Surgical treatment for primary spinal aneurysmal bone cysts: experience from Children's Hospital Boston

2012 ◽  
Vol 9 (3) ◽  
pp. 305-315 ◽  
Author(s):  
Georgios Zenonos ◽  
Osama Jamil ◽  
Lance S. Governale ◽  
Sarah Jernigan ◽  
Daniel Hedequist ◽  
...  
Keyword(s):  
Spinal Instrumentation ◽  
Neurological Deficits ◽  
Preoperative Embolization ◽  
Bone Cysts ◽  
Children's Hospital ◽  
Initial Intervention ◽  
Children’S Hospital ◽  
Aneurysmal Bone Cysts

Object Spinal aneurysmal bone cysts (ABCs) constitute a rare and clinically challenging disease, primarily affecting the pediatric population. Information regarding the management of spinal ABCs remains sparse. In this study the authors review their experience with spinal ABCs at Children's Hospital Boston. Methods The medical records of all patients treated surgically for primary spinal ABCs between January 1998 and July 2010 were retrospectively reviewed. Results Fourteen cases were identified (6 males and 8 females, ages 5–19 years old). The ABCs were located throughout the spine, with an equal number in the thoracic and lumbar spine, and rarely in the cervical spine. The majority of patients presented with back pain, but neurological deficits and spinal deformity were common. A variety of radiographic techniques were used to establish the diagnosis, including needle biopsy. Preoperative selective arterial embolization was performed in 7 cases (50%), and the majority of cases required spinal instrumentation along with resection. Mean follow-up was 55.9 months (range 15–154 months) after initial intervention. Two ABCs recurred (14%), at 9 months and 8 years after incomplete initial resection, and the patients underwent reoperation. Complete resection was ultimately achieved in all cases. All patients were asymptomatic and neurologically intact at their last follow-up evaluation, and showed no evidence of deformity or recurrence on imaging. Conclusions Computed tomography and MR imaging are adequate for an initial evaluation of spinal ABCs, although solid variants can present a diagnostic challenge. Given the high rates of recurrence with residual disease, complete obliteration of the lesion should be the goal of treatment. Preoperative embolization is often performed, although in the authors' opinion the degree of bleeding tends not to support its routine use. Long-term follow-up is warranted as recurrences can occur years after initial intervention. However, gross-total excision in conjunction with spinal stabilization, as needed, usually provides cure of the ABC and excellent long-term spinal alignment.

Long-term follow-up of Ethibloc injection in aneurysmal bone cysts

2009 ◽  
Vol 18 (6) ◽  
pp. 375-380 ◽  
Author(s):  
Harvey L. George ◽  
Puthanveettil Nithin Unnikrishnan ◽  
Neeraj K. Garg ◽  
Jayanth Sundar Sampath ◽  
Alf Bass ◽  
...  
Keyword(s):  
Bone Cysts ◽  
Long Term Follow Up ◽  
Aneurysmal Bone Cysts

Spondylolysis of C-2 in children 3 years of age or younger: clinical presentation, radiographic findings, management, and outcomes with a minimum 12-month follow-up

2014 ◽  
Vol 13 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Loyola V. Gressot ◽  
Sudhakar Vadivelu ◽  
Steven W. Hwang ◽  
Daniel H. Fulkerson ◽  
Thomas G. Luerssen ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Young Children ◽  
Neurological Deficits ◽  
Radiographic Findings ◽  
Children's Hospital ◽  
Very Young Children ◽  
The Mean ◽  
Neurologically Intact

