Type IIIB Research Articles

The Nightmare of Calcaneus: Gustilo-Anderson Type IIIB Fractures.

The aim of this study was to share the clinical results of open calcaneus fractures, which are extremely rare and have become the fearful nightmare of orthopedic surgeons. Between June of 2014 and June of 2020, of 196 patients with the diagnosis of calcaneus fracture, Gustilo-Anderson type IIIB, 17 patients (11 men and six women; mean age, 36.2 ± 4.6 years; range, 22-56 years) under prospective follow-up were retrospectively screened and included in the study. Patients were recorded in terms of classification of fracture, mechanism of injury, comorbidities, surgical progress, complications, and associated orthopedic and nonorthopedic injuries. Functional clinical results and quality of life of the patients were evaluated at the 6-month follow-up with the American Orthopedic Foot and Ankle Society score and the 36-Item Short Form Health Survey questionnaire. The most common etiologic factor was work-related injuries, with 29.4%. Although triple arthrodesis surgery was performed in three cases because of pseudoarthrosis, amputation was performed in one case because of deep infection. The most common soft-tissue defect was located at the medial aspect of the foot, which was commonly reconstructed with the used of reverse flow island sural flap. The average total American Orthopedic Foot and Ankle Society hindfoot score was 51.6 (range, 48-74) and similarly, physical component, pain, and social parameters on the 36-Item Short Form Health Survey were statistically significant compared to Turkish population normal scores. A long and laborious process is required for both the patient and the surgeon in the treatment of open calcaneal fractures. However, it is possible to obtain good results with soft-tissue reconstructions.

Molecular basis for inhibition of type III-B CRISPR-Cas by an archaeal viral anti-CRISPR protein

Despite a wide presence of type III clustered regularly interspaced short palindromic repeats, CRISPR-associated (CRISPR-Cas) in archaea and bacteria, very few anti-CRISPR (Acr) proteins inhibiting type III immunity have been identified, and even less is known about their inhibition mechanism. Here, we present the discovery of a type III CRISPR-Cas inhibitor, AcrIIIB2, encoded by Sulfolobus virus S.islandicus rod-shaped virus 3 (SIRV3). AcrIIIB2 inhibits type III-B CRISPR-Cas immune response to protospacers encoded in middle/late-expressed viral genes. Investigation of the interactions between S.islandicus type III-B CRISPR-Cas Cmr-α-related proteins and AcrIIIB2 reveals that the Acr does not bind to Csx1 but rather interacts with the Cmr-α effector complex. Furthermore, invitro assays demonstrate that AcrIIIB2 can block the dissociation of cleaved target RNA from the Cmr-α complex, thereby inhibiting the Cmr-α turnover, thus preventing host cellular dormancy and further viral genome degradation by the type III-B CRISPR-Cas immunity.

Low intensity extracorporeal shockwave therapy for chronic pelvic pain syndrome: Long-term follow-up.

Chronic prostatitis (CP) is one of the diseases that reduce the quality of life (QoL) of young men. To date, there is no consensus on the management of these patients. It is essential to continue research into the treatment of CP, despite the use of various therapies, including low-energy extracorporeal shockwave therapy (ESWT). The main objective of this study is to observe and record the clinical symptomatology of patients during a 48-week follow-up period after ESWT treatment. Between 2019 and 2021, 28 patients with type IIIB CP/chronic pelvic pain syndrome were enrolled. Patients underwent ESWT once weekly for 4 weeks (3,000 individual sessions, maximum total energy flux density 0.25 mJ/mm2, frequency 3 Hz). Participants were assessed at 0, 4, 12, 24, 36 and 48 weeks post-treatment using the visual analogue scale (VAS), National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI), and International Index of Erectile Function (IIEF)-5. The mean age of patients was 47.1 ± 13.7 years (range 28-4 years). The positive effect of LI-ESWT was reflected in improvements in VAS, NIH-CPSI, and IIEF-5 scores. Regression of patients' symptoms was observed as early as 4 weeks after treatment. The greatest progress was achieved at week 24. In addition, a slight worsening was observed at week 36 and 48, with stable progress. The treatment significantly improved the QoL of the patients, with the most significant improvement in the VAS score. In conclusion, this treatment approach is safe, most effective in the first 6 months. Thereafter, the efficacy of the treatment diminishes, but is sustained over a longer period.

