Thermoreversible helical fibers from photoreactive triphenylene-derived liquid crystals in liquid paraffin.

Liquid crystalline triphenylene derivatives, TPC1p-n (n = 6, 12, 14, 16) were prepared using p-alkoxycinnamate as the [2+2] photo-cyclization site. TPC1p-n (n = 12, 14, 16) showed Colr phase and gave crescent-shaped or helical fibers after UV-irradiated in liquid paraffin solutions at 90 and 110 °C in the Colr temperature range. The apparent photoreaction products were shown to be thermally reversible, i.e. they dissolved in liquid paraffin at high temperatures and reappeared on cooling, indicating that they were aggregates of oligomerized TPC1p-n. The reaction mechanism was discussed in terms of the structure of the liquid crystalline phase.

Nifekalant unmasked residual gap of superior vena cava isolation by suppressing immediate recurrence of intra-superior vena cava fibrillation.

Nifekalant successfully suppressed intra-superior vena cava fibrillation, which complicated the evaluation of the gap of superior vena cava isolation.

Angioplasty of the Occluded Persistent Sciatic Artery Using the Retrograde Approach from Superficial Femoral Artery.

Persistent sciatic artery is a rare developmental anomaly prone to atherosclerotic disease. We present a case of successful endovascular therapy for left persistent sciatic artery that was occluded at the distal site. The angioplasty was performed with both antegrade approach from contralateral common femoral artery and retrograde approach from ipsilateral superficial femoral artery. The guidewire was advanced via collateral channel and crossed through the lesion retrogradely. Rendez-vous technique was performed, and the lesion was successfully dilated by balloon inflation. Angioplasty of occluded sciatic artery can be performed successfully and effectively using the collateral channel.

[An endoscopic transnasal transsphenoidal biopsy for pituitary gland Langerhans cell histiocytosis in a child: a case report].

Endoscopic transnasal transsphenoidal surgery is gaining wide acceptance as the first-line treatment of pituitary tumors in adults, as this procedure is minimally invasive and associated with good outcomes. On the other hand, conventional transcranial and sublabial approaches are chosen for infants as their small anatomical structures pose some difficulty. This study reports on the case of a 5-year-old boy who had a Langerhans cell histiocytosis appearing in the sella. The biopsy was performed using the endoscopic transnasal transsphenoidal approach, and the patient has not exhibited any post-operative complications. Although the patient was a fairly young child, the endoscopic transnasal transsphenoidal approach resulted in fewer complications as compared to the transcranial and translabial approaches. Otolaryngologists, therefore, should view this approach more positively. The indication for pediatric skull base diseases can be expected to expand to include endoscopic transnasal surgery.

[A case of a large neonatal pharyngeal teratoma].

The congenital teratoma is a rare malformation. We present herein on the case of a large teratoma that arose from the epipharynx in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography at another hospital. The mother was transferred to our hospital for delivery. We explained about the ex utero intrapartum treatment (EXIT) because of the risk of airway obstruction at birth, but EXIT was refused by the parents. Delivery was accomplished by cesarean section at the 35 th week. Since the newborn had a severe respiratory disorder due to a large intraoral tumor and intratracheal intubation was very difficult, a tracheostomy was done under local anesthesia for 7 min. The tumor was removed successfully on the 9th day and 44th day of age.

Anesthetic management during tracheotomy in a child with respiratory distress caused by severe intubation-induced glottic stenosis.

We provided anesthetic management during a tracheotomy procedure for a child who demonstrated labored respiration during inspiration because of severe glottic stenosis and bilateral vocal cord paralysis caused by tracheal intubation. A 4-year-old boy developed acute respiratory depression associated with influenza pneumonia and had been under respiratory management with mechanical ventilation with tracheal intubation for 3 days. Following extubation, an upper-airway obstruction immediately appeared. The symptoms later worsened because of development of a common cold, and the patient underwent an emergency tracheotomy. For anesthetic management, we used a combination of ketamine with low-concentration sevoflurane inhalation. The tracheotomy was performed safely without respiratory complications by employing manual-assisted ventilation, while spontaneous breathing was preserved by use of a face mask.

Plasmablastic extramedullary plasmacytoma associated with Epstein-Barr virus arising in an immunocompetent patient with multiple myeloma.

