Birmingham Children's Hospital Research Articles

Reducing general anaesthetic episodes: 'piggyback' procedures - Birmingham Children's Hospital experience.

Introduction Birmingham Children's Hospital (BCH) has a well-established care pathway for joint procedures - 'piggybacks' - under general anaesthetic (GA). The premise behind these joint procedures is that dental treatment is undertaken at the same time as another planned GA, usually completed by the patient's primary medical or surgical specialty, or another speciality attending a dental GA list.Aim The aim of this paper is to share the recent BCH experience of joint procedures and provide a model for secondary and tertiary care providers across the UK to develop their own collaborative working approaches.Method Data were collected retrospectively from May 2021 to May 2023 on the department of paediatric dentistry at Birmingham Children's Hospital. Patients were included if they had any joint procedure undertaken.Results In total, 93 patients were treated as a 'piggyback' procedure during this period. The average age of patient treated was nine years and three months. A total of 39 patients had extractions only during this period, while 19 had scaling only and 12 had both scaling and extractions. Additionally, 269 primary teeth and 22 permanent teeth were extracted during this period.Conclusion BCH has a well-established pathway of care for 'piggyback' procedures. Future workforce planning and patient care pathways should consider joint procedures and further explore this initiative to continue to streamline patient care and reduce waiting lists and risks associated with GA.

Quality improvement using PDSA cycles improves performance on NPDA key care processes for Birmingham Children's Hospital

Quality improvement using PDSA cycles improves performance on NPDA key care processes for Birmingham Children's Hospital

O08 Mesenchymal stromal cells infusions in children with recessive dystrophic epidermolysis bullosa (MissionEB): a randomized controlled trial

Abstract Recessive dystrophic epidermolysis bullosa (RDEB) is one of the most severe forms of epidermolysis bullosa caused by loss-of-function mutations in the type VII collagen gene (COL7A1) leading to extensive skin and mucosal fragility and systemic complications. No active treatment is currently available. This study assesses whether repeated infusions of allogeneic umbilical cord-derived mesenchymal stromal cells are safe and can benefit children with RDEB. This is a double-blinded, randomized (1 : 1), placebo-controlled crossover trial with an internal dose de-escalation trial for safety and a further 12-month open-label study following review of the data. Around 36 participants with RDEB who are older than 6 months and younger than 16 years are being recruited at Great Ormond Street Hospital and Birmingham Children's Hospital. Participants will receive two intravenous infusions (days 0 and 14), at a dose of 2–3 × 106 cells kg–1 that could be adjusted to 1–1.5 × 106 cells kg–1 based on observed toxicities. Outcomes assessed at 3 and 6 months from day 0 in each crossover period include changes in EBDASI, iscorEB, pain, itch and quality of life scores. This is the largest cell-therapy study in children with RDEB. Previous nonrandomized trials have suggested that intravenous mesenchymal stromal cells are safe in children with RDEB and indicated early evidence of benefit. There was a need for a robust controlled study to validate these findings. The study is funded by the National Research Collaboration Programme (NIHR127963), an NHS England and NHS Improvement and National Institute for Health Research partnership, and Cure EB. ISRCTN registry (ISRCTN14409785).

The 72nd Bubble wrap x Birmingham Children's Hospital Emergency Department

The 72nd Bubble wrap x Birmingham Children's Hospital Emergency Department

Relationship between decrement time and recovery time in pediatric total intravenous anesthesia with propofol and remifentanil.

