scholarly journals Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome

2022 ◽  
Vol 52 (1) ◽  
pp. E16

OBJECTIVE In this study, the authors aimed to 1) retrospectively analyze the early functional outcomes in a cohort of very young children with craniofacial dysostoses who underwent robot-assisted frontofacial advancement (RAFFA) or robot-assisted midface distraction (RAMD), and 2) analyze the utility of robotic assistance in improving the accuracy and safety of performing transfacial pin insertion for RAFFA or RAMD. METHODS A retrospective analysis of a cohort of 18 children (age range 1–42 months at presentation), who underwent RAFFA or RAMD from February 2015 to February 2021 in the craniofacial unit at Amrita Institute of Medical Sciences and Research Centre in Kochi, India, was performed. Inclusion criteria were patients who had undergone RAFFA in a single stage or RAMD where the cranial vault had been addressed earlier, had been addressed on follow-up, or had not been addressed and had follow-up of at least 6 months. RESULTS Overall, 18 children with syndromic craniosynostosis underwent LeFort level III midface distraction, with or without RAFFA, from February 2015 to February 2021 at a single center in India. The patients’ ages ranged from 6 to 47 months at the time of the procedure. All patients had significant obstructive sleep apnea (OSA), significant ocular issues, and disturbed sleep as determined by the authors’ preoperative protocol. Clinically significant intracranial pressure issues were present in 17 patients. None of the patients had injury due to the transfacial pin trajectory such as globe injury, damage to the tooth buds, or the loss of purchase during the active distraction phase. The mean distraction achieved was 23 mm (range 18–30 mm) (n = 16/18). Of the 18 patients, 10 (56%) had an excellent outcome and 6 (33%) had a satisfactory outcome. In all cases, the degree of OSA had significantly reduced after surgery. Eye closure improved in all patients, and complete closure was seen in 11 patients. On follow-up, the functional gain remained in 14 of 16 patients at the final follow-up visit. The distraction results were stable during the follow-up period (mean 36 months [range 6–72 months]). CONCLUSIONS The early RAFFA and RAMD protocols investigated in this study gave a significant functional advantage in very young patients with craniofacial dysostoses. The results have demonstrated the accuracy and safety of robotic assistance in performing transfacial pin insertion for RAFFA or RAMD.

2013 ◽  
Vol 106 (8-9) ◽  
pp. 463
Author(s):  
M. Veyrier ◽  
C. Ducreux ◽  
R. Henaine ◽  
A. Bozio ◽  
F. Sassolas ◽  
...  

1998 ◽  
Vol 82 (3_suppl) ◽  
pp. 1107-1113 ◽  
Author(s):  
Bonnie C. Nicholson ◽  
Patricia C. Janz ◽  
Robert A. Fox

The effectiveness of a brief parental-education program for 40 families with very young children was studied. Families were assigned to either a parental-education or waiting-list control group. The parental-education program included information and strategies drawn from developmental and cognitive psychology and social learning theory. Analysis showed that participating parents significantly reduced their use of corporal and verbal punishment, changed their parenting attitudes, and improved their perceptions of their children's behavior in comparison to the control group. Effects were maintained at six weeks follow-up. Results supported tailoring parental-education programs to the unique needs of participants.


2014 ◽  
Vol 13 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Loyola V. Gressot ◽  
Sudhakar Vadivelu ◽  
Steven W. Hwang ◽  
Daniel H. Fulkerson ◽  
Thomas G. Luerssen ◽  
...  

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.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2472-2472
Author(s):  
Asim F. Belgaumi ◽  
Amani A. Al-Kofide ◽  
Nicey Joseph ◽  
Yasser Khafaga ◽  
Rubina J. Malik ◽  
...  