We encountered a case of plasmablastic extramedullary plasmacytoma with multiple myeloma. Histological findings revealed that the extramedullary plasmacytoma of this patient was of the plasmablastic type, which was positive for λ-stain and EBV-encoded RNA. In contrast, bone marrow aspiration demonstrated a common-type multiple myeloma, which was positive for λ-stain and negative for EBV-encoded RNA. This was a rare case of plasmablastic extramedullary plasmacytoma associated with Epstein-Barr virus arising in an immunocompetent patient with multiple myeloma.

Progressive leukoencephalopathy with intracranial calcification, congenital deafness, and developmental deterioration.

We report on a 12-year-old male with a unique cerebral white matter disease. His initial symptoms were congenital hearing loss and multiple intracranial calcifications on head CT. He developed severe intellectual disability and epilepsy. MRI showed signal abnormalities in the posterior limbs of the internal capsules, thalami, and cerebral white matter. The abnormalities were progressive over time. The neuropathology revealed diffuse and severe disruption of myelin and axons of the cerebral white matter and cerebrospinal tracts. We performed various metabolic examinations, detailed pathological investigations and genetic analyses, but could not identify the cause. To our knowledge his clinical course has not been described in the literature.

Tracheobronchial anomalies in syndromic craniosynostosis with 3-dimensional CT image and bronchoscopy.

Patients with syndromic craniosynostosis have many problems involving the upper airway, laryngotracheal airway, and tracheobronchial tree. Evaluation of tracheobronchial disorders in syndromic craniosynostosis is very important for accurately diagnosing these problems. We have used three-dimensional computed tomography (CT) imaging of the tracheobronchial tree (three-dimensional tracheal CT imaging) since a multidetector CT was installed in our hospital in 2004. In this study, we assessed the configuration of the tracheobronchial system using three-dimensional tracheal CT imaging and bronchoscopy.During the period from 2004 to 2009, 48 patients with syndromic craniosynostosis underwent surgery at our hospital. Of those, 16 patients required tracheostomy. These syndromic craniosynostosis cases were examined with three-dimensional tracheal CT imaging and bronchoscopy. All CT scans were performed on a multidetector CT (120 kV, 22-120 mA, 0.5 s/slice, 1-mm slice; Aquilion; Toshiba Co, Tokyo, Japan) without respiratory suppression.All 16 study patients presented with severe stenosis of the upper airway on bronchoscopic examination. Seven were confirmed to have abnormalities by both bronchoscopy and three-dimensional tracheal CT imaging. Four were confirmed to have abnormalities on either bronchoscopy or three-dimensional tracheal CT imaging, whereas 5 patients were apparently free of abnormalities as determined by both modalities.In conclusion, it was possible to accurately assess the configuration of the tracheobronchial system using both three-dimensional tracheal CT imaging and bronchoscopy. Our results suggest these examinations to be very useful for assessing the optimal timing of decannulation and respiratory function prognosis.

[A case report on cervical neuroblastoma with ataxia].

We present a case of pediatric primary cervical neuroblastoma (NB), which is extremely rare. A 3-year-old girl with ataxia but no nasal obstruction, dysphagia, or stridor was diagnosed with cervical NB. Diagnostic results including clinical chemistry, full blood count, and serology, were normal. Ataxia worsened within a few days after onset and was not cured by steroids or intravenous immunoglobulin, delaying a definite diagnosis until a tumor was detected. Opsoclonus-myoclonus syndrome is typified by opsoclonus with myoclonus and ataxia, primarily associated with neuroblastoma. Brain stem cell and cerebellum dysfunction is thought to be due to an autoimmune mechanism. Following chemotherapy and selective neck dissection, the girl has had no recurrence or adverse sequelae. Ataxia disappeared during chemotherapy. We suggest that neuroblastoma should be considered in any child with unexpected by prolonged ataxia.

Retrospective assessment of the dismasking flap procedure as a craniofacial approach.