Propofol target-controlled infusion is now used in many countries for the administration of total intravenous anesthesia (TIVA) and uses population pharmacokinetic data incorporated into the target-controlled infusion pump to administer a dose which will achieve a target concentration selected by the practitioner. Modern target-controlled infusion pumps can include an estimate of how long it will take for the plasma propofol concentration to fall to a value programmed into the pump, once the propofol infusion is stopped. This is known as decrement time. Many users consider decrement time to be equivalent to recovery time for the patient, despite the several assumptions that makes. To determine if the decrement time estimated by the target-controlled infusion pumps at the end of anesthesia could be reliably used to predict recovery time for individual patients. Target-controlled infusion pumps at Birmingham Children's Hospital are programmed with a propofol decrement concentration of 1.5 μg.mL-1 . Patients for a variety of surgical procedures were anesthetised with propofol-remifentanil TIVA. Eye opening was used as a surrogate of recovery time. Data were analyzed using median absolute deviation, Mann-Whitney U test, and linear regression to see if there was any correlation with decrement time and recovery time, total dose of propofol administered, duration of anesthesia and patient age. A total of 55 patients were audited. Median age was 7.2 years (range 0.9-15.9), and median weight was 26 kg (range 8.5-77). Median decrement time was 11.8 min (IQR 8.3-21), while median recovery time was 15 min (IQR 9-20). Recovery time was the same as decrement time in 2% of patients, less than in 40% of patients and more in 58% of patients. Two thirds of patients opened eyes at an estimated plasma propofol concentration between 1.2 and 1.8 μg.mL-1 . The median absolute deviation for the difference between decrement time and recovery time was 5.7 min, from a median of 1 min. Analysis demonstrated there was no statistical correlation between decrement time to 1.5 μg.mL-1 and recovery time, age, total propofol dose administered, or duration of anesthesia, for individual patients. There was variation between decrement time to 1.5 μg.mL-1 and recovery time of over 5 min in nearly half of patients. Decrement time to 1.5 μg.mL-1 must be used with caution when trying to predict recovery time for individual patients following TIVA.

Real world experience of efficacy and safety of rivaroxaban in paediatric venous thromboembolism

IntroductionPaediatric clinical practice for treatment of venous thromboembolism (VTE) is based on extrapolation from adult trials with minimal data on anticoagulation efficacy and safety in children. Based on EINSTEIN-Jr clinical trial data, rivaroxaban was approved to treat VTE and prevent its recurrence in children of all ages. AimTo report the safety and efficacy of rivaroxaban use in paediatric VTE and to present real-world data, specifically about very young children. MethodsWe conducted a retrospective observational study at Birmingham Children's Hospital. Data were collected from patients <16 years old who received rivaroxaban after its licensure in the period between March 2021 and June 2022. ResultsRivaroxaban was used for treatment of acute VTE in 64 patients. Thrombosis was CVC-related in 26 patients, unprovoked in 3, while the rest had one or more risk factors for VTE. Safety and efficacy of rivaroxaban were assessed in 52 patients after excluding patients who were on current rivaroxaban treatment and those who were lost to follow up or stopped rivaroxaban due to intolerance. No bleeding events were reported, and recurrence of thrombosis occurred in only 3.6 %. About 35 % had normalised re-imaging, 40.3 % improved, 9.6 % were unchanged and 11.5 % stopped rivaroxaban without re-imaging. Rivaroxaban was used for secondary VTE prophylaxis in 6 patients in our cohort with no recurrence of thrombosis or bleeding reports. ConclusionsOur real-world experience confirmed that rivaroxaban was well tolerated, effective and safe. Further real-world data and observational studies are essential to investigate the use of rivaroxaban among different risk groups.

OTHR-20. Precision neuro-oncology in the real world. Opportunity and challenges from a UK Oncology Centre

The last five years has shown advances in the molecular classification of brain tumour, molecular profiling techniques and an increased use of targeted therapies. We reviewed the molecular analysis pathways and use of targeted agents at Birmingham Children’s Hospital (BCH), a large (~55 new cases/year) neuro-oncology centre, between 2016-2021. Having previously been analysed locally by limited directed immuno-histochemical stains and referral for specific genetic tests, tissue is now referred for a range of second histopathological opinions and in depth molecular classification, via methylation array, panel sequencing, RNA fusion analysis, and whole genome sequencing. These are accessed through different evolving pathways and consent processes, including referral to other centres, national reference laboratories, clinical studies, and local genetics laboratories with links to national sequencing infrastructures. Different routes result in different reporting structures, timescales and with varying levels of interpretation, often without adequate access to clinical information and context. 21 patients were treated on five targeted agent clinical trials (Afatanib (n=6), Biomede (n=3), eSmart(n=1), PARC (n=7), Vinilo (n=5)), with one patient on both Afatanib and PARC trials. A further two patients visited other centres for trials. Eight patients received MAPK pathway inhibitors through compassionate access pathways, with benefit, including radiological response, in four. Cardiac toxicity was observed in three and retinal oedema in one. Two patients received immune checkpoint inhibition, with rapid fatal enlargement, either progression or pseudo-progression, in one case. These rapid changes in diagnostic and management options offer new opportunities for patients, but bring challenges to the delivery of neuro-oncology services, including the logistics of sample, report, clinical trial, compassionate access management and the increased multi-specialist support required for monitoring and management of toxicities. Integration of targeted agents into the appropriate part of a patient’s treatment strategy requires skilled interpretation of the benefits compared to conventional therapies.