Abstract Hodgkin Lymphoma (HL) is rarely seen in <5-year olds in developed nations. Even in developing countries, where a tendency towards younger age of presentation has been shown, this represents a minority of cases. Little is known about the biology and behavior of these very young patients with HL as compared to older children. Methods: We retrospectively reviewed HL cases diagnosed and treated at our institution between 1975 and 2003. HL was diagnosed histopathologically and staged clinically. The pediatric age group ranged from 0–14 years. Treatment strategy for these very young children was focused on the elimination of radiation therapy (XRT). Results: 69/368 (18.75%) patients were less than 5 years at diagnosis. When compared to older patients, there was a trend towards male predominance (M:F 4.31 v. 2.65; p=0.2), but no difference in the incidence of B-symptoms (26.1% v. 32.9%; p=1.0) and stage distribution (p=1.0). There was less mediastinal involvement (p=0.025) or bulky disease (p=0.01) in the younger patients. These patients had more mixed cellularity and less nodular sclerosis subtype (p=0.025). Fifty-five were treated with chemotherapy (CTX) alone, 12 with combined modality therapy (CMT) and 2 with XRT only. 35/55 CTX patients were treated with ABVD (20 per standard schedule, 13 modified and 2 unknown), 12 MOPP and 8 with hybrid or combination CTX (4 MOPP/ABVD, 3 COPP/ABVD and 1 unspecified). All CMT patients received ABVD (9 standard and 3 modified) and XRT (1500cGy/IF for 5, 1500cGy/EF for 4 and 2400cGy/EF, 2720cGy/IF and 3060cGy/IF for one each). The two XRT alone patients had stage I cervical disease and received 3900cGy and 3250cGy IFXRT. At ten years the EFS and OS for these patients under 5-years of age was 81.5% and 90.4%, respectively, compared to 75.5% and 90.5% for the children between 5 and 14 years of age (p>0.5 for both comparisons). OS (86.4% v. 100%; p=0.3, Log Rank test) and EFS (81.0% v. 90.9%; p=0.4, Log Rank test) for CTX v. CMT groups were not statistically significantly different. The CTX group had more B-symptoms (29.1% v. 16.1%) and higher stage disease (stage III/IV 47.3% v. 25%; stage IV 12.73% v. 0%). Conclusions: HL patients <5years old do not present with higher risk disease than older children. They can successfully be treated without XRT using CTX alone. XRT can be reserved for treating the few who relapse. This may result in reduction in XRT related toxicity, which can be significant in these very young children.


1994 ◽  
Vol 17 (1) ◽  
pp. 125-141 ◽  
Author(s):  
I. Ercan Alp

Development of working memory in the transitional period from infancy to preschool years was investigated from a neo-Piagetian perspective. A new task, the Imitation Sorting Task, was specifically designed for this purpose. The task involves a game of imitation. An increasing number of disparate objects are sorted into two containers and the child is asked to reproduce each demonstrated sorting. The number of objects in the largest set that the child can successfully sort in imitation determines the child's score on the task. The task was administered three times to children from 12 to 36 months of age. Scores increased in a linear fashion with age in all three administrations. Upon retesting within a few weeks after the original administration, children's score and rank remained very similar. Their score increased at the follow-up after 6 months, but their rank still remained similar to their original rank. The age-related increase in the scores appears to be about one unit every six months in this age range.


PEDIATRICS ◽  
2004 ◽  
Vol 114 (6) ◽  
pp. 1601-1605 ◽  
Author(s):  
S. A. Weinzimer ◽  
J. H. Ahern ◽  
E. A. Doyle ◽  
M. R. Vincent ◽  
J. Dziura ◽  
...  

2021 ◽  
Vol 15 (4) ◽  
pp. 322-330
Author(s):  
Lizz van der Heijden ◽  
Germán L. Farfalli ◽  
Inês Balacó ◽  
Cristina Alves ◽  
Marta Salom ◽  
...  

Purpose The main challenge in reconstruction after malignant bone tumour resection in young children remains how and when growth-plates can be preserved and which options remain if impossible. Methods We describe different strategies to assure best possible long-term function for young children undergoing resection of malignant bone tumours. Results Different resources are available to treat children with malignant bones tumours: a) preoperative planning simulates scenarios for tumour resection and limb reconstruction, facilitating decision-making for surgical and reconstructive techniques in individual patients; b) allograft reconstruction offers bone-stock preservation for future needs. Most allografts are intact at long-term follow-up, but limb-length inequalities and corrective/revision surgery are common in young patients; c) free vascularized fibula can be used as stand-alone reconstruction, vascularized augmentation of structural allograft or devitalized autograft. Longitudinal growth and joint remodelling potential can be preserved, if transferred with vascularized proximal physis; d) epiphysiolysis before resection with continuous physeal distraction provides safe resection margins and maintains growth-plate and epiphysis; e) 3D printing may facilitate joint salvage by reconstruction with patient-specific instruments. Very short stems can be created for fixation in (epi-)metaphysis, preserving native joints; f) growing endoprosthesis can provide for remaining growth after resection of epi-metaphyseal tumours. At ten-year follow-up, limb survival was 89%, but multiple surgeries are often required; g) rotationplasty and amputation should be considered if limb salvage is impossible and/or would result in decreased function and quality of life. Conclusion Several biological and technological reconstruction options must be merged and used to yield best outcomes when treating young children with malignant bone tumours. Level of Evidence Level V Expert opinion


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