The Value of Percutaneous Contrast-Enhanced Ultrasound in Sentinel Lymph Node Identification, Metastatic Status and Burden Diagnosis in Early Breast Cancer.

The aim of this study was to evaluate the value of percutaneous contrast-enhanced ultrasound (PCEUS) in the identification and characterization of sentinel lymph node (SLN). A total of 102 breast cancer patients were collected and underwent preoperative PCEUS, which was used to identify SLN and lymphatic drainage. SLNs were classified into 4 enhancement patterns, including 6 subtypes: homogeneous (I), featured inhomogeneous (II) including inhomogeneous hypoenhancement (IIa) and annular or semi-annular enhancement (IIb), focal filling defect (III) including filling defect area < 50% (IIIa) and filling defect area ≥ 50% (IIIb), and no enhancement (IV). The enhancement patterns of SLNs were compared with the final pathological diagnosis. The identification rate of SLNs using PCEUS was 100% (102/102); the rate of identification of LCs was 100% (102/102), and the coincidence rate was 98.0% (100/102). Four lymphatic drainage patterns (LDPs) including 5 subtypes were found: single LC/single SLN(74.5%), multiple LCs/ single SLN (13.7%) including 2 subtypes:2 LCs/1 SLN and 3 LCs/1 SLN, single LC/multiple SLNs (7.8%), and multiple LCs/multiple SLNs (3.9%). A total of 86.3% (44/51) of patients without axillary metastasis could be safely selected for types I, IIa, and IIb, while the axillary metastasis rates of types III and IV were 74.4%and 87.5%, respectively (P < .001). Compared with grayscale US, the PCEUS significant improvement in diagnosing metastatic SLNs (.794 versus .579, P < .001). For the SLN metastatic burden, Types I, IIa, IIb, and IIIa had ≤2 SLNs metastases, with a pathological coincidence rate of (64/67, 95.5%), and types IIIb and IV had >2 SLNs metastases, with a pathological coincidence rate of (25/35, 71.4%) (P < .001). The AUC of PCEUS for the diagnosis of SLN metastatic status and burden was .794 and .879, respectively (P < .001). PCEUS has a high identification rate for SLN and has good potential for diagnosing SLN metastatic status and burden by enhancement patterns.

Revision total hip arthroplasty with custom-made hip implant for Paprosky type IV femoral bone loss

Introduction Replacement of extensive Paprosky type IIIB and type IV bone loss is a challenge in revision total hip arthroplasty (THA).The purpose was to demonstrate the possibility of femoral reconstruction in proximal femur bone loss using a custom-made implant for revision THA.Material and methods We report a case of a 72-year-old patient with an extensive Paprosky type IV femoral defect, which was replaced using a custom-made modular component.Results The femoral defect was successfully augmented with a custom-made modular component, and the hip function was restored. The locking mechanism of the constrained system failed at 6 months with the joint remained stable. The patient could ambulate with additional support. VAS, HHS and HOOS scores measured before and after 2 years showed positive dynamics.Discussion Paprosky type IIIB and type IV defects are a challenge for revision hip arthroplasty. There is a variety of surgical options with outcomes being ambiguous. Modular and monoblock tapered stems, the technique of impacted bone graft have been reported to have excellent results in revision THA with Paprosky type III and IV defects. A custom-made femoral component was developed based on the principle of modular stems. Joint stability is a concomitant problem with a severe bone defect that can be addressed with a double mobility or constrained system. Both methods are associated with a sufficient number of complications.Conclusion Replacement of a Paprosky type IV femoral defect with a custom-made modular component demonstrated satisfactory outcomes at a two‑year follow-up. The patient had no complaints, could ambulate unassisted using an elbow crutch and positively evaluated the result of treatment.

