed during the second stage of auricular reconstruction were identified. Collectively, these included centralizing genioplasty (n = 1), fat transfer (n = 22), ear piercing (n = 7), and contralateral prominauris correction (n = 7). Six patients had correction for unilateral isolated microtia and in the remaining 28 patients, auricular reconstruction for microtia associated with a named syndrome. All patients reported a high rate of satisfaction with the result achieved and the majority (85%) reported no perceived need for additional surgical refinements to the ear or procedure(s) to achieve further facial symmetry. No peri- or post-operative complications were noted. Combining the final stage of autologous microtia reconstruction with other ancillary procedures affords a superior aesthetic outcome and decreased patient morbidity.
    Journal of Plastic Reconstructive & Aesthetic Surgery 08/2013; · 1.47 Impact Factor
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    ABSTRACT: Congenital melanocytic naevi (CMN) can be associated with neurological abnormalities and increased risk of melanoma. Mutations in NRAS, BRAF and Tp53 have been described in individual CMN samples, however, their role in the pathogenesis of multiple CMN within the same subject and development of associated features has not been clear. We hypothesised that a single post-zygotic mutation in NRAS could be responsible for multiple CMN in the same individual, as well as for melanocytic and non-melanocytic central nervous system (CNS) lesions. Fifty-five samples from 15 patients with multiple CMN were sequenced after site-directed mutagenesis and enzymatic digestion of the wild-type allele. Oncogenic missense mutations in codon 61 of NRAS were found in affected neurological and cutaneous tissues of 12/15 patients, but absent from unaffected tissues and blood, consistent with NRAS mutation mosaicism. In ten patients the mutation was consistently c.181C>A, p.Q61K, and in two c.182A>G, p.Q61R. All 11 non-melanocytic and melanocytic CNS samples from five patients were mutation positive, despite NRAS rarely reported as mutated in CNS tumours. Loss of heterozygosity was associated with onset of melanoma in two cases, implying a multi-step progression to malignancy. These results suggest that single post-zygotic NRAS mutations are responsible for multiple CMN and associated neurological lesions in the majority of cases.Journal of Investigative Dermatology ccepted article preview online, 7 February 2013; doi:10.1038/jid.2013.70.
    Journal of Investigative Dermatology 02/2013; · 6.19 Impact Factor
  • Journal of Plastic Reconstructive & Aesthetic Surgery 01/2013; · 1.47 Impact Factor
  • Journal of Plastic Reconstructive & Aesthetic Surgery 12/2012; · 1.47 Impact Factor
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    ABSTRACT: Use of the free groin flap, one of the first microvascular free flaps described, has been neglected recently because it has a short pedicle and varies anatomically. However, we have found its anatomical features and type of tissue ideal for volumetric enhancement in severe hemifacial asymmetry. We present a retrospective review of a consecutive series of 14 patients who had hemifacial augmentation with a free groin flap (mean age at operation 17 years, range 10-42) since 2001, and discuss the surgical technique. The most common cause of asymmetry was hemifacial microsomia (n=6). Anatomical variation of the vessels in the groin did not cause problems. Arterial anastomosis was to the facial artery in 13 patients; 12 patients had simultaneous hard tissue procedures. No flaps failed. The free groin flap is a useful adjunct in the management of hemifacial deficits in volume when free fat grafts will not provide enough bulk. Although the operation can take longer than non-vascularised grafts, little tissue is lost so long-term results may be more predictable. We have found the anatomy fairly consistent and the short pedicle caused no problems.
    British Journal of Oral and Maxillofacial Surgery 10/2012; · 1.13 Impact Factor
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    ABSTRACT: Poikiloderma with neutropenia (PN) is a rare disorder attributed to mutations in the C16orf57 gene(1,2) , with many similarities to other hereditary poikilodermas (HP)(3) . The risks ascribed to this condition are yet to be fully elucidated.x1We report the case of a patient with PN who developed a Squamous Cell Carcinoma (SCC) of the toe at an early age including studies demonstrating abnormal neutrophil function.
    British Journal of Dermatology 08/2012; · 3.76 Impact Factor
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    ABSTRACT: Congenital melanocytic nevi (CMN) are pigmented birthmarks that affect up to 80% of the skin surface area. The increased frequency of CMN in families of severely affected individuals is suggestive of a predisposing germline genotype. We noted a high prevalence of red hair in affected families, and considered a role for MC1R in this condition. A cohort of 166 CMN subjects underwent pigmentary phenotyping, with MC1R genotyping in 113. Results were compared with a local control group of 60 unrelated children and with 300 UK children without CMN. CMN subjects had higher prevalences of red hair and a red-haired parent than local controls and had a higher rate of compound heterozygosity and homozygosity for MC1R variants. The presence of a V92M or R allele (D84E, R151C, R160W, D294H) was associated with increasing size of the CMN, implying a growth-promoting effect of these alleles. Unexpectedly, the V92M and R151C alleles were also strongly associated with birth weight in the CMN cohort, a finding confirmed in the control group. The effect of germline MC1R genotype on development and severity of CMN led us to investigate potential broader effects on growth, revealing a role for MC1R in normal fetal development.
