The relationships among mastoid air cell development, tympanic sinus depth, and residual disease after surgery in children with congenital cholesteatoma.

Background: Mastoid development, tympanic sinus depth, and residual disease after surgery for congenital cholesteatoma are probably related, but these relationships have not been examined in detail.Aims/objectives: This study aimed to clarify the relationships between the abovementioned factors. Materials and Methods: The subjects were 31 patients with congenital cholesteatoma (stage III or IV in Potsic's staging system) that underwent mastoidectomy. The cross-sectional area of the mastoid air cells was measured as described previously. Tympanic sinus depth was classified into A-C using Marchioni et al.'s system.Results: Patients with deep tympanic sinuses or residual disease exhibited significantly greater mastoid air cell development. However, little residual disease was found in the mastoid air cells. Conversely, residual disease was observed more frequently in the patients with deep tympanic sinuses.Conclusions and significance: After surgery for congenital cholesteatoma, residual disease is more likely to occur in patients with marked mastoid growth, possibly because they have deep tympanic sinuses. Cases in which congenital cholesteatoma spreads to the mastoid air cells are classified as stage IV in Potsic's system, but our findings indicate that invasion into a deep tympanic sinus is more important than invasion into the mastoid air cells.

A quantitative study of the suppression of the development of the mastoid air cells by the presence of congenital cholesteatoma.

Background: The mastoid air cells are considered to develop better in children with congenital cholesteatoma but only a few quantitative studies have examined. Aims/objectives: The present study was undertaken in order to clarify the relationships between the development of the mastoid air cells in children with congenital cholesteatoma and clinical factors. Materials and methods: Mastoid air cell development was evaluated in 53 children with congenital cholesteatoma. The sizes of the mastoid air cells were measured on computed tomography scans of the temporal bone, and clinical factors, including age, the extent of invasion, and whether it was located close to the eustachian tube were evaluated. Results: The cross-sectional area of the mastoid air cells was significantly smaller on the affected side than on the unaffected side, especially in the older patients. The cases involving invasion into the mastoid portion also had smaller mastoid cells. Whether the cholesteatoma was located close to the eustachian tube had no effect. Conclusions and significance: The mastoid air cells on the affected side were less well developed. Our findings indicate that a high age and the presence of cholesteatoma in the mastoid region might be associated with suppressed mastoid pneumatization.

Practical analysis of pars flaccida cholesteatoma with classification and staging system proposed by Japan Otological Society: a comparative study.

BACKGROUND: The Japan Otology Society (JOS) proposed the classification and staging system for middle ear cholesteatoma. However, there was little analysis of the pathology of cholesteatoma using this staging system. AIMS/OBJECTIVES: To analyze the pathology of pars flaccida cholesteatoma using the staging system by JOS. MATERIAL AND METHODS: A total of 183 cases of fresh pars flaccida cholesteatoma treated between January 2009 and December 2015 were included. We used the staging system recommended by JOS (2015). The association of the following variables in each stage of pars flaccida cholesteatoma was examined: age, gender, preoperative hearing level, staging, statuses of mastoid cell growth and stapes, tympanic sinus invasion. RESULTS: Stage II disease showed the highest degree of progression, and peak incidence was observed in the third and fourth decades of life. Stage III significantly increased after the age of 40 years. The progression of the disease stage was significantly associated with deterioration of hearing level. Cholesteatoma invasion to tympanic sinus was recognized in 14.2% of cases. The state of the stapes is increasingly likely be to fracture as stage progresses. CONCLUSIONS: The JOS Staging System appropriately reflects the disease state, and it was found to be clinically meaningful in this study.

Elevation of Serum Acid Sphingomyelinase Activity in Acute Kawasaki Disease.

Kawasaki disease (KD) is an acute systemic vasculitis that affects both small and medium-sized vessels including the coronary arteries in infants and children. Acid sphingomyelinase (ASM) is a lysosomal glycoprotein that hydrolyzes sphingomyelin to ceramide, a lipid, that functions as a second messenger in the regulation of cell functions. ASM activation has been implicated in numerous cellular stress responses and is associated with cellular ASM secretion, either through alternative trafficking of the ASM precursor protein or by means of an unidentified mechanism. Elevation of serum ASM activity has been described in several human diseases, suggesting that patients with diseases involving vascular endothelial cells may exhibit a preferential elevation of serum ASM activity. As acute KD is characterized by systemic vasculitis that could affect vascular endothelial cells, the elevation of serum ASM activity should be considered in these patients. In the present study, serum ASM activity in the sera of 15 patients with acute KD was determined both before and after treatment with infusion of high-dose intravenous immunoglobulin (IVIG), a first-line treatment for acute KD. Serum ASM activity before IVIG was significantly elevated in KD patients when compared to the control group (3.85 ± 1.46 nmol/0.1 ml/6 h vs. 1.15 ± 0.10 nmol/0.1 ml/6 h, p < 0.001), suggesting that ASM activation may be involved in the pathophysiology of this condition. Serum ASM activity before IVIG was significantly correlated with levels of C-reactive protein (p < 0.05). These results suggest the involvement of sphingolipid metabolism in the pathophysiology of KD.

Incidence of intussusception as studied from a hospital-based retrospective survey over a 10-year period (2001-2010) in Akita Prefecture, Japan.

One concern about rotavirus vaccines is its possible association with intussusception. Thus, it is necessary to determine the baseline incidence for intussusception in the first year of life in places where rotavirus vaccines are introduced. However, few safety data exist for the period at which the first dose of Rotarix and RotaTeq are allowed to administer in Japan. The first dose of Rotarix is scheduled to administer at 6-20 weeks of age and that of RotaTeq is scheduled to administer at 6-24 weeks of age; the upper limits for these vaccines is later than the upper limit recommended by the World Health Organization by 5 and 9 weeks, respectively. We performed a retrospective cross-sectional study by reviewing medical charts of all hospitals that provided pediatric beds in Akita Prefecture, Japan, and identifying the cases of intussusception that met the Brighton criteria level 1 in these hospitals between January 2001 and December 2010. During this 10-year period, 122 children younger than 1 year of age were diagnosed with intussusception. The incidence of intussusception was estimated at 158 per 100,000 person-years among children younger than 1 year (95% confidence interval, 131-188), 10 per 100,000 person-years for children aged 0-2 months, 165 for children aged 3-5 months, and 300 for children aged 6-8 months. This rapid and substantial increase in the incidence of intussusception during the first year of life should be considered when formulating the immunization schedule for administering rotavirus vaccines in Japan.