A Photo Score for Aesthetic Outcome in Sagittal Synostosis:An ERN CRANIO Collaboration

European Reference Network (ERN) CRANIO is focused on optimizing care for patients with rare or complex craniofacial anomalies, including craniosynostosis and/or rare ear, nose, and throat disorders. The main goal of ERN CRANIO is to collect uniform data on treatment outcomes for multicenter comparison. We aimed to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of sagittal synostosis and aesthetic outcome of treatment. We conducted a retrospective study among nonsyndromic sagittal synostosis patients aged &lt;19 years. We included preoperative and postoperative photo sets from 6 ERN CRANIO centers. Photo sets included bird's eye, lateral, and anterior-posterior views. The sagittal synostosis photo score was discussed in the working group, and consensus was obtained on its contents. Interrater agreement was assessed with weighted Fleiss' Kappa and intraclass correlation coefficients.The photo score consisted of frontal bossing, elongated skull, biparietal narrowness, temporal hollowing, vertex line depression, occipital bullet, and overall phenotype. Each item was scored as normal, mild, moderate, or severe. Results from 36 scaphocephaly patients scored by 20 raters showed kappa values ranging from 0.38 [95% bootstrap CI: 0.31, 0.45] for biparietal narrowness to 0.56 [95% bootstrap CI: 0.47, 0.64] for frontal bossing. Agreement was highest for the sum score of individual items [intraclass correlation coefficients agreement 0.69 [95% CI: 0.57, 0.82]. This is the first large-scale multicenter study in which experts investigated a photo score to assess the severity of sagittal synostosis phenotypical characteristics. Agreement on phenotypical characteristics was suboptimal (fair-moderate agreement) and highest for the summed score of individual photo score items (substantial agreement), indicating that although experts interpret phenotypical characteristics differently, there is consensus on overall phenotypical severity.</p

A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis:a proposal for a Standard Operating Procedure

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications.METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures.RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation.CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.</p

A population-specific material model for sagittal craniosynostosis to predict surgical shape outcomes

Sagittal craniosynostosis consists of premature fusion (ossification) of the sagittal suture during infancy, resulting in head deformity and brain growth restriction. Spring-assisted cranioplasty (SAC) entails skull incisions to free the fused suture and insertion of two springs (metallic distractors) to promote cranial reshaping. Although safe and effective, SAC outcomes remain uncertain. We aimed hereby to obtain and validate a skull material model for SAC outcome prediction. Computed tomography data relative to 18 patients were processed to simulate surgical cuts and spring location. A rescaling model for age matching was created using retrospective data and validated. Design of experiments was used to assess the effect of different material property parameters on the model output. Subsequent material optimization—using retrospective clinical spring measurements—was performed for nine patients. A population-derived material model was obtained and applied to the whole population. Results showed that bone Young’s modulus and relaxation modulus had the largest effect on the model predictions: the use of the population-derived material model had a negligible effect on improving the prediction of on-table opening while significantly improved the prediction of spring kinematics at follow-up. The model was validated using on-table 3D scans for nine patients: the predicted head shape approximated within 2 mm the 3D scan model in 80% of the surface points, in 8 out of 9 patients. The accuracy and reliability of the developed computational model of SAC were increased using population data: this tool is now ready for prospective clinical application

Preliminary evaluation of long-term outcomes after stereotactic radiosurgery for cerebral arteriovenous malformations – when is the right time for re-irradiation?