Object Cervical spondylolysis is a rare condition that results from a pars interarticularis defect. The C-6 level is the most frequently involved site in the cervical spine. Its clinical presentations range from incidental radiographic findings to neck pain and, rarely, neurological deficits. Although 150 patients with subaxial cervical spondylolysis have been reported, a mere 24 adult and pediatric patients with C-2 spondylolysis have been described. The long-term outcomes of very young children with bilateral C-2 spondylolysis are of great interest, yet only a few longitudinal studies exist. Methods The authors retrospectively reviewed 5 cases of bilateral C-2 spondylolysis at Texas Children's Hospital and Riley Children's Hospital; these were combined with 5 other cases in the literature, yielding a total of 10 patients. Data regarding the patients' age, sex, C2–3 angulation and displacement, associated spine anomalies, neurological deficits, treatment, and most recent follow-up were recorded. Results The patients' ages ranged from 3 to 36 months (mean 12.9 months). There were 6 boys and 4 girls. The C2–3 angulation, displacement, and width of pars defect were measured when available. The mean C2–3 angulation was 9.5° (range 1–34°), the mean C2–3 displacement was 4.78 mm (range 1.1–10.8 mm), and the mean width of the pars defect was 4.16 mm (range 0.9–7 mm). One patient developed myelopathy and spinal cord injury. All 10 of the patients were treated initially with conservative therapy: 3 with close observation alone, 1 with a rigid cervical collar, 4 with a Minerva jacket, 1 with a sternal-occipital-mandibular immobilizer, and 1 with a halo vest. Three patients ultimately underwent surgery for internal fixation due to progressive instability or development of neurological symptoms. All patients were neurologically intact at the last follow-up (mean 44.3 months, range 14–120 months). Conclusions Based on the literature and the authors' own experience, they conclude that most very young children with C-2 spondylolysis remain neurologically intact and maintain stability in long-term follow-up despite the bony defect. This defect is often an asymptomatic incidental finding and may be managed conservatively. More aggressive therapy including surgery is indicated for those patients with a neurological deficit from spinal cord compromise secondary to stenosis and local C-2 kyphosis, progressive deformity, or worsening C2–3 instability.

scholarly journals Long term treatment with ataluren—the Swedish experience

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Eva Michael ◽  
Kalliopi Sofou ◽  
Lisa Wahlgren ◽  
Anna-Karin Kroksmark ◽  
Már Tulinius
Keyword(s):  
Upper Limb ◽  
Premature Stop Codon ◽  
Children's Hospital ◽  
Case Series Study ◽  
Long Term Treatment ◽  
Ambulatory Patients ◽  
Children’S Hospital ◽  
Over Time

Abstract Introduction Ataluren is a relatively new treatment for male patients with Duchenne muscular dystrophy (DMD) due to a premature stop codon. Long-term longitudinal data as well as efficacy data on non-ambulant patients are still lacking. Here we present the results from a long-term follow-up study of all DMD patients treated with ataluren and followed at the Queen Silvia Children’s Hospital in Gothenburg, Sweden, with focus on the evolution of patients’ upper motor and respiratory function over time. Methods This is a retrospective longitudinal case-series study of all male DMD patients treated with ataluren and followed at the Queen Silvia Children’s Hospital in Gothenburg, Sweden, since 2008. Results Our eleven patients had a median exposure to ataluren of 2312 days which is almost a fourfold higher than previous studies. Loss of ambulation occurred at a median age of 13.2 years. Patients who lost ambulation prior to 13.2 years of age had received ataluren for 5 years, whereas patients who continued to be ambulatory after 13.2 years of age had received ataluren for 6.5 years until loss of ambulation or last follow-up if still ambulatory. Four of six non ambulatory patients had Performance of the Upper Limb scores above the expected mean values over time. All but one patient maintained a pulmonary decline above the expected over time. All ambulatory patients increased in their predicted forced vital capacity (FVC) with 2.8 to 8.2% annually. Following loss of ambulation, 5 of 6 patients declined in predicted FVC (%), with annual rate of decline varying from 1.8 to 21.1%. The treatment was safe and well tolerated throughout the follow-up period. Conclusions This is the first study to present long-term cumulative treatment outcomes over a median period of 6.3 years on ataluren treatment. Our results indicate a delay in loss of ambulation, as well as a slower decline in FVC and upper limb motor function even after loss of ambulation. We suggest that treatment with ataluren should be initiated as soon as the diagnosis is confirmed, closely monitored and, in case of sustainable benefit, continued even after loss of ambulation.

scholarly journals Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up

2014 ◽  
Vol 13 (3) ◽  
pp. 260-272 ◽  
Author(s):  
Arnold H. Menezes ◽  
Raheel Ahmed
Keyword(s):  
Fibrous Dysplasia ◽  
Needle Biopsy ◽  
Bone Tumors ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Eosinophilic Granuloma ◽  
Bone Cysts ◽  
Aneurysmal Bone Cysts

