Radiographic diagnosis of congenital bony nasal stenosis.

Congenital bony nasal stenosis (CBNS) is a rare condition that causes respiratory distress in neonates. Between 1986 and 1996, we encountered 13 cases of CBNS. Recently, CT measurements have been used to evaluate the features of this type of stenosis, but no satisfactory investigation of the severity of bony nasal stenosis has been reported. We compared the nasal width (NW), facial width (FW), and interorbital distance (IOD) measured from occipitofrontal (Caldwell's) projections of plain radiographs in nine CBNS patients and nine normal infants. NW was significantly narrower in the CBNS patients than in the controls, and never exceeded the IOD in any of the CBNS patients. This measuring method can be used to diagnose CBNS, because NW in normal subjects of all age groups is always greater than IOD on Caldwell's view. We also investigated one patient radiographically, to determine how NW increased until the age of 4 years. However, there was no significant change in nasal width, although the FW increased significantly. This method seems useful for determining the severity of stenosis, and its simplicity makes it useful for routine investigation of breathing difficulty in neonates and infants. We review and discuss the CT images of CBNS reported in the literature.

Lingual cyst in infancy: importance of palpation for diagnosis.

Two infants, 5 and 12 weeks of age, with lingual cysts were presented. Histological findings were compatible with a salivary retention cyst in one and a thyroglossal duct cyst in the other. Both infants were admitted to our hospital because of severe stridor that had developed from one to two weeks of age. Their lingual cysts were easily recognizable by simple palpation and were confirmed by non-invasive imaging techniques, such as ultrasound sonography, computed tomography and magnetic resonance imaging. Lingual cyst in infancy may be large enough to cause stridor and dyspnea, and occasionally result in sudden infant death, although once diagnosis is made a radical operation can be easily performed. Therefore, it is important that palpation in the oral cavity should be performed with all infants with persistent stridor as a part of a physical examination.

Delayed post-neonatal intensive care unit hearing disturbance.

Ten children, who developed hearing disturbance after discharge from the neonatal intensive care unit (NICU), are reported. All were born at full term, between November 1988 and November 1992, weighed at least 2500 g and had normal auditory brainstem response (ABR) at discharge from the NICU. All ten underwent thorough evaluation to determine the cause of the subsequent hearing loss. All had severe cardiovascular and/or pulmonary disorders at birth, and persistent pulmonary hypertension of the newborn (PPHN) was diagnosed in eight. A total of 25 PPHN cases were treated at our facility during the 1988-1992 period, the rate of hearing disturbance in PPHN patients being 32%. The 10 infants in this study were mechanically ventilated for, on average, 30 days. High frequency oxygenation (HFO) was required in five cases, extra corporeal membrane oxygenation (ECMO) in six. During this period, only eight ECMO-treated infants survived. The rate of hearing loss in surviving cases was therefore very high, 75% (6 8). All 10 of our cases were treated with an aminoglycoside, furosemide and a muscle relaxant. We recommend follow-up at 6 and 12 months of age, as these children are at very high risk of developing hearing disturbance.

[Diagnosis of sinusitis in children with allergic diseases--comparison of waters projection radiographs and CT scans].

Sinusitis is common in children with allergic diseases, and the relationship between sinusitis and reactive airway diseases involving asthma has been reported. Most pediatricians and physicians base their diagnosis of sinusitis on findings from plain radiographs of the sinuses, especially Waters projection radiographs. We compared the diagnoses made by 11 pediatric allergists using 56 Waters projection radiographs with transverse CT findings. The ratio for the two diagnosis being consistent (normal plain radiographic findings and normal CT findings, or abnormal plain radiographic findings and abnormal CT findings) was approximately 60%. Sinusitis in children is often misdiagnosed on the basis of findings from Waters projection radiographs alone. Therefore, the use of CT findings for the diagnosis of sinusitis together with Waters projection radiographs is recommended.

[Hearing loss following discharge from the NICU].