OBJECT: In 1997 the authors presented the case of a 3-year-old girl who underwent complete resection of a huge tumor via a new technique using a dismasking flap. Since that report, 14 patients have undergone surgery using this technique. There have been few reports on long-term follow-up after a craniofacial surgical approach and reconstruction of a huge tissue defect. The authors report details of this procedure based on these cases, including long-term follow-up in the original case. METHODS: The dismasking flap is a cranioorbitofacial degloving method that uses a circumpalpebral approach with or without piriform margin incisions and presents a wide surgical field under direct vision. Fourteen patients have undergone surgery using the dismasking flap technique. These patients had multiple craniofacial bone fractures (3 cases) and malignant or premalignant craniofacial tumors (11 cases). Patients ranged in age from 3 to 62 years old. The longest follow-up period was 15 years. The results of these cases are reported regarding changes in the facial bones and soft tissues with growth, the various pathologies involved, and complications. RESULTS: Complications using this approach were lagophthalmos with ectropion of the lower eyelid, disturbance of the superior orbital nerve, disturbance of the inferior orbital nerve, maxillary hypoplasia, and blepharoptosis. In all affected patients these complications were almost always temporary. In 2 of the 14 cases, a repair operation for lagophthalmos was necessary, while others showed spontaneous improvement approximately 6 months to a year after the procedure. In the case with the longest follow-up duration, the patient's nose is asymmetrical, especially the alar portion, due to hypoplasia of the grafted bone and/or the fixation with titanium miniplates. CONCLUSIONS: This flap is very useful for en bloc resection of huge skull base tumors, multiple craniofacial bone fractures, and as a lateral approach to a deep portion of the middle cranial base. Careful attention is necessary, however, because one may encounter unexpected complications if one does not ensure adequate protection of the perioperative flap. Meticulous postoperative management is also essential.

[Tracheal cartilaginous sleeve in craniosynostosis].

Tracheal cartilaginous sleeve (TCS) is a congenital malformation involving fusion of the tracheal arches that may be isolated to a few tracheal arches, include the entire trachea, or extend beyond the carina into the bronchi. Tracheotomy was required in 9 of 23 craniosynostosis cases undergoing gradual distraction at Osaka City General Hospital from March 2002 to April 2006. TCS was diagnosed in 5 of 9 cases-four Pfeiffer patients and one Crouzon patient. Diagnosis was made intraoperatively during tracheotomy or at autopsy. 3D-CT was not useful in diagnosing TCS. Aggressive management of respiratory infection and pulmonary secretion, selection of appropriate tracheostomy tubes, and endoscopic evaluation are very important to care in managing TCS patients.

Seasonal expression of LHbeta and FSHbeta in the male newt pituitary gonadotrophs.

Seasonal changes in LHbeta and FSHbeta mRNA levels were examined in the pituitary gland of the adult male newt, Cynops pyrrhogaster, using in situ hybridization histochemistry and a quantitative real-time RT-PCR method. The annual fluctuation of LHbeta mRNA and FSHbeta mRNA levels in the pituitary gland displayed a close relationship with seasonal changes in testicular function. The values obtained by both methods showed similar fluctuation. The levels of LHbeta mRNA were always exceeded those of FSHbeta. The present immunoelectron microscopic observations support the data on the gene expression levels of the beta-subunits of LH and FSH. Gonadectomy in the summer increased the LHbeta and FSHbeta mRNA levels. Testosterone replacement inhibited the expression of LHbeta mRNA, but not of FSHbeta mRNA, suggesting that the expression of FSHbeta is regulated by some non-steroid factor, probably inhibin. In the case of gonadectomy during any other season, the LHbeta mRNA level increased, but not to the same extent as in summer, and androgen concentrations decreased to the minimum of the year. This finding provides new information about the regulation of annual changes in LHbeta and FSHbeta expression in the pituitary gonadotrophs.

[A device for tracheostomized children in home care].

Tracheomized children with deglutition disturbance require frequent suction--a procedure that may present problems for parents and other home-caregivers. We introduced a suction pump to drain the thoracic cavity continuously. This reduced the number of times of suction was required and raised no problems.

[A case of 9p-deletion syndrome with congenital median nasal fistula].

We report a case of 9p-syndrome with congenital median nasal fistula in a boy born to a 28-year-old mother as the second child by normal delivery. The fistula opened at the base of the bridge of the nose and ran between the nasal septum cartilage to the anterior cranial fossa. A frontal craniotomy and transcolumellar skin incision were conducted to extirpate the fistula. In the 10 months since, no fistula has recurred.