Object Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up. Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010. Results Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2–18 years). Chordomas received proton or LINAC irradiation, and as of 4–15 years of follow-up, no recurrence has been noted. Conclusions Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

scholarly journals Surgical perspective of aneurysmal bone cyst of the rib

2011 ◽  
Vol 1 (1) ◽  
pp. 8
Author(s):  
Magdi Ibrahim ◽  
Abdulla Allam
Keyword(s):  
Aneurysmal Bone Cyst ◽  
Bone Cyst ◽  
Synthetic Mesh ◽  
Long Term Results ◽  
Wide Resection ◽  
Bone Cysts ◽  
Perioperative Mortality ◽  
Aneurysmal Bone Cysts

A retrospective study of aneurysmal bone cyst (ABCs) of the rib was conducted to review their clinical, radiological and pathological features, as well as the early and long-term results of surgical management. From 2000 through 2010, 5 patients (3 male, 2 female, aged 17-40 years) with ABCs of the rib were treated in our department. All patients with aneurysmal bone cyst (ABCs) of the rib diagnosed radiologically and confirmed histologically. Surgical treatment consisted of wide resection in all patients with the use of synthetic mesh in two cases. There was no perioperative mortality. Follow-up at 1-10 years revealed no local recurrence. All aneurysmal bone cysts (ABCs) of the rib should be treated by wide resection with tumor-free margins in order to provide the best chance for cure.

scholarly journals P18 An audit on the delivery of Bristol Royal Children's Hospital paediatric orthopaedic services in response to BOAST COVID-19 guidance

BJS Open ◽  
2021 ◽  
Vol 5 (Supplement_1) ◽  
Author(s):  
Matthew Singh Benning ◽  
James Berwin ◽  
Thomas Knapper ◽  
Sebastien Crosswell ◽  
Charlotte Carpenter ◽  
...  
Keyword(s):  
Tertiary Referral Centre ◽  
Fracture Clinic ◽  
Children's Hospital ◽  
Asymptomatic Children ◽  
Retrospective Audit ◽  
Children’S Hospital ◽  
Paediatric Orthopaedic ◽  
Spread Of Infection

Abstract Introduction The COVID-19 pandemic raised concerns regarding the spread of infection by asymptomatic children. Guidance from the British Orthopaedic Association Standards for Trauma (BOAST) for the ‘management of patients with urgent orthopaedic conditions and trauma during the coronavirus pandemic’, helped structure our service in response to the pandemic. We assessed our compliance with ‘BOAST COVID-19 standards’ pertaining to children to determine whether it is possible to run a safe and effective paediatric orthopaedic service. Methods Between the 16th March and 30th April 2020, we performed a prospective audit of clinic and theatre data from the paediatric orthopaedic department at the Bristol Royal Children’s Hospital against the ‘BOAST COVID-19 standards’. We also performed a retrospective audit between 16th March and 30th April 2019 for comparison. Results Patients booked into acute fracture clinic (AFC) and fracture clinic follow-up (FFO) reduced by 40% and 48% respectively from 2019 to 2020. A virtual fracture clinic (VFC) was implemented with increasing trend in VFC consultations. From 2019 to 2020, the number of patient initiated follow-up appointments increased in AFC and FFO from 16% to 75% and 12% to 35% respectively. Radiography was reduced; only 17% and 39% of AFC and FFO patients respectively required radiographs. On-call referrals and trauma cases reduced by almost 50% with a similar case mix year-on-year. All elective operating was cancelled in 2020. Conclusion By reducing clinic admissions and theatre throughput, it was possible to run an effective paediatric orthopaedic service in a busy tertiary referral centre. Our aim now is to determine the long-term efficacy.

scholarly journals Impact of distance on postoperative follow-up in patients of pediatric surgery: a retrospective review

2020 ◽  
Vol 3 (4) ◽  
pp. e000195
Author(s):  
Meagan E Wiebe ◽  
Anna C Shawyer
Keyword(s):  
Postoperative Complications ◽  
Pediatric Surgery ◽  
Descriptive Statistics ◽  
Attendance Rates ◽  
Urban Center ◽  
Children's Hospital ◽  
Median Distance ◽  
Equivalent Rate ◽  
Children’S Hospital