Antiviral type III CRISPR signalling via conjugation of ATP and SAM

CRISPR systems are widespread in the prokaryotic world, providing adaptive immunity against mobile genetic elements1,2. Type III CRISPR systems, with the signature gene cas10, use CRISPR RNA to detect non-self RNA, activating the enzymatic Cas10 subunit to defend the cell against mobile genetic elements either directly, via the integral histidine–aspartate (HD) nuclease domain3–5 or indirectly, via synthesis of cyclic oligoadenylate second messengers to activate diverse ancillary effectors6–9. A subset of type III CRISPR systems encode an uncharacterized CorA-family membrane protein and an associated NrN family phosphodiesterase that are predicted to function in antiviral defence. Here we demonstrate that the CorA-associated type III-B (Cmr) CRISPR system from Bacteroides fragilis provides immunity against mobile genetic elements when expressed in Escherichia coli. However, B. fragilis Cmr does not synthesize cyclic oligoadenylate species on activation, instead generating S-adenosyl methionine (SAM)-AMP (SAM is also known as AdoMet) by conjugating ATP to SAM via a phosphodiester bond. Once synthesized, SAM-AMP binds to the CorA effector, presumably leading to cell dormancy or death by disruption of the membrane integrity. SAM-AMP is degraded by CRISPR-associated phosphodiesterases or a SAM-AMP lyase, potentially providing an ‘off switch’ analogous to cyclic oligoadenylate-specific ring nucleases10. SAM-AMP thus represents a new class of second messenger for antiviral signalling, which may function in different roles in diverse cellular contexts.

An archaeal virus-encoded anti-CRISPR protein inhibits type III-B immunity by inhibiting Cas RNP complex turnover.

CRISPR-Cas systems are widespread in prokaryotes and provide adaptive immune against viral infection. Viruses encode a type of proteins called anti-CRISPR to evade the immunity. Here, we identify an archaeal virus-encoded anti-CRISPR protein, AcrIIIB2, that inhibits Type III-B immunity. We find that AcrIIIB2 inhibits Type III-B CRISPR-Cas immunity in vivo regardless of viral early or middle-/late-expressed genes to be targeted. We also demonstrate that AcrIIIB2 interacts with Cmr4α subunit, forming a complex with target RNA and Cmr-α ribonucleoprotein complex (RNP). Furtherly, we discover that AcrIIIB2 inhibits the RNase activity, ssDNase activity and cOA synthesis activity of Cmr-α RNP in vitro under a higher target RNA-to-Cmr-α RNP ratio and has no effect on Cmr-α activities at the target RNA-to-Cmr-α RNP ratio of 1. Our results suggest that once the target RNA is cleaved by Cmr-α RNP, AcrIIIB2 probably inhibits the disassociation of cleaved target RNA, therefore blocking the access of other target RNA substrates. Together, our findings highlight the multiple functions of a novel anti-CRISPR protein on inhibition of the most complicated CRISPR-Cas system targeting the genes involved in the whole life cycle of viruses.

Congenital laryngo-tracheo-esophageal clefts: updates from a quaternary care pediatric airway unit

PurposeTo review the operative techniques, outcomes, and complications following surgery in pediatric patients with laryngo-tracheo-esophageal clefts (LTEC). We describe a new combined approach to treat long LTECs.MethodsTwenty-five patients underwent surgical repair for LTEC from March 2012 to July 2022 at our hospital. Every patient underwent a diagnostic endoscopy under general anesthesia and spontaneous ventilation to assess the LTEC and synchronous aero-digestive comorbidities/malformations. All patients underwent at least one surveillance endoscopy after the repair at our institution.ResultsThe patients had multiple other malformations, specifically gastro-intestinal, synchronous airway, and cardiac. The cleft distribution according to the modified Benjamin and Inglis classification was type I (n = 5, 20%), type II (n = 6, 24%), type IIIa (n = 8, 32%), type IIIb (n = 4, 16%), and type IVa (n = 2, 8%). The median follow-up was 44.6 months. Five patients (20%) had undergone previous cleft corrective surgery(s). Seven patients (28%) had partial to complete breakdown of the repair, needing additional intervention(s), and two required a combined—open plus endoscopic repair. Preoperatively, most patients (n = 18, 72%) needed a feeding assistance. At latest follow-up, feeding assistance was weaned off in 13 out of 18 patients, which was a 72% improvement. Ten patients (40%) needed ventilation assistance before the surgery. Post-operatively, ventilatory assistance was weaned off in 6 patients, meaning a 60% improvement.ConclusionLTEC are rare malformations, and their management needs precise diagnosis, appropriate surgical planning, and execution, and dedicated post-operative care. Primary and revision repair of long clefts with tracheal extension may require a combined approach.

