Clinical and imaging evaluation of Congenital Midnasal Stenosis.

OBJECTIVES: To illustrate the clinical and radiological presentation of a rare etiology of nasal obstruction in neonates, midnasal stenosis (MNS), including a comparison of nasal dimensions with those of normal infants. METHODS: We retrospectively reviewed medical charts and computerized tomography (CT) imaging of neonates with nasal obstruction diagnosed as stenosis in the midnasal area in a tertiary pediatric medical center. MNS was defined clinically by inability to visualize the middle turbinate with an endoscope despite the absence of stenosis of the anterior aperture or any gross septal deviation. CT measurements of the midnasal width were taken by an experienced neuroradiologist. We compared widths between the bony inferior turbinate to the bony septum in the narrowest area of symptomatic patients, to widths in a control group of asymptomatic children. RESULTS: Nine neonates from birth to three months old presenting with nasal obstruction, severe stertor, and blocked nasal passage at the midnasal level in endoscopic examination, were diagnosed with MNS. 6/9 had CT scans. Four had isolated unilateral stenosis, two unilateral MNS and contralateral choanal atresia, and three bilateral MNS. All patients were managed conservatively, initially with nasal saline irrigation and local steroids and topical antibiotics; Median time to resolution of symptoms was 14 days. When comparing the dimensions at the midnasal narrowest area of the stenotic group with a control group of 139 healthy children, the median bony width was 1.7 mm vs. 3.2 mm, respectively (p < 0.00001). Average dimensions according to age groups until the age of 12 months are given. CONCLUSION: In neonates with nasal obstruction, when choanal atresia and pyriform aperture stenosis are excluded, stenosis of the midnasal area should be considered. Most of these neonates can be managed conservatively. LEVEL OF EVIDENCE: 4.

Recurrent periorbital cellulitis associated with rhinosinusitis in children: Characteristics, course of disease, and management paradigm.

BACKGROUND: Recurrent periorbital cellulitis (RPOC) associated with rhinosinusitis is rarely noted and scarcely discussed in the literature. The aim of our study was to analyze the characteristics and disease course of a group of pediatric patients with RPOC. METHODS: The medical records of all pediatric patients with a diagnosis of RPOC treated in a tertiary children's hospital were retrieved. Included were patients with a history of two or more episode of RPOC. RESULTS: A total of 14 children were included. Mean follow up was 3 years (SD ±â€¯2.5). Median age of first POC episode was 12 months (range 5 months-12 years). Overall, 75 events of RPOCs were documented. A median of 3 events per patient was noted (range 2-16). Preseptal cellulitis was diagnosed in all but five patients, in whom orbital cellulitis or subperiosteal abscess were identified, one following failure of conservative treatment, and the remaining at first presentation or recurrence. Rhinorrhea was present in only five patients (35%). The majority of patients were treated with intra-venous antibiotics. Imaging studies were performed in all patients revealing ethmoidal sinusitis in all patients, with lamina papyracea dehiscence in two patients. Immune deficiency was diagnosed in one patient. Endoscopic sinus surgery was performed in seven patients, in four as preventive procedure and in three during an acute phase. Post-operative recurrent disease was noted in two patients that were operated during the acute phase. CONCLUSIONS: RPOC is a rare entity. Rhinosinusitis should be suspected in events of recurrent preseptal cellulitis even in the absence of nasal symptoms. CT scan is diagnostic for sinus origin of disease and possible anatomical abnormalities. In the majority of patients no evident etiology was identified. Elective Surgery or prophylactic antibiotic treatment should be strongly considered in this subgroup of patients as it seems beneficial.

Hyperactive auditory efferent system and lack of acoustic reflexes in Williams syndrome.

The auditory efferent system and acoustic reflexes have been investigated in patients with Williams syndrome (WS). Twenty-one patients aged 6-26 years with a genetically confirmed diagnosis of WS and with reported hyperacusis were compared with 21 normally developing age-matched subjects. The medial olivocochlear (MOC) efferent system was tested by stimulation of the contralateral ear with increasing levels of white noise, while recording transient evoked otoacoustic emissions (TEOAE) in the ipsilateral ear. The suppression effect on the amplitudes of the TEOAE was computed for each contralateral stimulus level. This measure reflects the strength of the MOC efferent system. In addition, the thresholds of ipsilateral and contralateral acoustic reflexes in response to 1, 2 and 4 kHz tones as well as to broadband stimuli were also recorded. Results showed that patients with WS had a significantly higher suppression effect of the MOC reflex on TEOAE. Ipsilateral and contralateral acoustic reflexes to tonal and broadband stimuli presented at maximum stimulus intensities were absent in 62-86% of the patients with WS. In the remainder, acoustic reflexes were elicited at lower auditory sensation thresholds than in controls. Hyperexcitability of the MOC efferent system coupled with absence of acoustic reflexes may contribute to the hyperacusis in WS and the consequent high-tone hearing loss induced by environmental noise. Both measures can be used for objective detection and thus, intervention of hyperacusis in the early stages of life.