ObjectiveCentralization of medical services in Canada has resulted in patients travelling long distances for healthcare, which may compromise their health. We hypothesized that children living farther from a children’s hospital were offered and attended fewer follow-up appointments.MethodsWe reviewed children less than 17 years of age referred to the general surgery clinic at a tertiary children’s hospital during a 2-year period who underwent surgery. Descriptive statistics were performed.ResultsWe identified 723 patients. The majority were male (61%) with a median age of 7 years (range 18 days to16 years) and were from the major urban center (MUC) (56.3%). The median distance travelled to hospital for MUC patients was 8.9 km (range 0.9–22 km) vs 119.5 km (range 20.3–1950 km) for non-MUC patients. MUC children were offered more follow-up appointments (72.7% vs 60.8%, p<0.05). No significant differences existed in follow-up attendance rates (MUC 88.5% vs non-MUC 89.1%, p=0.84) or postoperative complications (9.8% vs 9.2%, p=0.78). There were no deaths.ConclusionsPatients living farther from a hospital were offered fewer follow-up appointments, but attended an equivalent rate of follow-ups when offered one. Telemedicine and remote follow-up are underused approaches that can permit follow-up appointments while reducing associated travel time and expenses.

scholarly journals Measles Exposure Investigation in a Children’s Hospital Emergency Department— Denver Metropolitan Area, Colorado, 2019

2020 ◽  
Vol 41 (S1) ◽  
pp. s18-s19
Author(s):  
Ashley Richter
Keyword(s):  
Emergency Department ◽  
International Travel ◽  
Childhood Vaccination ◽  
Prevention Measures ◽  
Mmr Vaccine ◽  
Children's Hospital ◽  
Control Intervention ◽  
Children’S Hospital ◽  
Lost To Follow Up

Background: On December 14, 3 unvaccinated siblings with recent international travel presented to Children’s Hospital Colorado emergency department (CHCO-ED) with fever, rash, conjunctivitis, coryza, and cough. Measles was immediately suspected; respiratory masks were placed on the patients before they entered an airborne isolation room, and public health officials (PH) were promptly notified. Notably, on December 12, 1 ill sibling presented to CHCO-ED with fever only. We conducted an investigation to confirm measles, to determine susceptibility of potentially exposed ED contacts and healthcare workers (HCWs), and to implement infection prevention measures to prevent secondary cases. Methods: Measles was confirmed using polymerase chain reaction testing. Through medical record review and CHCO-ED unit-leader interviews, we identified patients and HCWs in overlapping ED areas with the first sibling, until 2 hours after discharge. Measles susceptibility was assessed through interviews with adults accompanying pediatric patients and HCW immunity record reviews. Potentially exposed persons were classified as immune (≥1 documented measles-mumps-rubella (MMR) vaccination or serologic evidence of immunity), unconfirmed immune (self-reported MMR or childhood vaccination without documentation), or susceptible (no MMR vaccine history or age <12 months). Susceptibility status directed disease control intervention, and contact follow-up was 21 days. Results: On December 14, all 3 siblings (ages 8–11 years) had laboratory-confirmed measles and were hospitalized. CHCO’s rapid isolation of the 3 cases within 5 minutes after presentation to the ED eliminated the need for exposure assessment on the day of hospitalization. However on December 12, the 1 ill sibling potentially exposed 258 ED contacts (90 patients, 168 accompanying adults) and 22 HCWs. The PH department identified 158 immune contacts (61%), 75 unconfirmed immune contacts (29%), and 19 susceptible contacts (8%); 6 contacts (2%) were lost to follow-up. Overall, 15 susceptible contacts received immune globulin (IG) postexposure prophylaxis and 4 contacts were placed on 21-day quarantine. Unconfirmed immune contacts self-monitored for measles symptoms and were contacted weekly by PH for 21 days. Moreover, 20 immune HCWs monitored symptoms daily; 2 susceptible HCWs were placed on 21-day quarantine. No secondary cases were identified. Conclusions: Rapid measles identification and isolation, high levels (90%) of immunity among contacts, prompt administration of IG, and effective collaboration between PH and CHCO prevented transmission.Funding: NoneDisclosures: None

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