Application value of high-frequency ultrasound combined with ultrasonography in the diagnosis of neonatal esophageal atresia.

To explore the application value of high-frequency ultrasound combined with ultrasonography in the diagnosis of neonatal esophageal atresia (EA). Seventy neonates with suspected EA who received healing in our hospital from August 2019 to April 2022 were retrospectively selected as the study subjects and their preoperative esophageal high-frequency ultrasound and ultrasound hydrography data were analysed. The diagnostic value of high-frequency ultrasound, ultrasound hydrography and combined detection in neonatal EA was analysed using intraoperative findings as the gold standard. (1) Among the 70 children with suspected EA, 62 were confirmed to be positive and 8 were negative; 59 were positive and 11 were negative by ultrasound hydrography alone; 61 were positive and 9 were negative by high-frequency ultrasound alone; 62 were positive and 8 were negative by combined detection.(2) The accuracy of combined detection was 97.14%, which was notably different from 92.86% by high-frequency ultrasound and 84.29% by ultrasound hydrography (P < 0.05).(3) The diagnostic AUC of ultrasound hydrography, high-frequency ultrasound, and combined detection for EA was 0.6125, 0.6500, and 0.6563, respectively (P < 0.05).(4) There was no significant variation in the distance between preoperative high-frequency ultrasound, ultrasound hydrography, and intraoperative measurements of distal and proximal blind ends of type IIIA and IIIB EA esophagus (P > 0.05). High frequency ultrasound and super fresh water injection angiography have good application value in the diagnosis of neonatal EA. There is no significant difference between the distance between the distal and proximal blind ends of the esophagus before and during the operation of type III EA and that during the operation of super fresh water injection angiography. However, in consideration of the risk of radiation and poisoning caused by esophagography, it is recommended that high-frequency ultrasound be selected first for diagnosis and if necessary, esophagography be supplemented for joint diagnosis.

Complications of transvaginal radiofrequency ablation of fibroids: A 5-year experience

IntroductionTransvaginal radiofrequency ablation is a relatively noninvasive approach for the treatment of fibroids in patients who do not wish to undergo conventional surgery. Information on potential complications of this novel technique is very scarce. MethodsRetrospective, descriptive, epidemiological study of 115 patients who underwent transvaginal radiofrequency ablation of fibroids and for whom complications were recorded. ResultsWe performed 115 transvaginal radiofrequency ablation procedures, we recorded a total of 11 complications (9.6%; 95% CI, 3.8–14.8). Of these, 8 (7.0%) were classified as Clavien-Dindo type I, 1 (0.9%,) as type II, and 2 (1.7%) as type IIIb (severe). No other complications were recorded in a year follow-up. ConclusionTransvaginal radiofrequency ablation is a treatment option that makes it possible to treat fibroids that are difficult to manage using other techniques. Few associated complications have been described, and most of them are mild.