Anomalous coronary artery in Tetralogy of Fallot-Feasibility of right ventricular outflow tract stenting as initial palliation.

This study addresses the outcome of right ventricle outflow tract (RVOT) stenting in Tetralogy of Fallot (ToF) with anomalous coronaries crossing the RVOT. RVOT stenting in ToF patients has emerged as an alternative to Blalock Taussig shunting. This is a single center study of patients who underwent RVOT stenting for symptomatic ToF at Birmingham Children's Hospital between 2005 and 2020. A total of 122 patients underwent RVOT stenting as initial palliation over a 15-year period, 10 patients had anomalous coronaries crossing the RVOT (study group) and 112 not (comparative group). Median age of the study group was 72.5 days (interquartile range [IQR]: 28-103) with a weight of 4.7 kg (IQR: 3.5-4.9). No significant differences were found between the two groups regarding the patients' weights and ages, procedure and screening times, or hospital stay. Four had valve sparing stenting. Oxygen saturations increased from a median of 75.5% (IQR: 70-82) to 94.5% (IQR: 90-95), p < 0.002. Postprocedure median hospital stay was 3 days (IQR: 2-6). Six patients underwent interstage catheterization reintervention and one needed early surgical palliation due to stent suboptimal position. Complete repair could be delayed for a median of 11.1 months (IQR: 5.6-19.2). At surgical repair, the patients had a median age of 12.3 months (IQR: 7.5-25.6) and weight of 7.7 kg (IQR: 6.8-10.8). There were no deaths. RVOT stenting in ToF with anomalous coronaries is safe and effective. Dilatable stents should be used when two-stage delayed conduit repair is the default approach.

Acute-on-chronic liver failure: A 20-year retrospective review of a tertiary paediatric liver centre.

Acute-on-chronic liver failure (ACLF) is an acute deterioration of pre-existing chronic liver disease related to a precipitating event. We characterised paediatric ACLF at Birmingham Children's Hospital (BCH) utilising European Association of Liver Disease CLIF criteria, including prevalence, triggers and outcomes. All BCH patients from 2000 to 2020 with CLD who underwent initial liver transplant or died on the transplant waiting list or whilst too unwell to be listed were reviewed. From 2000 to 2020, 24 (4%) children with ACLF were identified. Death occurred in 18 (75%). Transplant occurred in 9 (36%), 3 of which died. ACLF triggers were sepsis organism negative 11 (46%), sepsis organism positive 8 (33%) and GI bleed 5 (17%). Bilirubin at the time of transplant/death in those with ACLF who lived compared with those who died was 529umol/L (381) versus 665 (210) (p=0.38), creatinine 138 umol/L (147) versus 67 (46) (p=0.41), PT 33sec (14) versus (32 (15) (p=0.72), Grade 3, 4hepatic encephalopathy 1 (17%) versus 10 (56%) (p=0.17), vasopressor use 1 (17%) versus 17 (94%) (p=0.001) and ventilation 3 (50%) versus 17 (94%) (p=0.035). Acute-on-chronic liver failure whilst infrequent has high rates of mortality. The use of vasopressors and ventilation is more frequent in those who die from ACLF.

Ophthalmological Care of Patients With Craniofacial Disorders.

Patients presenting with craniofacial conditions present a unique challenge from an ophthalmological view point. There are no set guidelines as to their management or their long-term monitoring and follow-up. Largely, this should be the remit of a dedicated craniofacial team. Here we present pertinent ophthalmological pathology occurring in combination with craniosynostosis alongside the protocol employed in Birmingham Children's Hospital for the management of these patients.

Meet the BANN Committee members

Katie Elizabeth Herbert is a clinical nurse specialist in neurosurgery at Birmingham Children's Hospital. As Katie has recently joined the British Association for Neuroscience Nurses' committee, she will take this opportunity to introduce herself to the community.

