Aneurysmal bone cyst of the spine.

The authors present the case of an 11-year-old boy with a painful, rapidly expanding lesion in the posterior triangle of the neck. There was no history of cervical trauma. Computerized tomography of the neck revealed a unicameral (single-chambered) aneurysmal bone cyst involving the C3 vertebra. Treatment was by open resection and curettage; no recurrence was seen at six months. We discuss the natural history, differential diagnosis, radiographic appearance and treatment modalities for this unusual, benign, expanding, osteolytic lesion containing blood-filled cystic cavities.

Assessment of cochlear damage after pneumococcal meningitis using otoacoustic emissions.

Three cases are reported of children in Ghana with pneumococcal meningitis and differing degrees of hearing loss. The children were examined up to 12 d after admission by means of otoacoustic emissions. The technique is objective, non-invasive, quick (< 5 min per ear) and suitable for use in paediatric wards.

Hypoxia depresses nitric oxide output in the human nasal airways.

OBJECTIVES: The role of oxygen in the nasal air on nasal nitric oxide (NO) output was studied in 13 adult volunteers. METHODS: Nasal NO was measured while air containing oxygen (0%-100% in nitrogen) was aspirated through the nasal airway before and after the topical application of xylometazoline. RESULTS: The mean nasal NO output of the untreated nose was 507.8 +/- 161.9 nL/min (mean +/- SD) when 21% oxygen was aspirated through the nasal cavities in series and remained unaltered by 100% O2 (P = .79). Below 10% oxygen the reduction in nasal NO output correlated positively and significantly with the decrease in oxygen concentration (r2 = 0.14). NO output was 245.2 +/- 153.4 nL/min at 0% oxygen, a significant decline from 21% oxygen (P < .0001). Nasal vasoconstriction induced by xylometazoline and alterations in the blood oxygen content by a maximal breath-holding or breathing 100% oxygen did not alter nasal NO in hypoxia (P = .41). CONCLUSIONS: Nasal NO output is markedly depressed in hypoxia and is oxygen dependent at concentrations of less than 10%. Approximately 50% of nasally generated NO is produced from oxygen in nasal air or regulated by it.

Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management?

OBJECTIVE: To review and evaluate the place of surgical treatment in the management of rhabdomyosarcoma of the head and neck in children. DESIGN: Retrospective analysis of patient charts from January 1, 1972, to December 31, 1998. SETTING: Tertiary pediatric referral center. PATIENTS: Twenty-nine consecutive children with nonorbital head and neck rhabdomyosarcoma. INTERVENTIONS: Surgery, chemotherapy, and radiotherapy. MAIN OUTCOME MEASURES: Disease-free survival and long-term morbidity from treatment. RESULTS: Twenty patients had parameningeal and 9 had nonparameningeal head and neck tumors. All were treated with chemotherapy. For 18 patients, diagnostic biopsies were performed and they received radiotherapy. Eleven patients underwent surgery as definitive therapy. Using the Intergroup Rhabdomyosarcoma Study (IRS) staging system, 5 of these 11 patients had complete resection of tumor (IRS group I) and avoided radiotherapy. The other 6 patients required radiation because of compromised, regional, or incomplete resection of tumor. One had undergone regional resection with nodal involvement, and 2 had compromised resections with microscopic residual disease (IRS group II). Three had incomplete resections with gross residual tumor (IRS group III). Only 1 patient who underwent surgery ultimately died from recurrence at 2.7 years after an incomplete resection. The other 10 patients were relapse free at a median follow-up of 3.7 years (range 0.8-21.0 years). Long-term surgical morbidity was seen in 36% (4/11) of the patients and included facial nerve paralysis, trismus, and cosmetic deformity. CONCLUSIONS: Children with localized disease of the head and neck are able to undergo complete surgical resection, with low long-term surgical morbidity. By undergoing complete surgical resection, these children are able to avoid radiotherapy and its long-term complications, with no compromise in survival.

Pediatric vocal fold paralysis: a long-term retrospective study.