Disease pathology signatures in a mouse model of Mucopolysaccharidosis type IIIB

Mucopolysaccharidosis type IIIB (MPS IIIB) is a rare and devastating childhood-onset lysosomal storage disease caused by complete loss of function of the lysosomal hydrolase α-N-acetylglucosaminidase. The lack of functional enzyme in MPS IIIB patients leads to the progressive accumulation of heparan sulfate throughout the body and triggers a cascade of neuroinflammatory and other biochemical processes ultimately resulting in severe mental impairment and early death in adolescence or young adulthood. The low prevalence and severity of the disease has necessitated the use of animal models to improve our knowledge of the pathophysiology and for the development of therapeutic treatments. In this study, we took a systematic approach to characterizing a classical mouse model of MPS IIIB. Using a series of histological, biochemical, proteomic and behavioral assays, we tested MPS IIIB mice at two stages: during the pre-symptomatic and early symptomatic phases of disease development, in order to validate previously described phenotypes, explore new mechanisms of disease pathology and uncover biomarkers for MPS IIIB. Along with previous findings, this study helps provide a deeper understanding of the pathology landscape of this rare disease with high unmet medical need and serves as an important resource to the scientific community.

Both Acetabular and Femoral Reconstructions With Impaction Bone Grafting in Revision Total Hip Arthroplasty: Case Series and Literature Review

BackgroundExtensive bone loss on femur and acetabulum posed a big challenge to orthopedists in total hip revision surgeries. Impaction bone grafting (IBG) as a valuable bone preservation technique could effectively address this problem. Either IBG revision on the femoral or acetabular side was well studied, while its use on both sides in one operation was not. The aim of this study is to present the outcomes of IBG on both femoral and acetabular sides at first-time hip revision. MethodsWe retrospectively reviewed 8 patients (mean follow-up of 5.8 years) undergoing first-time revision with IBG on both acetabular and femoral sides at our institution. The Paprosky classification system was used to classify bone defects. Freeze-dried allografts and cemented prostheses were used in all patients. Postoperative complications and rerevision rates were reported. ResultsFive patients presented a Paprosky type IIC acetabular defect, 3 with a type IIIB, IIIA, and IIC defect, respectively. Three patients presented with a type IV femoral defect, 3 with a type IIIB defect, and 2 with a type II defect. Two patients developed complications, while one had an intraoperative femoral fracture and one had delayed wound healing. At the latest follow-up, no patient had rerevisions or operations related to the prosthesis. ConclusionsIBG in combination with cemented prosthesis is a profitable biological reconstruction revision technique that could provide satisfying midterm outcomes. We first propose the use of blood clots mixed with bone grafts for potential bone incorporation enhancement, while its specific effects need to be verified in further studies.

S1998 Klatskin Tumor Type III-B Presenting in an Elderly Patient with Concerning Abdominal Imaging

S1998 Klatskin Tumor Type III-B Presenting in an Elderly Patient with Concerning Abdominal Imaging

A novel ODAPH mutation causing amelogenesis imperfecta and its expression in human dental tissues

Background/purposeAmelogenesis imperfecta (AI), an assemblage of genetic diseases with dental enamel malformations, is generally grouped into hypoplastic, hypomaturation, and hypocalcified types. This study aimed to identify the genetic etiology for a consanguineous Iranian family with autosomal recessive hypocalcified AI. Materials and methodsDental defects were characterized, and whole exome analysis conducted to search for disease-causing mutations. Minigene assay and RT-PCR were performed to evaluate molecular consequences of the identified mutation and expression of the causative gene in human dental tissues. ResultsThe defective enamel of erupted teeth showed extensive post-eruptive failure and discoloration. Partial enamel hypoplasia and indistinct dentino-enamel junction were evident on unerupted teeth, resembling hypocalcified AI. A novel homozygous ODAPH (previously designated C4orf26) mutation of single-nucleotide deletion (NG_032974.1:g.5103del, NM_178497.5:c.67+1del) was identified to be disease-causing. The mutation would cause a frameshift to different ODAPH transcript variant (TV) products: p.(Ala23Hisfs∗29) for TV1 and p.(Gly23Aspfs∗140) for TV2. Both dental pulps of developing and exfoliating primary teeth expressed ODAPH TV2. ConclusionLoss-of-function ODAPH mutations can cause AI type IIIB (the hypocalcified, autosomal recessive type), rather than type IIA4 (the hypomaturation, pigmented autosomal recessive type). This study supports a hypothesis that the product of ODAPH TV2 is the single dominant ODAPH protein isoform critical for dental enamel formation and may also play an unappreciated role in development and homeostasis of dentin-pulp complex. Due to genetic heterogeneity and a nonideal genotype-phenotype correlation of AI, it is essential to perform genetic testing for patients with inherited enamel defects to make a definitive diagnosis.