Safeguarding children and families during the pandemic

Jane Powell and Julie Taylor at Birmingham Women's and Children's Hospital NHS Foundation Trust explain how a collaborative approach to safeguarding, maximising capacity within the service, will help to ensure better outcomes for children

Supratentorial vs infratentorial posterior calvarial distraction osteogenesis for the increase of ICV in children with syndromic or multi-suture craniosynostosis: a retrospective cohort study

PurposeCraniosynostosis is the premature and pathological fusion of calvarial sutures. One modality of surgical treatment of syndromic craniosynostosis is posterior calvarial distraction (PCD). This can be either supratentorial or infratentorial. Currently, supratentorial PCD may be regarded as safer but produces a smaller increase in calvarial volume compared to infratentorial PCD. This study quantifies and compares the effectiveness of supratentorial and infratentorial PCD to help guide surgical decision-making.MethodsThe CT and/or MRI scans of 47 cases of craniosynostosis who underwent PCD from the Birmingham Children’s Hospital (BCH) were converted to sagittal series multi-planar reformatted (MPR) scans for the manual calculation of ICV. The 47 cases were classified as having undergone either supratentorial or infratentorial PCD using lateral plain film radiographs, with 28 and 32 pairs of pre- and post-operative CT/MRI scans reviewed respectively.ResultsA statistically significant difference between supratentorial and infratentorial PCD was observed for the increase in supratentorial volume (STV) (P = 0.0458) and total intracranial volume (TICV) (P = 0.0437), but not for the increase in infratentorial volume (ITV) (P = 0.0697). The relationship for each volume trended towards convergence but was not achieved before the physical limit of 30 mm distraction had been reached. Intraclass correlation coefficient values for agreement of MRI and CT scans for STV, ITV and total ICV were 0.852, 0.864 and 0.854 respectively.ConclusionOur evidence suggests that supratentorial PCD is more effective for increasing ICV in a clinical setting. CT and MRI imaging modalities are acceptably clinically interchangeable for calculating ICV in craniosynostosis.

Parental Home Vision Testing of Children During Covid-19 Pandemic.

Background:The Covid-19 pandemic necessitated social distancing restrictions, which placed limitations on access to ophthalmic care to only those who had an imminent risk of sight loss. All other face-to-face consultations were converted to telephone consultations or were postponed. We investigated whether parents were able to test their child’s vision using available home vision testing applications, with an aim to aid decision making during a telephone consultation.Methods:Families with follow-up consultations at Birmingham Children’s Hospital were asked to test their child’s vision at home. Instructions for the use of Peek acuity, or iSight Pro, were emailed to a parent. Parents chose to use a particular app based on available devices at home. Parents were asked to test uniocular visual acuity twice. Home versus hospital acuity was correlated. Home acuity test-retest reliability was acquired. Parental feedback was obtained through questionnaires.Results:One hundred and three families were contacted, 15 families completed home vision testing. Ten families used Peek acuity, five families used iSight Pro. Uniocular visual acuity test-retest reliability was 0.03 LogMAR. Home-hospital acuity testing had a bias of 0.14 LogMAR, hospital acuity yielding a lower LogMAR score. Most families who completed testing found it easy to do; however, some struggled, and 81 families did not undertake home vision testing.Conclusions:Uptake of home vision testing was limited by parental engagement, most likely influenced by the current pandemic. Most families who undertook home vision testing were able to generate results that could be used for clinical decision making. Extending the impact of parental vision testing will require education by clinicians and further study to increase sample sizes and to improve confidence.

Five year clinical outcomes and evaluation following implantation of the Oticon™ wide bone anchored hearing system in 47 children

ObjectivesThe Oticon™ wide implant system was launched in 2009 and used at Birmingham Children's Hospital from 2014. To evaluate clinical outcomes of the Oticon™ wide implant (Oticon Medical), with a focus on skin complication rates and fixture loss over a 5-year period in a tertiary paediatric hospital in the UK. MethodsRetrospective 5-year longitudinal case record review of 47 children who were implanted with the Oticon™ wide implant system at Birmingham Children's Hospital (BCH) between January 2014 and January 2016. Results47 children (27 M:20F) were implanted with 70 Oticon wide implants 23 bilateral, 27 unilateral. Mean age at the time of implantation was 9y 6 m. The follow up was for a mean of 5.4 years. Significant soft tissue complications requiring treatment was found in 11% (n = 8) of loaded fixtures, abutment tightening on two patients, abutment exchange 6% (n = 4) and a 10% (n = 7) fixture failure. ConclusionThe Oticon™ wide implant system produces favourable results with regards to peri-abutment skin complications, fixture stability and revision surgery rates when compared to similar cohorts of children studied at Birmingham Children's Hospital.