We experienced 10 pediatric cases born between November of 1988 and November of 1992, whose ABR were normal at the time of discharge from the NICU, who subsequently developed hearing impairment. These children underwent thorough evaluations and the causes of their hearing loss are discussed herein. All 10 were born at full term, birth weights ranged from 2500g to 3826g, and all had severe cardiovascular and/or pulmonary disorders. Eight of the 10 had persistent pulmonary hypertension of the newborn (PPHN). During the four year period in which these 10 infants were born, a total of 25 PPHN cases were diagnosed in our NICU, i.e. the rate of hearing loss in PPHN infants was 32% (8/25). All 10 infants were intubated and maintained on mechanical ventilation for an average of 30 days. Five cases also required high frequency oscillation and six received extracorporeal membrane oxygenation (ECMO). During the same time period there were only eight surviving ECMO cases such that the rate of hearing loss was 75% (6/8). All 10 were treated with an aminoglycoside, furosemide and a muscle relaxant. As These infants are at very high risk for hearing impairment, follow-up at 6 and 12 months of age is essential.

Idiopathic deafness or hearing loss of unknown etiology following discharge from the NICU.

The clinical course, treatment and respiratory management of 6 infants who developed profound hearing loss (n = 5) or high tone hearing loss (n = 1) some months after discharge from the NICU are presented. All 6 infants had congenital and/or perinatal diseases causing hypoxia, and all were treated with gentamycin. All were nearly normal at discharge but later rose to 80-90 dB, or 60 dB in the case with high tone hearing loss. The hearing impairment was thought to be due to hypoxia, in combination with medications and invasive procedures. Follow-up audiologic testing is recommended after NICU discharge.

Management of acute otitis media. Assessment of therapeutic strategy in Japan today.

Currently, myringotomy is widely used in the routine initial treatment of acute otitis media in Japan. In an attempt to evaluate the validity of this strategy, a number of issues that demand reassessment or clarification have been identified. These issues and factors contributing to the persisting uncertainty about myringotomy are examined.

Choanal atresia and congenital pharyngeal stenosis.

From 1980 to 1992, 8 patients with choanal atresia were treated in our facility. The female to male ratio was 5:3. One patient, a girl, had unilateral osseous obstruction with membranous closure of the opposite naris while the other 7 had bilateral osseous obstruction. Seven of the 8 had other congenital anomalies, CHARGE association (2 cases), Apert's syndrome (1 case), meningocele and banded fingers (1 case), omphalocele (1 case), subdural hematoma (1 case), and nasal fistula (1 case). Of the 7 patients who underwent endonasal surgical repair, 3 (40%) experienced reclosure. The remaining one patient, whose obstruction was of the bilateral osseous type, was successfully operated on via a transpalatal approach. Surgical correction is particularly difficult in those with craniofacial abnormalities, e.g. Apert's syndrome and maxillary hypoplasia. We treated 5 congenital pharyneal stenosis cases (CPS). Our CPS cases were divided into Types 1, 2 and 3, the latter being a new type not previously reported on. We consider Type 3 to be the result of fetal tissue remnants. We performed uvula splitting surgery in 4 cases and obtained good results in 3.

[Congenital nasal stenosis--new concept and its cases].

Congenital nasal stenosis is a new disease concept which was reported, for the first time, by Knegt-Junk et al in 1988. The authors experienced 10 cases of this disease from 1986 to 1990. Radiography revealed that transverse width of the bony nasal passage of these patients was smaller than that of normal infants. Infants with nasal stenosis showed respiratory distress after birth and half of them had a low body weight and/or other congenital defects. Our patients were treated by nasal or oral intubation or by installation of nasal vasoconstrictor into the nasal passage. Four cases showed improvement up to 4 months of age, 6 others after 6 months of age. Tracheostomy was performed in one case. We must differentiate this disease from incomplete choanal atresia or nasal aperture stenosis. Our cases were similar to that of Knegt-Junk et al. We speculate that the congenital origin of this disease stems from a disturbance in embryologic development of the middle third of the face. Accordingly, we measured the diameter of the nasal passage radiography and recognized a narrow transverse width in the bony portion of the nose.