Clinical Phenotype of Pediatric and Adult Patients With Spinal Muscular Atrophy With Four SMN2 Copies: Are They Really All Stable?

The aim of this study was to provide an overview of the clinical phenotypes associated with 4 SMN2 copies. Clinical phenotypes were analyzed in all the patients with 4 SMN2 copies as part of a nationwide effort including all the Italian pediatric and adult reference centers for spinal muscular atrophy (SMA). The cohort includes 169 patients (102 men and 67 women) with confirmed 4 SMN2 copies (mean age at last follow-up = 36.9 ± 19 years). Six of the 169 patients were presymptomatic, 8 were classified as type II, 145 as type III (38 type IIIA and 107 type IIIB), and 8 as type IV. The remaining 2 patients were asymptomatic adults identified because of a familial case. The cross-sectional functional data showed a reduction of scores with increasing age. Over 35% of the type III and 25% of the type IV lost ambulation (mean age = 26.8 years ± 16.3 SD). The risk of loss of ambulation was significantly associated with SMA type (p < 0.0001), with patients with IIIB and IV less likely to lose ambulation compared to type IIIA. There was an overall gender effect with a smaller number of women and a lower risk for women to lose ambulation. This was significant in the adult (p = 0.009) but not in the pediatric cohort (p = 0.43). Our results expand the existing literature on natural history of 4 SMN2 copies confirming the variability of phenotypes in untreated patients, ranging from type II to type IV and an overall reduction of functional scores with increasing age. ANN NEUROL 2023;94:1126-1135.

Vegetation description around the savanna flux measurement site at Benfontein Nature Reserve, South Africa

A flux tower with eddy covariance instruments was recently erected at Benfontein Nature Reserve (BNR) outside of Kimberley. An understanding of the landscape within the flux tower footprint, in this case natural vegetation, is necessary to interpret the data collected by the eddy covariance instruments. The tower was erected as part of the Expanded Freshwater and Terrestrial Environmental Observation Network (EFTEON) infrastructure initiative to establish long-term monitoring platforms focused on socially relevant terrestrial landscapes and their coupled hydrological systems. We sought to describe the vegetation within the flux tower footprint to set a baseline of the vegetation in the landscape at the inception of the long-term monitoring of flux measurements at BNR. Woody vegetation was surveyed in five square 1 ha plots while the herbaceous layer was sampled across 105 circular plots following the Socio-Ecological Observatory for Southern African Woodland (SEOSAW) protocol. Woody vegetation was quantified in terms of abundance, diversity and biomass, together with Size Class Distribution (SCD) methods to describe the woody vegetation structure. The herbaceous layer was described by species count, basal cover, biomass, and diversity which was assessed using the Shannon-Weiner Diversity Index. Five tree species, dominated by Vachellia erioloba, were found in the plots. Recruits (0 and 1 m tall) were significantly more abundant than large trees (1.2–9.5 m high). The SCD for the woody vegetation yielded a Type IIIa curve i.e. populations missing one or more size classes, for all tree species except Ziziphus mucronata which yielded a Type IIIb curve representing a population missing small sized trees. The herbaceous layer was diverse, consisting of 10 grass and 32 forb species. Schmidtia pappophoroides was the dominant grass species in terms of count, basal cover and aboveground biomass. Herbaceous biomass was two times higher than that of woody vegetation, with the North plot contributing the highest biomass for herbaceous and woody vegetation. The results indicate that the vegetation around the flux tower represents a wooded grassland, which is a type of a savanna. These vegetation data will contribute to the interpretation of data collected by the flux tower instruments now and in the future. Furthermore, long-term data collection in this reserve is needed to capture the woody vegetation dynamics and its interaction with the herbaceous layer.