Ten minutes with Mrs Katya Tambe, Joint Head of Service, Ophthalmology Department, Nottingham University Hospitals NHS Trust, Nottingham, UK

![Graphic][1] ### Biography Katya Tambe is the Joint Head of Service (HoS) for the ophthalmology department at Nottingham University Hospitals NHS Trust (NUH). She was appointed as a consultant ophthalmologist...

Atypical malignancies after intestinal transplantation in children: A European single-centre experience.

Post-transplant malignancies, that is, lymphomas, are a recognized complication in intestinal transplant recipients but are mostly secondary to EBV infection. There is an increased risk for malignancies in unusual sites in intestinal transplant recipients as compared to other solid organ transplants and the general population. To evaluate the incidence, course, and outcome of unusual malignancies in children after ITx. Retrospective analysis of children who underwent ITx for primary digestive disorders at Birmingham Children's Hospital between January 1989 and December 2017. Ninety-eight intestinal transplants were performed in 90 children (49 males and 41 females) with an underlying primary digestive disorder. Median age was 2.7years (0.6-16.2), and median weight was 14.5kg (5.7-53.2) at the time of transplant. Within this cohort, we identified four cases of unusual malignancies at rare sites of presentation. One patient developed cerebral PTLD, two patients were diagnosed with SMT, located at the stomal orifice and in cervicothoracic paravertebral area, respectively, and the last patient developed a retroperitoneal angiosarcoma. Unfortunately, the overall patient outcome was poor in all but one child with SMT, who currently survives with cytotoxic T-cell therapy. Unusual malignancies can occur in approximately 5% of children following ITx. A high index of suspicion is required for a timely diagnosis and adequate treatment.

Bridging the gap: Improving patient safety through targeted in-situ simulation training in a paediatric intensive care unit and Learning from Excellence (LfE)

Background: Improving patient safety and reducing risk is important to a Paediatric Intensive Care Unit (PICU). Simulation-based education has generally focused on the management of clinical diagnoses, whereas the Quality and Safety Team has traditionally focused on collecting and analysing data about adverse events. There is a need to bridge the gap between the two streams - lessons learnt from adverse incidents and their impartation to staff in a targeted format during in-situ simulation training.Methods: Birmingham Children's Hospital PICU is a 31-bedded tertiary/quaternary unit with approximately 1500 admissions per year in the UK. All adverse incidents are collated (online IR1 with specific forms for incidents involving medications, accidental extubations, buzzer pulls, and extravasations) and analysed by the PICU Safety Group and trends are monitored. The PICU Simulation Team delivers in-situ simulation training for the multidisciplinary PICU staff weekly using interactive, computer-controlled manikins. Each training scenario and debriefing lasts 1 hour. A core team of multidisciplinary simulation facilitators runs the simulation training and the AI (advocacy-inquiry) debriefing model1 is used for conducting the debriefings. The ‘Simulation Group’ (efferent) and the ‘Risk Group’ (afferent) regularly discuss the priorities for the unit and the lessons learnt based on actual events or near-misses in the unit. It then implements the action points during targeted scenario training sessions. This may be the utilisation of a care bundle or activation of a ‘clinical pathway’. Any practical problem with implementation of these policies is fed back to the Risk Group to close the loop. A concept of ‘Learning from Excellence’ (LfE) has been introduced successfully and both ‘adverse incidents’ and LfE are used together as approaches to improve patient safety in the unit.Observation/Evaluation: Various simulation scenarios have been run since the start of the project. Examples include accidental extubations, delay in sepsis recognition and antibiotics prescription, ischaemic limb injury due to the indwelling arterial line, emergency chest reopening in post-operative cardiac surgical patients, child protection and safeguarding2. The learning gained during each debriefing is generalised to all the participants of the simulation session3 and then subsequently the salient points are shared by email with the entire unit. All staff members have to undergo simulation training. Scenarios are re-run back to back if the team does not achieve the expected outcomes. The anonymous feedback forms completed by the participants of the scenarios have shown they value this targeted training and that it has helped them implement good practice. Anecdotaly, the trend of ‘incident severity’ is believed to have been on the decline over a 7-year period in our PICU but long term monitoring will continue to identify any re-emerging or fresh trends.Conclusion: ‘Targeted’ simulation-based training is an important approach to enhance the safety culture in PICU. PICU Safety and Simulation Groups should develop a symbiotic relationship for this to succeed. Learning from Excellence can be effectively utilised to embed good practice in a clinical area.

