Multi-directional Cranial Distraction Osteogenesis for Treating Sagittal Synostosis with Frontometaphyseal Dysplasia: A Case Report.

Frontometaphyseal dysplasia (FMD), also known as Gorlin-Cohen syndrome, is a rare genetic syndrome. This syndrome affects the skeletal system and connective tissue, and causes a wide spectrum of manifestations of the skull, tubular bones, cardiovascular system, urinary system, and/or gastrointestinal system. Craniofacial findings of FMD are characterized by protruding supraorbital ridge, broad nasal bridge, hypertelorism, down-slanting palpebral fissures, and/or micrognathia. We describe a case of a 2-year-old girl diagnosed with sagittal synostosis accompanied with FMD. She presents anterior sagittal synostosis cranial form, compressed cerebrospinal fluid space (which suggested increased intracranial pressure), and the supraorbital hyperostosis. She underwent multi-directional cranial distraction osteogenesis in the calvaria and shaving of the supraorbital ridges. Despite concerns about bone fragility associated with FMD, the surgery was accomplished as usual. The patient had no intra- and postoperative complications. After 6 months of follow-up, the cranial shape has improved and the cerebrospinal fluid space has widened, but the supraorbital ridge has protruded again. Re-protrusion of the supraorbital ridge appears to be due to age-appropriate vigorous osteogenesis. The multi-directional cranial distraction osteogenesis procedure has been useful for treating sagittal synostosis even concomitant with FMD.

Nodular Pseudogout of the Skull Base Arising From the Temporomandibular Joint.

ABSTRACT: The authors report a case of a rare, large, nodular pseudogout which developed from the temporomandibular joint (TMJ).An 83-year-old female was referred to the hospital with swelling on her left cheek. Imaging studies revealed a large calcified mass surrounding the left mandibular condyle, partially destroying the adjoining bone and extending to the skull base. Magnetic resonance imaging (MRI) revealed soft tissue masses with low signal intensity on T1- and T2-weighted images and were enhanced after intravenous gadolinium injection.The mass was clinically and radiologically suspected to be a neoplastic lesion, such as chondrosarcoma or fibrous dysplasia. However, histological analysis showed that the mass contained granulomatous lesions with numerous multiple nodular rod-shaped and diamond-shaped crystal deposits, which validated the diagnosis of pseudogout. X-ray diffraction (XRD) was performed to identify the tumor's deposited materials and revealed that these were calcium pyrophosphate dihydrate (CPPD) crystals. This result confirmed the histopathological diagnosis of "tophaceous pseudogout."

Evaluation of algal photosynthesis inhibition activity for dissolved organic matter with the consideration of inorganic and coloring constituents.

The effect of waterborne ingredient on ecosystem has been of great interest. In the present study, the evaluation method using algal photosynthesis inhibition assay with dual-channel pulse amplitude modulation (PAM) system was established for a series of water samples to elucidate the potential effect of the total body of organic compounds including natural organic matter (NOM) on aquatic ecosystems. The more sensitive and less time-consuming monitoring method compared with algal growth inhibition assay was suggested, especially considering inorganic and coloring constituents. Algal photosynthesis inhibition activity was detected with high sensitivity for photosystem II (PSII) inhibitors, whereas the IC10 of the other chemicals was over the environmental standard concentration for Chlamydomonas moewusii (Chlorophyceae) and Pheodactylum tricornutum (Diatomea). The photosynthesis inhibition activity of Lake Biwa dissolved organic matter (LBDOM) and fulvic acid (LBFA) was significantly detected at ≥10 times the concentration and >10 mgC L-1, respectively, whereas prominent activity was confirmed for Suwannee River NOM (SRNOM) on the river original concentration (>30 mgC L-1) for both algae. Significant inhibition activity was detected in both algae at least in twice-concentration for water samples from a wastewater treatment pilot plant. There was no great difference in the activity between sewage secondary effluent and its filtrate with ultrafiltration (UF), and physically washing water for the UF membrane.