Two-Center Review of Posterior Vault Expansion following a Staged or Expectant Treatment of Crouzon and Apert Craniosynostosis

Background: The timing of posterior cranial expansion for the management of intracranial pressure can be "staged" by age and dysmorphology or "expectant" by pressure monitoring. The authors report shared outcome measures from one center performing posterior vault remodeling (PCVR) or distraction (PVDO) following a staged approach and another performing spring-assisted expansion (SAPVE) following an expectant protocol. Methods: Apert or Crouzon syndrome patients who underwent posterior expansion younger than 2 years were included. Perioperative outcomes and subsequent cranial operations were recorded up to last follow-up and intracranial volume changes measured and adjusted using growth curves. Results: Thirty-eight patients were included. Following the expectant protocol, Apert patients underwent SAPVE at a younger age (8 months) than Crouzon patients (16 months). The initial surgery time was shorter but total operative time, including device removal, was longer for PVDO (3 hours 52 minutes) and SAPVE (4 hours 34 minutes) than for PCVR (3 hours 24 minutes). Growth-adjusted volume increase was significant and comparable. Fourteen percent of PCVR, 33% of PVDO, and 11% of SAPVE cases had complications, but without long-term deficits. Following the staged approach, 5% underwent only PVDO, 85% had a staged posterior followed by anterior surgery, and 10% required a third expansion. Following the expectant approach, 42% of patients had only posterior expansion at last follow-up, 32% had a secondary cranial surgery, and 26% had a third cranial expansion. Conclusion: Two approaches involving posterior vault expansion in young syndromic patients using three techniques resulted in comparable early volume expansion and complication profiles. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.</p

Photographic protocol for image acquisition in craniofacial microsomia

Craniofacial microsomia (CFM) is a congenital condition associated with orbital, mandibular, ear, nerve, and soft tissue anomalies. We present a standardized, two-dimensional, digital photographic protocol designed to capture the common craniofacial features associated with CFM

Genome-wide association study identifies multiple susceptibility loci for craniofacial microsomia

Craniofacial microsomia (CFM) is a rare congenital anomaly that involves immature derivatives from the first and second pharyngeal arches. The genetic pathogenesis of CFM is still unclear. Here we interrogate 0.9 million genetic variants in 939 CFM cases and 2,012 controls from China. After genotyping of an additional 443 cases and 1,669 controls, we identify 8 significantly associated loci with the most significant SNP rs13089920 (logistic regression P 1�4 2.15 � 10 � 120) and 5 suggestive loci. The above 13 associated loci, harboured by candidates of ROBO1, GATA3, GBX2, FGF3, NRP2, EDNRB, SHROOM3, SEMA7A, PLCD3, KLF12 and EPAS1, are found to be enriched for genes involved in neural crest cell (NCC) development and vasculogenesis. We then perform whole-genome sequencing on 21 samples from the case cohort, and identify several novel loss-of-function mutations within the associated loci. Our results provide new insights into genetic background of craniofacial microsomia

Incident at the Rock Pile

Poetr

Incident at the Rock Pile

Poetr

Kafka und die Weltliteratur

Tagungsbericht zum internationalen Symposion an der Universität des Saarlandes, Saarbrücken, vom 20. bis 23. September 2004 Die Veranstalter des Saarbrücker Symposions 'Kafka und die Weltliteratur', Manfred Engel (Saarbrücken) und Dieter Lamping (Mainz), wußten, daß sie mit ihrer Tagung die vielfältigen Differenzen innerhalb der Kafka-Forschung nicht würden ausräumen können. Wohl aber hofften sie, die schmale Konsensbasis der Kafka-Forschung durch einen neuen Zugangsweg zu vergrößern: Statt den Autor, wie schon so oft, als (bewunderten) Einzelgänger innerhalb der klassischen literarischen Moderne zu betrachten und alle Anstrengungen auf eine Deutung der Einzeltexte zu konzentrieren, ging es in Saarbrücken erstmals darum, Kafkas Dichtungen in komparatistischer Hinsicht zu kontextualisieren

Franz Innerhofer als Erzähler : eine Studie zu seiner Poetik ; mit einer Forschungsübersicht und einer Werkbibliographie

Vollst. zugl.: Bamberg, Univ., Magisterarbeit, 199