Editor's Choice -- Type IIIb Endoleaks: Fabric Perforations of Explanted New Generation Endoprostheses

To analyse explanted endografts (EGs) and describe fabric degradation responsible for type IIIb endoleaks. As part of the European collaborative retrieval programme, 32 EGs with fabric defects upon macroscopic evaluation were selected. The explanted EGs were processed and studied based on the ISO 9001 certified standard protocol. It includes instructions on the collection, transportation, cleaning, and examination of explanted material. The precise analysis was performed with a digital microscope of 20× - 200× magnification. Possible perforation mechanisms were assessed in stress tests. The median time to explantation of the 32 EGs was 54 months. The explants included 65 separate EG modules, with 46 (70.8%) having a combined 388 fabric perforations. Each EG had a median of 4.79 mm2 (interquartile range [IQR] 9.86 mm2) of cumulated hole area (an average of 0.13% of an EG's area). There were 241 (62.1%) expanded polytetrafluoroethylene (ePTFE; 12 EGs) and 147 (37.9%) polyethylene terephthalate (PET; 20 EGs) fabric ruptures, with no difference in hole distribution between these types of material. Overall, 126 (32.5%) stent related and 262 (67.5%) non-stent related fabric perforations were identified. Perforations caused by fabric fatigue in ePTFE (151, 38.9%) and material kinking in PET (41, 10.6%) were the most common. The stent related perforations were larger in size (0.80 mm2) than non-stent related (0.19 mm2); p = .004. Wider interstent spaces and prolonged implantation duration associated with an increased risk of stent related perforation development; p < .001, p = .004, respectively. Large stent related perforations were also detected in the short term, suggesting mechanical issues as underlying causes. The fabric of EGs may degrade and lead to the development of perforations. The largest perforations are stent related. Their occurrence and size depend on the implantation time and the EG shape affected by arterial tortuosity. The conclusions are limited to the samples from a select explant group.

Comparative Evaluation of Custom-Made Components and Standard Implants for Acetabular Reconstruction in Revision Total Hip Arthroplasty

Background.The use of custom-made acetabular components is one of the promising methods for reconstruction of the acetabulum in cases of significant defects, including those associated with pelvic bone dissociation. It allows achieving stable fixation and restoring the biomechanics of the hip joint.&#x0D; Aimofthestudy to compare the results of using individually designed components, supportive antiprotrusion rings, augments, and hemispherical components in revision total hip arthroplasty for type IIIB bone defects according to Paprosky classification.&#x0D; Methods.The study analyzed the treatment outcomes of 90 patients with type IIIB bone defects who underwent revision total hip arthroplasty between 2017 and 2022. Patients were divided into three groups: the first group received individually designed acetabular components, the second group received augments with hemispheres, and the third group had antiprotrusion cages implanted. The analysis included the reasons for revision surgery, operation duration, blood loss volume, and type of revision procedure. Pain and functional outcomes were assessed with WOMAC, Harris Hip Score, and VAS scales.&#x0D; Results.3D-printed constructs were more frequently implanted in patients with pelvic bone dissociation. The first group showed a significantly positive dynamic in functional outcomes. Complications were diagnosed in 27 (30%) cases: joint instability (dislocation) in 10 (11.1%) patients, periprosthetic infection in 8 (8.8%), aseptic loosening in 4 (4.4%), and sciatic nerve neuropathy in 5 (5.5%) patients. The number of these complications was higher in the second and third groups of patients.&#x0D; Conclusion.Custom-made implants using 3D technologies are a preferable option for revision total hip arthroplasty in patients with type IIIB defects according to Paprosky classification, especially in cases of pelvic bone dissociation.

