Two-year clinical and economic burden, risk and outcomes following application of software-assisted hexapod ring fixation systems

Background Multiplanar external fixation systems that employ software-assisted deformity correction consist of rings connected by angled struts, defined as hexapod ring fixators (HRF). Costs and outcomes associated with the application of HRFs are not well documented. This study was designed to provide a nationwide baseline understanding of the clinical presentation, risks, outcomes and payer costs, and healthcare resource utilization (HCU) of patients requiring application of an HRF, from the day of, and up to 2 years, post-application. Methods Patients with HRF application (“index”) between 2007 and 2019 within the IBM Marketscan® Commercial Claims database were identified and categorized based on diagnosis: acquired deformity, arthropathy, congenital deformity, deep infection, nonunion, fracture, and other post-operative fracture sequelae. Demographics, comorbidities at index, complications post-index, HCU, and payments were analyzed. Payments were estimated using a generalized linear model and were adjusted for inflation to the 2020 consumer price index. Rates of deep infection and amputation were estimated up to 2 years post-index using Poisson regressions, and risk factors for each were estimated using logistic regression models. Results Six hundred ninety-five patients were included in our study (including 219 fractures, 168 congenital deformities, 68 deep infections, 103 acquired deformities). Comorbidities at index were significantly different across groups: less than 2% pediatrics vs 18% adults had 3 or more comorbidities, < 1% pediatric vs 29% adults had diabetes. Index payments ranged from $39,250–$75,350, with 12-months post-index payments ranging from $14,350 to $43,108. The duration of the HRF application ranged from 96 days to 174 days. Amputation was observed in patients with deep infection (8.9, 95% confidence interval (CI): 3.2–23.9%), nonunion (5.0, 95%CI: 1.6–15.4%) or fracture (2.7, 95%CI: 0.9–7.6%) at index. Complicated diabetes was the main predictor for deep infection (odds ratio (OR): 5.14, 95%CI: 2.50–10.54) and amputation (OR: 5.26, 95%CI: 1.79–15.51). Conclusions Findings from this longitudinal analysis demonstrate the significant heterogeneity in patients treated with HRF, and the wide range in treatment intensity, payments, and outcomes. Risks for deep infection and amputation were primarily linked to the presence of complicated diabetes at the time of HRF application, suggesting a need for careful management of comorbid chronic conditions in patients requiring HRF for orthopedic care.

Intraoperative cardiac function assessment by transesophageal echocardiography versus FloTrac/Vigileo™ system during pectus excavatum surgical repair

Background Pectus excavatum (PE), a congenital deformity of the chest wall, can lead to cardiac compression and related symptoms. PE surgical repair can improve cardiac function. Intraoperative transesophageal echocardiography (TEE) has been successfully employed to assess intraoperative hemodynamic variations in patients undergoing PE repair. FloTrac/Vigileo™ system (Edwards Life-sciences Irvine, CA) (FT/V) is a minimally invasive cardiac output monitoring system. This retrospective study aimed to assess hemodynamic changes in surgical repair of PE using FT/V and concordance with parameters measured by TEE. Results N=19 patients submitted to PE repair via Ravitch or Nuss technique were enrolled. Intraoperative cardiac assessments simultaneously obtained via TEE and FT/V system were investigated. The agreement between TEE-derived cardiac output (CO-TEE) and FT/V system parameter (COAP) was evaluated. The relationship between COTEE and COAP was analyzed for all data using linear regression analysis. A significant correlation between COAP and COTEE values (R = 0.65, p < 0.001) was found. Bland-Altman analysis of COAP and COTEE showed a bias of 0.13 L/min and a limit of agreement of − 2.33 to 2.58 L/min, with a percentage error of 48%. Intraoperative measurements by TEE and FT/V both showed a significant increase in CO after surgical correction of PE (p < 0.005). Conclusions FT/V system compared to TEE in hemodynamic monitoring during PE surgery yielded clinically unacceptable results due to a high percentage error. After surgical correction of PE, CO, measured by TEE and FT/V, significantly improved.

The use of intraoperative neurophysiological monitoring in dorsal resection of hemivertebrae