    Journal of Investigative Dermatology 05/2012; 132(8):2026-32. · 6.19 Impact Factor
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    ABSTRACT: Lipofibromatosis is a rare benign fibrofatty tumor of childhood. The typical presentation of this tumor is as a poorly demarcated and slow-growing mass involving the subcutaneous or deep soft tissues. Lipofibromatosis was first described in 2000, and since then, a small number of cases have been reported in the literature. We report a case of a 19-month-old boy who presented with a swelling of the anterior aspect of the right knee since birth, which had increased in size out of proportion with his growth. Magnetic resonance imaging was extremely useful because it showed the lipomatous nature of the mass, narrowing the differential diagnosis to the pediatric fibrofatty soft tissue tumors. The histologic biopsy revealed the specific diagnosis of lipofibromatosis. We describe the radiologic and pathologic features of this entity and discuss the differential diagnosis in a young child with a fat-containing limb mass.
    Journal of Pediatric Surgery 05/2012; 47(5):1028-31. · 1.31 Impact Factor
  • Benjamin Soukup, Syed A Mashhadi, Neil W Bulstrode
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    ABSTRACT: This study aims to assess the health-related quality-of-life benefit following auricular reconstruction using autologous costal cartilage in children. In addition, key aspects of the surgical reconstruction are assessed. After auricular reconstruction, patients completed two questionnaires. The first was a postinterventional health-related quality-of-life assessment tool, the Glasgow Benefit Inventory. A score of 0 signifies no change in health-related quality-of-life, +100 indicates maximal improvement, and -100 indicates maximal negative impact. The second questionnaire assessed surgical outcomes in auricular reconstruction across three areas: facial integration, aesthetic auricular units, and costal reconstruction. These were recorded on a five-point ordinal scale and are presented as mean scores of a total of 5. The mean total Glasgow Benefit Inventory score was 48.1; significant improvements were seen in all three Glasgow Benefit Inventory subscales (p < 0.0001). A mean integration score of 3.8 and a mean aesthetic auricular unit reconstruction score of 3.4 were recorded. Skin color matching (4.3) of the ear was most successfully reconstructed and auricular cartilage reconstruction scored lowest (3.5). Of the aesthetic units, the helix scored highest (3.6) and the tragus/antitragus scored lowest (3.3). Donor-site reconstruction scored 3.9. Correlation analysis revealed that higher reconstruction scores are associated with a greater health-related quality-of-life gain (r = 0.5). Ninety-six percent of patients would recommend the procedure to a friend. Auricular reconstruction with autologous cartilage results in significant improvements in health-related quality-of-life. In addition, better surgical outcomes lead to a greater improvement in health-related quality-of-life. Comparatively poorer reconstructed areas of the ear were identified so that surgical techniques may be improved. Therapeutic, IV.
    Plastic and Reconstructive Surgery 03/2012; 129(3):632-40. · 3.33 Impact Factor
  • Adel Fattah, Neil J Sebire, Neil W Bulstrode
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    ABSTRACT: Current techniques of autologous ear reconstruction involve the soft tissue coverage of a carved costal cartilage framework. However, assessment of the morbidity associated with this donor site has been little documented. This study describes a method to reconstruct the defect and analyses the outcomes with or without donor site reconstitution. The donor site was reconstituted by wrapping morcelised cartilage in a vicryl mesh. Twenty-one patients with reconstitution and nine without were recruited to the study. Scar quality and length, dimensions of donor defect and visible deformity were recorded according to a modified Vancouver scar scale. Patients were also assessed by the SF36 questionnaire, a well-validated health survey. In a subset of our study group, we assessed the fate of the donor site reconstitution by direct visualisation in situ and histological analysis. Fifteen donor sites of patients without donor site reconstitution were compared to 23 reconstructed donor sites. In those without, all had a palpable defect with nearly half exhibiting visible chest deformity. In contrast, those that had rib reconstitution did not demonstrate significant chest wall deformity. Intraoperative examination demonstrated formation of a neo-rib, histologically proven to comprise hyaline cartilage admixed with fibrous tissue. Analysis of SF36 results showed a higher satisfaction in the reconstituted group, but in both groups, the donor site was of little overall morbidity. Although there is little difference between the groups in terms of subjectively perceived benefit, rib reconstitution is objectively associated with better costal margin contour and less chest wall deformity.
    Journal of Plastic Reconstructive & Aesthetic Surgery 09/2009; 63(9):1459-65. · 1.47 Impact Factor
  • Veronica Kinsler, Neil Bulstrode
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    ABSTRACT: Recent advances in research have prompted this review of the role of surgery in the management of congenital melanocytic naevi (CMNs). Good data on the incidence of neurological and malignant complications of CMNs have re-fuelled the debates on whether surgery decreases the risk of malignant melanoma and whether early surgery is advantageous. We conclude the following: 1) untreated CMNs can lighten spontaneously, sometimes dramatically, 2) routine surgery has not been demonstrated to reduce the risk of malignancy and is, therefore, for cosmetic reasons only, 3) early surgery has not been shown to be advantageous and carries increased anaesthetic risk and 4) there is some evidence that surgical intervention may adversely affect the behaviour of the CMN cells. Our current practice is based on the following guidelines: 1) patients are treated in a multidisciplinary-team setting which includes the specialties of paediatric dermatology, plastic surgery and neuroradiology with access to neurology, neurosurgery and oncology, 2) serial photographs are taken at yearly intervals to assess spontaneous lightening. 3) all routine surgery is delayed for at least the first year. 4) patients with facial CMNs (either the principal lesion or large satellites) are offered surgery for cosmetic reasons, 5) patients with a single, easily excisable CMN are offered surgery for cosmetic reasons and 6) all families are made aware of the possibility of spontaneous lightening and the possibility that surgery may have effects on the behaviour of naevus cells.
    Journal of Plastic Reconstructive & Aesthetic Surgery 03/2009; 62(5):595-601. · 1.47 Impact Factor