P20.02.B ELECTRO-CLINICAL, NEUROIMAGING FEATURES AND NEUROPATHOLOGICAL CHARACTERIZATION OF POLYMORPHOUS LOW-GRADE NEUROEPITHELIAL TUMOR OF THE YOUNG (PLNTY)

Abstract BACKGROUND Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) has been recently recognized by 2021 WHO Central nervous system classification. It is an epileptogenic tumor type, affecting mostly children and young adults, with peculiar radiological, and pathomolecular features. PLNTY is a very rare tumor, described in only 52 cases up to now. Herein, we describe 13 patients with electro-clinical and neuroimaging findings, associated to histopathological and molecular findings. MATERIAL AND METHODS We selected PLNTY patients from our Cancer Registry (Apr 2017-Dec 2022), and collected electro-clinical, radiological and histo-molecular data. Minimum follow-up was 6 months. Exams included EEG, long-term scalp video-EEG, pre-surgical brain computer tomography and Magnetic Brain Imaging (MRI). Immunohistochemistry included CD34, IDH1-R132H, GFAP, synaptophysin, ATRX, p53 and FGFR3. IDH1-2, BRAF (V600E) sequencing, Kiaa1549:BRAF, FGFR3:TACC3, FGFR2:kii1598 and FGFR2:CTNNA3 fusion analyses will be performed. RESULTS We describe 13 patients, median age 20.6 years (range 12.3-46.4). Most patients showed memory/executive impairment and had epilepsy, with an onset from 5 months-21 years before surgery. The main feature of seizures was a difficult localization and even lateralization, both semiologically and electrographically. Post-surgical seizure control was excellent.The tumors (n=11/13 temporal) presented as solid or solid-cystic cortical mass with no mass effect and unclear hedges. Calcifications were present in 4, and cysts in 6 cases. Microscopically the tumors showed infiltrative growth pattern, oligodendroglial-like cells, strong and often diffuse CD34 immunostaining, and frequent intra-tumoral calcifications. Malformation of cortical development, namely FCD type IIIB, was associated to PLNTY in 2 cases. Proliferation activity (MIB-1 LI) was very low (1%) in all cases except one (MIB-1 LI which reached 3%). CONCLUSION This study significantly extends the number of reported PLNTY associated to epilepsy, mostly characterized by bilateral interictal epileptiform discharges and difficult to lateralize seizures.The MRI features were heterogeneous and the differential diagnosis should include also dysplasia and glioneuronal tumors. PLNTY, despite its name, occurs also in adults, and electro-clinical and radiological diagnosis can be challenging. As recently described, PLNTY can show the MAPK pathway activating alterations, that are under investigation in our cohort to eventually clarify their role in the evolution of the disease. SUPPORT the study is partially supported by the Health Italian Minister (RC).

Relationship between number of debridements and clinical outcomes in open tibia fractures requiring free flap coverage: A retrospective cohort study

IntroductionOpen fractures with degloving injuries are commonly managed by serial debridements prior to definitive flap coverage in the United States (US). Some international institutions minimize the number of debridements prior to coverage. The purpose of this study was to compare clinical outcomes in patients with open tibia fractures requiring free flap coverage from institutions with differing debridement philosophies. MethodsThis was a multi-site retrospective cohort study of patients treated at two US tertiary care facilities (serial debridement cohort) and one in India (early total debridement cohort) with Type IIIB or IIIC open tibia fractures requiring free tissue transfer. The number of debridements prior to flap coverage were recorded and primary outcomes were rates of infection, non-union, and flap failure. Fischer's exact tests were used to compare outcomes between the cohorts. Results80 patients were included, 44 from India and 36 from the US. Patients in the serial debridement cohort underwent more debridements prior to flap coverage (mean 3.64 vs 1.84, p < 0.001) and had significantly higher rates of infection and non-union compared to patients in the early total debridement cohort (p < 0.05). There were no differences in rates of flap failure between the cohorts. ConclusionsPatients with open tibia fractures treated at institutions favoring serial debridements underwent more debridements and had higher rates of infection and non-union compared to patients treated at a center favoring early total debridement. Serial debridements may not be necessary prior to flap coverage for open tibia fractures and the current practice should be further investigated.