BACKGROUND: Congenital disorders of vertebrae formation are a common pathology in children. Intraoperative neurophysiological monitoring is a mandatory procedure, although it may not be effective enough due to the immature neural structures and the use of inhalation anesthetics in young children. AIM: To study aims to investigate the characteristic features of intraoperative neurophysiological monitoring in children with a congenital deformity of the spine during dorsal resection of the hemivertebrae. MATERIALS AND METHODS: 42 patients aged 117 years with a congenital deformity of the spine underwent 46 resections of the abnormal vertebra from an isolated dorsal approach (egg-shell technique). Intraoperative neurophysiological monitoring at the stages of the operation included a muscle relaxant test (TOF), transcranial electrical stimulation of the motor cortex (TCeMEP), control of the approach to the nerve (N. Proxy), correct placement of the pedicle screw (Screw Integrity), and EMG recording of the electromyogram. The accuracy of the screw placement was assessed by the Gerzbien method, and the presence of neurological disorders was tested by the Frenkel scale. The effect of inhalation anesthetic (sevoran) on motor evoked potentials was monitored by regulating its delivery, and the dependence on the age of patients was evaluated. RESULTS: The average age of patients was 7.7 4.5 years, and the TOF value was 80.5 17%. In 41 patients, the N. Proxy test was unremarkable, while in one patient, the 812 mA value did not require a change in the trajectory of the screws. From the beginning of sevoran and intraoperatively, motor evoked potentials from all tested muscles were recorded in 54.8% of patients; in children over 8 years old, this was observed in 92.8%, in children under 8 years old in 35.7% of cases in their age groups. In other patients, motor evoked potentials were most often not recorded from the muscles of the thigh and lower leg after sevoran administration. In children over 8 years old in 7.2%, under 8 years old in 83.3% of patients; Interestingly, in 7.2% of patients who are under 8 years of age, motor evoked potentials were not initially recorded from any muscle. Withdrawal of sevorane in 30.9% of patients allowed intraoperative motor evoked potentials to be obtained from all tested muscles in 100% of cases. For adequate management of anesthesia, 5 patients (50%) 14 years old and one patient 6 years old (5.6%) did not receive sevoran, and motor evoked potentials were recorded from the abdominal muscles. This allowed to assess the conduction only at the thoracic level and are required increased vigilance of surgeons when carrying out any corrective manipulations. CONCLUSIONS: Intraoperative neurophysiological monitoring with dorsal hemivertebra resection is an effective method that allows controlling the neurological complications during manipulations on the spine.

A case of congenital deformity

The fact that a low fluid content in the fetal bladder, or oligohydramnia, is usually accompanied by deformities, is confirmed by the following interesting case.

Use of torso orthoses in the treatment of congenital spinal deformities: A literature review

BACKGROUND: Congenital malformations of the spine require urgent treatment, despite the popularity of this problem. The choice of the term and method of surgical treatment of congenital spinal deformities with vertebral malformations does not have diametrically opposed views in the domestic and foreign literature, but the scientific discussion continues regarding the use of orthotics. Currently, several designs of orthoses on the trunk are known worldwide, which can be used not only for idiopathic but also for congenital scoliosis. А unified view of the use of this type of complex treatment is necessary, but there are opposite opinions in the scientific literature. AIM: This study aimed to analyze publications that present the use of torso orthoses in the treatment of congenital malformations of the spine. MATERIALS AND METHODS: We searched for data in the open electronic databases of PubMed and e-LIBRARY using the following keywords and phrases: congenital deformity of the spine, congenital scoliosis, orthosis, and effectiveness of orthosis. The search covered 30 years of literature. RESULTS: Several authors have reported a significant correction of the congenital curves using orthotics, but this issue has not been sufficiently studied and is still at the research stage. Some specialists consider the formation or progression after the operation of compensatory counter-curve above or below the established metal structure, which can be corrected not only by repeated surgery, but also by using orthoses. CONCLUSIONS: There is a clear trend toward complex treatment of children with isolated congenital malformations of the spine, including a reasonable combination of surgical treatment and orthotics. In Europe and Russia, preference is given to the Rigo system Cheneau brace.

Comparative analysis of the quality of life of children with congenital scoliosis after surgical treatment: Extirpation of the hemivertebra from dorsal and combined access

BACKGROUND: Congenital scoliosis with disorders of the formation of the vertebrae is the most common cause of pronounced deformities of the spine in early childhood. This pathology can be treated surgically using various techniques that differ in invasiveness, severity of the condition in the postoperative period, achieved result of deformity correction, and nature of the long-term prognosis. Numerous studies have assessed the quality of life of patients who underwent surgery for acquired deformities, trauma, and degenerative and neoplastic diseases of the spine in adults. However, features of the childs quality of life following surgical technique for congenital scoliosis have not been sufficiently studied. AIM: This study aimed to compare the quality of life of children with congenital scoliosis of the thoracolumbar localization after extirpation of the hemivertebra from the dorsal and combined approaches. MATERIALS AND METHODS: An intergroup prospective analysis of the results of a survey of 60 patients with congenital deformity of the spine against the background of an isolated violation of the formation of the thoracic or lumbar vertebra was carried out. Patients underwent standard surgical treatment. Patients were divided into two groups according to the surgical approach to the abnormal vertebral body: dorsal group (n = 28) and combined group (n = 32). The average age of the patients was 75 (minmax, 26196) months. The follow-up period was 18 months after surgery. To assess the quality of life, a specialized Russian version of the PedsQL v4.0 questionnaire and a modified visual analog scale were used. RESULTS: After surgical treatment of congenital spinal deformity, quality of life indicators decreased more than two times than the results of a preoperative survey. At 18 months postoperatively, the physical activity and psychoemotional state were restored to the preoperative level, while patients of the combined group had a higher satisfaction score on the quality of life (p 0.05). CONCLUSIONS: The combined approach provides the best correction of congenital deformity of the spine and allows maintaining of the achieved result throughout the observation period. In the early postoperative period, the combined group demonstrated a significant decrease in the level of satisfaction with the quality of life, while the pain syndrome was higher than that in the dorsal group. Dynamic observation revealed the leveling of these differences and a subsequent increase in the level of satisfaction with the quality of life of these patients.