OBJECTIVE: To review our experience of pediatric vocal fold paralysis (VFP), with particular emphasis on etiological factors, associated airway pathologic conditions, and treatment and prognostic outcomes. DESIGN: Retrospective case review of a cohort of patients presenting with VFP. SETTING: Tertiary referral center. PATIENTS: A consecutive sample of 102 patients presenting with VFP to Great Ormond Street Hospital for Children, London, England, over a 14-year period from 1980 to 1994. RESULTS: There was an almost equal distribution of unilateral (52% [n = 53]) and bilateral (48% [n = 49]) VFP. Iatrogenic causes (43% [n = 44]) formed the largest group, followed by idiopathic VFP (35% [n = 36]), neurological causes (16% [n = 16]), and finally birth trauma (5% [n = 5]). Associated upper airway pathologic conditions were noted in 66% (n = 23) of patients who underwent tracheotomy. Tracheotomy was necessary in only 57% (n = 28) of children with bilateral VFP. Prognosis was variable depending upon the cause, with neurological VFP having the highest rate of recovery (71% [5/7]) and iatrogenic VFP the lowest rate (46% [12/26]). CONCLUSION: Recovery after an interval of up to 11 years was seen in idiopathic bilateral VFP; this has significant implications when considering lateralization procedures in these patients.

Sinus histiocytosis with massive lymphadenopathy. Report of a case associated with necrosis.

We describe a case of a 21-year-old man who had a generalized lymphadenopathy and arthritis. Inguinal and cervical lymph nodes showed changes typical of sinus histiocytosis with massive lymphadenopathy. Extensive coagulative necrosis of the intrasinusal histiocytes is described, a feature which, to our knowledge, is unreported previously.

Solitary infantile myofibroma compromising the airway.

Infantile myofibromatosis is an uncommon and benign condition presenting in the neonatal period. It is self-limiting disease that may present as a localised or generalised process. Various examples of this entity have been reported in the literature. This report describes a neonate with a rapidly growing oropharyngeal lesion obstructing the airway that had the typical histological features of an infantile myofibroma. This case report highlights that a solitary myofibroma may be incredibly extensive making complete excision impossible and can be particularly challenging to manage in terms of airway stabilisation.

Factors contributing to limited open-set speech perception in children who use a cochlear implant.

Cochlear implants have enabled many children with severe to profound sensorineural hearing loss to develop speech perception skills. However, some children experience few gains while others develop high levels of speech perception. We identified potential factors contributing to poor performance with an implant by studying implanted children who do not develop functional speech perception. Five children were identified as developing no open-set word recognition skills after at least 2 years of implant use. This study group was compared to a randomly selected control group (n=10) and an age-matched control group (n=5). Pre-implant factors were examined using a Graded Profile Analysis and post-implant factors were assessed in a retrospective chart review. A greater number of pre-implant concerns were raised in the study group than in randomized controls (P<0.01). Chronological age and duration of deafness were pre-implant concerns in all study group subjects. A greater number of post-implant concerns were found in the study group than in randomly selected controls (P<0.005). We conclude that while appropriate selection of candidates for cochlear implantation is important in predicting speech perception outcomes, post-implant follow-up is also essential and must include regular monitoring of equipment, monitoring of stimulation levels with use of objective measures of stimulation levels if necessary, and consistent habilitation.

The role of a graded profile analysis in determining candidacy and outcome for cochlear implantation in children.

Assessment of candidacy for cochlear implantation in children continues to present a challenge to cochlear implant programs. The efficacy of implantation depends on a number of factors and as a result a multidisciplinary approach has been adopted. At the Cochlear Implant Program at The Hospital for Sick Children, Toronto, we use a graded profile analysis (GPA) which has been adapted from the Children's Implant Profile (CHIP) developed by Hellman (S.A. Hellman, P.M. Chute, R.E. Kretschmer, M.E., Nevins, S.C. Parisier, L.C. Thurston, The development of a Children's Implant Profile, Am. Ann. Deaf. 136 (1991) 77-81). This structured assessment allows each potential candidate to be 'scored' in each category of assessment giving a potential range of -14 to +14. In this retrospective study of 109 candidates we examine the relationship between GPA scores and decision to implant. For those patients who were implanted, the relationship between GPA score and speech perception outcomes was also evaluated. Three distinct groups of children emerged from the analysis. The first group had scores less than 5 and were not considered to be suitable for implantation. Within the second group who scored between 5 and 8, there was no clear relationship between decision to implant and score. The last group, who scored 9-14, was considered to be generally suitable for implantation provided that there were no medical contraindications and the parents consented to implantation. There was a significant association between rate of improvement of speech perception and GPA score. Those patients with scores of 9 to +14 improved at a faster rate than group 5-8 (P < 0.05). The implications of these findings are discussed.

Recurrent laryngeal papillomatosis: a case of florid papillomatosis following a remission of 30 years.