[Bilateral acute profound deafness after MMR vaccination--report of a case].

In April 1989 the MMR vaccination program had started, and until October, 1989, 630,000 children received vaccination. In is, however, well known that many children developed various complication including aseptic meningitis after vaccination, and the MMR vaccination program has discontinued. This report described a case of bilateral acute profound deafness most likely due to MMR vaccination. The cause of this deafness was presumed to be mumps vaccination. The bases of the presumption are as follows: the meningitis after MMR vaccination was elicited by PCR method to be caused by mumps vaccine, and the complication of CNS after measles vaccination occurs within 14 days after injection, while the onset of vomiting and gait disturbance of the case was 24 days after vaccination.

[Surgical treatment of subglottic stenosis in children--laryngotracheal plastic surgery with autogenous costal cartilage graft].

We performed laryngotracheal plastic surgery by transplantation of autogenous costal cartilage graft in 9 cases of subglottic stenosis (2 with congenital type and 7 with acquired type). Of 9 cases, 3 cases had the lesion after long-term intubation, 2 cases after tracheotomy and 2 cases after laryngo-tracheo-bronchitis. Five cases aged one year and the remaining 4 cases 2 years, 3 years, 4 years and 6 years old, respectively. We could successfully decannulate in all the 9 cases after our plastic surgery. The period from surgical treatment to decannulation varied from 9 days to 6 months. However, 8 cases were decannulated in 2 months after surgery. Cotton et al (1978) did not use any stents in their treatment for subglottic stenosis, but we did in all of our cases. We preferred stents of the silicone Y-tube, and the recommendable duration of cannulation is 4 weeks.

[Factors on delayed recovery of otitis media with effusion in children--clinical and bacteriological study].

The resolution of middle ear effusions (MEE) of children with otitis media with effusion (OME) who underwent myringotomy for the bacteriological examination was analyzed in terms of the culture results and the clinical features. The present study consisted of 193 children (258 ears), and the MEE from 77 ears (30%) were culture positive and the respiratory pathogens were detected from 44 ears (17%). Each child was then assigned to receive either a more than two-week course of antibiotics, cefaclor (CCL) or not. At one month following entry, 53 (55%) out of 97 ears in CCL-treated group were effusion-free compared with 31 (40%) out of 78 ears in the control group (P less than 0.05). In the control group, the resolution of MEE was significantly poor in the recurrent cases and the cases with pathogen positive-MEE. The presence of accompanying diseases such as adenoid vegetation, chronic sinusitis and allergy, however, was not related to the resolution of MEE. On the other hand, the cure rate of the cases with pathogen positive-MEE and recurrent cases in the CCL-treated group showed significant improvement. Furthermore, the cases accompanying adenoid vegetation and chronic sinusitis tended to become effusion-free after the antibiotic treatment. Therefore, the persistent bacterial infection in the middle ear and/or surrounding organs such as adenoid plays possibly an important role in the delayed recovery of OME. Antibiotics treatment could increase, to some extent, the resolution of MEE in cases with OME.

Immunological study on association between mumps and infantile unilateral deafness.

The incidence of unilateral deafness in primary school children has been reported to be about 0.1%, but the cause of most of these cases has not been clarified, because parents and children themselves cannot recognize the onset of hearing loss occurring in one ear alone. This study was performed to clarify the possibility that mumps causes infantile unilateral deafness. The results were: 1) on dividing a total of 78 cases of infantile unilateral deafness according to age from 3 to 6 years, mumps infection rate in the 4-year-old group was nearly twice that in a randomly selected group; 2) on checking the neutralizing or ELISA IgG test of 31 cases with no history of mumps only, the 4-year-old group demonstrated a positive reaction to mumps more than the randomly selected group did. The above results suggested that unilateral deafness may be caused by asymptomatic mumps infection. To confirm this, we studied 5 infantile cases of sudden hearing loss by means of ELISA IgM antibody test and found a bilateral sudden deafness in children aged 1.9 years, caused by asymptomatic mumps infection.