AIM The purpose of the study is to evaluate the results of stereotactic radiosurgery for cerebral arteriovenous malformations (AVMs) in patients followed-up for at least two years. MATERIAL AND METHODS From a group of 91 patients irradiated with single-fraction radiosurgery, a subset of 58 patients followed-up for at least two years was selected. The median follow-up was 5.7 years. There were 8 Spetzler-Martin grade I lesions, 26 grade II, 20 grade III, and 4 grade IV. In 27 (42.6%) embolization was performed before radiosurgery. In 31 (53.5%) at least one intracranial hemorrhage was diagnosed before the treatment, in 18 (31%) the presenting symptom was epilepsy, in 38 (66.6%) headaches, in 14 (24%) paresis, in 9 (15.5%) visual deficits and in 5 (8.6%) speech disturbances. The patients were irradiated with single doses of 5–20 Gy (mean and median dose 16 and 17.5 Gy, respectively). The treatment was performed with a linear accelerator equipped with a micromultileaf collimator. RESULTS The actuarial one-year obliteration rate was 11%, two-year – 38%, three-year 47%, four-year – 67%, and five-year – 72%. The median time to obliteration was 3.17 years. In five patients obliteration occurred at least four years after the irradiation. Two patients from this group were qualified for repeat radiosurgery, both of them after 7 years from the initial treatment. In both cases the AVMs finally occluded. CONCLUSIONS Long-term follow-up is required in patients after stereotactic radiosurgery for cerebral AVMs because obliteration can occur after the postulated 2–3 year latency period. Repeat radiosurgery is an effective method of treatment but an optimal time for re-irradiation is not clearly defined.CEL Celem pracy jest ocena wyników leczenia metodą radiochirurgii stereotaktycznej malformacji tętniczo-żylnych u chorych obserwowanych co najmniej przez dwa lata. MATERIAŁ I METODA Z grupy 91 chorych napromienianych stereotaktycznie pojedynczą dawką promieniowania wyselekcjonowano 58, u których okres obserwacji wynosił co najmniej 24 miesiące. Mediana czasu obserwacji wynosiła 5,7 roku. W badanej grupie było 8 chorych, u których rozpoznano AVM I stopnia w skali Spetzlera-Martina, 26 – II stopnia, 20 – III stopnia i 4 – IV stopnia. U 27 (42,6%) przeprowadzono wcześniej zabieg embolizacji, u 31 (53,5%) przed leczeniem wystąpiło co najmniej jedno krwawienie, u 18 (31%) obserwowano napady padaczkowe, u 38 (66,6%) bóle głowy, u 14 (24%) niedowłady, u 9 (15,5%) zaburzenia widzenia, u 5 (8,6%) zaburzenia mowy. Chorych napromieniano dawkami 5–20 Gy (średnia i mediana odpowiednio 16 i 17,5 Gy) za pomocą przyspieszacza liniowego wyposażonego w mikrokolima- tor wielolistkowy. WYNIKI Aktualizowany roczny odsetek obliteracji wynosił 11%, dwuletni - 38%, trzyletni - 47%, czteroletni - 67% i pięcioletni - 72%. Mediana czasu do obliteracji wynosiła 3,17 roku. U 5 chorych do obliteracji doszło w okresie powyżej 4 lat po leczeniu. U 2 chorych powtórnie przeprowadzono radiochirurgię stereotaktyczną, u obu po 7 latach od pierwotnego leczenia, w obu przypadkach uzyskując obliterację. WNIOSKI 1. Chorzy po radiochirurgii stereotaktycznej wymagają długiego czasu obserwacji, gdyż do obliteracji może dojść w późniejszym niż postulowany 2–3-letnim okresie latencji. 2. Powtórna radiochirurgia stereotaktyczna jest leczeniem skutecznym, optymalny czas powtórnego zabiegu nie jest jednak dobrze zdefiniowany

Opracowanie modelu protezy nerwu obwodowego z polimeru naturalnego

Presented are the results of investigations into the preparation of a peripheral nerve prosthesis. The prosthesis is built up of a multichannel core having a diameter of 2 to 5 mm. The core prepared by freeze drying is housed in a polymeric sleeve. The prosthesis core is made of microcrystalline chitosan (MCCh) while the sleeve is prepared from poly(DL-lactide-coglycolide) copolymer. The usefulness of the prepared biomaterial was assessed by in vivo testing on animals.W pracy przedstawiono badania prowadzące do opracowania modelu protezy nerwu obwodowego. Proteza zbudowana jest z wielokanałowego rdzenia o średnicy od 2 do 5 mm wytworzonego metodą liofilizacji i osadzonego w tulei polimerowej. Rdzeń protezy wytworzono z mikrokrystalicznego chitozanu (MKCh), natomiast tuleję stanowi błona uzyskana z resorbowalnego kopolimeru poli(DL-laktyd-ko-glikolid). Opracowany biomateriał poddano ocenie przydatności w warunkach in vivo z wykorzystaniem zwierząt

Polymer hollow fiber-encapsulated peripheral nerve extracts change their activity towards injured hippocampal neurites in rats

The regeneration of the adult mammalian central nervous system (CNS) requires changes of the nonpromising environment. Applying peripheral nerve grafts and their extracts are both the useful method to induce regeneration of injured CNS neurites. Our previous reports showed that degeneration of peripheral nerves enhanced their neurotrophic activity in a time-dependent manner. Electrophoretical analysis of proteins obtained from degenerating sciatic nerves revealed significant changes in fractions of low molecular mass.The aim of the present work was to examine the influence of fractionated extracts from 7-day-predegenerated and non-predegenerated peripheral nerves upon injured hippocampal neurites in adult rats. The extracts were closed in fibrin-filled connective tissue chambers (CTC) or within CTC-wrapped polymer hollow fibers (PHF) of 30 kDa cut-off. The cell bodies of regrowing fibers were labeled with FITC-HRP.The CTCs appeared to be useful tool for implantation of artificial grafts into mammalian CNS. Full-spectrum nerve extracts induced strong regeneration of injured hippocampal neurites. The number of labeled cells within hippocampus was significantly lower in PHF groups than in CTC ones, indicating that low-mass proteins present in peripheral nerve extracts are not sufficient to induce successful regeneration

Dead-ended autologous connective tissue chambers in peripheral nerve repair - early observations

The effects of the repair of nerve gap injuries are still unsatisfactory, despite the great progress in microsurgery. Until now, there is no effective method to induce the regeneration of the transected peripheral nerve when its distal stump is missing. The aim of this work was to examine whether the implantation of dead-ended connective tissue chambers can promote the outgrowth of injured peripheral neurites. This method differs from all previous nerve guides because it totally eliminates the distal part of the nerve and restricts the influence of surrounding tissues. We have also tried to establish whether some neurotrophic factors can be applied by means of these chambers. The results of this work show that dead-ended autologous connective tissue chambers can be a useful tool in peripheral nerve injuries treatment, even when the distal part of the nerve is missing