Laryngeal papillomatosis is the commonest benign tumour affecting the larynx. Two forms are found i.e. juvenile onset and adult onset. Typically the juvenile onset form has a greater rate of recurrence and often remits with the onset of puberty (Corbitt et al., 1988). The human papilloma virus (HPV) is the causative agent (Abramson et al., 1987; Corbitt et al., 1988), specifically types HPV6 and 11. Attempts have been made to correlate the clinical behaviour of these two modes with the viral serotype and other aetiological factors such as smoking and hormonal factors (Abramson et al., 1987; Rimmel et al., 1992). Studies, however have shown that there is considerable variation in behaviour (Steinberg et al., 1983; Corbitt et al., 1988; Crissman et al., 1988). It is widely accepted that the disease 'burns' itself out, particularly with respect to the juvenile form. It is interesting and unusual therefore when the disease reappears after many years of remission. The following case report illustrates this point.

Vocal fold palsy after use of the laryngeal mask airway.

We report two cases of left vocal fold palsy following use of the laryngeal mask airway. In both cases anaesthesia was uneventful with a duration of about 60 minutes. It is proposed that high intra-cuff pressures induced during anaesthesia resulted in distension of the hypopharynx and subsequent neuropraxia of the motor branches of the recurrent laryngeal nerve and the external branch of the superior laryngeal nerve.

Closed rhinoplasty approach for excision of nasal dermoids.

OBJECTIVE: To assess the efficacy of excision of nasal dermoids through a closed rhinoplasty incision. This is the first description of the use of this approach for excision of superficial nasal dermoids. METHODS: Three boys aged five, nine and 12 years presented with midline nasal dermoids with minimal cutaneous involvement. Magnetic resonance imaging demonstrated distinct, cystic, superficial nasal masses. The cysts were excised through a closed rhinoplasty approach. In each case, completeness of extirpation was judged by histopathological examination of the excised specimen. Aesthetic outcome was recorded photographically. RESULTS: All three patients' cysts were completely excised, with excellent cosmetic results. CONCLUSIONS: The closed rhinoplasty incision is another approach in the surgeon's armamentarium for excision of small, superficial nasal dermoid cysts. In well selected cases, this approach gives optimal cosmetic results, provides adequate exposure with minimal dissection, and allows total extirpation.

Paediatric eosinophilic oesophagitis presenting to the otolaryngologist.

INTRODUCTION: The prevalence of eosinophilic oesophagitis is increasing. A Pubmed search for 'eosinophilic oesophagitis' and 'eosinophilic esophagitis' yielded 345 publications since 1976. Only seven were in otolaryngology journals.1-7 Patients typically present with dysphagia, vomiting, dyspepsia or food impaction and are therefore usually referred to a paediatric gastroenterologist; otolaryngologists are not usually involved in management. A missed diagnosis may result in oesophageal stricture. METHODS: Two patients, aged two and four years, were referred to the paediatric otolaryngology department with intermittent upper oesophageal food impaction. A paediatric gastroenterologist was involved in the investigation. Histological examination of oesophageal biopsies demonstrated changes consistent with eosinophilic oesophagitis. RESULTS: Both patients were expediently diagnosed, investigated and managed. CONCLUSION: A diagnosis of eosinophilic oesophagitis must be considered in patients presenting with food bolus impaction. Early involvement of a paediatric gastroenterology team in the diagnosis is recommended in children presenting with oesophageal symptoms, in order to avoid delayed diagnosis.

Oesophageal perforation: an unusual complication of a hypoglycaemic episode.

A case of spontaneous oesophageal rupture following vomiting, secondary to a hypoglycaemic episode is reported. The case is of interest in its presentation and physical signs. It reflects the difficulty in diagnosing a condition associated with considerable morbidity and mortality. The importance of recognition of a pattern of symptoms, physical signs and radiographic findings is emphasized.

Disseminated ascariasis: a case report.

A very unusual case of ascariasis is reported. There was massive migration of worms from the small bowel to the liver, and probably thence to the heart and lungs, where adult worms were demonstrated. The cause of death was pulmonary embolism, adult worms blocking the main pulmonary arteries with superimposed thrombosis. This is believed to be unique.

Intra-ocular tuberculosis associated with a penetrating injury. A case report.

A case of histologically proven intra-ocular tuberculosis is described. The condition was preceded by penetrating trauma, and no evidence of systemic tuberculosis other than a positive Mantoux test was found. Numerous acid-fast bacilli were seen in pathological sections of the eye. We suspect that a tuberculosis bacillaemia from an undetected healed focus coincided with the trauma and thereby produced intra-ocular tuberculosis.

Two histologically different, synchronous carcinomas in a single male breast. A case report.

A rare case of male breast carcinoma is reported. The operative specimen revealed two histologically distinct tumours, a papillary carcinoma and a colloid carcinoma. Features may support the theory of multicentric origin of breast cancer.