Median cervical cyst is characterized by a hemispheric, flat, smooth, and soft tumor and usually is located in the center of the anterior neck. The diagnosis of median cervical cyst is easy in adult patients, but not in children. We treated nine children with a median cervical cyst who visited our department between January 1989 and September 2003. Among these patients, three children underwent surgery and none had recurrence. Paracentesis or incision is sometimes performed for the treatment of the cyst. However, since the cyst recurs easily with these procedurs, total excision by surgery is recommended.
The patient is a six-year-old boy with Prader-Willi syndrome (PWS). He started GH therapy when he was five years of age. Although he showed mild sleep problems before GH therapy, he did not have upper airway obstruction. Nine months after the initiation of GH therapy, he began to show severe sleep apnea. Otorinolaryngologic study revealed marked enlargement of the adenoid. After adenoidectomy, he showed remarkable improvement in sleep apnea. Although the causal connection between GH therapy and adenoid enlargement is not clear, in this patient, GH therapy might have been associated with the cause of airway obstruction.
Children with Cranio-facial anomalies frequently show upper airway obstruction with a wide variety of symptoms, ranging clinically from mild nasal obstruction to severe respiratory distress. Case 1 was a 2 m/o boy with Hallermann-Streiff syndrome who had developed and suffered from feeding difficulty and obstructive sleep apnea for a month. Endoscopic findings showed bilateral nasal stenosis and macroglossia with mandibular dysostosis caused his respiratory dysfunction. Case 2 was a 3 m/o girl with Crouzon syndrome. We found nasal cavity and nasopharyngeal stenosis due to midface retrusion synchronized with respiration. Stenting technique on nasopharyngeal airway relieved the respiratory embarrassment in both cases.
Upper airway compromise can be life-threatening condition and may need rapid evaluation and immediate intervention to change the life time prognosis especially at the birth period. We report two neonatal cases with cyanosis and breathing difficulty, who were saved by emergency airway intervention. Case 1 was a female neonate with congenital subglottic stenosis. She had marked cyanosis at admission. Although a manual attempt to insert an intubation tube failed because of mechanical resistance, laryngoscopy-guided intubation was successful. Case 2 was a male neonate with agnathia. Since he had difficulty with intratracheal intubation, he ultimately underwent successful tracheostomy.
From April 1996 through November 2003 we had four cases of cochlear implant for children with congenital inner ear malformation. We reported on each operation technique used and the postoperative hearing level of these four cases. Three these cases had the Mondini deformity. The other had a common cavity deformity. CSF gusher in cochleostomy occurred in one of the three Mondini deformity cases. It is important to bear in mind abnormalities in the intratympanic course of the facial nerve, constriction of internal auditory meatus, cochlear nerve defect, CSF gusher in cochleostomy and meningitis after cochlear implantation, when the operation is being performed on children with inner ear malformation.
Twenty patients including five children with bilateral anomaly of the external auditory meatus and fifteen with unilateral anomaly consulted our hospital during the last eleven years. Three of 5 children with bilateral anomaly could not be diagnosed at an earlier stage since they did not have either malformation of the auricle or craniofacial deformities. We reported, here, specific time courses of speech and language development observed in the four children in the process of treatment. In particular a case was found to have mental retardation and speech impairment like the children with moderate hearing loss who could not be diagnosed at an earlier stage.
Cornelia de Lange syndrome (CdLS) is a multisystem syndrome involving multiple congenital anomaly, growth retardation, and neurodevelopmental delay. We reviewed seven patients with CdLS to evaluate their hearing levels, and otolaryngologic symptoms. Four patients had stenosis of the external auditory canals. Four were suffering from otitis media with effusion. We measured ABR in seven patients, which showed severe bilateral hearing loss in two, severe and moderate loss in one, moderate bilateral loss in two, moderate and mild loss in one, and mild bilateral loss in one patient. Although we attempted to fit hearing aids, the patients had difficulty tolerating the devices.
We reported 9 cases with congenital cytomegalovirus (CMV) infection. They were all symptomatic at birth. Mean gestational age was 38.6 weeks. Median birth weight was 2,547 g (4 cases were under - 2S.D. ) and the median circumference of the head was 30.8 cm (6 cases were under -2S.D. ). Initial ABR was recorded from 1 to 19 months of age, and 2 cases previously had no response on both sids on 100 dB stimulation. Of 5 cases who had 2nd ABR recording,4 cases were lately no response at either side. Therefore, we consider microcephaly or intrauterine growth retardation are risks for sensorineural hearing loss.