P61 An innovative multi-speciality clinic for patients with autoinflammatory conditions

Abstract Background Systemic autoinflammatory diseases (SAIDs) are rare, complex, and can often be debilitating. Establishing clinical and genetic diagnoses is equally complex and lengthy, causing significant stress to children and families. Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) recommendations emphasise the importance of access to genetic expertise alongside a tertiary paediatric rheumatology multidisciplinary team. Methods We established a 2-monthly combined clinic for children with diagnosed and suspected autoinflammatory conditions at Birmingham Children’s Hospital (BCH). These are run by a consultant paediatric rheumatologist with special interest in unexplained periodic fever syndromes and autoinflammatory disorders, in collaboration with a consultant clinical geneticist interested in genetic causes for autoinflammation. The BCH paediatric rheumatology department operates a truly multidisciplinary approach with a collocated team. This allows ease of access to clinical nurse specialists, physiotherapy and occupational therapy, as well as effective provision of transitional care, a dedicated research nurse and research coordinator. To avoid multiple appointments and delays in diagnosis, upon receiving referrals for possible SAIDs, a standardised letter along with fever diary, blood forms and instructions for examining clinicians is sent to patients and the referring clinician. In establishing fevers and fever-patterns, possible evidence of inflammation and exclusion of infection prior to initial assessment, the process is streamlined and appointments reduced. A multi-speciality assessment with paediatric rheumatology and genetics is conducted, collaborating with immunology and other appropriate specialists. If SAID is suspected, appropriate panel testing is arranged, followed by whole exome sequencing if results are inconclusive. Patients are also recruited to national and international research studies if relevant, including BCH Rare diseases centre Stars Together Registry &amp; NIHR BioResource rare diseases studies. Results In the past 3 years, the service has seen 96 new patients. Current patients have a variety of diagnoses, including: cryopyrin-associated periodic syndrome (CAPS)(NLRP3 mutations), familial Mediterranean fever (FMF) , TNF receptor-associated periodic syndrome (TRAPS), PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis), ADA2 mutation, NOD-2 mutation, Otulin-related autoinflammatory syndrome (ORAS), Aicardi-Goutieres syndrome (AGS), DNASE1L3 mutation, complement factor I deficiency, C2 deficiency, SLC29A3 mutation, Bechet’s disease and undifferentiated SAIDs. This does not include patients who have primary immunodeficiencies, non-inflammatory vasculopathies or other SAID mimics. Drug therapies include: NSAIDs, short-term corticosteroids, Colchicine, Infliximab, Tocilizumab, Methotrexate, Adalimumab, Mycophenolate mofetil (MMF), Ruxolitinib and Anakinra, all supported by clinical nurse specialists. Conclusion This cohesive service provides single-point access to multidisciplinary paediatric rheumatology and genetics, along with national and international high quality research. A unified approach avoids the need for multiple appointments, allowing prompter diagnosis, initiation of appropriate treatment, and potentially earlier disease control. This aims to prevent disease-related damage, working towards optimal support for families and improving quality of life. A dedicated multi-speciality clinic also provides excellent training opportunities, both between specialities and for junior trainees. Conflicts of Interest The authors declare no conflicts of interest.

Craniofacial Approaches to Pediatric Orbital Tumors.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. A total of 9 patients were identified between the years 2008 to 2017. Pathologies included rhabdomyosarcoma, juvenile ossifying fibroma, optic nerve glioma, and retinoblastoma. Surgical approaches to the orbit included supraorbital bar osteotomy (transcranial) or lateral orbitotomy (extracranial). Surgical team members included neurosurgery, craniofacial surgery, and ophthalmology. This study aims to review the role of surgery in management as well as the specific indications for performing transcranial or extracranial osteotomies. It also highlights the excellent access achieved with the use of these osteotomies in certain cases, especially when compared with transconjunctival or transcutaneous approaches.