The Prevalence and Morphology of Supernumerary Teeth in Children With Nonsyndromic Cleft Lip and Palate

Introduction: Cleft lip with or without cleft palate (CL/P) is congenital deformity associated with hyperdontia. Objective: To determine the prevalence and characteristics of supernumerary teeth in patients with CL/P. Design: Retrospective descriptive and correlation clinical study. Patients: One hundred thirteen children with cleft (age ranged 9.3-19.2; 67 males and 46 females) treated in Clinic of Congenital Facial Deformities Medical University of Lublin were included in the study. Methods: Records evaluation was conducted regarding age, gender, cleft type (Q36, Q37— International Classification of Diseases 10th revision), cleft side, and incidence of supernumeraries. In all supernumerary teeth, size, shape, and developmental degree were analyzed and correlation between the incidence of hyperdontia with different variables was checked. Correlations were detected using chi-square and the Yates correction. Results: The majority of the examined group were males—59.29% with Q37 (67.26%) and the cleft on the left side (62.83%). Hyperdontia was noted in 26.55%. Only upper lateral incisors were affected. They usually had atypical shape (56.67%), reduced size (83.33%), and delayed development (56.67%). Conclusions: The prevalence of supernumerary permanent teeth in patients with cleft was higher than in the general population. Anomaly was more frequent in male patients and occurred mainly on the cleft side. The severity of the cleft did not influenced the frequency of supernumerary teeth, their shape, size, and developmental degree. Supernumerary teeth were characterized by reduced crown size, abnormal structure, incorrect inclination, and delayed development phase.

Ophthalmic manifestations of proboscis lateralis

Proboscis lateralis (PL) is a rare congenital malformation of the craniofacial structure with varied clinical associations. None of the studies documented a discrete review of ophthalmic presentations in PL. The principal aim of the present study is to explore the ophthalmic manifestations of PL. The ancillary goal is to derive a relationship between congenital deformity in PL and various ophthalmic anomalies. Databases were searched in order to obtain articles related to PL. A qualitative systematic analysis of 100 subjects was performed. In PL, eyelid coloboma (32.6%) is the most common ocular feature, followed by hypertelorism (25.3%), iris coloboma (22.4%), lacrimal system abnormality (20.7%), malpositioned eyebrow (14.4%) and retinochoroidal coloboma (12.9%). Sinonasal deformity is the most common systemic abnormality, detected in 87.9% of cases of PL, as compared with central nervous system involvement (56.2%) and other anomalies. The analysis showed a strong significant association between brain abnormalities and hypertelorism (p=0.000) and between brain abnormalities and micro-ophthalmia/anophthalmia (p=0.000). Statistically significant association was noted between cumulative ocular abnormalities and cumulative systemic abnormalities (p=0.001). The present study on PL reviewed the salient features of this rare congenital disorder. The study outcome provides a new aspect to concomitant ocular abnormalities. This study supports the view that other congenital anomalies in cases of PL had significant influence on certain ophthalmic anomalies.

Successful Early Non-Operative Management of Congenital Knee Dislocation: Demonstration via a Case Report

Congenital knee dislocation (CKD) is a deformity in which unnatural hyperextension is possible at the knee much beyond the normal 0° full extension while the flexion is severely restricted. Incidence of this rare congenital malformation is 1/100,000 live births. We present a clinical case of unilateral CKD in a 2-week-old female child and our approach of treatment using serial casting. We have been able to demonstrate that if initiated early, within first few weeks of life, the congenital deformity of knee can be managed conservatively without any surgical intervention even in higher grades.

Comprehensive management of Crouzon syndrome: A case report with three-year follow-up

Crouzon syndrome is one of the most common craniosynostosis facial syndromes caused by a mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. Less commonly, there is a mutation of the FGFR3 gene which results in Crouzon syndrome syndrome with acanthosis nigricans. It involves the premature fusion of sutures of the cranial vault, base, orbital and maxillary region. The clinical presentation of this congenital deformity depends on the pattern and timing of sutural fusion. The present report describes the features and management of this syndrome in an 18-year-old woman. The patient presented with a hypoplastic maxilla, deficient midface, exorbitism due to shallow orbits, severe crowding and bilateral crossbite. A multidisciplinary approach involving orthodontics and surgical intervention with distraction osteogenesis brought about marked improvement in the facial profile, occlusion and upper airway. The aesthetics and function were greatly enhanced, and the results were found to be stable at the end of three years.